Heart pathology

Muez G. ( M.D.)
 Congestive Heart Failure (CHF)

A condition when cardiac output (CO) is insufficient for metabolic requirements of

the body.
Because many patients present without signs or symptoms of volume overload, the
term “heart failure” is preferred over the older term “congestive heart failure.
 epidemiology
• Globally, >26 million people are affected by HF
• The prevalence of HF increases significantly with age
• The lifetime risk of HF at age 55 years is 33% for men and 28% for women
• Despite the higher incidence in men, women constitute more than half of the
population with HF (higher prevalence) due to their higher life expectancy
• Studies have shown that age-adjusted rates of HF hospitalization are highest for
black men, followed by black women, white men, and white women
• As developing nations undergo socioeconomic development, the epidemiology
of HF is becoming similar to that of Western Europe and North America, with
coronary artery disease emerging as the most common cause of HF.
o Heart failure is the end result of many different forms of heart
disease
Occurs usually as a consequence of progressive deterioration of
myocardial contractile function (systolic dysfunction) as often occurs in
ischemic injury & pressure or volume overload.
Can also result from an inability of the heart chamber to relax, expand
& fill sufficiently during diastole to accommodate an adequate
ventricular blood volume (diastolic dysfunction)
E.g. massive left ventricular hypertrophy, myocardial fibrosis or
constrictive pericarditis
Heart failure may result because of greatly increased demands for
blood by the tissues, a process sometimes referred to as high output
failure.
 Pathophysiology & Progression to Failure
As the heart begins to fail, a number of local adaptive responses are triggered in an
attempt to maintain normal cardiac output
These include neurohumoral reactions as well as molecular and morphologic
changes within the heart.
1. Activation of neurohumoral systems
Release of the norepinephrine by adrenergic cardiac nerves → increase heart
rate &
augments myocardial contractility & vascular resistance
Activation of the renin-angiotensin-aldosterone system
Release of atrial natriuretic peptide (ANP)
2. The Frank-Starling mechanism
• The increased preload of dilation helps to sustain cardiac performance by
enhancing contractility.
3. Myocardial structural changes

Augmented muscle mass (hypertrophy) with or without cardiac chamber dilation, in

which the mass of contractile tissue is augmented.

Cardiac Hypertrophy
• The major initial structural adaptation to a chronically increased mechanical work is
hypertrophy of individual muscle fibers
• In the case of a cardiac chamber subjected to a pure pressure load (e.g., hypertension,
valvular stenosis), the hypertrophy is characterized by an increase in the diameter of
individual muscle fibers.
• Cross-sectional area of myocytes is expanded but not cell length
• This pattern of fiber enlargement results in the development of classic concentric
hypertrophy (thickness of the ventricular wall increases without an increase in the size
of the chamber) .
When the heart is subjected to an abnormal volume load (e.g., valvular
regurgitation or abnormal shunts), the length of individual muscle
fibers also increases
• This pattern is called Eccentric hypertrophy characterized by dilation
with increased ventricular diameter .
 The development of hypertrophy initially serves as a positive,
adaptive response However, sustained cardiac hypertrophy often
evolves to cardiac failure Despite the potential hemodynamic benefits
associated with hypertrophy, the development of hypertrophy comes at
a significant cost to the cell Oxygen requirements of the hypertrophic
myocardium are increased, owing to increased myocardial cell mass
and increased tension of the ventricular wall accompanied by
decreased capillary density, Molecular signals that lead to the
development of hypertrophy may also be accompanied by the
expression of certain proteins that lead to:

− Impaired myocyte contractility

− Increased fibrous tissue and even
− Premature myocyte death
 Left-Sided Heart Failure
Left-sided heart failure is most often caused by:
 Systemic hypertension
 Mitral or aortic valve disease
 Ischemic heart disease
 Primary diseases of the myocardium
• The morphologic & clinical effects of left-sided CHF primarily result
from:
− Progressive damming of blood within the pulmonary circulation
− Diminished peripheral blood perfusion & flow
Morphology
• Heart
Depending on the cause of the disease process, abnormalities such as myocardial
infarction or valvular deformity may be present.
The failing left ventricle is hypertrophied and often dilated as well
The microscopic changes are nonspecific: primarily myocyte hypertrophy and
variable degrees of interstitial fibrosis
• Secondary enlargement of the left atrium with resultant atrial fibrillation may
compromise stroke volume or cause blood stasis & possible thrombus formation
Lungs
• The lungs are boggy and congested and the cut surface shows a frothy mixture of
surfactant-rich fluid and blood
• Microscopically - alveolar capillaries are congested (transudation of fluid), initially
limited to perivascular interstitial spaces, causing widening of the alveolar septa. In
time, it overflows into the alveoli (pulmonary edema).
 Kidneys
Decreased CO → acivation the renin-angiotensin- aldosterone system →
retention of
water & salt with consequent expansion of interstitial fluid & blood volume ,If the
perfusion deficit of the kidney is severe, impaired excretion of nitrogenous
products may cause azotemia (prerenal).
• Brain
Cerebral hypoxia may give rise hypoxic encephalopathy
 Clinical features of Left-sided heart failure
The most common & earliest manifestation of left ventricular failure is
Dyspnea, or a sense of breathlessness and cough
− The dyspnea is initially exertional
• Orthopnea
• Paroxysmal nocturnal dyspnea
Right- Sided Heart Failure
• The most common cause of right-sided heart failure is left ventricular
failure
• May also occur in the absence of left-sided heart failure in patients
with intrinsic diseases of the lung parenchyma and/or pulmonary
vasculature (corpulmonale) and in patients with pulmonic or tricuspid
valve disease
• It sometimes follows congenital heart diseases, in which there is a
left-to-right shunt
 Morphology of right side failure
Heart
• Isolated right heart failure is most often caused by lung disease, so
most cases exhibit only hypertrophy and dilation of the right atrium
and ventricle

Liver & portal system

• Liver is increased in size & weight (congestive hepatomegaly)
• With long-standing cardiac failure, the liver may become fibrotic and,
in extreme cases, frankly cirrhotic (cardiac cirrhosis)
 Spleen
• Right sided heart failure leads to elevated pressure in the portal vein & its
tributaries
• Congestion results in a tense, enlarged spleen (congestive splenomegaly)
Subcutaneous Tissues
• Peripheral edema of dependent portions of the body (pedal & pretibial edema) is
the hallmark of right-sided heart failure
• Generalized massive edema is called anasarca
 Clinical features
• It is the major cause of systemic venous congestion and soft tissue
edema
• •Systemic venous congestion → distended neck veins and an enlarged
liver & spleen
• It is also associated with an increased frequency of DVT and
pulmonary embolism
• Pleural effusions, pericardial effusions and ascites
 In many cases of chronic CHF, patients present with the clinical
syndromes of both right-sided & left-sided heart failure.
CONGENITAL HEART DISEASE

• Congenital heart disease (CHD) is a general term designating developmental

abnormalities of heart & great arteries present from birth
• May have serious consequences
• Incidence 6-8/1000 live births
• Cause unknown in >90%
• 2-10X increased incidence in siblings or child of affected parent
Etiology & Pathogenesis
1. Genetic Factors
- Trisomy 21 (associated with Down syndrome) is the most common
known genetic cause of congenital heart disease

2. Environmental factors: - Congenital rubella infection

- Teratogens

3. Multifactorial: genetic, environmental & maternal factors

• Classification of Congenital Heart Disease
Congenital heart diseases can be subdivided into three major groups:
 Malformations causing a left-to-right shunt (Acyanotic congenital
heart diseases)
 Malformations causing a right-to-left shunt (cyanotic congenital heart
diseases)
 Malformations causing obstruction
frequency
• Ventricular septal defect (VSD) 42%
• Atrial septal defect (ASD) 10%
• Pulmonary stenosis 8%
• Patent ductus arteriosus (PDA) 7%
• Tetralogy of Fallot 5%
• Coarctation of aorta 5%
• Aortic stenosis 4%
• Transposition of the great arteries 4%
Left-to-Right Shunts
• Represent the most common type of congenital cardiac
malformation
• These include atrial septal defects (ASDs), ventricular septal defects
(VSDs), and patent (persistent) ductus arteriosus (PDA)
• These malformations may be asymptomatic at birth, or they may
cause fulminate congestive heart failure
• Left-to-right shunts increase pulmonary blood flow. When pulmonary
circulation is exposed to increased pressure and volume it can result
in: → right ventricular hypertrophy and, potentially, failure
• Pulmonary arteries respond to increased pressure and volume by
medial hypertrophy and vasoconstriction
• Eventually pulmonary vascular resistance increases toward systemic
levels, reversing the shunt to right-to-left with unoxygenated blood in
the systemic circulation resulting in late cyanotic congenital heart
disease or Eisenmenger syndrome
 Atrial Septal Defect (ASD)
• Abnormal communication in atrial septum (AS) allows blood flow between left
atrium right atrium

• Classified by size & location in AS

– Secundum ASD - (90%)
– Primum ASD
– Sinus venosus ASD
• Clinical features
Functional consequence
- Left to right shunt: acyanotic
- Increased pulmonary flow
• May be diagnosed in adults > 30 yrs old (low pressure, low flow)
• Murmur
• Less than 10% develop pulmonary hypertension
 Ventricular Septal Defect (VSD)

Defect in ventricular septum allows flow between left and right left ventricle
VSD is the most common congenital heart defects at birth
Morphology
• Classified by size and location
− Perimembranous (or membranous) – the most common (90%). occur in the
region of the membranous interventricular septum
− Infundibular (subpulmonary or supracristal VSD) – involves the RV outflow
tract
− Muscular VSD – can be single or multiple
Clinical features
• Most manifest in the pediatric age group
• Most are associated with other congenital cardiac anomalies such as
Tetralogy of Fallot
• The functional consequences of a VSD depend on:
 size of the defect
 whether there are associated right-sided malformations
 Patent Ductus Arteriosus (PDA)

• PDA results when the ductus arteriosus remains open after birth
• 90% cases of PDA are isolated
• The remainder are most often associated with VSD, coarctation of the aorta, or
pulmonary or aortic valve stenosis
 Clinical Features
• PDA causes a high-pressure left-to-right shunt, audible as a harsh
waxing and waning
murmur sometimes referred to as a "machinery" murmur.
• A small PDA causes no symptoms
• it may lead to Pulmonary hypertension causing cyanosis and
congestive heart failure
• The high-pressure shunt also predisposes affected individuals to the
development of infective endocarditis.
 Right-to-left shunts
• Cyanosis & dusky blueness of the skin and mucus membrane develops → because
poorly oxygenated blood enters the systemic circulation (cyanotic congenital heart
disease)
• Examples include: tetralogy of Fallot, transposition of the great vessels, persistent
truncus arteriosus, tricuspid atresia and total anomalous pulmonary venous
connection
• Bland and septic emboli arising in veins can directly enter the systemic circulation
(paradoxical embolism) and result in brain infarction and abscess
• Clinical features arise associated with severe, long standing cyanotic heart
disease:
→ cyanosis, clubbing of the tips of the fingers & toes and polycythemia
Tetralogy of Fallot (TOF)
• The most common form of cyanotic heart disease
• 4 components:
– VSD, Pulmonary stenosis, Overriding aorta, RV hypertrophy
• Functional consequences:
– Right to left shunt
– Decreased pulmonary flow
Clinical Features
• Consequences depend primarily on the severity of the subpulmonary stenosis
− Mild stenosis, resembles an isolated VSD, and the shunt may be left-to-right,
without cyanosis (so-called pink tetralogy)
− As the obstruction increases in severity, right-to-left shunting develops, producing
cyanosis classic (TOF)
− Thus, most patients with tetralogy of Fallot are cyanotic from birth or soon
thereafter
• These patients develop complications of chronic cyanosis, such as erythrocytosis
with hyperviscosity and digital clubbing
• Increased risk for infective endocarditis, systemic emboli, and brain abscesses
Congenital Obstructive Lesions

• Congenital obstruction to blood flow can occur at the level of the heart valves or within
a great vessel
• Common examples include aortic or pulmonary valve stenosis or atresia, and
coarctation of the aorta
• Obstruction can also occur within a chamber, as with subpulmonary stenosis in TOF
Coarctation of Aorta
• Localized obstruction due to curtain-like fold of aortic media that
narrows the lumen
• Most cases of coarctation of the aorta can be placed into one of two
major categories:
− Infantile form and Adult form (more common)
Clinical features
• Clinical manifestations depend on the severity of the narrowing and the patency of the
ductus arteriosus
 Infantile form – coarctation of the aorta with a PDA usually leads to
manifestations early in life; it may cause signs and symptoms immediately after
birth
- Many infants with this anomaly do not survive the neonatal period without intervention
 Adult form – in coarctation of the aorta without PDA, there will be
hypertension of the upper extremities, by contrast, blood pressure is
low and pulses are weak in the lower extremities.
• Erosions (“notching”) of the under surfaces of the ribs
− Particularly characteristic in adults is the development of collateral
circulation between the pre coarctation arterial branches and the post
coarctation arteries through enlarged intercostal and internal
mammary arteries and the radiographically visible erosions
("notching") of the undersurfaces of the ribs.
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