CMS 251 Unit 1 Cardiovascular System

UNIT 1: CARDIOVASCULAR PATHOLOGY
MODULE: CLINICAL PATHOLOGY

UNIT 1 - The Cardiovascular System
By OKINDA, B, Carey Francis
September-October 2015
OUTLINE
Topic Sub Topics Hours
1. Introduction to Review of Anatomy and Physiology 2
CVS Pathology Pathophysiology and Investigations in CVS Disorders
2. The Heart Congenital Disorders 1
Cardiac Failure 2
Ischaemic Heart Disease 1
Valvular Heart Disease 1
Acute Rheumatic Fever and Rheumatic Heart Disease 1
Myocardial and Pericardial Disorders 1
3. Arteries Hypertension and Hypertensive Heart Disease 1
Aneurysms 1
Atheroma/atherosclerosis and Arteriosclerosis 1
4. Veins DVT and PE 1
Varicosities and Haemorrhoids 1
Tumours of Blood Vessels 1
TOTAL 15
Lesson 1: Introduction to Pathology of the Cardiovascular System
Learning Outcomes
At the end of the lesson the learner shall be able to: -

1. Describe the anatomy and physiology of the cardiovascular system
2. Describe mechanisms of cardiovascular disease
3. Discuss investigations in cardiovascular disease
1.0. INTRODUCTION
 CVS pathology is the study of causes and effects of disease on the CVS (heart and blood
vessels - arteries, veins and the capillaries)
2.0. THE HEART
 Pumps sufficient oxygenated blood containing nutrients, metabolites and hormones to

meet the moment to moment metabolic needs and preserve a constant internal milieu
 Has 3 layers – endocardium, myocardium and pericardium (outer covering)
 Has 4 chambers with 4 valves (aortic, pulmonary, tricuspid and the mitral)
 Heart muscle is specialized with 2 essential characteristics - contractility and rythymicity
Carey F. Okinda Page 1

 Conducting system (SAN), AVN, the Purkinje tissues/fibres, bundle of His) contains
specialized cells for initiation and transmission of signals in a co-coordinated manner.
 Physiological function of the heart is maintained by healthy muscles, efficient valves, the
conducting system and co-ordination of chambers and normal peripheral resistance
Diagram 1.1: Normal Heart
Functioning of the Heart
 Heart has 3 major types of cardiac muscle - atrial, ventricular & the specialized
excitatory and conductive muscles
 Muscle is organized in two syncytium with the many cells connected in series with
intercalated discs with specialized structures such as fascia adherens (mechanical links),
mascula adherens /desmosome (lattice structure and site for cytoplasmic filaments) and
gap junction (makes the adjacent cells loose and is permeable to ions). This
arrangement facilitates the all or nothing principle.
The Cardiac Cycle
 Comprises of
i) Phase I – Atrial Contraction
o Period of rapid refilling of ventricles in the first 1/3 of diastole, blood moves slowly
into the ventricles in the middle of 1/3 of diastole and atrial contraction pushes
more blood into the ventricles (last 1/3 of diastole)
ii) Phase II – isovolumic ventricular (isometric) contraction
o Emptying ventricles during the beginning of ventricular contraction when no
emptying takes place - isovolumic or isometric (i.e. is there is no increase in
tension of muscle but no shortening of muscle fibres)
iii) Phase III – ventricular systole

o Period of rapid ejection in the first 1/3 due to rise in left ventricular pressure (>80
mmHg) and right ventricular pressure (>8 mmHg) forcing open the mitral and
tricuspid valves respectively
o Slow ejection in the lase 2/3 of 30% of ventricular emptying
iv) Phase IV – period of isovolumic ventricular (isometric) relaxation
o Ventricular relaxation allows ventricular pressure to fall
o Increased pressure in the distended arteries pushes blood back towards the
ventricles closing the aortic and pulmonary valves
o Ventricular muscles contract but ventricular volume stays (isometric relaxation)
v) Phase V – ventricular diastole relaxation (period of ventricular diastole relaxation
overlaps with atrial contraction.
3.0. CARDIAC OUTPUT
 Normal CO for young healthy male adult is 4 – 8 litres/minute (average 5.6 litres/min)
with females at 10% less
 Five basic mechanisms controlling CO include heart rate, ventricular filling pressure,
ventricular distensibility, systemic vascular resistance and ventricular contractility.
 Cardiac output (CO) = Heart rate (HR) x Stroke volume (SV) of the left ventricle
 SV is the diastolic volume of the ventricle minus the volume of blood in the ventricle at
the end of systole
 Cardiac Index (CI) is the cardiac output per square metre of body surface area. The
normal is 3.0 litres/minute and changes with age.
Ejection Fraction
 EDV is the volume of blood in the ventricles at the end of diastole when the filling of
ventricles increases volume of each ventricle to 120 – 130 mls while the ESV is the blood
remaining in the ventricles at the end of systole (usually 50 – 70 mls).
 Ejection Fraction = 70 x 100 = 58.3% (60%)
120
4.0. THE LAWS
1. Poiseulle’s Law Blood flow = Pressure x diameter of blood vessel

Length of vessel x viscosity of blood
2. Starling’s Law – increase in dilatation leads to increased filling, contraction and stroke
volume
3. Frank-Starling – within physiological limits, the heart pumps all the blood that comes to it
without allowing excessive damming of blood in the veins. The greater the heart is filled
during diastole, the greater will be the amount of blood pumped into the aorta.
4. Laplaces Law – the circumferential force tending to stretch the muscle fibres in the vessel
wall is proportional to the diameter of the muscle x the pressure inside the vessel (F = D x
P). The wall tension require to counteract a given pressure in a spherical cavity is
proportional to the radius of the cavity.

5.0. PRINCIPAL MECHANISMS OF CARDIOVASCULAR DISEASE
 Many diseases can involve the heart and blood vessels but generally cardiovascular
dysfunction results from five main mechanisms: -
i) Failure of the pump
ii) An obstruction to flow
iii) Regurgitation flow
iv) Disorders of cardiac conduction
v) Disruption of the continuity of the circulatory system
6.0. INVESTIGATIONS IN CARDIOVASCULAR DISEASE
1) IMAGING
a) Chest X-Ray
 Is taken in postero-anterior (PA) direction at maximum inspiration.
 The heart is close to the X-ray film to minimize magnification of the chest with
respect to the thorax.
 Look at the heart size, calcification and lung fields
Interpretation

Heart Size
i) The cardio-thoracic Ratio (CTR)

 The maximum transverse diameter of the heart is compared with the maximum
 Is usually less than 0.5 (50%) except in neonates, infants, athletes and patients with
skeletal deformities (Scoliosis, funnel chest)
 Pericardial effusion or cardiac dilation increases the ratio.
ii) Patterns of specific chamber enlargement seen on the chest X-ray

a. Left Atrial dilatation
 Prominence of the left atrial appendage on the left heart boarder.
 A double atrial shadow to the right of the sternum.
b. Left ventricular enlargement - increased CTR and smooth elongation & increased
convexity of the heart border.
c. Right Atrial enlargement - right boarder of the heart projects into right lower lung
field.
d. Right ventricular enlargement - increased CTR & upward displacement of the apex of
the heart.
e. Ascending aortic dilatation/enlargement - prominence of the aortic shadow
f. Dissecting of the ascending aorta - widening of the mediastinum
Calcification
 Occurs due to tissue degeneration
 Seen on the lateral or a penetrated PA view but best studied by CT scanning
 Calcification can be seen in:- Pericardial
 Valvular
Lung Fields
 Increased in vascularity an in size of hilar vessels seen when there are left to right shunts.
 When there is pulmonary ligaemia there is a paucity of vascular markings and a
reduction in diameter of arteries.
 Prominence of pulmonary arteries hili and pruned (reduced in size) at the peripheral
Lung fields as seen in pulmonary arterial hypertension.
Kerley Lines
 Septal lines seen when the interlobular septa in the pulmonary interstitium become
prominent due to lymphatic engorgement or oedema of the connective tissues
 Kerley A lines
o Are 2-6 cm long oblique lines that are < 1 mm thick and course towards the hilar
o Represent thickening of the interlobular septa that contain lymphatic connections
between the perivenous and bronchoarterial lymphatics deep within the lung
parenchyma
o On CXR they are seen to cross normal vascular markings and extend radially from the
hilum to the upper lobes

 Kerley B lines
o These are 1-2 cm thin lines in the peripheries of the lung
o Are perpendicular to, and extend out to the pleural surface
o Represent thickened sub pleural interlobular septa and are usually seen at the lung
bases.
 Kerley C lines
o Short lines which do not reach the pleura (i.e not B or D lines) and do not course
radially away from the hila (i.e not A lines).
 Kerley D lines
o Are exactly the same as Kerley B lines, except that they are seen on lateral chest
radiographs in the retrosternal air gap
Causes
 Pulmonary oedema, neoplasm, pneumonia (viral, mycoplasma and Pneumocystis),
interstitial Pulmonary Fibrosis, pneumoconiosis and sarcoidosis
Pleural Effusion
 Abnormal pulmonary vasculature
 Fluid level
 Opacity
 Loss of costo-phrenic and cardio-phrenic angles
b) MRI (Magnetic Resonance Imaging)

 Non-invasive imaging technique where a powerful magnetic field is used
 Cardiac MRI that uses radio waves, magnets, and a computer to create pictures of
the heart. This gives a 3D image of the moving as well as still pictures of the heart.
c) Nuclear Imaging
 Primarily used is Ischaemic Heart Disease
 Myocardial structure/function can be assessed by radio-nucleide imaging techniques

 Thallium (with behaves as potassium) is taken up by healthy myocardium
 Ischaemia or infarction produces unclear image with a “cold” spot.
d) CT Scanning
 Useful showing the size and shape of the cardiac chambers as well as the thoracic
abdominal aorta and Mediastinum.
2) ELECTROCARDIOGRAM (EGG)
 Is a recording of the electrical activity of the heart (sum of the depolarization and
repolarization potential of the myocardial cells)
 Important in diagnosing rhythm and conduction problems
 Depolarization initiating each heart beat begins at Sino-Atrial node and spreads as an
advancing wave through the atria which are depolarized simultaneously to the A-V
Node
 Depolarization spreads from the atria to ventricles via the Bundle of HIS that begins at
A-V Node passing into the interventricular septum where it divides into right and left
branches
EGG Waveform
Terminology
 P wave - 1st deflection corresponds to depolarization and the atria

 QRS Complex - 3 deflections - 1st downwards (Q wave), 2nd upwards (R wave) and 3rd
downwards (S wave) corresponds to depolarization of the ventricle.
 T Wave - repolarization of ventricular muscles.
 Upward deflection is a depolarization wave is moving towards recording electrodes
 Downward deflection repolarization wave is moving away from the recording electrodes.
Time intervals
 All EGG recorders run a standard paper speed of 25 mm/s.
 EGG paper is standardized so that 5 large squares pass under the recorder stylus each
second.
 One large square is equivalent to 0.2 sec.
 Each large square is subdivided into five small squares each equivalent to 0.04s.
 Heart rate can be calculated from the number of squares between QRS complexes.
 Time taken by each part of the depolarization sequence in each cardiac cycle is

 Calculated by the number of small squares it occupies.
PR interval
 Time taken for depolarisation to spread from SAN to Atria to AVN through the Bundle
of HIS bundle to the ventricle
 Shown by number of small squares between beginning of P wave and the beginning of
the ORS complex
 Normal upper limit 0.20 seconds
 Width of QRS complex indicates the time taken by the depolarisation wave to spread
throughout the ventricles
QT intervals
 Time taken for the whole depolarization sequence in ventricles.
 Obtained from number of squares between beginning of QRS complex and of T wave.
Abnormalities of Conduction
(a) AV Node/Bundle of HIS

 Prolonged PR interval
1) 1st degree heart block - P Wave is followed by QRS complex.
2) 2nd degree heart block - some P waves are followed by QRS complexes but others
are not. There are 3 varieties of 2nd degree heart block
3) 3rd degree (complex) – no P waves are conducted, ventricular escape rhythm
controls the heart with a slow rate, QRS complexes are wide and abnormal.
(b) His bundle branches
 Right bundle - branch block, broadening of QRS complex
 Left bundle branch block - wide QRS complex, inverted T wave
3) ECHOCARDIOGRAPHY
- Use echoes of ultrasound waves to map the heart and study its function.
4) PHONOCARDIOGRAPHY
 Application of a sensitive microphone to the chest which allows heart sounds and
murmurs to be recorded.

5) CARDIAC CATHETERIZATION
 Is introduction of a thin radio-opaque tube (catheter) into the circulation which allows
measurement of pressure in the right heart chambers, left ventricle, aorta and
pulmonary artery
 Blood samples can be taken to measure the concentration of Ischaemic metabolites e.g.
lactate oxygen content.
 Radio opaque contrast material is injected.
6) URINALYSIS
 Amount, haematuria, culture, microscopy and proteins
7) TOTAL BLOOD COUNT

 Red blood cells, white blood cells and platelets
8) Urea And Electrolytes

9) C-Reactive Proteins
10) Blood sugars
11) Liver Function Tests
12) Blood Cultures
13) Blood Lipid Profiles
 Total cholesterol - below 200 milligrams per deciliter (mg/dL), or 5.2 millimoles per
liter (mmol/L).
 Low-density lipoprotein (LDL) cholesterol - less than 130 mg/dL (3.4 mmol/L), and
under 100 mg/dL (2.6 mmol/L) is even better.
 High-density lipoprotein (HDL) cholesterol - 60 mg/dL (1.6 mmol/L) or higher,
though it's common that HDL cholesterol is higher in women than men.
 Triglycerides - be less than 150 mg/dL (1.7 mmol/L)

Lesson 2: Congenital Heart Diseases
Learning Outcomes
At the end of the lesson the learner shall be able to: -

1. Describe the pathology of congenital heart disorders
1.0. INTRODUCTION
 CHD is the abnormality of the heart or blood vessels present from birth. It is the most
important cause of heart disease in the early years of life and the incidence is higher in
premature infants.
 Malformations occur during the stage of cardiac development (3rd - 8th week of
gestation).
 Cardiac abnormalities could be incompatible with intrauterine life, manifest shortly after
birth when foetal circulation changes to the postnatal circulation, cause cardiac
malfunction only in adult life or be entirely innocent.
 Congenital anomalies are morphologic defects that are present at birth. These
anomalies may occur are malformations, disruptions, deformities, sequences and
syndromes.
1) Malformations - primary errors of morphogenesis with an intrinsic abnormal
development process as a result of multiple causes.
2) Disruptions result from secondary destruction of an organ or body region that was
previously in normal development due to extrinsic disturbance in morphogenesis.
3) Deformations result from extrinsic disturbance of morphogenesis through local or
generalized compression of the growing foetus by abnormal biomechanical forces
e.g. uterine constraints such as maternal factors (which ones?) and foetal factors
4) Sequence – a pattern of cascade anomalies (examples?)
5) Syndrome - collection of congenital anomalies
2.0. DEVELOPMENT OF THE HEART
 Remarkable development of the heart occurs in 6 – 7 days but becomes obvious at day
18 or 19 in the cardiogenic area of the mesoderm layer where a paired mass of
specialized cells called the heart cords form
 After a short time a hollow centre develops in each cord to form a heart tubes
 Heart tubes begin to migrate towards each other during day 21 and soon fuse to form a
single median endocardial heart tube
 The process of fusion is accompanied by dilatations and constrictions of the tube so that
when fusion is completed during the 4th week five distinct regions can be seen
 These regions are the truncus arteriosus, bulbous cordis, ventricle, atrium and sinus
venosus

3.0. AETIOLOGY
1. Idiopathic/unknown (90%)
2. Genetic – aberrations, gene mutations and multifactorial inheritance. Examples -
chromosomal abnormalities e.g. Trisomy 21 (Down’s syndrome)
3. Environmental factors such as infections in the mother during pregnancy e.g. rubella,
drugs and alcohol and cigarette smoking, radiation, maternal diabetes
4. Multifactorial causes
4.0. PATHOGENESIS
 Timing of prenatal teratogenic determines occurrence and type of anomaly produced

 Embryogenic period which takes first 9 weeks (early - 1st 3 weeks) and foetal period (10
weeks to birth) determine the outcomes as organogenesis occurs mainly during
embryogenic whereas during the foetal period there is growth and development of
organs with reduced susceptibility to teratogenic agents but susceptible to growth
retardation.
5.0. CLINICAL EFFECTS/FEATURES
 Children with significant congenital anomalies have disturbance in the haemodynamics

of blood flow, failure to thrive, cyanosis, increased risk to recurrent or chronic infections
and high risk of infective endocarditis.
6.0. CLASSIFICATION
1. Malposition of the heart

2. Shunts (Cyanotic Congenital Heart Disease) - Left-to-right shunts & Right-to-left shunts
3. Obstructions (Obstructive Congenital Heart Disease)
7.0. MALPOSITIONS
1. Ectopia Cordis
 Birth defect in which the heart is abnormally located outside the thoracic cavity and has
defective heart muscles and coverings
 Most commonly the heart protrudes outside the chest through a split sternum and less
often the heart may be situated in the abdominal cavity or neck.
 Condition is fatal in first days of life. It is associated with other malformations such as
Tetralogy of Fallot, pulmonary atresia, atrial and ventricular septal defects, and double
outlet right ventricle and non-cardiac malformations e.g. cleft palates
 Most cases result in stillbirth or death shortly after birth
 Depending on the position of the heart from birth ectopia cordis can be classified into
four categories namely - cervical, thoracic, thoracoabdominal and abdominal.

Diagram 2.1: Ectopia Cordis
2. Malposition (Dextrocardia)
 Presence of the heart ion the right hemithorax with the apex of the heart points to the
right side of the chest
 Usually associated with major cardiac anomalies e.g. transposition of great arteries.
Diagram 2.2: Dextrocardia
8.0 SHUNTS (CYANOTIC CONGENITAL HEART DISEASES)

8.1 Introduction
 A shunt is an abnormal communication between heart chambers, between blood

vessels or between the heart chambers and blood vessels
 Pressure differences in heart chambers determines the direction of shunting of the
blood - left-to-right shunting (more common) or right-to-left shunting.
8.2 Classification
1. Left-to-right shunts (late cyanosis or acyanotic heart diseases)

a. Atrial Septal Defect (ASD)
b. Ventricular Septal Defect (VSD)
c. Patent Ductus Arteriosus (PDA)
d. Atrioventricular Septal Defect (AVSD)
2. Right-to-left shunts (early cyanosis or cyanotic heart diseases ) – 5TS
a. Tetralogy of Fallot (TOF)
b. Transposition of great arteries

c. Truncus arteriosus and stenosis
d. Tricuspid atresia and stenosis
e. Total anomaly of pulmonary venous drainage/connection
8.3 LEFT-TO-RIGHT SHUNTS (Acyanotic Heart Disease)
 Cause cyanosis several months or years after birth.
1. Atrial Septal Defect (ASD)
 An abnormal opening in the atrial septum that allows free communication between the
left and right atria
 Usually asymptomatic until in adulthood when pulmonary hypertension (in 10% cases) is
induced causing late cyanotic heart disease and right-sided heart failure
 Effects are produced due to left-to-right shunt at the atrial level with increased
pulmonary flow
 Result in hypertrophy of the right atrium and ventricle, enlargement and haemodynamic
changes in tricuspid and pulmonary valves, reduction in size of left atrium and left
ventricle and reduction in size of the mitral and aortic orifices.
Diagram 2.3: ASD
Features
 Explain the pathophysiology of these
1. Right ventricular hypertrophy features.
2. Cardiac failure  How will use elicit them on physical
3. Cyanosis (late) examination
4. Haemodynamic changes + Murmur
5. Failure to thrive
2. Ventricular Septal Defect (VSD)
 Most common congenital anomaly of the heart usually recognized early in life

 There is incomplete closure of the ventricular septum allowing free communication
between the left & right ventricles
 30% cases occur in isolation but it is frequently associated with other structural
anomalies especially the Tetralogy of Fallot
 Smaller defects of less than 0.5 cm in diameter close spontaneously
 Clinical features range from asymptomatic murmurs to late cyanosis and fulminant
chronic heart failure depending on the size of the defect
 Effects produced due to left-to-right shunt at the ventricular level, increased pulmonary
flow and increased volume in the left side of the heart
 Result in hypertrophy and dilatation of the RA and RV, endocardial hypertrophy of the
LV and enlargement and haemodynamic changes in all the heart valves
Diagram 2.4: VSD
Features
1. Hypertrophy and dilatation of the right atrium
2. Hypertrophy and dilatation of the right ventricle  Explain the
3. Murmur pathophysiology of
4. Cardiac failure these features.
5. Failure to thrive  How will use elicit them
on physical examination
Diagram 2.5: Effects of VSD

3. Patent/Persistent Ductus Arteriosus (PDA)
 Accounts for 10% of CHD and usually occurs as an isolated anomaly in 85-90% cases
 Ductus arteriosus (DA) is a normal vascular connection between the aorta and the
bifuractaion of the pulmonary artery which allows communication between the aorta
and the pulmonary artery during foetal life
 Normally at term the ductus closes within the first 1-2 days of life as a result of muscular
contraction due to the effect of relatively high oxygen tension and reduced local
prostaglandin E (PGE2) synthesis
 Persistence of DA beyond 3 months of life is usually permanent & abnormal
 May be associated with VSD, coarctication of the aorta and pulmonary or aortic stenosis.
 There is an accompanying left ventricular hypertrophy and pulmonary artery dilatation.
Diagram 2.6: PDA
Pathophysiology
 PDA allows shunting of blood from the high pressure aorta to the low pressure
pulmonary artery, increasing volume of blood passing through the lungs & returning to
the left atrium.
 This increased preload leads to left atrial dilation, increased LA pressure, increased PV
pressure and ultimately pulmonary congestion (left-sided heart failure).
 Bulging of the aorta and pulmonary artery proximal to the PDA occurs as a result of
increased blood volume and turbulent flow.
 Pressure difference between the aorta and pulmonary artery (greatest during systole),
and consequently continuous flow of blood through the PDA produces the characteristic
continuous murmur
 Increased flow through the pulmonary artery can result in pulmonary hypertension.
 When the pressure in the pulmonary artery equals or even exceeds that of the aorta,
either the diastolic portion of the murmur or the complete murmur may disappear due
to flow reversal (reverse shunting PDA)
 Blood then bypasses the lungs and the patient presents with cyanosis and a
compensatory polycythaemia.

Effects
1. Loud murmur (machinery murmur)  Why is PDA classified as a left-
2. Pulmonary hypertension to-right shunt disorder?
3. Right ventricular hypertrophy
4. Right atrial hypertrophy
5. Dilated ascending aorta
Diagram 2.7: Effects of PDA
8.0. RIGHT-TO-LEFT SHUNTS (Cyanotic Congenital Heart Disease)
 There is shunting of blood from the right side of the heart to the left side allowing entry
of poorly oxygenated blood into the systemic circulation
 Results in early cyanosis hence the description of congenital cyanotic heart disease.
 The shunts can allow movement of emboli from venous sources to pass directly into the
systemic circulation resulting in what we would call paradoxical emboli.
1. Tetralogy of Fallot (TOF)
 TOF accounts for 10% of children born with heart abnormalities

 It is composed of four (tetralogy) cardinal anomalies namely: -
i) VSD
ii) Dextraposition of the aorta (displacement of the aorta to the right side) so as it
overrides the VSD
iii) Pulmonary stenosis with ventricular outflow obstruction
iv) Right ventricular hypertrophy
 Severity is determined by the extent of RV outflow obstruction & size of the VSD.

Diagram 2.8: Tetrology of Fallot (TOF)
1 - Pulmonary stenosis (a form of right ventricular outflow tract obstruction)

2 - Right ventricular hypertrophy
3 - Overriding aorta
4 - Ventricular septal defect
Effects
 Hypertrophy of the RA and RV, cyanosis, failure thrive, cardiac failure and murmurs
2. Transposition of Great Arteries (TGA)
 Aorta arises from the RV while the pulmonary artery emanates from the left ventricle.
 TGA is common in children of diabetic mothers
 2 common types are regular transposition (commonest) where the aorta is displaced
anteriorly and the to the right of the pulmonary trunk type) and corrected transposition
 Poor prognosis that depends on severity of tissues hypoxia and the ability of the right
ventricle to maintain aortic blood flow.
Diagram 2.9: Transposition of Great Vessels

3. Truncus Arteriosus
 Rare abnormality of poor prognosis associated with numerous connected heart defects
 Embryological structure called the truncus arteriosus does not divide properly into the
pulmonary artery and aorta resulting in a single large common vessel receiving blood
from both the left and right ventricle
 There is an associated VSD
 Presents with early cyanosis due to the right-to-left shunt but the flow later reverses and
the patient develops LVH with pulmonary vascular hypertension
Diagram 2.10: Truncus Arteriosus
Clinical Features
 Cyanosis, cardiomegaly, biventricular hypertrophy, cardiac failure, loud 2nd heart sound,
systolic murmur, wide pulse pressure and bounding pulse
4. Tricuspid Atresia and Stenosis
 Often associated with pulmonary stenosis and atresia with an inter-atrial defect (ASD)
through which right-to-left shunting of blood occurs
 There is absence of tricuspid orifice in tricuspid atresia and a small tricuspid ring with
malformed valve cusps in tricuspid stenosis
 Children with tricuspid atresia are cyanotic since birth and live for a few weeks or
months.
Features
 Progressive cyanosis, poor feeding, tachypnoea, murmur, left ventricular hypertrophy,
normal heart size

5. Total Anomaly of the Pulmonary Venous Drainage (TAPVD)
 Rare cyanotic CHD in which all four pulmonary veins are malpositioned and make
anomalous connections to the systemic venous circulation
 There are no pulmonary veins directly joining the left atrium hence drainage is into the
left innominate vein or to the coronary sinus.
Features
 Volume and pressure hypertrophy of the right atrium and right ventricle, cyanosis,
murmur (systolic ejection) right ventricular heave, RHV, cardiomegaly, cardiac failure,
splitting of S2, S3 gallop, Failure to thrive
9.0. OBSTRUCTIVE CONGENITAL ANOMALIES
 Result in obstruction to blood flow from the heart and are classified as obstruction in
the aorta e.g. coarctication of the aorta, obstruction to outflow from the left ventricle –
aortic stenosis and atresia and obstruction to outflow from the right ventricle –
pulmonary stenosis and atresia
1. Coarctication of the Aorta
 Aorta is compressed or contracted and 50% cases occur as isolated defects with the
remaining occurring with multiple other anomalies of the heart
 There is localized narrowing of the aorta in any part with the constriction being more
often distal to the ductus arteriosus (post-ductal or adult type) or occasionally proximal
to the ductus arteriosus (pre-ductal or infantile type) on the transverse aorta.
 Causes of Death: Chronic cardiac failure, aortic dissection, intracranial haemorrhage and
infective endocarditis
Diagram 2.11: Coarctication of the Aorta

2. Aortic Stenosis and Atresia
 The most common abnormality of the aorta is bicuspid aortic valve, which has less
functional significance but predisposes to calcification. Complete aortic atresia is rare
and incompatible with neonatal survival. Aortic stenosis may be congenital or acquired.
 Congenital aortic stenosis is of three types –
1) Valvular stenosis where there valves cusps are irregularly thickened and malformed
2) Subvalvular where there is a thick fibrous ring under the aortic valves causing
subaoratic obstruction and
3) Supravalvular stenosis that has a fibrous constriction above the sinuses of valsalva.
Effects
1. Left ventricular hypertrophy (pressure overload)
2. Post-stenotic dilatation of the aortic root
3. Infective endocarditis
4. Sudden death (rare)
3. Pulmonary Stenosis and Atresia
 Commonest form of obstructive congenital heart disease where there is fusion of the
cusps of the pulmonary valve forming a diaphragm like obstruction to blood flow and it
may also occur as a component of TOF or may occur in conjunction with transposition
abnormalities
 There is no communication between right ventricle and the lungs so blood bypasses the
right ventricle through an inter-atrial septal defect and enters the lungs via the PDA.
 WHAT ARE THE FEATURES?

Lesson 3: Cardiac Failure (Heart Failure)
Learning Outcomes
At the end of the lesson the learner should be able to: -

1. Define cardiac failure
2. Describe the causes of cardiac failure
3. Describe the pathology of cardiac failure with respect to each cause
1.0. DEFINITION
 Cardiac failure is a
i) Situation when the ventricular myocardium fails to maintain a circulation adequate
for body requirements despite adequate venous return
ii) situation when the heart is unable to deliver adequate supply of oxygenated blood
for meeting metabolic needs of peripheral tissues both at rest and during exercise
iii) State in which an increase in filling pressure and therefore fibre length causes a fall
rather than a rise in cardiac output
iv) Syndrome of ventricular dysfunction
 Heart failure is a clinical syndrome in which patients have the
o Symptoms typical of heart failure (breathlessness, fatigue, tiredness, ankle swelling)
o Signs typical of heart failure (tachycardia, tachypnoea, pulmonary rales, pleural
effusion, raised jugular venous pressure, peripheral oedema, hepatomegaly)
o Objective evidence of a structural or functional abnormality of the heart at rest
(cardiomegaly, third heart sound, cardiac murmurs, abnormality on the
echocardiogram, raised natriuretic peptide concentration)
2.0. RISK FACTORS
 Age, hypertension, physical inactivity, diabetes, obesity, smoking, gender , nutrition ,

family history of heart failure, valvular heart disease, infection, coronary artery disease,
high cholesterol and triglycerides, excessive alcohol consumption, prior heart attack,
certain exposures, such as to radiation and some, types of chemotherapy,
3.0. CAUSES OF CARDIAC FAILURE
 Causes
i) Intrinsic pump failure
ii) Increased work load on the heart - Pressure overload and Volume overload
iii) Impaired filling of the cardiac chambers
iv) Multifactorial ( a combination of the above factors)
3.1. Pump Failure
 Most common and important cause of heart failure

 Frequently results from weakness of ventricular contractions.

Causes
1. Myocardial weakness
2. Cardiac rhythm disorders
3. Reduced or poor myocardial response
4. Multifactorial (multiple causes)
Myocardial Weakness
 Leads to unsatisfactory pumping action of the heart muscles due to reduced

contractibility of myocardium leading to secondary reduction of Blood supply.
Causes
 Classified based on aetiology or function.
a) Aetiological Classification
i) Myocardial Ischaemia and infarction
ii) Infections
iii) Nutritional Deficiency states- Beri Beri (Thiamine)
iv) Systemic CT disorders - rheumatoid arthritis, S.L.E1 and polyarteritis Nodosa.
v) Cardiomyopathies - reduces the contractibility of the myocardium
vi) Metabolic/endocrine - diabetes mellitus, hyperthyroidism and hypothyroidism],
adrenal cortical insufficiency and acromegaly.
vii) Storage disorders - Glycogen storage disease
viii) Infiltrations – amyloidosis, sarcoidosis, heamochromatosis
ix) Sensitivity & Toxic reactions - drugs e.g. cytotoxic drugs, alcohol, cobalt, barbiturates
x) Physical agents - Irradiation
b) Functional Classification
 Based on whether the chambers are dilated or not

 Dilatation can be generalized or focal
 Myocardial weakness may be due to hypertrophic and/or restrictive cardiomyopathy
Pathology of Myocardial Weakness
1. Expulsion of blood by the ventricles during systole is reduced due to the weak pumping
action of the ventricles leaving a residual blood volume.
2. During diastole the chambers dilate to contain both residual and incoming blood
causing dilatation of the ventricles putting the ventricles at a greater disadvantage as
more force will be required to pump out the increased volume of blood (Frank-Starling
Law). But due to the weakness of the myocardium, this is will not achieved and
therefore blood pools in the ventricles.
3. If the destruction is not halted, dilatation of the ventricles and failure are progressive.
1
Systemic Lupus Erythromatosus

4. Ventricular dilation (left ventricle and right ventricle) leads to the stretching of the
respective valves (mitral and tricuspid) resulting in valve incompetence of the Mitral and
Tricuspid valves respectively.
5. This worsens the situation due to reduced cardiac output and damming of blood in
veins which increase systemic venous pressure (systemic venous pressure) slowing the
general circulation.
CARDIAC RHYTHM DISORDERS
 Effective pumping action of the heart is achieved by alternate relaxation and contraction
allowing blood to enter the chambers (diastole) and force it out during contraction
(systole)
 This is achieved by the co-ordination, conduction and rhythmicity of the cardiac muscle
together with the efficiency of the conducting system of the heart
Circus Movement
 Is cardiac impulse conduction around the heart without stopping hence there is
continuous impulse conduction due to an enlarged heart (long pathway), slow
conduction e.g. failure of the purkinje tissue, decreased refractory period which results
from epinephrine, sympathetic stimulation and irritation of the heart by disease and
transmission of impulses in figures of 8’s for example in ventricular fibrillation
Rhythm Disorders
 Arrhythmias can be can disorders of impulse conduction at sites such as the SAN, AVN,
atria, Ventricles and Purkinje tissues or disorders of impulse formation in the form of
abnormal site of origin or abnormal rate of impulse discharge.
Tachycardia
 Is a rhythm rate greater than 100 beats per minute

 Causes include: exercise, anxiety, any disorder that increases the SNS stimulation
 Examples - atrial fibrillation, atrial flutter, paroxysmal Tachycardia and atrial tachycardia
Pathology
 Tachycardia impairs diastolic refilling of ventricles and shortens the coronary artery
diastolic filling reducing blood supply to the heart
 This results in decreased SV and CO thus decreasing blood supply to the myocardium
resulting in ischaemia which reduces the performance of the heart
 Fewer impulses reach the ventricles to effect contraction and therefore the SV and CO
reduce compromising blood supply and there is irregular ventricular response to
transmission of impulses from the atria.
 Resulting incompetent emptying of the ventricles causes pooling of blood in the heart
chambers leading to dilatation and hypertrophy of the ventricles and cardiac failure

Atrial Flutter
 Is an impulse frequency of 125– 300 beats per minute, usually regular but can become
irregular if there is fluctuating heart block.
 Causes include digoxin toxicity, cardiomyopathy, chronic ischaemic heart disease and
rheumatic heart Disease (RHD)
Atrial fibrillation
 Is an impulse transmission of 350 – 600 beats per minute (irregular) and force made
worse on exercise
 Causes include Rheumatic Heart Disease (RHD), coronary Heart disease, hypertensive
heart disease, thyrotoxicosis, cardiomyopathies, constrictive pericarditis, pulmonary
embolism and alcohol abuse
Paroxysmal Tachycardia
 Is an impulse transmission of 150 – 250 beats per minute and it is intermittent
Bradycardia
 Is an impulse rate of below 60 beats per minute

 Causes
i) Physiological (athletes and during sleep)
ii) Pathological - cardiac (acute myocardial infarction, drugs e.g. beta blockers, digoxin,
heart block) and non-cardiac (hypothyroidism, obstructive jaundice, ICP2)
Pathology
 In partial heart block at SAN some impulses reach ventricles to effect contraction but
stroke volume cardiac output and heart rate are reduced but in total heart block at SAN
no impulses pass to effect ventricular contraction hence the ventricles contract at 25
beats/min (normal for ventricular tissue)
 This is inadequate to sustain required blood supply.
Heart Block
 Interferes with conduction process and impulses are blocked from getting through the
ventricular myocardium thus ventricles contract at much slower rate than normal
 Can occur at the
i) SAN, AV – Block; 1st degree there is delayed impulse transmission from AVN to
ventricles;
ii) 2nd degree there is intermittent failure of impulse transmission (Mobitz I block,
Mobitz II block and 2:1 or 3:1 (advanced) block and
iii) 3rd degree where there is complete A–V block
2
Increased intracranial pressure

Causes
 Myocardial infarction, digoxin toxicity, idiopathic fibrosis, congenital heart disease, aortic
valve disease, infiltration - tumours, syphilis, endocarditis, inflammation - rheumatoid
arthritis, ankylosing spondylitis, Reiter’s syndrome and sarcoidosis, rheumatic fever and
diphtheria
3.2. Increased Workload on the Heart
3.2.1 Pressure Overload
 Situation where there is increased resistance to the expulsion of blood from the
ventricles or inflow of blood into ventricles.
Causes
1. Left Ventricle - aortic stenosis and systemic hypertension
2. Right ventricles - pulmonary hypertension, mitral stenosis and lung Disease
Pathology
 Considered in two groups of ventricular outflow obstruction and ventricular inflow
obstruction
Ventricular Outflow Obstruction
 Result from hypertension (pulmonary and systemic), aortic and pulmonary Stenosis
 Pathology
i) Obstruction to out flow of blood from the ventricles causes increased afterload
(ventricular) with the response of ventricular hypertrophy but the ventricular capacity
remains normal (Starling’s Law)
ii) Increased in ventricular muscle bulk causes muscles stiff and this will require higher
atrial pressure for refilling and so there occurs atrial hypertrophy
iii) With the increased load due to increased afterload the ventricles dilate needing high
wall tension to maintain the systolic pressure (Laplace’s Law)
iv) Coronary vessels are unable to supply the increased muscle bulk with adequate
blood so the muscle fibres become ischaemic and die off
v) Ischaemic muscle tissue is replaced by fibrous tissue, which has poor contractibility.
Ventricular Inflow Obstruction
 Result from mitral stenosis, tricuspid stenosis, cardiac tumours, external pressure or
constriction e.g. constrictive pericarditis and endomyocardial fibrosis
 Pathology
i) Obstruction of in flow of blood from the atria causes increased afterload (atrial) with
the response of atrial hypertrophy but the atrial capacity remains (Starling’s Law)
ii) Increase in atrial muscle bulk makes them stiff and this will require higher systemic
venous pressure for refilling and emptying and so there occurs atrial hypertrophy

iii) With increased load (due to increased afterload) the atrial dilatation requires high
wall tension to maintain the systolic pressure (Laplace’s Law) hence there occurs
pooling of blood in the systemic and pulmonary vessels. This reduced ventricular
filing
iv) Reduced ventricular filling cardiac output is reduced
v) Coronary vessels are unable to supply the increased muscle bulk with adequate
blood so the muscle fibres become ischaemic and die off. The ischaemic muscle
tissue is replaced by fibrous tissue, which has poor contractibility
vi) The increased atrial action causes hypertrophy and dilatation, which result in Atrial
fibrillation
3.2.2 Volume Overload
 Occurs when the ventricles are required to expel more than the normal amount of blood
 Causes
i) Incompetent valves that allow blood to flow back into the chambers increasing the
blood volume e.g. aortic regurgitation and pulmonary regurgitation.
ii) States with high general circulation (High Output States) such as severe anaemia,
thyrotoxicosis, Beriberi and patent Ductus Arterious (PDA).
iii) Hypoxia resulting from lung disease (increase circulation) e.g. cor pulmonalae which
leads to an increase in circulation.
iv) Arterio-venous shunts between the left and right sides of the circulation causing
cyanosis and hence hypoxia which causes increased circulation
Pathology
 Based on the effects of ventricular hypertrophy and dilatation, Frank-Starling’s Law and
Laplace’s Law
3.3. Impaired Filling of the Cardiac Chambers
 CO is decreased and cardiac failure ensues due to extra cardiac causes or defects in the
filling of the heart chambers as seen in cardiac tamponade and constrictive pericarditis
3.4. Multiple Factors
 Involves a combination of the above-mentioned factors.
4.0. COMPENSATORY (ADAPTIVE) MECHANISMS
 Functioning of the heart is guided by intimate integrating four principle determinants

that regulate SV and CO
 There are two intrinsic factors - preload (ventricular end-diastolic volume) and afterload
(intraventricular systolic tension during ejection) and two extrinsic autonomic
modulations - contractility (variable force of ventricular contraction independent of
loading) and heart rate (frequency of contraction).

 Compensatory changes in heart failure can be: -
i) Local - chamber enlargement, myocardial hypertrophy and increased heart rate
ii) Systemic Changes - activation of the sympathetic nervous system and RAAS, release
of ANP and ADH
Basic Adaptive Mechanisms
 CVS maintains arterial pressure and perfusion of vital organs when there is huge
haemodynamic burden or disturbance in myocardial contractility through a number of
adaptive mechanisms meant to sustain adequate cardiac performance.
 Mechanisms may be adequate to maintain the overall heart performance at relatively
normal levels, but their capacity to sustain the performance may ultimately be exceeded
and pathologic changes, such as apoptosis, cytoskeletal alterations, and extracellular
matrix (particularly collagen) synthesis and remodeling, may occur, causing structural
and functional disturbances
 Main adaptive mechanisms include
i) Frank-Starling mechanism
ii) Myocardial structural changes (dilatation and hypertrophy)
iii) Activation of neuro-hormonal systems (adrenaline, RAA and ANP).
Frank-Starling Principle
 Describes the relationship between preload and cardiac performance

 Sates that, normally, systolic contractile performance (represented by stroke volume or
CO) is proportional to preload within the normal physiologic range
Compensatory Enlargement of the Heart
 Prevents heart failure or postpones heart failure

 Achieved through structural changes in three processes namely: - hypertrophy (from
increases pumping) dilatation (accommodates excessive blood) and remodelling (change
in structure of myocytes)

5.0. PATHOPHYSIOLOGY OF CARDIAC FAILURE
i) Systolic dysfunction
 HF with reduced EF (ejection fraction)
 Ventricles contracts poorly and empty inadequately, leading initially to increased
diastolic volume and pressure and decreased ejection fraction
ii) Diastolic dysfunction

 Ventricular filling is impaired, resulting in reduced ventricular end-diastolic volume,
increased end-diastolic pressure, or both
 Contractility and hence EF remain normal; EF may even increase as the poorly filled LV
empties more completely to maintain CO
 Markedly reduced LV filling can cause low CO and systemic symptoms.
iii) Cardiac response

 If ventricular function is impaired, a higher preload is required to maintain CO
 Ventricles are remodelled over time
 LV becomes less ovoid and more spherical, dilates, and hypertrophies while the RV
dilates and may hypertrophy
 Initially compensatory, these changes eventually increase diastolic stiffness and wall
tension (ie, diastolic dysfunction develops), compromising cardiac performance,
especially during physical stress. Increased wall stress raises O2 demand and accelerates
apoptosis (programmed cell death) of myocardial cells
iv) Haemodynamic responses

 With reduced CO, O2 delivery to the tissues is maintained by increasing O2 extraction
and sometimes shifting the oxyhemoglobin dissociation curve to the right to favour O2
release
 Reduced CO with lower systemic BP activates arterial baroreflexes, increasing
sympathetic tone and decreasing parasympathetic tone. As a result, heart rate and
myocardial contractility increase, arterioles in selected vascular beds constrict,
venoconstriction occurs, and Na and water are retained
 These changes compensate for reduced ventricular performance and help maintain
hemodynamic homeostasis in the early stages of HF
 However, these compensatory changes increase cardiac work, preload, and afterload;
reduce coronary and renal perfusion; cause fluid accumulation resulting in congestion;
increase K excretion; and may cause myocyte necrosis and arrhythmias.
v) Renal responses
 Decreased perfusion of the kidneys activates the renin-angiotensin-aldosterone system
causing a cascade of potentially deleterious long-term effects
 Angiotensin II worsens HF by causing vasoconstriction, including efferent renal
vasoconstriction, and by increasing aldosterone production, which enhances Na
reabsorption in the distal nephron and causes myocardial and vascular collagen
deposition and fibrosis

 Angiotensin II increases norepinephrine release, stimulates release of vasopressin, and
triggers apoptosis
 Angiotensin II may be involved in vascular and myocardial hypertrophy, thus
contributing to the remodelling of the heart and peripheral vasculature, potentially
worsening HF.
vi) Neurohumoral responses

 Help increase heart function and maintain BP and organ perfusion
 Chronic activation of these responses is detrimental to the normal balance between
myocardial-stimulating and vasoconstricting hormones and between myocardial-
relaxing and vasodilating hormones.
6.0. MANIFESTATIONS OF CARDIAC FAILURE
 Depend on the rate of development of casual factors and the side of the heart affected.
 Development of causal factors - acute or chronic cardiac failure
 Side of the heart involved - Left ventricular failure - LVF), right side (Right Ventricular
Failure - RVF) and total (congestive) cardiac failure, CCF (LVF + RVF)]
Grading Of Cardiac Failure - New York Heart association (NYHA) Classification
Grade I No limitation of physical activity. Ordinary physical activity does not

cause undue fatigue, palpitation, or dyspnoea.
Grade II Slight limitation of physical activity. Comfortable at rest, but ordinary

physical activity results in fatigue, palpitation, or dyspnoea.
Grade III Marked limitation of physical activity. Comfortable at rest, but less than
ordinary activity results in fatigue, palpitation, or dyspnoea.
Grade IV Unable to carry on any physical activity without discomfort. Symptoms

at rest. If any physical activity is undertaken, discomfort is increased.

6.1. ACUTE CARDIAC FAILURE
 Causal factors develop rapidly or suddenly

 Causes – massive myocardial infarction, gross pulmonary embolism, cardiac arrhythmias,
acute bacterial toxaemia, rheumatic fever and rapture of ventricles and valve cusps
 In severe cases of acute cardiac failure (due to myocardial infarction) there is marked
reduction in cardiac output with selective peripheral vasoconstriction following
sympathetic activity causes cardiogenic shock
6.2. CHRONIC HEART FAILURE
 Causal factors develop gradually (slowly)

 Causes - myocardial ischaemia due to atheroma, severe systemic hypertension, chronic
valvular disease/lesions and chronic lung disease causing hypoxia leading to Pulmonary
Hypertension
 In this regard cardiac output is diminished and tissue hypoxia results
6.3. LEFT SIDED HEART FAILURE (LEFT VENTRICULAR FAILURE, LVF)
 More common that right ventricular failure

 LVF leads to RVF then total heart failure (CCF).
Causes of LVF
1. Ischaemic Heart Disease (IHD) particularly Myocardial Infarction
2. Chronic Hypertension/Hypertension
3. Aortic valvular disease due to rheumatic endocarditis, aortic stenosis (calcific), syphilitic
heart disease and congenital heart disease
4. Mitral incompetence/mitral valve disease
5. High output conditions – severe anaemia, AR, fever, thyrotoxicosis, A-V malformations,
Beri Beri
6. Cardiomyopathy
7. Adhesive mediastino-pericarditis
Pathology
i) During systole the LV fails to expel all the blood it receives hence contains an
increasing volume of blood at the end of systole
ii) During the next diastole there is accumulation of the residual blood (left during
systole) and the incoming blood during diastole. Increased diastolic volume causes
dilatation of the ventricle further increasing inadequacy of contraction.
iii) Ventricular dilation causes stretching of valve rings (mitral 10cm) resulting in
incompetence (mitral regurgitation - MR)
iv) MR allows some blood expelled during systole passes through the valve to the left
atria increasing pressure here (left atria) causing venous congestion in the pulmonary
system causing oedema of the lungs (pulmonary oedema)
v) Pulmonary congestion leads to shortness of breath, orthopnoea, PND and
haemoptysis

vi) This retrograde loss of blood through the leaking valve further compromises the
ventricular output and cardiac output.
vii) Decreased CO causes renal ischaemia (acute tubular necrosis, oliguria), CNS ischaemia
-anoxic neuronal changes (dizziness, confusion), bowel ischaemia – mucosal or
transmural necrosis (GI bleeing, sepsis) and skeletal ischaemia (weakness, fatigue,
reduced exercise tolerance)
viii) With the situation persisting there is ventricular dilatation and hypertrophy.
Clinical Features (Manifestation)
 Result from insufficient blood flow through various body organs/tissues and pulmonary
congestion
 Include or involve the heart (size, abnormal heart sounds, pulse), lungs (dyspnoea,
orthopnoea, paroxysmal nocturnal dysnpoea/PND, cough, and cyanosis), pedal oedema,
kidneys, brain and the liver
1) The Heart
i) Size - Cardiomegaly
 Increase in the heart size due to dilatation and hypertrophy of heart chambers.
 Assessment based on subjective visual impression, physical examination
(palpation of apex beat), determination of the cardio-thoracic ratio and volume
measurement (length x width x depth x 0.63)
ii) Abnormal Heart Sounds

 There may be a third or fourth heart sound
 Third Heart sound (S3 Gallop) occurs due to rapid ventricular filling (can be due to
young age (normal), constrictive pericarditis, mitral stenosis and/or regurgitation
and valvular heart disease – mitral/aortic regurgitation
 Fourth heart sound (S4 Gallop) occurs in situations of increased atrial activity due
to LV disease, LVH, dilated heart cavity, pulmonary stenosis, pulmonary
hypertension and acute myocardial infarction

2) The Pulse
i) The Pulse rate – there can be tachycardia or bradycardia
ii) Pulse rhythm and pulse character - pulsus paradoxicus (Kussmal’s sign) or pulsus
alternans
3) The Lungs
 Effects include dyspnoea, cyanosis, cough and crepitations

 Congestion and oedema occur in the pulmonary venous circulation and the alveolar
capillaries as the fluid collects in alveoli (pulmonary) and in severe cases rhexis of red
blood cells into the capillaries occurs causing haemorrhage into alveolar spaces
resulting in haemoptysis
i) Dyspnoea
 Occurs due to inadequate oxygenation of blood flowing though functionally
impaired lungs, anoxaemia of respiratory centre and the carotid sinus and
decreased vital capacity of lungs due to vascular distension
 Explain the pathophysiology of orthopnoea. How will you determine

orthopnoea
ii) PND (Paroxysmal Nocturnal Dyspnoea)
 Pulmonary congestion and oedema are worsened by severe functional imbalance
of ventricles
 Paroxysmal (nocturnal) dyspnoea is a sudden-onset of severe shortness of breath
and coughing, awakening the patient.
 Factors that produce paroxysmal dyspnoea include:
a) Depression of respiratory centre during sleep (decreases arterial oxygen)
b) Decreased ventricular function due to decreased sympathetic tone (decrease
myocardial contractility and hence cardiac output) and
c) Redistribution of fluid to the chest.
 Pathophysiology of PND
a) Excessive sympathetic activity causes venoconstriction so blood moves from
the systemic veins to the pulmonary circulation
b) During sleep, irritability of CNS decreases hence accumulation of oedema with
provoking defence system e.g. cough
c) Decreased muscular activity allows pooling of blood in veins and change in
position or movement expels blood causing sudden increase volume in the
lungs
d) Reabsorption of interstitial fluid in recumbence causing increase blood
volume
e) In active state, hydrostatic pressure at the capillary level is high leading to
fluid effusion into the interstitial spaces. At night (inactive) the reverse
happens leading to net fluid flow in the vascular system, heart and pulmonary
circulation leading to congestion causing paroxysmal dyspnoea
f) The patient lying down improves the venous return from the limbs worsening
the situation

iii) Cyanosis - may be present or not
iv) Cough
 Occurs as a result of irritation of mucosa (oedema fluid)
 May be productive of blood-streaked, frothy sputum due to pulmonary
congestion and oedema
v) Pulmonary oedema occurs due to venous congestion in the lungs and causes
wheezy respirations “Cardiac asthma” Rhonchi, basal crepitations and Chyne-strokes
respiration in chronic pulmonary oedema
4) Kidneys
 Reduced CO causes low glomerular filtration rate (GFR) reducing the renal blood
flow, which results in renal anoxia and vasoconstriction reflexes
 There is sodium retention leading to oedema formation.
5) Brain
 Reduced CO compromises blood flow to the brain resulting in cerebral anoxia,
irritability, and loss of attention span, restlessness, stupor and coma.
6) Liver
 Increased systemic venous pressure causes hepatic congestion (Tender
hepatomegaly) with minor abnormalities such increased SGOT, SGPT, serum Bilirubin
and abnormalities in BSP excretion
6.4. RIGHT VENTRICULAR FAILURE (RVF)
 Usually combined with LVF and pure RVF occurs in few instances
 Usually caused by left ventricular failure (LVF)
 When caused by pulmonary diseases it is described as the heart of pulmonary disease
(cor pulmonale).
Causes
1. Myocardial Infarction (not severe than the left ventricle)
2. Chronic Destructive Pulmonary Disease - chronic Bronchitis, emphysema, pulmonary
fibrosis, pulmonary abscess and pulmonary tuberculosis (PTB).
3. Massive pulmonary embolism
4. Pulmonary hypertension following LVF secondary to IHD
5. Viral myocarditis
6. Constrictive pericarditis
7. Valvular lesions (Tricuspid stenosis and congenital pulmonary stenosis)
8. Left sided failure
9. Congenital heart disease
Pathology
i) LVF causes increase left atrial pressure and the pressure in the pulmonary arterial
pressure which increases the workload on the RV leading to RVH and eventually failure.

ii) Failing right ventricle is unable to expel all the blood received hence becomes dilated.
iii) Dilatation results in the stretching of the tricuspid valve ring leading to tricuspid
regurgitation and blood accumulates in the right atrium, systemic and portal venous
systems leading to systemic venous congestion and causing “Cardiac” type of oedema.
iv) There is increased diastolic volume which causes visceral congestion and effusions,
peripheral congestion and oedema (stasis, pitting oedema and distended neck veins).
Manifestations (Features)
 Disturbance involves damming of blood in the spleenic, systems and portal system and
inadequate flow from lungs to left ventricle
 Venous congestion and stagnation occurs throughout the body causing renal anoxia,
which results in sodium and water retention hence increasing the blood volume.
The Heart - As LVF
Liver
 Congested and enlarged (hepatomegally). In severe cases there is central haemorrhagic
necrosis of liver and healing occurs by formation of a Fibrous tissue a situation that
causes “Cardiac Cirrhosis”
Raised JVP and Oedema

 There is congestion of the peripheral venous system resulting in raised jugular venous
pressure and pitting pedal oedema.
Kidneys
 Congestion and Renal anoxia causes disturbed renal function
 Pedal oedema
Brain - As in LVF
Portal system
 Spleen – may become congested and enlarged
 Therefore is a systemic venous congestion syndrome
6.5. CONGESTIVE (TOTAL) HEART FAILURE (CCF)
 Involves failure of both right and left ventricles which may fail spontaneously for
example in severe myocardial infarction, severe toxic myocarditis e.g. Diphtheria, beri
beri and congestive cardiomyopathy
Causes
1. Increased workload for both ventricles e.g. RHD with lesions involving mitral and Aortic
valves
2. Increased CO e.g. in severe anaemia and thyrotoxicosis (In high output failure - the fall
in cardiac output is relative from a previously high cardiac output). But may still be low
output failure with an abnormally low output

3. Ventricular stiffness that follows poor response to SAN and hypertrophic
cardiomyopathy
NB: Thromboembolic phenomenon is common in CCF due to blood stagnation. This
increases the risk of pulmonary embolism
Low Output Failure – Causes

1. Myocardial disease
2. Ischaemic heart disease (IHD)
3. Myocarditis
4. Cardiomyopathy
5. Arrthymias
6. Hypertension
7. Valve stenosis
8. Cor pulmonalae
Cardinal Signs of CCF

 Pedal oedema, raised JVP, tender Hepatomegally, cardiomegaly, gallop rhythm and
basal crepitations
7.0. STAGES OF CARDIAC FAILURE
8.0. CAUSES OF CARDIAC ENLARGEMENT
Enlargement of the heart occurs due to increased workload (volume and pressure).
1. Left Ventricular Hypertrophy (LVH)

 Common causes of marked left ventricular hypertrophy include: -
i) Systemic Hypertension
ii) Aortic stenosis and regurgitation or mitral regurgitation
iii) Mitral insufficiency
iv) Coartication of the Aorta

v) Collusive coronary artery disease
vi) Congenital abnormalities e.g. septal defects - PDA
vii) High Cardiac output states- thyrotoxicosis, severe anaemia and A – V fistula
 Mild left ventricular hypertrophy is caused by hypertrophic cardiomyopathies and
left ventricular failure of any cause
2. Right Ventricular Hypertrophy (RVH)

i) Left ventricular hypertrophy (LVH)
ii) Chronic Lung disease – e.g. chronic emphysema, bronchioectasis, pneumoconiosis,
pulmonary vascular disease
iii) Pulmonary stenosis and insufficiency
iv) MitraI regurgitation (MR), Mitral Stenosis (MS)
v) Congenital heart disease (C.H.D) with shunts
vi) Pulmonary stenosis (PS)
vii) LVH/LVF
3. Compensatory Dilatation
 Follows valve incompetence or shuts and is usually accompanied by hypertrophy of the
respective ventricles.
 Causes
i) Valvular insufficiency – mitral and/or aortic regurgitation in Left ventricular dilatation
and tricuspid and/or pulmonary regurgitation in right ventricular dilatation.
ii) Left-to-right shunts e.g. VSD
iii) Conditions with high output states – give examples
iv) Myocardial diseases e.g. cardiomyopathy (which type?)
v) Systemic hypertension
9.0. LUNG-HEART INTERACTIONS
 Normal pulmonary circulation is high capacitance, low resistance and the right ventricle
is thin
 LVF causes pulmonary congestion which decreases PO2 resulting in impaired left
ventricular function. CLVF causes chronic pulmonary congestion and vascular changes
(pulmonary hypertension) which results in RVH (also occur in VSD).
 RVH or pulmonary disease lead to high pulmonary vascular resistance (PVR) resulting in
high pulmonary artery and high right ventricular pressures, which affect LV function
 Congenital heart disease e.g. VSD causes a left-right shunt which leads to increased
right ventricular pressure.
10.0. DIAGNOSIS - Framingham Criteria

 Involves simultaneous presence of at least 2 major or 1 major + 2 minor criteria
 Major criteria - PND; neck vein congestion; rales; radiographic cardiomegaly; acute
pulmonary oedema; s3 gallop; increased CVP > 16 cm at right atrium; hepatojugular
reflux; > 4.5 kg weight loss in 5 days of diuresis
 Minor criteria - bilateral ankle oedema; Nocturnal cough; Dyspnoea on ordinary
exertion; Hepatomegaly; Pleural effusion; Reduced vital capacity; Tachycardia > 120 bpm

Framingham Criteria for Congestive Heart Failure – MODIFIED
Major Description
Paroxysmal nocturnal dyspnea
Orthopnea
Weight loss in response to treatment
Neck vein distention
Rales
S3 gallop
Hepatojugular reflux
Cardiomegaly
Pulmonary oedema
Minor Dyspnea on exertion
Nocturnal cough
Hepatomegaly
Bilateral ankle oedema
Tachycardia
Chest radiograph
Pleural effusion
Vital capacity decreased one third from maximum
11.0. COMPLICATIONS
i) Renal failure
ii) CVA (stroke) Explain the pathophysiology of
iii) Valvular heart disease these complications
iv) Hepatic failure
v) Cardiac arrhythmias
vi) Anaemia
vii) Venous stasis
viii) DVT
ix) Pulmonary embolism
x) Cardiac arrest

Lesson 4: Ischaemic Heart Disease (IHD)
Learning Outcomes

1. Describe blood supply to the heart
2. Evaluate risk factors in and causes of IHD
3. Describe the pathophysiology and pathology of IHD
1.0. INTRODUCTION
 IHD is a situation when there is diminished myocardial blood supply due to arterial
blood flow obstruction or vasoconstriction
 Can be an acute or chronic state of cardiac disability arising from an imbalance between
the supply of oxygen and myocardial demand for these nutrients
 Obstruction or narrowing of the coronary arterial system is the most common cause of
myocardial anoxia hence the term coronary artery disease is used synonymously with
IHD.
2.0. BLOOD SUPPLY TO THE MYOCARDIUM
Diagram 4.1: Blood supply to the Myocardium (Anterior)
Coronary Circulation
 Two coronary (the left and right) arteries responsible for blood supply

 Dominant artery is the one that gives off the AV nodal artery and supplies the posterior
descending artery
 In majority of individuals the right coronary artery is dominant while in the remaining
the circumflex artery is dominant
 Some individuals have collateral channels that connect the major coronary arteries.
 Coronary arteries are good examples of end arteries but there exists a collateral cardiac
and extra-cardiac collateral circulation with a rich anastomososes even though the
blood vessels involved are usually very small and can only open if occlusion of the
coronary arteries is gradual
 Coronary veins run parallel to major coronary arteries draining blood into the coronary
sinus, which empties blood directly into the right atrium.
3.0. RISK FACTORS
1. Fixed factors e.g. age, male sex and positive family history
2. Potentially changeable with treatment
a. Strong Association - hyperlipidaemia, cigarette smoking, hypertension and diabetes
mellitus
b. Weak Association – personality, obesity and physical inactivity, gout, contraceptive
pill and heavy alcohol consumption
4.0. CAUSES OF IHD
1. Reduced coronary blood flow due to obstruction

 Atheroma/artherosclerosis, arteritis e.g. inflammation, thrombosis, vascular spasms,
embolus, coronary ostial stenosis (e.g. syphilis), coronary arteritis (e.g. polyarteritis),
aneurysm – coronary artery, trauma – contusion, compression - tumours
2. Decrease in the flow of oxygenated blood

 Anaemia, carbohyhaemaoglobinaemia and hypotension – coronary perfusion
pressure
3. Increased demand for oxygen

 Increased cardiac output - thyrotoxicosis and myocardial hypertrophy - aortic
Stenosis, hypertension
5.0. PRESENTATION
 Depends on the characteristics of the lesion in the coronary arteries in terms of onset,
duration, degree, location and extent
 This influences the effects of myocardial ischaemia which may present as: -
asymptomatic state, angina pectoris, myocardial infarctions (acute and chronic), cardiac
arrhythmias, cardiac failure and sudden death

ANGINA PECTORIS
1.0 INTRODUCTION
 Angina pectoris is a clinical syndrome associated with transient sudden, severe

paroxysmal substernal pain due to diminished blood flow through the coronary artery
(inadequate perfusion). Angina means strangling
 Pain is prompted by exertion, cold and emotional stress and lasts a short time, radiates
to the shoulder (jar, check, left arm), relieved by rest and drugs (vasodilatation -TNT)
 Angina occurs because myocardial cells become ischaemic but the damage is reversible
 Platelets and fibrin aggregate blocking the lumen and the platelet constituents (TXA 2,
histamine and serotonin) promote vasospasm which worsens the situation.
 Main risk factors are myocardial infarction, cardiac failure and sudden death
2.0 CAUSES
i) Coronary artery disease resulting in impaired perfusion – atheroma, syphilis, valve

disorders (AS, AR, severe MS) and vasospasm
ii) Myocardial infarction - promotes Angina by decreasing blood supply to the surviving
myocardium around the infarction. It also relieves angina by eliminating the dead tissue
3.0 PREDISPOSING FACTORS
 Include those that result in increased myocardial oxygen demand such as: -
i) Increased ventricular preload e.g. exercise, anaemia and thyrotoxicosis
ii) Increased ventricular afterload e.g. hypertension, valvular lesions ( AS), obstructive
cardiomyopathy
iii) Increased ventricular wall tension due to dilation and hypertrophy
iv) Decreased heart function e.g. myocarditis and tachycardia
 Factors prompting attacks include physical activity, exposure to cold, exercises, injury,
shock and coronary artery spasm
Pathology
a. Coronary artery shows arteriosclerosis, patchy fibrous intimal thickening, calcification,
accumulation of lipid debris and fibrosis
b. Myocardium exhibits ischaemic changes and fibrosis
c. ECG shows abnormal conduct
Classification
1. Class 0: Asymptomatic
2. Class 1: Angina with strenuous Exercise
3. Class 2: Angina with moderate exertion
4. Class 3: Angina with mild exertion
1. Walking 1-2 level blocks at normal pace
2. Climbing 1 flight of stairs at normal pace
5. Class 4: Angina at any level of physical exertion

4.0 PRESENTATION and CLINICAL PATTERNS OF ANGINA
 There are 3 overlapping clinical patterns of angina pectoris namely stable (typical)
angina, Prinzmetal’s variant angina and unstable (crescendo) angina
Stable (Typical) Angina
 Most common pattern (also described as classical or exertional)

 Characterized by attacks of pain following emotional or physical exertion due to chronic
stenosing coronary atherosclerosis and relieved by rest
 ECG shows depression of the ST segment due to poor perfusion of the subendocardial
region of the left ventricle
 No elevation of enzymes in blood because there is no irreversible myocardial injury
Prinzmetal’s variant Angina
 Characterized by pain which occurs at rest with no relationship with physical activity
 Mainly due to sudden vasospasm of the coronary trunk induced by coronary
atherosclerosis or release of humoral vasoconstrictors by mast cells in the coronary
adventitia
 ECG shows ST segment elevation due transmural ischaemia
 Patients respond well to vasodilators.
Unstable (Decrescendo) Angina
 Also called pre-infarction angina or acute coronary insufficiency due to multiple factors.
 Most serious variety characterized by more frequent onset of pain, prolonged duration
pain, often occurring at rest
 Indicates impending myocardial infarction and has multiple aetiology.
5.0 INVESTIGATIONS
 Why are these investigations above necessary?
1) ECG
2) Coronary angiography
 What parameters will you look for when the results are
out?
3) Chest X-Ray
4) VDRL  What are the important findings on examination of
5) Haemogram + ESR cardiovascular system of a 50 year old man who
6) Echocardiography presents with angina pectoris
MYOCARDIAL INFARCTION (MI)

1.0 INTRODUCTION
 MI is a lethal disease of modern times which occurs as a result of ischaemia and affects
mainly the ventricular myocardium
 Magnitude of infarction depends on amount of collateral flow, metabolic requirements
of the cells and duration of ischaemia
 Atheroma of the coronary vessels accounts for the majority of cases but rarer causes

2.0 INCIDENCE
 Higher in industrialized countries due to association with atherosclerosis

 Affects more males than females
3.0 CAUSES
– See the causes of ischaemic heart disease
4.0 PATTERNS AND TYPES OF INFARCTS
 Classified according to the

i) Anatomic regions of the left ventricle involved (anterior, posterior or inferior, lateral,
septal and circumferential; combinations – anterolateral, posterolateral and
anteroseptal)
ii) Degree of thickness of the ventricular wall involved (full thickness or transmural,
subendocardial)
iii) Laminar or age (newly-formed or acute/recent/fresh; advanced –
old/healed/organized)
 3 main patterns namely regional infarct, transmural infarct and subendocardial infarct
Diagram 4.2: Blood Vessel Blockage Sites
Regional myocardial infarcts (RMI)
 Accounts for 90% cases. It results from occlusion of a single vessel

 Occupies the segment of myocardium normally supplied by a particular coronary artery
 May involve a variable thickness of the myocardial wall
 Important arteries whose occlusion result in regional infracts of the heart are: -
1) Left anterior artery - supplies the anterior and lateral wall of LV, part of inter-
ventricular septum and the apex
2) Left circumflex artery that supplies the posterior wall of LV.
3) Right coronary artery supplying RV
4) Left circumflex and right coronary supplying the posterior part of intra-ventricular
septum.
Transmural Infarct
 Results from occlusion of a single coronary vessel and involves full thickness of the
myocardial wall
 Majority result from thrombosis complicating atheroma.
Subendocardial Infarcts
 Affect the inner wall of left ventricle and account for 10% cases of myocardial infarcts
 Result from generalized widespread atherosclerosis in all coronary vessels but with no
specific occlusion
 Subendocardial region is most vulnerable part of the myocardium because
i) Collateral supply developed tends to supply the subendocardial part of the
myocardium
ii) It is under the greatest tension from compressive forces of the myocardium.
 May be confined to the inner half of the myocardium and may be regional or
circumferential.
5.0 CLINICAL PRESENTATION
 May present as acute or chromic myocardial infarction

 Acute myocardial infarction is the most important consequence of coronary artery
disease.
Diagnosis
 Based on three types of features – clinical features, ECG changes and serum enzymes
determinants.
Clinical Features
 Chest pain(what characteristics?), indigestion, apprehension, oliguria, low grade fever,
shock and acute pulmonary oedema
ECG Changes
 ST segment elevation, T wave inversion and wide deep Q waves
Serum cardiac Markers

 Certain proteins and enzymes are released into blood from the necrotic heart muscle
after myocardial infarction

6.0 PATHOLOGY
i) Structural changes
 Infarcts with variation in size > 2 cm affecting the inner part of myocardium
 Majority of the infarcts are transmural (whole thickness)
ii) Macroscopy
a) Congestion (Blotchy congestion)
b) Pale myocardium
c) Haemorrhagic margins
d) Softened patch (dead tissue)
e) Colour change from grey brown to yellow green
f) Red zone of vascular granulation (later)
Diagram 4.3: Myocardial Infarction
iii) Microscopy
a) Coagulative necrosis changes
b) Polymorphonuclearr leucoytes (neutrophils, monocytes)
c) Digestion of tissues by macrophages
d) Show necrotic changes at the margins
7.0 DIFFERENTIAL DIAGNOSIS
1. Aortic dissection
2. Pulmonary embolism  What are the differentiating
3. Spontaneous pneumothorax features of these conditions?
4. Pericarditis  What investigations will be
5. Oesophageal rupture crucial in differentiating these
6. Peptic Ulcer disease diagnoses
7. Pancreatitis

8.0 COMPLICATIONS
 Explain how these complications occur? Pathophysiology

 How will they present?
 How will you investigate for these complications?

Lesson 5: Valvular Heart Disease (VHD)
Learning Outcomes

1. Describe the causes and mechanisms of valvular damage
2. Explain the pathology and clinical presentation of valvular heart disease
3. Investigate valvular heart disease
4. Explain complications of valvular heart disease
1.0 INTRODUCTION
 Valvular heart diseases comprise of the disorders of the heart valves

 Normal function of the heart depends on the mechanical efficiency of the valves whose
malfunction contributes immensely to the disability of heart function.
2.0 VALVE DEFORMITY
 A valve deformity can be a stenosis or regurgitation

 Stenosis - a reduction in valve aperture, increases pressure load in preceding chambers
 Regurgitation – result in failure of the valve to close completely and increases volume
load on both sides of the valve
Diagram 5.1: Valve Deformities
3.0 CAUSES OF DEFORMITY/DISORDERS
1. Congenital – usually associated with other congenital disorders

2. Acquired - E.g. Rheumatic fever (the commonest cause)
a. Post-inflammatory scarring
b. Degenerative changes with aging
c. Dilatation of the valve ring e.g. in ventricular dilatation
d. Degeneration of collagen support tissue of the valve
e. Acute destruction by acute necrotizing inflammation

4.0 THE MITRAL VALVE
4.1. Introduction
 Consists of 2 leaflets/cusps (a larger anterior & a small posterior), annules, the chordae
tendineae and papillary muscles
 Normal function depends on mechanical efficiency of the cusp, chordae, papillary
muscle, pliability and size of fibrous ring/annules and adequacy of LV contraction
 Normal size of circumference is 5 – 12 cm
 Atrial surface of the leaflets has two zones peripheral smooth or body zone and central
rough or coaptation zone separated by a gently curved coaptation line
 Coaptation zone (depth and length) of the valve is critical to valve competency
 Has a cross-sectional area of 5 cm2 and allows ventricular filling at peak rate of 500 –
1000 mls/s
Diagram 5.2: Mitral Valve
4.2. Mitral Stenosis (MS)
 Rheumatic Heart disease is a major cause of MS affecting more female than males
 In 2/3 cases the aortic valve is also affected
Aetiology
a. Congenital
b. Acquired - rheumatic fever /rheumatic heart disease, calcification, infective endocarditis,
rheumatoid arthritis and Systemic Lupus Erythromatosus (S.L.E.)
Pathophysiology
 Disturbance in LV filling due to reduced mitral valve area causes a reduction in peak LV
filling rate and loss of normal period of diastasis
 During exercise as HR increases and a pressure gradient develops with an increase in
mean LA pressure in an effort to improve ventricular filling. This results in LA
hypertrophy and dilatation

 Chronic LA hypertension causes elevation of pulmonary capillary, venous and arterial
pressures favouring transudation of fluid resulting in oedema (pulmonary oedema)
 Pulmonary hypertension leads to RV hypertrophy, dilatation and failure.
 There will be congestion in systemic veins (raised JVP, hepatomegally & pedal
oedema)
 There is reduced CO due to poor LV filling and RV failure eventually ending up in cardiac
failure.
Pathology
 There is distortion of normal mitral valve anatomy with fusion of commisures

1) The cusps:
i) Thickened, distorted and vascularized throughout (normally they are vascularized
at the base only)
ii) Dense fibrous tissue with infiltrations of lymphocytes and plasma cells
iii) Fused along the free margins forming a “Button Hole” or “Fish mouth” opening
iv) Thrombus formation and calcification
v) Great thickening and rigidity causes stenosis and regurgitation
2) Chordae - thickening, fusion and contraction
3) Valve ring is calcified
Clinical Features
Symptoms
 Reduction exercise tolerance, breathlessness, fatigue, heaviness of limbs, palpitations,
cough (blood-tinged, frothy, frank haemoptysis), haemoptysis (due to chest infection,
pulmonary infarction, acute pulmonary oedema and rupture of small blood vessels in
lungs), angina (due to pulmonary hypertension, RVF and previous coronary embolism),
nocturnal dyspnoea (late complain), recurrent chest infection
Physical Examination (Signs)

i) General Examination
 Weight loss, peripheral cyanosis, “malar flash”, rapid, irregular pulse (atrial
fibrillation), rapid with a normal character (but amplitude may be reduced) or slow
rising (small volume, slow rising) and raised JVP if there is right ventricular failure
ii) The Pericardium

 “Tapping apex” due to a palpable first heart sound, left parasternal heave, loud Hs
(first heart sound) with a rumbling mid-diastolic murmur/thrill.
iii) Effects (Other features)
1. LA myocardial hypertrophy is limited causing increase in pressure in LA and

accumulation of blood in LA and pulmonary veins leading to pulmonary venous
congestion. There is also dilatation of the left atrium

2. Increased pulmonary venous pressure (PVP) causes pulmonary arterial hypertension
leading to right ventricular hypertrophy (RVH) exhibiting features such as dyspnoea,
persistent cough, pulmonary oedema, paroxysmal nocturnal dyspnoea (PND) and
haemoptysis due to engorged blood vessels
3. Right ventricular hypertrophy results in tricuspid regurgitation
4. Congestive cardiac failure
5. Thrombosis
6. Systemic Embolism (worst being cerebral infarction)
7. Atrial fibrillation
8. Pulmonary hypertension results in pulmonary valve regurgitation that produces an early
diastolic murmur (Graham-Steell murmur)
Effects
1) Left atrial dilatation and hypertrophy
2) Left ventricular hypertrophy and dilatation
3) Diastolic murmur
Investigations
1. Chest X-ray
a. Heart size is normal or increased (commonly left atrial enlargement)
b. Calcification may be visible
c. Lung fields show dilated veins with an increase in size of main pulmonary artery
(pulmonary hypertension)
d. Evidence of pulmonary oedema - lymphatic lines, generalized hazy shadowing and
obvious interstitial oedema
e. Pulmonary haemosiderosis in long standing cases
2. E.C.G. shows atrial fibrillation and left atrial hypertrophy (bifid “p” wave)
3. Echocardiogram
4. Cardiac catheterisation.
5. Full haemogram and ESR
Complications
1) Atrial fibrillation
2) Systemic embolism
3) Pulmonary hypertension
4) Pulmonary infarction
5) Chest infection
6) Infective endocarditis
7) Tricuspid regurgitation
4.3 Mitral Regurgitation (MR)
Aetiology
 RHD (accounts for 50%) and prolapsing mitral valve are the most common causes
 Any disorder that causes dilatation of the left ventricle causes mild mitral regurgitation

Table 5.1: Causes of MR
Structure Anatomical Pathogenesis

Affected Fault
1. Valve cusps Congenital cleft Atrial Septal Defects (ASD)

Redundant cusps - Marfan’s syndrome,
- Floppy valve syndrome, loss of collagen
Perforation - Infective endocarditis
Distortion/Scarring - Rheumatic fever
Iatrogenic - Floppy Valve
2. Chordae Redundant chordae - Marfan’s syndrome, Floppy valve
Ruptured chordae - Floppy valve, Marfan’s syndrome
- Infective endocarditis/Rheumatic
Chordal shortening - Rheumatic, endomyocardial, fibrosis
3. Papillary muscle Dysfunction - IHD and cardiomyopathy
Prolapsing mitral valve ring - Various
Rupture - Acute myocardial infarction
4. Valve ring Dilatation - severe LV disease
Calcification - Various
Pathophysiology
 Incompetence of the mitral valve allows regurgitation of blood into the left atrium
during systole producing LA dilatation.
 During diastole the additional blood volume freely moves into the left ventricle
stretching the left ventricle. This increased volume load leads to LV dilatation and
hypertrophy and eventually left ventricular failure.
 Pressure rise in the left atrium during ventricular systole leads to pulmonary
congestion and oedema.
 There is increased volume in the atria and ventricle leading to dilatation and
hypertrophy of left ventricle.
Clinical Features
i) Symptoms - As in mitral stenosis
ii) Physical Examination - Signs

 Laterally displaced apex beat (diffuse and thrusting), soft 1st heart sound, systolic
thrill, a prominent 3rd heart sound (resulting from sudden rush of blood into the
dilated LV in early systole), apical pan systolic murmur (regurgitation occurs
throughout systole) radiating to axilla

Effects
1. Regurgitation of blood into left atrium during ventricular systole
2. Left ventricular Dilatation and Hypertrophy
3. Right ventricular hypertrophy and dilatation
4. Congestion of the lungs and pulmonary hypertension
6. Left ventricular failure leading/right ventricular failure/CCF.
Investigations
1. Chest X-ray – shows left atrial and left ventricular enlargement, increased cardio-thoracic
ratio (CTR), valve calcification
2. ECG (bifid p wave)
3. Echocardiogram – dilated left atrium and left ventricle
4. Cardiac catheterisation – prominent left atrial systolic pressure
5. Full Haemogram + ESR
5.0 THE AORTIC VALVE
5.1. Anatomy
 Consists of 3 semi lunar segments/cusps (2 posterior cusps - the left and right and one
anterior cusp)
 Cusps are larger, thicker and stronger attachments and opposite the cusps of the aorta
there are 3 slight dilatations (aortic sinuses)
 The orifice of the aorta surrounds the cusps
 Aortic valve disease is a common cause of sudden cardiac deaths
Diagram 5.3: Aortic Valve
5.2 Aortic Stenosis (AS)
Introduction
 An important cause of cardiac disability that represents a fixed obstruction to the left
ventricular ejection at the level of the valve cusps
 Becomes symptomatic when the valve orifice is reduced to 1 cm2 (normal is 3 cm2)

Diagram 5.4: Aortic Stenosis
Causes
1. Valvular
a. Calcified bicuspid valve
b. Rheumatic(post inflammatory scarring)
c. Senile degeneration (wears & tear) which results from arteriosclerotic degeneration
and calcification
d. Congenital – Valve with a single commissure and Bicuspid valve
e. Infective endocarditis (rare)
f. Hyperlipidaemia (rare)
2. Fixed sub-aortic stenosis (sub-valvular) for example congenital fibrous ridge or
diaphragm.
3. Supravalvular stenosis e.g. congenital fibrous diaphragm above the aortic valve
4. Hypertrophic cardiomyopathy e.g. septal muscle hypertrophy obstructs left ventricular
outflow.
Pathophysiology
 Pressure resulting from the aortic stenosis leads to development of a pressure gradient
between the left ventricular cavity and aorta.
 This resistance is fixed hence differs from the increased peripheral resistance of systemic
hypertension which fails during exercise (pressure overload)
 The resultant obstructed left ventricular emptying leads to increased left ventricular
pressure and compensatory left ventricular hypertrophy.
 Left ventricular hypertrophy (LVH) causes an increase in the diastolic stiffness of the
cavity and therefore end-diastolic pressure increase causing pulmonary vascular
congestion.
 The increased ventricular wall thickness (hypertrophy) results in relative ischaemia of left
ventricular myocardium leading to – angina, arrthymias and left ventricular failure.

Pathology
1) Post-Rheumatic aortic stenosis the cusps are thickened, rigid and partly adherent.
2) Calcification - the cusps are thickened by fibrosis, have irregular nodules and are not
fussed or vascularized
Clinical Features
i) Symptoms
 Breathlessness (paroxysmal nocturnal dyspnoea – PND), chest pain due to Ischaemic
heart disease (IHD) and angina and syncope
ii) Signs
 The pulse - slow rhythm, low volume, slow rising/plateau
 Palpation - normally placed apex beat is not usually displaced because hypertrophy
(as opposed to dilatation) does not produce noticeable cardiomegaly, apical
heaving; systolic thrill
 Auscultation -first heart sound is normal or reduced and a 4th heart sound is present
with a systolic murmur that is a low-pitched ejection murmur that radiates to the
carotids (Diamond shaped – crescendo-decrescendo pattern).
Effects
1. LVH/D as a result of pressure overload and ischaemia.

2. Angina and myocardial infarction
3. Ventricular fibrillation
4. Pulmonary congestion and oedema
5. Right ventricular failure
6. Congestive cardiac failure.
Investigations
1. Chest X-ray shows: -

a. LVH & D
b. Dilated aortic root and ascending aorta
c. Calcification of the aorta (may be present)
d. CTR increases if heart failure is present
2. ECG shows: -
a. Left ventricular hypertrophy (LVH)
b. Bifid “p” wave
c. Conduction disturbances such as complete heart block and prolonged PR interval.
3. Echocardiogram reveals disruption of normal anatomy of the heart and thickened,
calcified and immobile aortic valve
Differential Diagnosis
1. Hypertrophic cardiomyopathy

2. Congenital stenosis
3. Heart block
4. Fixed sub-valvular stenosis
Causes of Death
1. Pulmonary oedema
2. Ventricular fibrillation
3. Left ventricular failure (LVF)
5.3 Aortic Regurgitation (AR)
 AR increases the workload (volume) on the left ventricle.

 Commonest causes are Rheumatic fever and Infective endocarditis.
Causes
1. The Cusps
a. Distortion of cusps as in rheumatic fever and rheumatoid arthritis
b. Perforation of cusps as in infective endocarditis and trauma
2. Valve Ring Dilatation - dissecting aneurysm, Marfan’s syndrome, syphilis, Ankylosing
spondylitis and Reiter’s syndrome
3. Loss of support associated with Ventricular septal defect (VSD)
4. Stretching/Distortion of roof of aorta in rheumatic heart disease (RHD), which causes
Fibrous thickening and distortion and calcification.
Diagram 5.5: Aortic Regurgitation
Pathophysiology
 There is reflux of blood from the aorta into the left ventricle during diastole
 The total volume of blood pumped into the aorta must increase hence there is increased
volume of blood in left ventricle causes increased ventricular mass and size of chamber.
 There is associated with increased left ventricular stroke volume
 The aortic run off of blood during diastole reduces the diastolic blood pressure
compromising coronary perfusion

 The large ventricular size makes it mechanically less efficient (ischaemic)
 Walls become stiffer and end diastolic pressure increase leading to pulmonary
congestion (pulmonary oedema).
 The large SV and peripheral dilatation up-stroke with a larger blood volume leads to a
high systolic and low diastolic pressure leading to a bounding pulse or collapsing pulse.
Pathology
 Cusps are thickened with fused commisures and have rolled edges with calcification.
 Thrombosis may be present or absent
 In Infective endocarditis the cusps are destroyed and perforation may be present.
Clinical Features
i) Symptoms
 Dyspnoea, breathlessness and chest pain (angina pectoris)
ii) Physical Examination - Signs

 Signs due to hyper-dynamic circulation and reflux of blood into left ventricle are
pulse is bounding/collapsing/water hammer; Quincke’s sign (capillary pulsation in
nail beds), De Musset’s sign (head nodding with each heart beat), Durozier’s sign
(systolic bruit over femoral arteries), pistol shot femorals (a sharp bang heard on
auscultation over the femoral ) and Corrigan’s sign (visible arterial pulsation in the
neck)
 Palpation - apex beat displaced laterally and downwards and there is a diastolic thrill
 Auscultation - a high pitched diastolic murmur (Austin Flit murmur)
1. PDA
2. Coronary A – V fistula
3. Ruptured sinus of valsalva aneurysm.
Investigations
1. Chest X-ray
2. ECG
3. Echocardiogram
5. Full haemogram
6.0 TRICUSPID VALVE
6.1. Anatomy
 Has three triangular cusps (anterior, inferior and median with small intermediate
segments seen in the angles between the cusps

 The anterior cusps are the largest
 Central parts of the cusps are thick and strong while the margins are thin and
translucent
 Bases of the cusps are attached to the fibrous ring.
6.2. Tricuspid Stenosis (TS)
Causes
1. Rheumatic heart disease

2. Usually coexists with mitral valve disease or aortic valve disease
Pathophysiology
 Causes obstruction to right ventricular filling with a diastolic pressure gradient across
the valve
 Causes increased right atrial pressure causing fluid retention (ascites and peripheral
oedema)
 There is systemic venous congestion producing hepatomegally
 Results in reduced cardiac output.
Clinical Features
i) Symptoms
 Abdominal pain due to hepatomegally, abdominal swelling due to ascites and leg
swelling (oedema)
ii) Signs
 Raised jugular venous pressure (JVP), oedema/ascites, presystolic pulsation over the
liver, murmur (rumbling, mid-systolic murmur best heard at the lower left sternal
boarder and is loud on inspiration) and hepatomegally
Investigations
1. Chest X-ray
2. ECG
3. Echocardiogram
4. Cardiac Catheterisation.
6.3. Tricuspid Regurgitation (tr)
 Frequently functional and occurs in association with dilatation of the LV cavity

 Common in patients with cor pulmonalae, myocardial infarction & pulmonary
hypertensive disease.
Causes
1. Organic - rheumatic fever, infective endocarditis, ischaemia of myocardium, Ebstain’s
anomaly (congenitally malpositioned valve), prolapsing cusp, endomyocardial fibrosis

2. Congenital
3. Functional causes such as right ventricular dilatation, congenital heart disease with left
to right shunt and chronic Cor pulmonalae
Pathophysiology
 There is severe and chronic elevation of the venous pressure (right atrial and systemic
congestion)
Clinical Features
 Symptoms of right ventricular failure
 Signs - raised JVP, oedema/ascites, hepatomegally, pulsation of the liver and pansystolic
murmur.
7.0 THE PULMONARY VALVE
7.1. Pulmonary Stenosis (PS)
 There is obstruction to blood flow between the RV and the main pulmonary artery.
Causes
1. Infundibular Stenosis – this is the narrowing of the valve below the pulmonary valve.
a. Accompany valve Stenosis
b. Congenital abnormalities e.g. ventricular septal defect (VSD)
2. Valvular Stenosis - abnormal valve, congenital
3. Supravalvular Stenosis – narrowing above the pulmonary valve.
Pathophysiology
 During ventricular systole the valve domes upwards but its excursion is limited
 A jet of blood passes through the narrowed valve and is very turbulent with disturbed
pattern of flow.
 The disturbed floe causes dilatation of the pulmonary artery above the valve (post-
stenotic dilatation).
Pathology
 Valve is thickened and the valve commissures are fused along part of their length
leaving a central or slightly eccentric orifice.

Lesson 6: Acute Rheumatic Fever (ARF) & Rheumatic Heart Disease (RHD)
Learning Outcomes
At the end of the lesson the student should be competent to: -
1. Describe the pathogenesis, pathophysiology and pathology of ARF and RHD

2. Investigate and diagnose ARF and RHD
3. Discuss complications and prevention measures of ARF
Acute Rheumatic Fever (ARF)

1.0 INTRODUCTION
 Rheumatic fever is an immunologically mediated inflammatory disorder of connective

tissue that occurs as a sequael of infection by beta haemolytic Streptococci Group
A type 12 affecting especially the heart, joints and subcutaneous tissues
 ARF is predominantly an illness of childhood (children & young adults)
 Occurs in individuals who are genetically susceptible resulting from an abnormal
immune reaction as a delayed non-suppurative sequel of upper respiratory tract
infection with group A streptococci
 ARF is an acute febrile illness with lesions occurring in the heart, skin, joints, tendons
and fascia, subcutaneous tissues, respiratory system and the central nervous system
2.0 AETIOLOGY
 Associated with -haemolytic Streptococci group A type 12 infection

 Strep may share antigens with human tissues particularly the heart muscle and valves
 Usually occurs usually 2 – 4 weeks after a throat infection by streptococcus pyogenes
3.0 PATHOGENESIS
 Based on: -
3.1. Immunological Factors
 Has two components

i) Infective complement - streptococci produce toxic products (streptolysins S and O)
that cause tissue injury
ii) Autoimmune component - the host reaction where there is inflammation mediated
by antigen-antibody complexes that is capable of causing damage through
sensitized T-lymphocytes
The Streptococcus (The infection) – The Infective Component
 Attaches firmly to pharyngeal cells with assistance of lipotechoic acid producing a brisk
antigenic response
 Enters the body through mucosa of the URT, through wounds, breaches of body surface.

 Numerous surface antigens provoke formation of antibodies, which react with the
antigens to form antigen-antibody complexes.
 Cause local inflammatory reaction (lesion) and spreads along the lymphatic and tissue
planes to reach bloodstream.
 Protected from phagocytosis by the M-protein (main virulent factor)
 Produce toxins and enzymes – Toxins - streptolysins (streptolysin O, SLO and
streptolysin S, SLS) cause haemolysis and damage of many cells such as the white blood
cells, liver and heart muscles. Enzymes produced digest macromolecules, activate
fibrinolysis, hyaluronidase breaks down the connective tissue and streptodornase
liquefies purulent discharges.
Diagram 6.1: The Mechanism
IMMUNE
Throat infection due to - RESPONSE
haemolytic streptococcus
(Group A)
Cell mediated and

Cross-reaction with Cardiac and Antibodies to
connective tissues of susceptible streptococcal antigens
individuals
Streptococcal antigens
Host Reaction – Autoimmune component
1. Autoimmune reaction resulting from invasion by the streptococci organisms to which

the host responds by producing corresponding antibodies resulting in high anti
streptococcal titres.
2. Antigen-antibody complexes formed trigger tissue destruction resulting in an
inflammatory response with the appearance of the circulating immune complexes.
3. Individuals with autoimmune tendencies tend to be more adversely affected and this is
supported by the presence of antibodies and the capacity for experimental induction.
3.2. Epidemiological Factors
 Suggestive of familial occurrence and in crowding conditions, seasonal prevalence of

rheumatic fever and streptococcal pharyngitis, rheumatic fever follows streptococcal
infection by 2 – 3 weeks, age 6 – 15 years (peak 8 years), Anti-streptolysin O titres rises
during rheumatic fever attack, prophylactic antibiotic therapy in military camp reduces
upper respiratory tract infections and rheumatic fever, socio-economic considerations,
geographical distribution and individual susceptibility

4.0 PATHOLOGY
 Characterized with exudative and proliferative inflammatory lesions in the connective

tissues especially the heart, joints and subcutaneous tissues
 Manifestations occur as a result of cardiac damage and inflammation at local sites
 Classical features are Aschoff Nodules (Dr. Ludwig Aschoff, German Pathologist, 1905)
Aschoff Nodules
 Pathognomonic feature of ARF
 It is degenerated collagen surrounded by activated histiocytes and lymphoid cells
 Nodes are widespread in connective tissue of the joints, tendons, blood vessels and the
heart (Heart – myocardial tissue and valves)
 Development of the Aschoff nodule takes place in 3 stages
i) Early (Exudative or degenerative) stage - non-specific mucoid degeneration of cells
(neutrophils, lymphocytes, plasma cells) in 4th week of illness resulting in oedema of
the connective tissue. There is increased acid mucopolysaccharide that destroys
collagen fibres. Fibrinoid degeneration occurs too.
ii) Intermediate (proliferative or granulomatous) stage - entails formation of well-

defined nodules – Aschoff nodules during the 4th – 13 week period.
iii) Late (healing or fibrosis) stage - healing of tissues destroyed occurs in about 12 – 16
weeks after the illness. There progressive fibrosis hyalinization and accumulation of
cells (lymphocytes, monocytes, initially leucocytes) with the resultant fibrinous
Aschoff nodule. The nodules heal by fibrosis
Diagram 6.2: Aschoff Nodules
5.0 PATHOLOGICAL LESIONS/CHANGES
 Grouped into three categories

1. General manifestations
2. Cardiac manifestations in the heart walls (pancarditis – endocarditis, myocarditis and
pericarditis), heart valves (rheumatic valvulitis) and blood vessels (rheumatic arteritis)
3. Extra-cardiac manifestations – depict inflammation at local sites
a. Joints – arthralgia and arthritis
b. Serous membranes
c. Skin – subcutaneous nodules, erythema marginatum
d. CNS - chorea

General Manifestations
 Fever with sweating, malaise, raised ESR, and raised C - reactive protein and neutrophil
leucocytosis
Cardiac Lesions (Manifestations)
i) Heart Walls - Pancarditis

a. The Pericardium
 Pericarditis occurs during the early phase of the illness
 Features of pericarditis include pericardial effusion and fibrinous pericarditis
b. Myocardium - myocarditis is usually mild and occurs during the acute phase of the
illness.
c. Endorcarditis
 Diffuse (widespread) inflammation with development of inflammatory oedema
and light cellular infiltration
 Development of Aschoff nodules affects heart valves leading to valvular heart
disease
 Inflammation leads to ulceration of the valve surface, which encourages
accumulation of platelets, exudates and fibrin thrombosis forming small masses
called vegetations
 Endocardial thrombotic vegetations develop on valves
d. The Heart Valves (Rheumatic valvulitis)

 Injured valve surfaces heal by fibrosis and compensatory mechanisms of the
heart, cause destruction of the heart valves (mainly mitral and aortic)
 Valve abnormalities include thickening of the valve leaflets, impaired valve
closure due to ring dilatation and deformity, shortening and fusion of chordae,
fusion of leaflet & chordal and fibrosis and calcification destroys the structure of
the valves
Macroscopy
 Increased weight of heart, cardiac hypertrophy, flabby myocardium, small pale focal
lesions
 Thickening of the valves and loss of translucency
 Small, multiple wary vegetations along the line of closure of the leaflets and cusps.
Microscopy
 Aschoff Nodules, highly vascularized valve cusps, oedema with infiltration with
polymorphs, macrophages, lymphocytes and plasma cells
 There is proliferation of fibroblasts and fibrinoid necrosis of valve cusps
ii) Blood vessels - Rheumatic Arteritis

 Inflammation involving the coronary, renal, mesenteric and cerebral arteries

Extra-Cardiac Manifestations
 Local inflammation is usually encountered in joints and adjacent musculofascial tissues,

serous membranes, the skin and the central nervous system
i) The Joints
 Involvement of joints and adjacent musculofascial tissues causing arthritis with
effusion, muscle pains and weakness
 Inflammatory changes in the synovium with cellular infiltration resembling Aschoff
nodules
a) Polyarthritis
 Usually involves two or more joint at a time
 Migrating polyarthritis commonly affects larger joints (wrist, elbow, knee &
angle) while hip s and small joints of the hands are occasionally affected
 Usually lasts about 4 weeks and resolves without any residual damage
b) Arthralgia
 Causes minor discomfort to severe pain
ii) Serous Membranes

 Involvement of serous membranes in the pericardium and sometimes the pleura with
resultant pleural effusion
 Lungs are congested; firm and rubbery (rheumatic pneumonitis) with the alveolar
ducts being lined with fibrin
iii) The Skin
a) Subcutaneous Nodules
 Firm painless subcutaneous lesions varying in size (0.5 – 2 cm) palpable over bony
prominences that are subject to pressure with a predilection of arms and legs or
tendons on the extensor surfaces of the elbows, knees, the occiput and the scapulae
 Consist of eosinophilic hyaline swelling of collagen with cells (lymphocytes, plasma
cells, macrophages and fibroblasts)
 Occur in 3rd week and last 1-2 weeks and are usually associated with carditis
Diagram 6.3: Subcutaneous Nodules
b) Erythema marginatum
 Is a non-pruritic erythymatous rash that begins as a non-itchy faint red macule and
the erythema spreads on forward while the centre returns to normal

 They are associated with carditis and subcutaneous nodules.
 The lesions may be raised or flat with and irregular margin outline mainly on the
trunk and proximal parts of the extremities and are migratory with the skin rash
fading in 24 hours with no residual scarring
Diagram 6.4: Erythema Marginatum
iv) Central Nervous System – The Brain

 Sydenhan’s3 chorea results from encephalitis
 Lesions located in the cerebral hemispheres, brainstem and basal ganglia consist of
small haemorrhages, oedema and perivascular infiltration by lymphocytes
 Chorea is characterized by distorted and involuntary jerky movements of the trunk and
the extremities accompanied by some degree of emotional instability.
6.0 CLINICAL FEATURES
 Depend on organs involved
6.1. General Features
 Sudden onset include fever, joint pains, general malaise and loss of appetite.
6.2. Cardiac Features
 Cardiomegaly, congestive cardiac failure (CCF), pericardial effusion, ECG changes (raised
ST segment in pericarditis and inverted or flat T-wave in myocarditis), AV block, cardiac
arrthymias and changing murmurs (Diastolic mitral - Carey Coomb’s murmur)
6.3. Extra-Cardiac Features
a) Respiratory System – epistaxis, tachypnoea

b) Musculo-skeletal system - fleeting polyarthritis (knees, elbows, ankles, wrists), swollen,
red and tender joints
3
[St. Vitu's dance] named after Dr. Thomas Sydenham (1686)

c) The Skin - erythema marginatum (trunk and limbs) and subcutaneous nodules (tendons
and joints bony prominences)
d) The Central Nervous System - chorea
7.0 DIAGNOSIS
 Based on the Ducket Jones Criteria that comprises of the major and minor criteria
 Made when there is evidence of strep infection and 2 major criteria and 0 minor or 1
major and 2 minor criteria are evident.
Major Criteria
1. Subcutaneous nodules
2. Pancarditis (friction rub, murmur, cardiomegaly, CCF, and ECG changes)
3. Arthritis (migratory polyarthritis, swollen, tender, red)
4. Chorea
5. Erythema marginatum
Minor Criteria – LEAF

1. Leucocytosis
2. ESR – raised - acute phase proteins (ESR, positive C-reactive proteins, leucocytosis)
3. Arthralgia (joint pain without arthritis)
4. Fever
5. Prolonged P-R interval on ECG
6. Previous rheumatic fever or rheumatic heart disease
Evidence of antecedent Strep infection:

 ASO/Strep antibodies/Strep group A throat culture/Recent scarlet fever/anti-
deoxyribonuclease B / anti-hyaluronidase

8.0 INVESTIGATIONS
1. Chest X-Ray
2. ECG
3. Throat swab – culture
4. Blood culture
5. Total blood count (TBC)
6. C-R proteins
7. ASOT titres
8. Urinalysis
9. Renal function tests
10. Liver biochemistry
9.0 COMPLICATIONS
1. Heart Failure
Rheumatic Heart Disease (RHD)

1.0. INTRODUCTION
 Occurs as an aftermath of destructive effects of rheumatic fever on the endocardium

and heart valves(post inflammatory scarring)
 Destruction results in healing by fibrosis of the damaged surfaces resulting in valve
disorders and incompetence (stenosis and regurgitation)
 RHD can be acute or chromic RHD
2.0. ACUTE RHD
 Presents as acute rheumatic fever (ARF) which occurs mainly in children

 Presents with cardiac and extra-cardiac features
 Recurs in 50 – 70% of young children and causes chronic rheumatic valvulitis
3.0. CHRONIC RHD
 Occurs mainly in adults as a sequale of ARF with destruction of heart valves

 Presents mainly as valvular heart (mitral, aortic, tricuspid and pulmonary)
4.0. MITRAL VALVE
 Thickening of valve leaflets especially at the lines of fusion, fusion of the commissures
and thickening, shortening and fusion of the chordae tendinae resulting in mitral
stenosis (MS), mitral regurgitation (MR) or both.

Diagram 6.5: Mitral Valve Destruction
5.0. AORTIC VALVE
 Thickening of the valve cusp especially along the lines of fusion and fusion of
commissures resulting in aortic stenosis (AS) and aortic regurgitation (AR) or often both
 Damage to valves produces changes in the heart with AS causing left ventricular
hypertrophy and AR left ventricular hypertrophy and dilatation.
Infective Endocarditis
1.0. INTRODUCTION
 Endocarditis is an inflammatory condition of the mural endocardium characterized by

large crumbling vegetations, toxaemia and bacteraemia
 Infective endocarditis is caused by infection of the heart valves or other areas of the
endocardium
 Growth of microorganisms on an endothelium occurs in a pre-existing cardiac lesion
 Offending organism is usually present in masses of thrombus (vegetation)
2.0. TYPES OF ENDOCARDITIS
1. Infective (microbial)
 Mainly bacterial or fungal
 Destroys valve tissue in contrast with non-infective
 Forms thrombosis with microorganisms deep within it (vegetations)
 Associated with thrombus formation
2. Non-infective (non-microbial) endocarditis

 Verrucous (acute rheumatic fever)
 Atypical verrucous (Libman-Sacks in S.L.E)
 Non-bacterial thrombotic endocarditis (NBTE)
3.0. AETIOLOGY
 Low virulence pathogenecity normal commensal organisms of the skin, mouth, urinary
tract and gut

 Organisms enter the blood in inconsequential events of bacteraemia and become
entangled in platelet aggregations on the surface of the abnormal endocardium and
grow to cause persistent infection
 Valvular abnormalities produce turbulent flow, which damages the endocardium causing
deposition of platelets and fibrin forming vegetations
 Vegetations fall downstream from an area of relatively higher pressure.
 Organisms include - aalpha –haemolytic streptococci low virulence organisms e.g. S.
viridans (mouth and pharynx commensals), S. sanguis and S. feacalis, Staphylococcus
aureas; Streptococcus boris (GIT), Staphylococcus epidermidis(skin) –indwelling venous
catheters, artificial pacemaker wires; Streptococcus pneumonia; Haemophilus ssp;
Diptheroids - skin/GIT; Colliform bacilli; Bacteroides; Coxiella burnetti; Neiserria; Gram
negative bacilli- pseudomonas aeruginosa; Fungal – drug addicts/Immunosuppresed
e.g. Candida, Aspergilla’s, Histoplasma; Rickettsia
4.0. PREDISPOSING FACTORS
1. Conditions causing bacteraemia, septicaemia and pyaemia e.g. dental carries/extraction,

boils/Carbuncles, U.T.I, pneumonia, tonsillectomy/Adenoidectomy, surgery (G.I.T, G.U.T,
billiary and open Heart) and drug addicts
2. Cardiac lesions - valve abnormalities, abrasions, mechanical/biological prosthetic valves,
3. Immunosuppression
4. Haemodynamic factors
5. Portals of entry of the organisms – blood.
5.0. PATHOGENESIS
 Involves three interactive processes

1) Host factors that predispose the endothelium to infection
o Include damaged endothelium, prosthesis, and infection and valve abnormalities
while systemic host factors are usually loss of systemic host defences
2) Circumstances enhancing bacteraemia
o Persistent bacteraemia challenges antibody production by B-lymphocytes and
plasma cells. The increased antibody levels and hypergamonaglobinaemia leads
to formation of rheumatoid factors and antiglobulins (False positive for syphilis
VDRL, ANF).
o Tissue damage is caused by excess circulating antigen-antibody complexes
3) Tissue tropism and virulence of circulating microorganisms
o Blood borne organisms adhere to the vegetations and provoke further deposition
of platelets, fibrin and macrophages
o A 24h lag period before rapid bacterial growth occurs
o The vegetation lesion grows and may become obstructive, erosive and
proliferative
.
6.0. CLASSIFICATION
1. Acute bacterial/Infective endocarditis (ABE and SABE)

2. Sub-acute bacterial/Infective endocarditis

Pathogenesis of ABE and SABE
 Jet and venture effects damage the endothelial surface which is exposed to platelet
activity and results in fibrin thrombosis
 Microbes in the thrombus multiply and bacteraemia occurs
 High titres of agglutinating antibodies lead to clumping of bacteria (sticking to the
thrombus).
Acute Infective Endocarditis
 Usually caused by virulent pyogenic bacteria e.g. Staphylococcus aureus and

Streptococcus feacalis present in a local suppurating lesion and causes focal destruction.
 Bacteria proliferate and mix with the leucocytes forming large and crumbling
vegetations that are deposited on the diseased valves resulting in rapid destruction of
cusps and chordae and spread of suppuration to adjacent heart muscle causing acute
heart failure
Subacute Infective Endocarditis (SABE)
 Caused by low-grade bacteria e.g. Staphylococcus viridans, coliform bacteria,

Staphylococcus albus and results in formation of large vegetations gradually increasing
damage to the valve cusps with minimal spread to adjacent structures causing gradual
cardiac failure.
Special Considerations
 Endocarditis in drug addicts is often due to Staph aureaus or fungal (from skin,
contaminated drugs or cutting materials). Right sided endocarditis is more common in
addicts but left sided endocarditis forms the bulk of cases
 Fungal endocarditis is associated with bulkier vegetations that obstruct the valves,
embolize and obstruct large vessels. It occurs as a complication of open heart surgery or
narcotic addiction. It may present with signs of emboli such as hemiplegia.
7.0. PATHOLOGY
Macroscopy (Gross Appearances)
i) The Heart
 Features of chronic rheumatism or features of congenital VHD e.g. floppy mitral
valve, bicuspid aortic valve and calcific aortic Stenosis
ii) Vegetations
 Pathognomonic lesion comprising of large masses of thrombus adherent to valve
cusps or endocardium
 May be single, sessile, polypoidal or cauliflower and may spread outside the cusp.
 Size is influenced by haemodynamics of blood circulation and organism responsible.

iii) Invasion by microorganisms
 Organisms invade the underlying cusps causing necrosis leading to aneurysm
formation, rupture of cusps and rupture of chordae
iv) Destructive effects of lesions

 Depend on the size of the emboli (mall and big emboli)
 Cause infarcts in internal organs due to embolism in the liver, spleen, petechial
haemorrhage, retinal haemorrhage, splinter haemorrhage, Janeway lesions and
subconjuctiral haemorrhage
v) Renal Glomerulonephritis
 Due to glomerular lesion causing haematuria, uraemia and renal failure
Microscopy
1. Vegetations - composed of platelets, fibrin, and colonies of microorganism, scanty

polymorphs and calcification. Below the vegetation there is heavy inflammation and
vascularization.
2. Cusps - hyperaemic, vascularized, thickened, fibrosed, oedematous with necrotic tissues
3. Cellular infiltration with polymorphs, macrophages and giant cells
4. The Kidneys are described as “flea-bitter” because of the pinpoint red spots on
subcapsular (small haemorrhages at site of tuff capillaries) due to immune – complex
deposition. They allow blood into glomeruli and renal tubules causing haematuria.
8.0. CLINICAL FEATURES
i) Cardiac Failure
 Results from volume overload on LV and myocardial damage due to embolic and
immune mechanisms.
ii) Systemic emboli
 Involves the spleen, mesenteric arteries, kidney (58% cases) and cerebral lesions
(hemiplegia, blindness, and dementia)
iii) Immunological Complications
 High levels of circulating immune – complexes are associated with the arthritis,
subungual splinter haemorrhages, purpura and glomerulonephritis. The Osler’s
nodes (small red tender nodes) are embolic in origin.
9.0. CRITERIA FOR DIAGNOSIS (DUKE’S CRITERIA)
Major criteria
1) Two positive blood cultures for organisms typical of endocarditis
2) Three positive blood cultures for organisms consistent with endocarditis
3) Serologic evidence of Coxiella burnetii (or one positive blood culture)
4) Echocardiographic evidence of endocardial involvement

Minor criteria
1) Predisposing heart disorder
2) IV drug abuse
3) Fever ≥38° C
4) Vascular phenomena - arterial embolism, septic pulmonary embolism, mycotic
aneurysm, intracranial haemorrhage, conjunctival petechiae and Janeway lesions
5) Immunologic phenomena - Glomerulonephritis, Osler nodes, Roth spots, Rheumatoid
factor
6) Microbiologic evidence of infection consistent with but not meeting major criteria
7) Serologic evidence of infection with organisms consistent with endocarditis
Definite Diagnosis
 2 major or 1 major + 3 minor or 5 minors
Possible Diagnosis
 1 major + 1 minor or 3 minor
10.0. INVESTIGATIONS
1. Full heamogram and ESR - reduced haemoglobin (Hb), increased wbc’s, reduced
platelets and increased C – reactive proteins
2. Blood cultures At least six samples
3. Liver biochemistry (LFTS) - reduced Serum alkaline Phosphotase
4. Immunoglobins and complement - raised Serum Ig, reduced total complement and C3
complement due to immune-complex formation, circulating immune complexes and
rheumatoid factor
5. Serological tests
6. Urea/Electrolytes
7. Urinalysis
8. ECG – evidence of myocardial infarction
9. Echocardiography
10. Chest X-Ray - evidence of Heart failure and emboli in right-sided endocarditis.
11.0. COMPLICATIONS
1. Intracardiac
 Severe valve deformities and obstruction of valves or outlet tract, rupture of chordae
tendinae, perforation of cusps/leaflet, abscess, fistula, obstruction, embolism into
coronary artery (ischaemic heart disease) and cardiac failure
2. Extra-cardiac
 Systemic emboli to major organs - Kidney (renal failure), Liver (hepatic failure), Retina
(retinopathy) and Brain (cerebro-vascular accident – CVA)
 Mycotic aneurysm formation, pyemia and septicaemia
 Glomerulonephritis (secondary to immune complexes)
 Anaemia
 Other toxic or allergic inflammation of vessel walls leading to petechiae and/or
splinter haemorrhages in the skin, mucosa, conjunctiva and retina.
Lesson 7: Disorders of the Myocardium and Pericardium
Learning Outcomes
At the end of the session the learner should be able to: -

1. Describe the pathology and pathophysiology of the disorders of the myocardium and
pericardium
2. Outline the pathology, features and effects of cardiomyopathy
THE MYOCARDIUM
1.0 ANATOMY
 Muscle tissue of the heart composed of syncytium of branching and anastomosing,

transversely striated muscles fibres
 Consists of two layers – the superficial and deep layers. The superficial layer is the same
in the ventricles and atria but the arrangement of the muscles in the deep layer is more
complex in the ventricles where the left ventricle has large deep layer
 Very rich in mitochondria that provide the ATP required for cardiac function
 Has plentiful sarcoplamic endothelium an equivalent of endoplasmic reticulum of other
cells that is that houses ribosomes responsible for synthesis of proteins.
2.0 DISORDERS OF THE MYOCARDIUM
1. Cardiomyopathy
a. Dilated (Congestive) cardiomyopathy
b. Hypertrophy cardiomyopathy
c. Restrictive cardiomyopathy
2. Myocarditis
3. Myocardial Ischaemia
4. Miscellaneous - fatty infiltration, fatty change and atrophy
CARDIOMYOPATHIES
1.0 INTRODUCTION
 Cardiomyopathy is a general term indicating disease of the cardiac muscle

 Can be primary cardiomyopathy (cause is unknown) or secondary cardiomyopathy
(cause is known)
 WHO definition excludes heart muscles diseases of known aetiologies
2.0 PRIMARY CARDIOMYOPATHY
 A group of myocardial diseases of unknown aetiology.

 Classified based on predominant clinical presentations and pathophysiology
i) Dilated (Congestive) cardiomyopathy – there is ventricular dilatation
ii) Hypertrophic cardiomyopathy – myocardial hypertrophy
iii) Restrictive(obliterative) cardiomyopathy – impaired ventricular filling

Dilated (Congestive) Cardiomyopathy (DCM)
 Characterized by gradual progressive cardiac failure associated with dilatation and

hypertrophy of the four heart chambers
 Because of dilatation of the chambers and the ensuing heart failure, DCM is also called
congestive cardiomyopathy
 Patients present with unexplained heart failure usually between ages of 30 – 60 years
Diagram 7.1: Dilated Cardiomyopathy
Causes
 Unknown but associated factors exist including familial (autosomal dominant), viral
infection – Coxsackie’s virus , HIV, alcohol toxicity (chronic alcoholism) and peri-partum
Pathogenesis
1. Genetic defect along the family pedigree
2. Post viral myocarditis
3. Effects of alcohol or alcohol metabolites
4. Dilated heart discovered within several months before or after delivery due to effects of
hypertension, volume overload and nutritional effects
Pathology
 Macroscopy - cardiomegaly, increased weight of the heart, dilatation of the heart
chambers, thickening of ventricular walls and thrombosis (mural)
 Microscopy- hypertrophy of heart muscle cells, degenerative changes and cellular
infiltration with mononuclear inflammatory cells
Clinical Features
1. Right ventricular failure
2. Left ventricular failure
4. Cardiac arrhythmias
5. Embolism (how will this present and what is the mechanism)
Investigations
1. Chest X-ray – cardiac enlargement

2. ECG – diffuse non-specific ST segment and T wave abnormalities

3. Echocardiogram – dilatation of the left ventricle and/or right ventricle with poor global
contraction
4. Full haemogram
1. Cardiovascular disease (ischaemic, rheumatic, congenital, systemic hypertension)

2. Generalized disease e.g. sarcoidosis
3. Connective tissue disorders e.g. systemic lupus erythromatosus, systemic sclerosis
4. Neuromuscular disease e.g. muscular dystrophy
5. Alcohol excess
6. Glycogen storage disease
7. Cytotoxic drug therapy - cyclophosphamide
Hypertrophic Cardiomyopathy (HCM)
 An inherited disorder of the heart muscle characterized by a variable hypertrophy of the

ventricles without a cardiac or systemic cause.
 Exhibits massively thickened (hypertrophied) inter-ventricular septum that results in
distorted ventricular contraction with abnormal valve movement during systole
Diagram 7.2: Hypertrophic Cardiomyopathy
Causes
1. Familial (autosomal dominant)
2. Collagen disease/storage disease
3. Increased circulating catecholamines
4. Infants of diabetic mothers
Pathogenesis
1. Autosomal dominant resulting from mutations in the genes controlling sarcomeric
proteins on chromosome 14.
2. Collagen disease and myocardial ischaemia cause fibrosis of the intracardial arteries
and compensatory hypertrophy
3. Increased circulating catecholamines may cause hypertrophy of the myocardial fibres

Pathology
 Microscopy - cardiomegaly, hypertrophy, asymmetrical septal hypertrophy
 Microscopy - myocardial cell disorganization, hypertrophy of muscle cell with large
prominent nuclei and replacement fibrosis
Clinical Features
 Cardiac arrhythmias, cardiac failure, syncope, dyspnoea, chest pain and disturbed
systolic ventricular function (double apical pulsation, jerky carotid pulse, ejection systolic
murmur, pansystolic murmur and fourth heart sound)
Complications
1. Atrial Fibrillation
2. Mural Thrombosis
3. Embolization
6. Chronic Heart failure
7. Sudden death
Investigations
1. Chest X-ray
2. ECG – is diagnostic as it shows ventricular hypertrophy
3. Genetic analysis
Differentials
1. Hypertensive heart disease
2. Aortic stenosis
Restrictive Cardiomyopathy
 Characterized by restriction in ventricular filling due to reduction in ventricular volume

 Restriction stems from fibrosis of the ventricular muscle
 Myocardial relaxation is restricted during diastole
 It is associated with Idiopathic/familial states, amyloidosis, sarcoidosis, Loeffler’s
endocarditis, endomyocardial fibrosis and endocardial fibroelastosis
Diagram 7.3: Restrictive Cardiomyopathy

Pathogenesis
 Endomyocardial fibrosis (EMF) is characterized by fibrosis of the endocardium and

tissues underlying the myocardium of inflow tracts of both or either ventricles. The
fibrosis involves the papillary muscles and chordae tendinae resulting in mitral and/or
tricuspid incompetence. The fibrous tissue restricts ventricular muscle contraction.
 Endocardial fibroelastosis is rare and involves formation of a diffuse layer dense, white
avascular tissue composed of elastic fibres.
Clinical Features
 Dyspnoea, fatigue, embolic features, features of constrictive pericarditis such as a high
JVP with diastolic collapse – Friedreich’s sign and elevation of venous pressure with
inspiration – Kussmaul’s sign; Heart – cardiomegaly with a third or fourth heart sound
Investigations
1. Chest X-ray – confirms enlarged heart
2. ECG – low voltage and ST segment and T wave abnormalities
3. Echocardiogram – asymmetrical myocardial thickening, impaired ventricular filling
4. Endomyocardial biopsy
Complications
1. Cardiac Failure
2. Valvular heart disease
3. Thrombosis (mural)
4. Embolism
4.0 SECONDARY CARDIOMYOPATHY
Introduction
 A group of myocardial diseases with known aetiologies or clinical associations but they
are poorly defined
 Excludes well defined entities such as ischaemic, hypertensive, and valvular, pericardial,
congenital and inflammatory conditions of the heart.
The Disorders
 Main disorders include: -

i) Nutritional disorders such as thiamine deficiency, Beri beri heart disease and those
associated chronic alcoholism
ii) Toxic chemicals - Cobalt , Arsenic, Lithiu, Hydrocarbons
iii) Drugs – cyclophosphamide, catecholamines
iv) Metabolic diseases – diabetes mellitus, amyloidosis, glycogen storage disease,
hyperthyroidism and hypothyroidism
v) Neuromuscular diseases e.g. muscular dystrophy
vi) Connective tissue diseases - Rheumatoid arthritis, S.L.E

MYOCARDITIS
Definition - Myocarditis is an inflammatory lesion of the myocardium
Aetiology
1. Infections
a. Viruses - Coxsackie Group A, B, Echovirus type 8, Infleunza, Adenoviruses, Polio, HIV
b. Bacterial toxins/bacteria – Staphylococcus, Syphilis, Streptococcus, Diphtheriae
c. Protozoal - Trypanosomiasis (T. cruzi – Chaga’s disease)
d. Parasites - Trichinosis spiralis and Toxoplasmosis
e. Fungal -Candida albicans, Aspergillus
2. Poisons and chemicals - Drugs - cytotoxics – daunorubicin; Alcohol
3. Physical agents - severe hypothermia, irradiation
4. Hypersensitivity reactions/connective tissues disorders - Rheumatic fever, Rheumatoid
arthritis and S.L.E
5. Endocrine/metabolic disorders - Diabetes mellitus, hypothyroidism, hyperthyroidism
6. Idiopathic
Features
1. Acute unexplained heart failure
2. Cardiac Arrthymias
3. Chest pain
4. Gallop rhythm
5. Cardiac enlargement
Viral Myocarditis
 Presents as acute myocarditis and is usually accompanied by mild acute pericarditis
Incidence
 Infants, Outbreaks in nurseries
 Young adults
Pathology
 Macroscopy - Shows widespread interstitial oedema
 Microscopy - infiltration of the myocardium by macrophages and lymphocytes with
minimal plasma cells and eosinophils
Toxic Myocarditis
 Major feature of diphtheria and may be seen in pneumococcal pneumonia, typhoid

fever, septicaemia, severe acute bacterial infections
 Pathology
 Macroscopy - gross oedema with fibres that are swollen and glassy

 Microscopy- numerous small foci of coagulative necrosis, loss of striations and
nuclei; cellular infiltration with macrophages, lymphocytes and occasionally
polymorphs
Suppurative Myocarditis
Aetiology
1. Pyogenic bacteria - Staphylococcus aureus (localized infection) and Streptococcus
pyogenes (spreading infection)
2. Occurs in septicaemia and pyaemia
Hypersensitivity
 Results from hypersensitivity reactions to antigens shared by causal strep and heart
muscle e.g. in rheumatic fever. It may complicate rheumatoid arthritis, S.L.E, syphilitic
gumma and sarcoidosis
THE PERICARDIUM
1.0 ANATOMY
 Comprises of fibrous and serous pericardium membranes enclosing the heart

 Holds about 50 mls of fluid usually formed by the serous pericardium
 Fibrous pericardium (outer pericardial layer) limits sudden distension of the heart
 Serous pericardium is a thin delicate membrane found within the fibrous pericardium
and covers the heart. Has an outer or parietal layer that lines the fibrous pericardium
and the inner or visceral layer that covers the outer surface of the heart and adjoining
parts of the great vessels.
 Has two recesses – (1) the transverse sinus, which is behind the commencement of aorta
and pulmonary trunk in front of the two atria, and (2) the oblique sinus found behind
the left atrium extending to oesophagus and descending thoracic aorta.
2.0 THE PERICARDIAL FLUID
 Forms a thin film on the surface of the pericardium and acts as a lubricant facilitating
movements of the heart within the pericardial cavity.
 Limits distension of the heart contributing to haemodynamic interdependence of the
ventricles and acts as a barrier to infections.
3.0 PERICARDITIS
Inflammation of the pericardium
Aetiology
1. Idiopathic
2. Infections
i. Bacterial

a) Complication of septicaemia, pyaemia, empyema; ulcerating ca bronchus and
ulcerating ca oesophagus
b) Pyogenic cocci - Streptococcus pyogenes, Streptococcu pneumoniae, Staphylococcu
aureus
c) Tuberculosis
ii. Viral – Echovirus, Coxasackie virus
iii. Protozoal/Parasitic – amoebiasis, toxoplasmosis, ecchninococcal
iv. Fungal - Histoplasmosis (H. capsulatum), Actinomycosis
3. Myocardial infarction
4. In association with connective tissue disorders such as S.L.E, Rheumatoid arthritis, Acute
rheumatic fever and Polyarteritis nodosa
5. Metabolic – uraemia and hypothyroidism
6. Neoplastic – primary and secondary
7. Physical agents – radiation, blunt trauma
8. Haemorrhage due to trauma, aortic dissection and anticoagulant therapy
9. Drug induced
3.1. ACUTE PERICARDITIS
Introduction
 Acute inflammatory process involving the serosal lining of the pericardium

 Characterized by active hyperemia, inflammatory oedema, leucocyte emigration and
exudate accumulates in the pericardial sac with fibrin deposition on the surface giving
the “bread and butter” appearance
 Inflammation is usually fibrinous accompanied by an effusion, (serous, haemorrhagic or
purulent)
 Exudation fluid accumulates in the pericardial sac (pericardial effusion) increasing the
pericardial pressure interfering with atrial filling and circulation in general (cardiac
tamponade)
 Fibrin deposited is removed by process of organization with subsequent fibrous
thickening of the pericardial layers with formation of adhesions (constrictive pericarditis)
leading to cardiac failure.
Aetiology
1. Infective (Infections)
a. Bacterial as a complication of septicaemia, pyaemia, bacterial pneumonia, empyema,
ulcerating ca oesophagus/ca bronchus and tuberculosis OR pyogenic cocci –
Streptococcu pyogenes, Streptococcu pneumoniae and Staphylococcu aureus
b. Viral - Group B coxsackie, Echovirus
c. Parasites
2. Non-Infective
a. Acute and previous rheumatism
b. Immunological
c. Myocardial infarction
d. Metabolic
e. Ureamia following nephrotic syndrome where pericarditis is usually a terminal event
as a result of metabolic derangement
f. Complication of malignancy, trauma
3. Idiopathic
Pathology
 Diagnostic feature at autopsy is usually the “bread and butter” appearance

 Effusion can be serous (in acute rheumatism, myocardial infarction) , haemorrhagic (in
tuberculosis, uraemia, infective microbes, secondary metastatic tumour) and purulent
(suppuration) in septic pericarditis, pyogenic pericarditis
 Pericarditis can be: -
i) Serous (as in non-bacterial inflammation, rheumatic fever, s.l.e, tumours, myocardial
infarction and viral infection)
ii) Serofibrinous/fibrous – is the most frequent )as in rheumatic fever (commonest
cause), myocardial infarction and ureamia
iii) Suppurative/suppurative – septic/pyogenic (mode of infection - direct extension,
blood stream, lymphatics; organisms - strep., staph., meningococcus,
mycobacterium, gonococcus)
iv) Haemorrhagic (as in tuberculosis, tumours, infective microbes)
3.2. CHRONIC PERICARDITIS
 Results from inadequately treated bacterial pericarditis especially TB

 TB Pericarditis follows chronic pulmonary tuberculosis and the presumed route of
infection is by lymphatic or extension from the infected pleura. The exudate formed is
turbid or blood stained. There are tubercles visible on pericardial surface. Calcification
may lead to constrictive pericarditis
 Pericardits presents as CCF, low stroke volume and a small heart
Pericardial Effusion
 Occurs when an inflammatory exudate collects in the closed pericardium

 May give rise to mechanical embarrassment of the circulation by reducing ventricular
filling leading to cardiac tamponade
 Pathophysiology & Clinical features include raised JVP, raised JVP with sharp diastolic
collapse (Friedreich’s sign), a paradoxical pulse (BP falls during inspiration) and
Kussmal’s sign (increased neck vein distension during inspiration)

Lesson 8: Hypertension and Hypertensive Heart Disease
Learning Outcomes

1. Describe the role of risk factors in causation of hypertension.
2. Describe the pathological processes in hypertension
3. Describe the complications and investigations in hypertension
BLOOD PRESSURE
1.0 INTRODUCTION
 BP is the force exerted by the blood against any unit area of the vessel wall. It is usually
measured in millimetres of mercury
 Normal BP is a systolic pressure of 100 – 140 mmHg and diastolic at 60 –90 mmHg
 Systolic pressure is produced by transmission of left ventricular systolic pressure while
vascular tone and an intact aortic valve maintain the diastolic pressure
 Hypertension increases the risk of cardiovascular disease mainly left ventricular failure
and ischaemic heart disease that could result in cardiac failure or/and sudden death and
cerebrovascular accident (CVA, stroke) due to cerebral haemorrhage or infarction
 Mathematically speaking, Blood Pressure (BP) = Cardiac Output (CO) x Peripheral
Resistance (PR). Therefore an increase in CO, or PR or both increases BP (PR is the total
peripheral resistance [sum total])
2.0 BLOOD PRESSURE CONTROL MECHANISMS
i) Nervous mechanism – comprises of the baroreceptor mechanisms, central nervous

system (vasomotor centre, sympathetic autonomic nervous system and the vagus
nerve) and chemoreceptors
ii) Capillary fluid drift activation
iii) Kidneys - Rennin-Angiotensin-Aldosterone (R.A.A) mechanism.
iv) Hormonal mechanisms
2.1. The Nervous Mechanism
 Comprises baroreceptors and chemoreceptors through the vagus and glossopharyngeal

nerves
 Aortic baroreceptor conveys information through the vagus nerve while the carotid
body sends through the Hering’s nerve to the glossopharyngeal nerve. This is also called
pressure buffer system (Buffer nerves). Baroreceptor stimulation causes vasodilatation
and decreases the heart rate and strength of contraction.
 Chemoreceptors control the blood pressure through effects of oxygen lack on arterial
pressure. They are capable of detecting oxygen level in blood and carbon dioxide
concentration in blood via the Hering’s and vagus nerves, which in turn influence the
response through the autonomic nervous system.

Diagram 8.1: BP Control by Baroreceptors (Pressure receptors)
Decreased BP Increased BP
Baroreceptors CNS ANS
Increased BP Decreased BP
2.2. Capillary Fluid Shift Mechanism
 Works by altering the amount of fluid present in the capillaries in that when the capillary
pressure falls too low, there is absorption of fluid from the tissues into the circulation
through the process of osmosis hence elevating the blood volume and in turn the blood
pressure and when the capillary pressure rises too high, fluid is lost out of the circulation
reducing blood volume and pressure.
Diagram 8.2: Capillary Fluid Shift Mechanism

Increased blood volume Increased extra-cellular fluid volume leads to
Changes in pressure after the capillary
Increased
Increased mean Increased Increased
blood
circulating filling venous cardiac
pressure
pressure return output
2.3. Kidneys
 Regulates BP through the RAAS
2.4. Hormonal Mechanism
 Involves three hormone mechanisms namely norepinephrine/epinephrine, vasopressin

and renin-Angiotensin (see RAA system)
3.0 SYSTEMIC HYPERTENSION
3.1. Introduction
 Hypertension is a high arterial blood pressure.

 Normotension is SBP of 140 mmHg and below and DBP of 90 mmHg and below (WHO)
 Therefore, hypertension is SBP of ≥160mmHg and ≥ DBP of 95 mmHg
 Borderline hypertension is SBP of > 140 mmHg and < 160 mmHg and/or DBP of > than
90 mmHg and < 95 mmHg

3.2. Risk and Associated Factors
1) Family history and genetic background – hypertension has a polygenic susceptibility

2) Environmental factors
a) Salt intake – the cell membrane transport defect therefore increased intracellular
sodium and high intake changes the cell physiology
b) Diet and obesity
c) Alcohol consumption (causes skin vasodilatation and vasoconstriction in muscle bed)
d) Coffee – 200 mg caffeine increase BP by 10/8mmHg for 1 – 2 hours.
e) Abnormal blood lipids
f) Stress/noise – acute pain or stress can raise blood pressure
3) Age
4) Gender – women withstand raised BP before menopause
5) Glucose intolerance (Insulin resistance).
3.3. Classification
 Based on clinical course and the cause

 Clinical course (benign and malignant)
 Benign hypertension is a stable elevation of blood pressure over years (long clinical
course) associated with few symptoms and causes disabilities when it is poorly
controlled
 Malignant hypertension is rapid elevation of blood pressure. Usually causes eye
damage with retinal haemorrhage, exudates and papilloedema, renal damage and
hypertensive encephalopathy
 Cause (primary/essential/idiopathic or secondary) hypertension
 Primary hypertension is elevated blood pressure with no known cause and accounts
for 90% of the cases but secondary hypertension, which accounts for 10% of the
cases, have known causes.
 Combination of the classifications
i) Primary (Essential) hypertension (90%) - benign (90%) and malignant (10%)
ii) Secondary hypertension (10%) - benign (80%) and malignant (20%)
3.4. Aetiology
Primary Hypertension
 Has a multifactorial aetiology and that no single aetiology can be identified that there is
no obvious hence the term essential hypertension (a diagnosis of exclusion)
Secondary Hypertension
1) Renal
a) Unilateral - renal artery stenosis, pyelonephritis, obstructive nephropathy, tumours,
tuberculosis and irradiation
b) Bilateral – glomerulonephritis, interstitial nephritis, pyelonephritis, polycystic kidney,
analgesic induced, collagen vascular disease (Systemic Lupus Erythromatosus (S.L.E),

Polyarteritis nodosa (P.N)), gouty, diabetes mellitus, chronic renal failure of any
cause, tumours and nephropathies
2) Adrenal disorders - primary aldosteronism (Conn’s syndrome), Cushing’s syndrome,
phaechromocytoma, congenital adrenal hyperplasia and acromegaly
3) Drug associated - oral contraceptives, corticosteroids and sympathomimetics
4) Others such as acute lead poisoning, pre-ecclampsia, pregnancy and coartication of the
aorta
3.5. Pathophysiology
 Involves two basic mechanisms - volume loading and vasoconstriction
Figure 8.2: Pathophysiology of Hypertension
Volume Loading
 Occurs when excess extra-cellular fluid volume accumulates in the body if all other
functions of the circulation are normal
Vasoconstriction
 Caused by a continuous infusion of a vasoconstrictor agents or excess secretion of a
vasoconstrictor by one of the endocrine organs.
 Vasoconstrictors include: - angiotensin II, norepinephrine and epinephrine
3.6. Pathology
 Mainly affects blood vessels, the heart, systemic arterial tree, brain and the kidneys.

1) Blood Vessels
 Changes are widespread from the aorta to 1 mm diameter vessels

 Changes in the large vessels are the same in all types of hypertension but vary in small
vessels particularly the arterioles in benign and malignant hypertension
 Develop degenerative changes in response to persistent elevation in BP resulting in
reduced vascular lumen, ischaemia, increased fragility and haemorrhage
 Aorta is mainly involved (atheroma, aneurysms, dissection).
 Severely elevated BP damages the tunica intima of small vessels resulting in fibrin
accumulation in the vessels, local oedema and intravascular clotting. Increased intra-
arterial pressure damages the endothelium and angiotensin II induces endothelial wall
contraction allowing plasma to leak through interendothelial spaces
 Plasma constituents deposited in the vessel wall cause medial necrosis
 Effects of vessel pathology include
i) Ischaemia
ii) Aneurysm formation
iii) Rupture of aneurysm
iv) Development and rupture of “berry aneurysm” causing sub-arachnoid haemorrhage.
a) Large and Middle sized arteries

 As a result of expose to increased intra-luminal pressure: -
i)Hypertrophy/thickening of the media due to an increase in size and number of
smooth muscle cells and increased elastic tissue
ii) Arteriosclerosis: Long standing hypertension leads to hypertrophic changes that
culminate in fibrous replacement of smooth muscle. The elastic tissue breaks way
and partial reabsorption may occur.
iii) Thickening and rigidity of arterial walls –reduces capacity of the vessels to expand
and contract
iv) Medial smooth muscle is replaced by collagen causing dilatation and lengthening
of the aorta and its branches accompanied by loss of arterial compliance (vessels
become elongated and tortuous)
 Differential diagnosis is senile arteriosclerosis. The difference is: - patient is
normotensive, features are less pronounced and the media is fibrosed but not
thickened
b) Small Arteries and Arterioles

 Are vessels of less than 1 mm diameter
i) Benign Hypertension
 Show features of arteriosclerosis - medial thickening, pronounced intimal
thickening, lumen narrowing and hyaline thickening (hyaline arteriosclerosis –
DDX - DM)
 Common sites - abdominal viscera, retina, adrenal glands and the kidneys
(affects the afferent arteries severely). It rarely affects the heart, skin and
skeletal muscles
 Effects include accentuation of atheroma, IHD, cerebral infarction, ischaemia of
lower limbs and mesesenteric ischaemia

ii) Malignant Hypertension
 Shows fibrinoid necrosis of small arterial walls and arterioles resulting in: -
necrosis of vessel wall causing cell damage, gross thickening due to permeation
of the necrotic tissue by plasma contents; reduction of lumen diameter (lesion
affects the whole thickness and circumference), intravascular thrombosis and
formation of small infarcts; passage of blood through damaged arterial bed
causes red cell fragmentation (macro-angiopathic haemolytic anaemia) caused
by intravascular deposition of fibrin
 Vascular changes are widespread and mainly notable in internal organs such as
the brain, kidneys (severely affected), the gut and pancreas.
2) The Heart
 Changes result of pressure overload causing hypertensive heart disease which include
LVF and RVF (cor pulmonale) due to ventricular hypertrophy and dilatation, myocardial
ischaemia and infarction and cardiac arrthymias
3) The Kidney
 Due to involvement of small blood vessels supplying the kidneys

 Atherosclerosis results in ischaemia of the nephron, glomeruli and the renal tubular
system causing benign hypertensive nephrosclerosis
4) The Brain
 Destruction of vessels in the brain results in intracerebral haemorrhage and formation of

microinfarcts
3.7. Pathophysiologic Abnormalities in Hypertension
1) Cardiac Output
 Increased in patients with labile and borderline hypertension while in established
hypertension, the CO is normal and increased PR sustains the BP
2) Vessel resistance
 Increased due to hypertrophy of the vessels
3) Cell membrane abnormalities

 Abnormalities in membrane transport systems affected are channels for calcium,
sodium and potassium; exchangers for sodium & hydrogen and sodium & calcium
and pumps for calcium and sodium/potassium
 All these functions are related to the membrane lipids
4) Central Nervous System

 The vasoactive agents have a central action
 Angiotensin II acts centrally to produce thirst and water resistance

5) Sympathetic Nervous System
 There is sympathetic over-reactivity and stimulation causes release of rennin.
 There are catecholamines circulating in the body and the rennin-angiotensin system
is activated too
3.9 Clinical Features What are the clinical features of hypertension?
The Brain
 Hypertensive encephalopathy is characterized by epileptiform fits and transient
paralysis.
Eye changes
 Lesions in small arteries in the retina result in oedema, haemorrhage, infarcts and
exudate formation causing blindness
 Papilloedema may be associated with cerebral oedema
Keith-Wagner Classification
Grade 1 - Increased tortuosity of retinal arteries and increased

reflectiveness (silver wiring)
Grade 2 - Grade 1 plus the appearance of arteriovenous nipping
produced when thickened retinal arteries pass over the retinal
veins.
Grade 3 - Grade 2 plus flame-shaped haemorrhages and soft “cotton
3.10 Investigationswool” exudates
1. Urinalysis (Sugar, proteins, blood, microscopy, culture)
Grade 4 Grade 4 - Grade 3 plus papilloedema (bulging and blurring of edges of
2. Blood
the optic disc)
a. Full haemogramme and ESR
b. Urea & electrolytes
c. Fasting blood sugars
d. Fasting blood lipids
e. Blood uric acid levels
f. Creatinine clearance
g. Serum catecholamines (VMA –vinillyl mendelic acid)
h. Hormonal assay- cortisol and aldosterone
3. Imaging
a. Chest X-ray  What are the parameters of
b. ECG measurements?
c. Renal ultrasound  What is the significance of these
d. Imaging – MRI, IVP/IVU investigations?
e. Renal angiography
4. Renal biopsy
5. Fundoscopy

3.11 Complications
1. Cardiovascular System - congestive cardiac failure (CCF), myocardial infarction/IHD,

peripheral vascular disease and arteriosclerosis
2. Central nervous system - cerebrovascular accident (CVA) and hypertensive
encephalopathy
3. Eyes - Retinopathy
4. Genitourinary tract (G.U.T) – nephropathy and renal failure
5. Gastrointestinal tract (G.I.T) - liver infarcts and pancreatic infarcts
6. Respiratory System - Pulmonary oedema
7. In pregnancy
a. Small for gestational age (SGA)  What is the pathophysiology of
b. Intrauterine foetal death (IUFD) these complications
c. Prematurity  How will you establish the presence
d. P.E.T of these complications
e. Loss of foetus
4.0 HYPERTENSIVE HEART DISEASE
 Results from systemic hypertension of prolonged duration

 Manifests as LVF or RVF (cor pulmonale) or CCF, IHD
Left Ventricular Failure (LVF)
 Pressure overload in the systemic hypertension is associated with hypertrophy of the LV
Cor Pulmonale
 Cor pulmonale (Cor = heart: pulmonale = lung)

 Pulmonary heart disease is the disease of the right side of the heart resulting from
disorders of the lung. It is the right sided part of hypertensive heart disease
 May be acute or chronic depending on rapidity of development
 Acute cor pulmonale
o Occurs following massive pulmonary embolism resulting in sudden dilatation of the
pulmonary trunk, lungs and right ventricle.
 Chronic cor pulmonale
o More common and often preceded by pulmonary hypertension caused by various
chronic lung diseases such as chronic emphysema, chronic bronchitis, pulmonary
tuberculosis (PTB), cystic fibrosis and Pickwickian syndrome (hyperventilation in
marked obesity)

5.0 HYPOTENSION
5.1. Introduction
 Hypotension is a physiologic state in which the arterial blood pressure is abnormally

low. For an adult, hypotension exists when the systolic pressure is less than 90 mmHg
and the diastolic pressure is less than 60 mmHg
 Because arterial pressure is determined by cardiac output, venous pressure and systemic
vascular resistance a reduction in any of these variables can lead to hypotension.
5.2. Risk Factors
1) Age - older than 65 (orthostatic, or postural, hypotension) and neurally mediated

hypotension (primarily affects children and younger adults)
2) Medications- alpha blockers, have a greater risk of low blood pressure.
3) Certain diseases - Parkinson's disease, diabetes and heart conditions
5.3. Causes
 Reduced cardiac output, hypovolemia, blood volume redistribution, reduced systemic
vascular resistance and vascular obstruction (e.g., pulmonary embolism)
5.4. Pathophysiology
 Results from cardiac factors(reduced CO) and vascular factors (vasodilatation)
5.5. Effects (Pathology)

5.6. Clinical Features
5.7. Complications
 Organ damage(heart, brain, renal)

Lesson 9: Aneurysms
Learning Outcomes
At the end of the learner should be able to: -

1. Explain the causes and pathogenesis of aneurysms
2. Discuss pathology and features of aneurysms
3. Outline the complications of aneurysms
1.0 INTRODUCTION AND ANATOMY
 Based on the size of the blood vessels and the histological features
 Arteries are divided into 3 main categories namely large (elastic) arteries e.g. the aorta;
medium sized (muscular) arteries e and small arteries and arterioles- these
 Capillaries are about the size of the RBCs , have a layer of endothelium but no media
 Blood from capillaries return to the heart via post-capillary venules and then veins.
 Histologically, all arteries have three coats called- tunica intima (smooth muscle layer),
tunica media (muscular layer rich in elastic tissue) and tunica adventitia (poorly defined
layer found in the connective tissue in which elastic and nerve fibres, small and thin
walled nutrient vessels, the vaso vasora are dispersed)
Diagram 9.1: Cross Section of Blood Vessels
2.0 DISORDERS OF ARTERIES
 Main diseases incldue artheosclerosis, arteritis (vasculitis) and aneurysms

 Main pathological processes are atheroma (elastic arteries), calcification (muscular
arteries) and arteriosclerosis (small arteries)
 Disorders include
1) Congenital Disorders e.g. congenital or “Berry” aneurysm, hypoplasia of aorta, A-V
fistula or aneurysm
2) Degenerative Changes - atherosclerosis, arteriosclerosis and Marfan’s syndrome

3) Inflammation (Arteritis) -, Polyarteritis nodosa (P.N), Thrombo-angitis obliterans -
T.A.O (Buerger’s disease), Syphilitic, Rheumatic, Rheumatoid, Takayashu's disease
4) Neutrophil Vascular Disorders e.g. Raynaud’s disease
5) Systemic Hypertension (already considered)
3.0 ANEURYSMS
3.1. Introduction
 Is a permanent, abnormal, irreversible localized dilatation of arteries/blood vessel
 Dilatation that is localized in a blood vessel
3.2. Risk Factors
 Advancing age, alcohol consumption (especially binge drinking), atherosclerosis,

cigarette smoking, use of illicit drugs, such as cocaine or amphetamine, hypertension
(high blood pressure), trauma (injury) to the head and infection
3.3. Aetiology
 Congenital(deficiency of media and elastic lamina), Atheroma, Syphilis, Trauma.,

Hypertension, Infection (Staphylococcus aureas, pyogenic abscess), Connective tissue
disorders e.g. Marfan’s syndrome
3.4. Pathogenesis
 An arterial lesion weakens the media locally

 Force expanding the aneurysm is the blood pressure.
 The stretching of the blood vessel results in further weakening
 Once started the aneurysm expands and commonly it ruptures
3.5. Classification
 Based on shape, pathology, size and composition
1) Shape
a) Fusiform aneurysm – results from symmetrical stretching involving the whole
circumference.
b) Saccular aneurysm - involves part of the circumference which dilates
Diagram 9.2: Types of Aneurysms

c) Cylindrical
d) Varicose/sepenteine – tortuous dilatation of arteries
e) Racemose – interconnecting small arteries and veins
2) Pathological mechanisms
 Congenital, Berry aneurysm , atherosclerosis (arteriosclerotic), syphilitic, mycotic
(weakening resulting from infection by microbes) and dissecting aneurysm
3) Size
a) Small aneurysms have a diameter of less than 15 mm
b) Larger aneurysms include those classified as large (15 to 25 mm.)
c) Giant (25 to 50 mm.)
d) Super giant (over 50 mm.)
4) Composition of the wall

a) True aneurysm – composed of all the layers of the vessel wall
b) False aneurysm (pseudoaneurysm) – have a fibrous wall
3.6. Complications of Aneurysms
1. Local pressure effects

2. Rupture – haemorrhage
a. Cerebral berry aneurysm – subarachnoid haemorrhage

b. Dissecting aneurysm of thoracic aorta – blood into pericardium – cardiac failure
(cardiac tamponade)
c. Abdominal aortic aneurysm – massive retroperitoneal haemorrhage
3. Thrombosis and embolism
4. Ischaemia
3.7. Individual Aneurysms
1) Cerebral Aneurysm
 Affects major cerebral arteries
 Commonly called “berry aneurysm” which is usually symptomless and the diagnosis
is made at autopsy but it may rupture and bleed into the subarachnoid space.
 Bulging, weakened area in the wall of an artery in the brain, resulting in an
abnormal widening, ballooning, or bleb
 More frequently occurs in an artery located in the front part of the brain that
supplies oxygen-rich blood to the brain tissue
 Common type of cerebral aneurysm is called a saccular, or berry aneurysm. Others
include fusiform (associated with atherosclerosis) and dissecting aneurysms
 Sites - anterior communicating artery (30 - 35%), bifurcation of the internal carotid
and posterior communicating artery (30 - 35%), bifurcation of middle cerebral
(20%), basilar artery bifurcation (5%) and posterior circulation arteries (5%)
Diagram: Cerebral Aneurysm
Features
 Symptoms of an unruptured cerebral aneurysm include, but are not limited to, the
following Headaches (rare, if unruptured), eye pain, vision deficits (problems with
seeing) and eye movement deficits
 Subarachnoid haemorrhage (SAH), due to rupture of the aneurysm
 Features of SAH include initial sign (rapid onset of severe headache), stiff neck, nausea
and vomiting, changes in mental status, such as drowsiness, dilated pupils, loss of
consciousness, motor deficits (loss of balance or coordination), photophobia, back or
leg pain

2) Mycotic (Infective) Aneurysm
 An infected embolus leads to localized infection and destruction of the tunica media
weakening it e.g. direct extension of organisms from vegetations in bacterial
endocarditis (Staphylococcus aureas), Infection from abscess, TB (fatal haematemesis)
3) Syphilitic (Luetic) Aneurysm

 Complicates syphilitic aortitis commonly affecting the aortic arch and the ascending
aorta as a result of losing the elastica and muscle of the arteries
 Forms a saccular aneurysm and occasionally fusiform
 Pathogenesis
o Inflammation infiltrates around the vasa vasorum of the adventitia followed by
endarteritis obliterans which results in ischaemic injury to the media causing
destruction of the smooth muscle and elastic tissue of the media and scarring
o Most frequent in the ascending aorta and aortic arch
 Effects
1) Pressure/compression effects
a) Syndrome of superior mediastinal compression
b) Displaced great veins with thrombosis causing congestion of head and neck
vessels and enlargement of collateral veins
c) Oesophagus causing dysphagia
d) Bronchus causing chronic cough and suppurating pneumonia
e) Trachea causing dyspnoea
f) Left laryngeal nerve – left vocal cord paralysis, aphonia and horse voice
2) Ruptures - massive haemorrhage into the trachea, oesophagus, pericardium,
pleural cavity and peritoneum
3) Embolism/thrombosis
4) Cardiac dysfunction – when the aortic root and valve are involved, syphilitic
aneurysm produces aortic incompetence and cardiac failure. narrowing of the
coronary ostia aggravates cardiac disease
4) Atheromatous (Atherosclerotic) Aneurysm

 Common in advanced age affecting more males than females mainly affecting the
abdominal aorta and common iliac artery
 Causes fusiform aneurysm, which may rupture when still small
 Results from an artheromatous plague weakens the media or extends into the media.
 Pathogenesis - severe atherosclerotic lesions which cause thinning and destruction
of the medial elastic tissue resulting in atrophy and weakening of the arterial wall.
There is also degeneration of the media.
Effects
1. Rupture into the peritoneum causing peritoneal and intraperitoneal haemorrhage
leading to an acute abdomen.
2. Thrombo-embolic
3. Pressure/compression effects – ureter
4. Arterial occlusion - inferior mesenteric artery

5) Dissecting Aneurysm
 Not a true aneurysm because the vessel is not dilated
 Aetiology - trauma, hypertension, necrosis, connective tissue disorder, congenital,
atheroma and physical exertion e.g. pregnancy
 Pathogenesis
o A tear occurs in the media and blood enters and tracks between the inner and
outer parts of the media dissecting the wall into inner and outer layers
o The blood tends to encircle the aorta and may pass along the entire length of the
bifurcation tracking distally and proximally
o In majority of the cases, the primary lesion causes necrosis of tunica media with
the tunica intima becoming hypertrophic and sclerosed
 Effects
1) Rupture - externally (haemorrhage) or internally (double barrel aorta),
2) Ischaemia due to obstruction – leads to renal infarction, cerebral infarction and
infarction of the spinal cord
3) Thrombosis
4) Cardiac disease
5) Haemorrhage into the Mediastinum, pleura, peritoneum and retro-peritoneal
6) Pressure effects leading to ischaemia which causes myocardial infarction and
renal necrosis
Diagram: Dissecting aneurysm
6) Artero-venous Aneurysm
 This is an abnormally acquired communication between a vein and an artery due to

simultaneous laceration that allows blood to pass from the artery to the vein
producing a local dilatation of the vein, which pulsates as forcefully as the artery
 A thrill can be felt or a bruit can be heard over the aneurysm. It is also called
cirsoid/racemorse aneurysm, false/traumatic.

Lesson 10: Atheroma (Atherosclerosis/Arteriosclerosis)
Learning Outcomes

1. Outline the causes, risk and predisposing factors of atheroma
2. Describe the pathogenesis and pathology of atheroma
3. Outline the effects and complications of atheroma
1.0 INTRODUCTION
 Atherosclerosis is a disease of the intima associated with deposition of sterols,

triglycerides and lipoproteins resulting in narrowing of the vessel lumen, thrombosis or
obstruction in large and medium sized arteries
 Arteriosclerosis affects the media causing proliferation or hyaline changes that result in
an increase in wall thickness and decreased vessel elasticity (arteriosclerosis = hardening
of vessels)
 Atheroma is an intimal plague (patch) created by the focal deposition of lipids in the
subendothelial connective tissue of the inner intima due to accumulation of lipids,
proliferation of smooth muscle cells and formation of fibrosis tissue. They have a soft
lipid rich part (athere = porridge) and a hard (sclerotic) fibrous component. The principal
changes occur largely within the intima of the medium and large arteries.
Diagram 10.1: Atheroma Plaques
2.0 LIPID METABOLISM
 There are two important pathways of lipid metabolism namely exogenous and
endogenous
i) Endogenous (dietary lipids)
o Digested to release triglycerides (TG) and cholesterol esters which combine with
phospholipids and specific proteins to become water soluble chylomicrons
o The TG component of chylomicrons can move from the circulation into cells under
the influence of lipoprotein lipase enzyme found on endothelial surface of cells.

o Within the cell it is then converted to glycerol and non-esterified fatty acids (a
major source of energy)
o Chylomicron without TG is called chylomicron remnant particle (CMR) which is rich
in cholesterol and attaches to liver receptors to enter the hepatocytes
ii) Endogenous lipids
o Lipids produced by the liver from building blocks of glycerol and fatty acids from
the fat stores or synthesized from glucose and cholesterol derived from
lipoproteins (e.g. CMR) or locally synthesized
o Glycerol and fatty acids combine to produce TG
o The liver releases VLDL rich in TG and 25% cholesterol
o Loss of TG produces IDL and further loss of TG results in release of cholesterol rich
LDL. LDL is removed from circulation and broken down to amino acids and
cholesterol.
3.0 AETIOLOGY
 Poorly understood or unknown but risk factors exist based on epidemiological studies,
intervention trials and biochemical investigations
 Risk factor detection and uses ischaemic heart disease (IHD) as an indicator
4.0 THE RISK FACTORS
4.1 Major Risk Factors
Major Constitutional Risk Factors

1) Age – prevalence increases with advancing age
2) Sex - affects more M > F up to the age of 55 years after which the incidence is the same
3) Genetic Factors - genes associated with predisposition to hypertension, diabetes, LDL
receptor changes, altered activation of nicotine (reduces likelihood of smoking), and
altered ion channels proteins which influence arrhythmias.
4) Familial and racial factors - related to other risk factors such as diabetes, hypertension
and hyperlipoproteinaemia.
Major Acquired Risk Factors (Hard risk factors)

1) Hypertension - major risk factor
2) Hyperlipidaemia - cholesterol is essential building material for cell membranes and
hormones. Is transported by lipo-proteins in blood (HDL from peripheral to the liver and
LDL from the periphery to the liver and other systems). Oxidation of LDL encourages
atherosclerosis.
3) Cigarette smoking - encourages thrombosis by increasing the aggregation of
thrombocytes and diminishes coronary flow. Smoking encourages oxidation of LDL.
Women who smoke have increased risks
4) Diabetes mellitus - increased aggregation of platelets, increased LDL & decreased HDL.

4.2 Minor Risk Factors (Soft risk factors)
 Have a lesser role in aetiology of atherosclerosis.

1) Environmental factors - high prevalence in developed than developing countries
2) Diet - obesity and fatty acids and cholesterol
3) Hormonal - exogenous hormones (e.g. oral contraceptives) or endogenous
oestrogen deficiency (post-menopausal women)
4) Physical inactivity - enhances fat deposition
5) Stressful life style - Type A personality (aggressive, competitive drive, ambitiousness
and as sense of urgency increases) as compared to type B personality of relaxed and
happy-go-lucky type.
5.0 SITES
 Common sites in order of decreasing frequency – aorta (abdominal, arch, transverse);

proximal coronary arteries, descending thoracic aorta, femoral and popliteal arteries,
internal carotid artery, vertebral, basilar and middle cerebral arteries (Circle of Willis)
6.0 PATHOGENESIS
 Explain involves various theories
i) Reaction to Injury Theory (Virchow, Duguid and Rokitansky)

 Explains that some change or damage to the vascular endothelium causes increased
permeability of the vessel wall to proteins and lipids leading to aggregation of
platelets and monocytes
 Aggregated platelets and monocytes release substances to promote smooth muscle
proliferation and the influx of more leucocytes
 The leucocytes release various enzymes and growth factors which promote smooth
muscle cell proliferation. Monocytes migrate from the blood into the subendoethelial
layers where they become macrophages and ingest lipids
ii) Insudation or Infiltration Theory (Virchow)

 Suggested that leakage of plasma proteins and lipids from the blood to the sub
endothelial tissue stimulates intimal cell proliferation a form of low-grade
inflammation
iii) Encrustation Theory (Karl von Rokitansky)

 Suggested that thrombi forming on damaged endothelium could be organized to
form a plaque
 Process of Atheroma formation

1) Endothelial cell damage
2) Focal degenerative changes in the subendothelial tissue of intimal ground
substances and fat deposition
3) Fatty deposits

4) Fatty macrophages (foam cells)
5) Release of fats stimulates connective tissue proliferation
6) Capillaries from – Haemorrhage
7) Low grade inflammatory reaction
8) Cell proliferation
7.0 CELLS INVOLVED IN ATHEROGENESIS
 Include the endothelium, smooth muscle, monocytes/macrophage, platelets and

lymphocytes
Diagram 10.2: Cells Involved in Atherogenesis
1) Endothelial Cells
 Modify transport lipoproteins, participate in adherence of leucocytes, form
vasoactive substances, participate in procoagulant and anticoagulant activity and
form growth factors
2) Smooth Muscle - principal source of CT in fibrous plaques, forms growth factors.
3) Monocytes/Macrophages
 When activated can secrete growth factors for connective tissue cells e.g. fibroblasts
and smooth muscles
 Scavenge cells can be injurious to neighbouring cells e.g. endothelium and smooth
muscle and cause mitogenic stimulation of smooth muscle cells.
4) Platelets
 Are a rich source of growth factors and participate in coagulation and thrombosis
5) Lymphocytes: Suggests involvement of immune or auto-immune responses
8.0 PATHOLOGY
 Principal lesions are fatty streaks, fibrous plaque and complicated lesion
Fatty Streaks
 Found throughout the arterial tree at all stages
 Consist of monocytes-derived macrophages that have entered the intima

 Macrophages take up large amounts of lipid in the form of lipid droplets containing
cholesterol
 Can regress and disappear, progress to become fibrous plaque or remain unchanged.
Fibrous Plaque & Complicated Lesion

 Complicated lesion is a fibrous plaque that has become altered by calcification, or
developed cracks/fissures, undergone ulceration leading to haemorrhage & thrombosis
 Thrombosis leads to clinical sequels such as MI, cerebral infarction and gangrene.
Macroscopy
 Slightly raised yellow spots in the luminal surface, spots enlarge, coalesce forming
irregular yellow streaks (PLAQUE)
Microscopy
 Lipid droplets, smooth muscle cells and macrophages, dense connective tissue matrix,
intimal thickening (ischaemia causes necrosis – aseptic necrosis). Plaque – are disc-like
yellowish smooth glistering surface that enlarges and intimal thickening occurs
 They can be yellow or white depending on the amount of connective tissue present.
9.0 CLINICO-PATHOLOGICAL CONSEQUENCES
1) Reduction of blood flow through arteries

2) Predisposition to thrombosis
3) Bleeding into a plaque – coronary arteries leading to myocardial infarction
4) Weakening of vessel walls
10.0 COMPLICATIONS
1) Ischaemia
2) Haemorrhage
3) Rupture
4) Ulceration
5) Occlusive thrombosis, which leads to ischaemia, necrosis, infarction and embolism.
6) Embolism
7) Aneurysm
8) Gangrene
9) Hypertension

Lesson 11: Disorders of Veins
Learning Objectives
At the end of the lesson the learner should be able to:-

1. Explain the pathology of thrombophlebitis and phlebothrombosis
2. Discuss the pathology of varicose veins
3. Discuss the pathology of haemorrhoids
1.0 ANATOMY – STRUCTURE & FUNCTION
 Veins have the basic structure similar to that of arteries as they comprise of the intima,
media and adventitia, which are less clearly defined as in arteries
 Have a large calibre with low venous pressure insufficient to return blood to the heart
 Structure varies depending on the mechanical conditions e.g. intra-luminal pressure. It
changes when mechanical conditions are altered. The structure also will vary as one
ascends the venous tree. Veins collapse when not filled with blood.
 Walls of the veins are thinner; the three tunicae (intima, media and adventitia) are less
clearly demarcated. The elastic tissue is scanty and not clearly organized into internal
and external lamina. The media has small amounts of smooth muscle cells with
abundant collagen
 All veins except vena cavae and common iliac veins have valves which are made of
delicate folds of intima. The valves are located every 1 – 6 cm to the point of a tributary.
 The valves are well developed in the lower limbs. Veins prevent retrograde venous blood
flow.
Diagram 11.1: Anatomy of Veins

2.0 DISEASES OF THE VEINS
1. Thrombosis
2. Phlebitis
3. Thrombophlebitis and phlebothrombosis
4. Varicosities - varicose veins, varicoceole, oesophageal varices and haemorrhoids
3.0 THROMBOPHLEBITIS AND PHLEBOTHROMBOSIS
 Thrombophlebitis refers to the primary inflammation of the vessel wall followed by

thrombosis upon the inflammation
 Phlebothrombosis refers to a condition in which a thrombus forms in the vessel wall due
to secondary infection leading to acute inflammation of the vein.
Aetiopathogenesis
 Thrombosis that precedes thrombophlebitis is initiated by the Virchow’s triad

 Predisposing factors include cardiac failure, malignancy, and use of oestrogen-
containing compounds, post-operative state and immobility
 Most common in the deep veins of the legs with the other sides being periprostatic
venous plexus in the males, pelvic veins in the females and near the foci of infection in
the abdominal cavity (acute appendicitis, peritonitis, acute salpingitis and pelvic
abscess).
Effects
 Local - oedema, heat, swelling, tenderness, redness and pain.
 Systemic - embolic phenomenon with pulmonary thrombo-emboslim being the most
common and most important. Others are bacteraemia and septic embolization to brain
meninges.
4.0 VARICOSITIES
Introduction
 Varicosities are abnormal dilated and tortuous veins
 Usually due to chronic continuous increase in pressure of blood in the veins.
 Physiologically, a varicose vein is a superficial vein that permits blood flow in the reverse
direction due to incompetent valves in the veins
 Varicose veins are usually dilated, lengthened and tortuous
 Veins involved include
i) Lower extremities (involved most frequently) – called varicose veins.
ii) Lower oesophagus (oesophageal varices)
iii) Anal region (haemorrhoids)
iv) Spermatic cord (varicocoele)

4.1. VARICOSE VEINS
 Are swollen and enlarged veins, usually blue or dark purple in colour
 May also be lumpy, bulging or twisted in appearance. They mostly occur in the legs.
Predisposing Factors
 Pregnancy, pelvic tumours, age, sex, race, weight, height, diet, side (left > right), bowel
habit, occupation, heredity, clothes, erect stance
Aetiopathogenesis
 Involves various factors such as

i) Familial weakness of vein walls
ii) Increased intraluminal pressure due to prolonged upright posture (e.g. police,
nurses, surgeons), compression of iliac veins (e.g. pregnancy, intravascular
thrombosis, growing tumour)
iii) Hormonal effects on smooth muscles
iv) Obesity and chronic constipation.
Increased Pressure
 Effects of pregnancy – the presenting part presses on the iliac veins impending blood
flow leading to pooling with subsequent dilatation of veins in the lower limb because of
the ever-increasing pressure.
 In obstruction, the obstruction of the main vein leads to increase I pressure in the
collateral veins leading to dilatation and destruction of valves hence the varicosity and
incompetence of valves in the veins.
Diagram 11.2: Aetiopathogenesis of Varicose Veins
Pathology
1. Weakness of veins and vein wall damage
2. Valve incompetence and valve failure
3. Obstruction
4. Increased pressure

Diagram 11.3: Pathology of varicose veins
Classification
 Two classes namely primary varicose veins and secondary varicose veins
 Primary varicose veins - results from the changes in the vein wall, progressive venous
dilatation and valvular failure. Congenital predisposition and occupation influence
development of the varices
 Secondary varicose veins - occur due to thrombosis with resulting valvular damage,
increased venous pressure in the superficial veins leading to varicosities and
arteriovenous malformations
Sites
1. Legs
2. Arms
3. Scrotum
4. Lower end of the oesophagus
Types of varicose veins
 There are several types of varicose veins, such as:

i) Trunk varicose veins are near to the surface of the skin and are thick and knobbly.
They are usually visible, often quite long and can look unpleasant.
ii) Reticular varicose veins are red and are sometimes grouped close together in a
network.
iii) Telangiectasia varicose veins, also known as thread veins or spider veins, are small
clusters of blue or red veins that sometimes appear on your face or legs. They are
harmless and, unlike trunk varicose veins, do not bulge underneath the surface of
the skin.

Features
Diagram 11.4: Features of Varicosities
Gravitational Varicosity
Usually occurs in the long saphenous vein. It is commoner in females than males
Predisposition
1. Hereditary
2. Continuous standing without much movement leads to increase in pressure hence
dilatation and tortousity of the veins. Veins have valves and depend on muscular activity
for movement of blood upwards and due to inactivity, stasis, pooling and dilatation of
the veins is usually the rule.
3. Pregnancy – the gravid uterus usually causes pressure on the pelvic veins leading to
increased pressure in the venous system at the point of the legs. Usually such
varicosities are particularly worse in individuals with hereditary predisposition.
4. Obesity – usually inactive and therefore the venous return is usually defective because it
depends on muscular activity and hence stasis, pooling of blood and dilatation of veins
occur leading to varicosity. They usually tend to have more fat than musculature and
hence muscular contractibility is even further defective
Complications
1. Valve atrophy
2. Replacement of elastic tissue by fibrous tissue
3. Necrosis (varicose ulcer)
4. Haemorrhage
5. Thrombosis
6. Embolism

4.2. VARICOCELE
 Is a gravitational varicosity that involves dilatation of veins draining the testis

 The veins draining the testis and the epidydimis form a bulky plexus called the
pampiniform plexus
 The distention and pooling of blood depresses the optimal temperature for
spermatogenesis hence the patient presents with seminalysis that reveals oligospermia
and azospermi
Diagram 10.5: Varicocele
4.3. HAEMORRHOIDS (ANAL PILES)
 Haemorrhoids = Greek – haima = blood; rhoos = flowing; Piles = Latin (pila – a ball).
 Haemorrhoids are veins occurring in relation to the anus. This is the dilatation and
turtuosity of the haemorrhoid plexus situated around the ano-rectal junction.
 Haemorrhoids or piles are the varicosities of the haemorrhoidal veins
 Common in elderly persons and women mainly due to increased venous pressure.
Aetiology
1. Hereditary - congenital weakness of vein walls and abnormally large arterial supply
2. Morphological - gravity aid
3. Anatomical – collecting radicles of superior haemorrhoidal vein lie unsupported in the
very loose submucous connective tissue of the ano-rectum.
4. Portal hypertension
5. Chronic constipation
6. Venous stasis
7. Tumours
8. Exacerbating factors – straining, constipation, diarrhoea, dysentery

Classification
 Haemorrhoids may be external or internal in relation to the anal orifice

 External haemorrhoids involve the inferior haemorrhoidal plexus and are covered by the
skin
 Internal haemorrhoids involve the superior haemorrhoidal plexus and are covered by
mucous membrane. When the two are associated, they are referred to interoexternal
(mixed) haemorrhoids.
Diagram 11.6: Types of haemorrhoids
Internal Haemorrhoids
This is a dilatation of the internal venous plexus within an enlargement displaced anal
cushion. The existing communication between the internal and external plexus leads to
dilatation of the internal plexus with a possibility of involvement of the external plexus.
Pathology
 The haemorrhoids are arranged in 3 groups at 3, 7 and 11 o’clock positions following

the arterial supply of the anus (2 divisions on the right branch and a single left branch).
Smaller secondary haemorrhoids exit between the three primary ones.
Diagram 11.7: Haemorrhoids
 Each principal haemorrhoid has three parts: -

i) The pedicle (situated at the anorectal ring, covered with a pale mucosa, pulsating
artery is felt, usually seen on autopsy)

ii) Internal haemorrhoid (commences just below the anorectal region, bright red or
purple in colour, covered by mucous membrane, variable size)
iii) An external associated haemorrhoid (lies between dentate line anal region, covered
by the skin, blue veins seen unless when fibrosis is present)
Clinical Features
 May be symptomatic of some other conditions (symptomatic haemorrhoids) such as Ca

rectum, pregnancy, straining at micturition and from chronic constipation
 Bleeding - slight and bright red and occurs during defecation
 Prolapse – 2nd and 3rd degree haemorrhoids
 Heaviness in the rectum (3rd degree)
 Discharge - mucoid (mucous from the engorged mucous membranes) and leakage of
ingested liquid paraffin
 Pruritis – due to the discharge
 Pain
 Anaemia
Diagnosis
1. History
2. Physical examination - Per rectal examination – inspection and digital examination
3. Protoscopy
4. Sigmoidoscopy
Grading of Haemorrhoids
 First degree haemorrhoids: these bleed but do not prolapse

 Second degree haemorrhoids: these prolapse but reduce spontaneously
 Third degree haemorrhoids: these prolapse but can be reduced manually
 Fourth degree haemorrhoids: these are permanently prolapsed and cannot be reduced
Diagram 11.8: Grading of Haemorrhoids

Complications
1. Profuse Haemorrhage
2. Strangulation
3. Thrombosis
4. Ulceration – accompanies thrombosis and strangulation
5. Gangrene
6. Fibrosis/scarring
7. Suppuration/inflammation
8. Pyephlebitis (portal pyemia).
External Haemorrhoids
 External comprise of distinct clinical entities.

1. A thrombosed external haemorrhoid (perianal haematoma) is a small clot in the perianal
subcutaneous connective tissue formed due to backpressure on anal veins due to
straining, coughing and lifting heavy weights.
2. Dilatation of the veins of anal verge
3. Sentinel pile.
4.4. OESOPHAGEAL VARICES
 Oesophageal varices are swollen veins in the lining of the lower oesophagus near the
stomach
Causes
1) Portal hypertension, which is most commonly caused by liver cirrhosis.
2) Portal vein thrombosis (blood clots inside the portal vein)
3) Portal vein obstruction
4) Idiopathic portal hypertension

Risk factors
 Size of the varices—the larger they are, the more easily they can rupture
 Red colour signs—during an endoscopic examination, the varices may reveal red
markings or spots
 High portal vein pressure
 Severe cirrhosis
 Continued alcohol use—consuming alcohol despite pre-existing liver problems
 Bacterial infection
 Oesophageal varices are unlikely to display symptoms unless they have ruptured
 When ruptured
o Hematemesis
o Abdominal pain
o Light-headedness
o Melena (black stools)
o Bloody stools (only in severe cases)
o shock (only in severe cases, due to blood loss)
4.5. VENOUS THROMBOSIS
 Formation of a semi-solid coagulum within flowing blood in the venous system

 Thrombosis of the deep veins of the leg is complicated by the immediate risk of
pulmonary embolus and sudden death
 Patients are at risk of developing a post-thrombotic limb and venous ulceration
Aetiology
 Revolves around three factors (Virchow’s triad) namely changes in the vessel wall
(endothelial damage), changes in flow of blood (stasis) and changes in blood
composition (e.g. coagulability of blood - thrombophilia)
 There are many predisposing causes of venous thrombosis
 Development of DVT is multifactorial but immobility remains the most important factor
Pathology
A thrombus often develops in the soleal veins of the calf, initially as a platelet aggregate.
Subsequently, fibrin and red cells form a mesh until the lumen of the vein wall occludes.
4.5.1. DEEP VENOUS THROMBOSIS (DVT)
Definition
 DVT is a clot that most commonly occurs in one leg, but can also occur in the arm,
abdomen or around the brain
 Symptoms can develop slowly or suddenly; involve the foot, ankle, calf, whole leg/arm
 These include pain, swelling, discoloration (bluish, purplish or reddish skin colour) and
warmth..
Risk Factors
1) Immobility
 Hospitalization, being paralyzed, prolonged sitting, limb immobilized by plaster cast
(< 1 month)
2) Surgery and Trauma

 Major surgery (especially of the pelvis, abdomen, hip, knee), bone fracture or cast,
catheter in a big vein (central venous catheter), major trauma (< 1 month), acute
spinal cord injury (< 1 month), recent surgery (< 1 month) andlimb trauma and/or
orthopaedic procedures
3) Increased oestrogens
 Birth control pills, patches, rings, pregnancy, including up to 6 weeks after giving
birth, oestrogen and progestin hormone therapy
4) Medical conditions
 Cancer and chemotherapy, heart failure (< 1 month), inflammatory disorders (lupus,
rheumatoid arthritis, inflammatory bowel disease), renal disease - nephrotic
syndrome, coagulation abnormalities, stroke (< 1 month), serious lung disease
including pneumonia (< 1 month), abnormal pulmonary function (COPD), indwelling
central venous catheter, acute infection /severe sepsis (< 1 month), hypertension,
hyperlipidemia, autoimmune disease, including systemic lupus erythematosus and
myeloproliferative disorders
5) Other risk factors:

 Previous blood clot, family history of clots, clotting disorder (inherited or acquired),
obesity (BMI > 25 kg/m2), older age (over 40 - incidence increase with age), cigarette

smoking, varicose veins, previous DVT or family history of thrombosis and pregnancy
or postpartum period
PATHOGENESIS
 Virchow’s triad
INVESTIGATIONS
1) Blood tests:
a. D-dimer is a substance found in blood which is often increased in people with blood clots. A
blood test can be used to rule out presence of a DVT. If the D-dimer test is negative and you
are determined to have a low-risk for DVT (based upon the history and physical
examination), further testing with an imaging study to rule out a blood clot may not be
needed. However, if the suspicion that you have a blood clot is intermediate or high, an
imaging study needs to be done.
2) Imaging studies which diagnose DVT:
a. Doppler ultrasound (Duplex) is a painless and non-invasive test used to diagnose DVT.
b. Contrast venogram is often reserved for situations in which a Doppler ultrasound is not
feasible.
c. Magnetic resonance imaging (MRI) uses a strong magnet to create an image of inside the
body
d. Computer tomography (CT) venography or MRI venography are the preferred tests
to look at blood clots in the pelvis or the abdomen.
Hamilton Score
Characteristics Score
Plaster immobilization of lower limb 2
Active malignancy (within 6 months or current) 2
Strong clinical suspicion of DVT by emergency department physicians and 2
no other diagnostic possibilities
Bed rest (>3 days) or recent surgery (within 4 weeks) 1
Male sex 1
Calf circumference >3 cm on affected side (measured 10 cm below tibial 1
tuberosity)
Erythema 1
A score of 2 represents unlikely possibility for deep venous thrombosis (DVT)
A score of 3 represents likely probability for DVT.

Modified Wells Score
Clinical Characteristics Score
Active cancer (patient receiving treatment for cancer within previous 6 months or 1
currently receiving palliative treatment)
Paralysis, paresis, or recent plaster immobilization of lower extremities 1
Recently bedridden for 3 days or more, or major surgery within previous 12 weeks 1
requiring general or regional anaesthesia
Localized tenderness along distribution of deep venous system 1
Entire leg swollen 1
Calf swelling at least 3 cm larger than that on asymptomatic side (measured 10 cm 1
below tibial tuberosity)
Pitting oedema confined to symptomatic leg 1
Collateral superficial veins (non-varicose) 1
Previously documented DVT 1
Alternative diagnosis at least as likely as DVT -2
A score of 2 indicates that probability of deep venous thrombosis (DVT) is likely
A score of <2 indicates that probability of DVT is unlikely.
Wells Criteria /scoring for DVT

Present Score
Lower limb trauma or surgery or immobilisation in a plaster cast +1
Bedridden for more than three days or surgery within the last four week +1
Tenderness along line of femoral or popliteal veins (NOT just calf tenderness) +1
Entire limb swollen +1
Calf more than 3cm bigger circumference, 10cm below tibial tuberosity +1
Pitting oedema +1
Dilated collateral superficial veins (non-varicose) +1
Past Hx of confirmed DVT +1
Malignancy (including treatment up to six months previously) +1
Intravenous drug use +3
Alternative diagnosis as more likely than DVT -2
Pre-test Clinical probability of a DVT with score:
DVT "Likely" if Well's > 1
DVT "Unlikely" if Wells< 2
COMPLICATIONS
What are the complications of DVT?

4.6. PULMONARY EMBOLISM
 Acute respiratory consequences of pulmonary embolism include the following increased

alveolar dead space, hypoxemia and hyperventilation
 Additional consequences that may occur include regional loss of surfactant and
pulmonary infarction (see the image below)
 Arterial hypoxemia is a frequent, but not universal, finding in patients with acute
embolism
 Mechanisms of hypoxemia include ventilation-perfusion mismatch, intrapulmonary
shunts, reduced cardiac output, and intracardiac shunt via a patent foramen ovale.
 Pulmonary infarction is an uncommon consequence because of the bronchial arterial
collateral circulation.
Haemodynamic consequences
 Pulmonary embolism reduces the cross-sectional area of the pulmonary vascular bed,
resulting in an increment in pulmonary vascular resistance, which, in turn, increases the
right ventricular afterload
 If the afterload is increased severely, right ventricular failure may ensue
 In addition, the humoral and reflex mechanisms contribute to the pulmonary arterial
constriction. Following the initiation of anticoagulant therapy, the resolution of emboli
usually occurs rapidly during the first 2 weeks of therapy; however, it can persist on
chest imaging studies for months to years.
 Chronic pulmonary hypertension may occur with failure of the initial embolus to
undergo lyses or in the setting of recurrent thromboemboli.
Wells criteria / scoring for PE

Present Score
Clinical Signs and Symptoms of DVT? +3
PE is No. 1 Dx or Equally likley Dx +3
Heart Rate > 100 +1.5
Immobilization at least 3 days, or Surgery in the Previous 4 weeks +1.5
5 Previous, objectively diagnosed PE or DVT? +1.5
Haemoptysis? +1
Malignancy with treatment within 6 months, or palliative? +1
Pre-test clinical probability of a PE:
Wells Score > 4 - PE likely. Consider diagnostic imaging.
Wells Score 4 or less - PE unlikely. Consider D-dimer to rule out PE.
Disorders of Lymphatics and Blood Vessel Tumours
The lymphatic system is made up of lymphatic capillaries, lymphatic vessels and the lymph
nodes. The lymphatic capillaries resemble blood capillaries and the larger lymphatics are
identical to veins but are lined up by a single layer of endothelium with thinner muscle

walls. Lymphatic capillaries and lymphatics form plexuses around tissues and organs.
Lymphatic capillary walls are permeable to tissue fluid, proteins and particular matter.
Lymphangitis
Lymphangitis is inflammation of the lymphatic that can be acute or chronic.
ACUTE LYMPHANGITIS
Acute lymphangitis results from many bacterial infections most commonly beta haemolytic
streptococci and staphylococci and is often associated with lymphadenitis.
CHRONIC LYMPHANGITIS
 Chronic lymphangitis is due to persistent and recurrent acute lymphangitis or from

chronic infections like tuberculosis, syphilis and actinomycosis and usually results in
permanent obstruction due to fibrosis.
Lymphoedema
 Lymphoedema is swelling of soft tissues due to localized increase in the quantity of

lymph. It can be primary (idiopathic) or secondary (obstructive).
Primary (Idiopathic) lymphoedema
Primary lymphoedema occurs without any underlying secondary cause e.g. congenital
lymphoedema.
Secondary lymphoedema
Is the more common form of lymphoedema resulting from obstruction of the lymphatic
channels due to: -
a) Lymphatic invasion by malignant tumour
b) Surgical removal of lymphatics
c) Post-irradiation fibrosis
d) Parasitic infestations e.g. filariasis
e) Lymphangitis causing scarring and obstruction
Obstructive lymphoedema occurs when the obstruction is wide spread since collaterals
develop.
Tumours of Blood Vessels
1. Name the tumours that affect blood vessels

2. How will these tumours present?
3. How will you investigate for them?
4. What is Kaposi’s sarcoma (KS)?
5. How many forms of KS are there?
6. What are the complications of KS?

CMS 251 Unit 1 Cardiovascular System

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CMS 251 Unit 1 Cardiovascular System

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UNIT 1: CARDIOVASCULAR PATHOLOGY

MODULE: CLINICAL PATHOLOGY

Lesson 1: Introduction to Pathology of the Cardiovascular System

At the end of the lesson the learner shall be able to: -

2.0. THE HEART

 Pumps sufficient oxygenated blood containing nutrients, metabolites and hormones to

Carey F. Okinda Page 1

Diagram 1.1: Normal Heart

Functioning of the Heart

The Cardiac Cycle

Carey F. Okinda Page 2

3.0. CARDIAC OUTPUT

4.0. THE LAWS

1. Poiseulle’s Law Blood flow = Pressure x diameter of blood vessel

Carey F. Okinda Page 3

6.0. INVESTIGATIONS IN CARDIOVASCULAR DISEASE

Carey F. Okinda Page 4

i) The cardio-thoracic Ratio (CTR)

ii) Patterns of specific chamber enlargement seen on the chest X-ray

Carey F. Okinda Page 5

b) MRI (Magnetic Resonance Imaging)

Carey F. Okinda Page 6

 P wave - 1st deflection corresponds to depolarization and the atria

Carey F. Okinda Page 7

(a) AV Node/Bundle of HIS

Carey F. Okinda Page 8

7) TOTAL BLOOD COUNT

8) Urea And Electrolytes

Carey F. Okinda Page 9

At the end of the lesson the learner shall be able to: -

2.0. DEVELOPMENT OF THE HEART

Carey F. Okinda Page 10

 Timing of prenatal teratogenic determines occurrence and type of anomaly produced

5.0. CLINICAL EFFECTS/FEATURES

 Children with significant congenital anomalies have disturbance in the haemodynamics

1. Malposition of the heart

Carey F. Okinda Page 11

Diagram 2.2: Dextrocardia

8.0 SHUNTS (CYANOTIC CONGENITAL HEART DISEASES)

 A shunt is an abnormal communication between heart chambers, between blood

1. Left-to-right shunts (late cyanosis or acyanotic heart diseases)

Carey F. Okinda Page 12

8.3 LEFT-TO-RIGHT SHUNTS (Acyanotic Heart Disease)

 Cause cyanosis several months or years after birth.

1. Atrial Septal Defect (ASD)

Diagram 2.3: ASD

2. Ventricular Septal Defect (VSD)

Carey F. Okinda Page 13

Diagram 2.4: VSD

Carey F. Okinda Page 14

Diagram 2.6: PDA

Carey F. Okinda Page 15

Diagram 2.7: Effects of PDA

8.0. RIGHT-TO-LEFT SHUNTS (Cyanotic Congenital Heart Disease)

1. Tetralogy of Fallot (TOF)

 TOF accounts for 10% of children born with heart abnormalities

Carey F. Okinda Page 16

1 - Pulmonary stenosis (a form of right ventricular outflow tract obstruction)