Anatomy and Physiology of the valves of the heart

The four valves are known as:

The tricuspid valve
The pulmonic or pulmonary valve
The mitral valve
The aortic valve

1) TRICUSPID VALVE
This valve is located between the right atrium and the right ventricle.
Closes off the upper right chamber (or atrium) that holds blood coming in from
the body.
Opens to allow blood to flow from the top right chamber to the lower right
chamber (or from right atrium to right ventricle).
Prevents the back flow of blood from the ventricle to the atrium when blood is
pumped out of the ventricle.
2) PULMONIC OR PULMONARY VALVE
The pulmonary valve is located between the right ventricle and the pulmonary
artery.
Closes off the lower right chamber (or right ventricle).
Opens to allow blood to be pumped from the heart to the lungs (through the
pulmonary artery) where it will receive oxygen.
3) MITRAL VALVE

 This valve is located between the left atrium and the left ventricle.
Closes off the upper left chamber (or left atrium) collecting the oxygen-rich blood
coming in from the lungs.
Opens to allow blood to pass from the upper left side to the lower left side (or
from the left atrium to the left ventricle).
4) AORTIC VALVE
The aortic valve is located between the left ventricle and the aorta.
Closes off the lower left chamber that holds the oxygen-rich blood before it is
pumped out to the body.
Opens to allow blood to leave the heart (from the left ventricle to the aorta and on
to the body).

Diseases of the heart valves

1) Regurgitation (or leakage of the valve)
This means the valve doesn't close completely, causing the blood to flow backward
through the valve. This results in leakage of blood back into the atria from the
ventricles (in the case of the mitral and tricuspid valves) or leakage of blood back into
the ventricles (in the case of the aortic and pulmonary valves).
2) Stenosis (or narrowing of the valve).
With stenosis, the valve opening is narrowed and the valve doesn't open properly,
inhibiting the ability of the heart to pump blood across the narrowed valve due to the
increased force required to pump blood through the stiff (stenotic) valve(s).
3) Atresia.
This means the valve opening doesn't develop at all, preventing blood from passing
from an atria to a ventricle, or from a ventricle to the pulmonary artery or aorta.
Blood must find an alternate route, usually through another existing congenital
(present at birth) defect, such as an atrial septal defect or a ventricular septal defect.

Aortic Stenosis
Definition
Aortic stenosis is caused by narrowing of the orifice of the aortic valve and leads
to obstruction of left ventricular outflow.

Etiology
Up till today, the exact aetiology for Aortic Stenosis is still not well known. However,
aortic stenosis can be either congenital or acquired.
Aortic stenosis is most commonly caused by age-related progressive calcification of a
normal (three-leafed) aortic valve (>50% of cases) with a mean age of 65 to 70 years
old. Other causes of aortic stenosis include calcification of a congenital bicuspid
aortic valve (30-40% of cases) and acute rheumatic fever post-inflammatory (less
than 10% of cases).

Age < 70 years

Age >70 years

Bicuspid AV (50%)

Degenerative (48%)

Postinflammatory (25%)

Bicuspid (27%)

Degenerative (18%)

Postinflammatory (23%)

Unicommissural (3%)

Hypoplastic (2%)

Hypoplastic (2%)

Normal aortic valves have three leaves (tricuspid), but some individuals are born with
an aortic valve that has two leaves (bicuspid). Typically, aortic stenosis due to
calcification of a bicuspid valve appears earlier, in the 40s or 50s, whereas aortic
stenosis due to calcification of a normal tricuspid aortic valve appears later, usually in
the 70s and 80s.

Congenital valvular aortic stenosis

Congenitally unicuspid, bicuspid, tricuspid, or even quadricuspid valves may be the
cause of aortic stenosis. In neonates and infants younger than 1 year, a unicuspid
valve can produce severe obstruction and is the most common anomaly in infants
with fatal valvular aortic stenosis. In patients younger than 15 years, unicuspid valves
are most frequent in cases of symptomatic aortic stenosis.
In adults who develop symptoms from congenital aortic stenosis, the problem is
usually a bicuspid valve. Bicuspid valves do not cause significant narrowing of the
aortic orifice during childhood. The altered architecture of the bicuspid aortic valve
induces turbulent flow with continuous trauma to the leaflets, ultimately resulting in
fibrosis, increased rigidity and calcification of the leaflets, and narrowing of the
aortic orifice in adulthood.
Congenitally malformed tricuspid aortic valves with unequally sized cusps and
commissural fusion (functionally bicuspid valves) can also cause turbulent flow
leading to fibrosis and, ultimately, to calcification and stenosis. Clinical
manifestations of congenital aortic stenosis in adults usually appear after the fourth
decade of life.

Acquired valvular aortic stenosis

The main causes of acquired aortic stenosis include degenerative calcification and,
less commonly, rheumatic heart disease.
Degenerative calcific aortic stenosis (also called senile calcific aortic stenosis)
involves progressive calcification of the leaflet bodies, resulting in limitation of the
normal cusp opening during systole. This represents a consequence of long-standing
hemodynamic stress on the valve and is currently the most frequent cause of aortic

stenosis requiring aortic valve replacement. The calcification may also involve the
mitral annulus or extend into the conduction system, resulting in atrioventricular or
intraventricular conduction defects.
Risk factors for degenerative calcific aortic stenosis include hypertension,
hypercholesterolemia, diabetes mellitus, and smoking. The available data suggest that
the development and progression of the disease are due to an active disease process at
the cellular and molecular level that shows many similarities with atherosclerosis,
ranging from endothelial dysfunction to, ultimately, calcification.
In rheumatic aortic stenosis, the underlying process includes progressive fibrosis of
the valve leaflets with varying degrees of commissural fusion, often with retraction of
the leaflet edges and, in certain cases, calcification. As a consequence, the rheumatic
valve often is regurgitant and stenotic. Coexistent mitral valve disease is common.
Other, infrequent causes of aortic stenosis include obstructive vegetations,
homozygous type II hypercholesterolemia, Paget disease, Fabry disease, ochronosis,
and irradiation.
It is worthwhile to note that although differentiation between tricuspid and bicuspid
aortic stenosis is frequently made, it is often difficult to determine the number of
aortic valve leaflets. A study comparing operatively excised aortic valve structure
evaluation by cardiac surgeon versus pathologist found that valve structure

determination was frequently incongruous.

Hemodynamic parameter of blood flow in Aortic Stenosis

Pathophysiology
When the aortic valve becomes stenotic, resistance to systolic ejection occurs and a
systolic pressure gradient develops between the left ventricle and the aorta. In normal
condition, the pressure in left ventricle is equal to the pressure in aorta. This outflow
obstruction leads to an increase in left ventricular (LV) systolic pressure. As a
compensatory mechanism to normalize LV wall stress, LV wall thickness increases

by parallel replication of sarcomeres, producing concentric hypertrophy. At this stage,

the chamber is not dilated and ventricular function is preserved, although diastolic
compliance is reduced.
Eventually, however, LV end-diastolic pressure (LVEDP) rises, which causes a
corresponding increase in pulmonary capillary arterial pressures and a decrease in
cardiac output due to diastolic dysfunction. The contractility of the myocardium may
also diminish, which leads to a decrease in cardiac output due to systolic dysfunction.
Ultimately, heart failure develops thus causing remodelling of the heart.
In most patients with aortic stenosis, LV systolic function is preserved and cardiac
output is maintained for many years despite an elevated LV systolic pressure.
Although cardiac output is normal at rest, it often fails to increase appropriately
during exercise, which may result in exercise-induced symptoms.
Diastolic dysfunction may occur as a consequence of impaired LV relaxation and/or
decreased LV compliance, as a result of increased afterload, LV hypertrophy, or
myocardial ischemia. LV hypertrophy often regresses following relief of valvular
(also called valvular) obstruction. However, some individuals develop extensive
myocardial fibrosis, which may not resolve despite regression of hypertrophy.
In patients with severe aortic stenosis, atrial contraction plays a particularly important
role in diastolic filling of the left ventricle. Thus, development of atrial fibrillation in
aortic stenosis often leads to heart failure due to an inability to maintain cardiac
output.

Grading of Aortic Stenosis

Grade

Mild

Aortic jet velocity, Mean aortic valve Aortic valve area,

m/s
pressure gradient, cm2
mm Hg
< 3.0

< 25

1.5

Moderate

3-4

25-40

1.0-1.5

Severe

>4

> 40

< 1.0

Clinical signs and symptoms

Symptoms related to aortic stenosis depend on the degree of valve stenosis. Most
people with mild to moderate aortic stenosis are asymptomatic. Symptoms usually
present in individuals with severe aortic stenosis, though they may occur in those
with mild to moderate aortic stenosis as well.
Shortness of breath
Diastolic dysfunction and decreased cardiac output with exercise. If early indications
of aortic stenosis are not recognized, can delay diagnosis and treatment.
Chest pain
Angina pectoris in patients with aortic stenosis is typically precipitated by exertion
and relieved by rest due to increased left ventricular workload and oxygen
consumption. Thus, it may resemble angina from coronary artery disease.
Syncope
Syncope from aortic stenosis often occurs upon exertion when systemic
vasodilatation in the presence of a fixed forward stroke volume causes the arterial
systolic blood pressure to decline. It also may be caused by atrial or ventricular
tachyarrhythmias.
Syncope at rest may be due to transient ventricular tachycardia, atrial fibrillation, or
(if calcification of the valve extends into the conduction system) atrioventricular
block. Another cause of syncope is abnormal vasodepressor reflexes due to increased
LV intracavitary pressure (vasodepressor syncope).
Heart failure
Diastolic dysfunction resulting in pulmonary congestion and dyspnea. Presence of
swellings at legs or feet (edema) may indicate heart failure.

Physical Examination
Auscultation
The typical murmur of aortic stenosis is a high-pitched, "diamond shaped" crescendodecrescendo, midsystolic ejection murmur heard best at the right upper sternal border
radiating to the neck and carotid arteries (see figure below). In mild aortic stenosis,
the murmur peaks in early systole, however as the disease progresses the peak moves
to later in systole since longer time is required to complete LV systole and aortic
valve closure is delayed. The intensity of the murmur typically increases as disease
progresses, however when heart failure develops and cardiac output declines, the

murmur becomes softer. Thus the intensity

of the murmur is not a good indicator of
disease severity.
Auscultation at the cardiac apex may reveal a murmur that may sound midsystolic or
holosystolic and may mimic the murmur of mitral regurgitation. However this is
commonly the result of radiation of the murmur of aortic stenosis to the apex rather
than coexistent mitral regurgitation. This finding is referred to as "Gallavardin
dissociation". To determine if the apical murmur is indeed due to mitral regurgitation
or radiation of the murmur of aortic stenosis, dynamic auscultation can be undertaken
(see section on dynamic auscultation). The murmur of hypertrophic cardiomyopathy
can also at times mimic the murmur of aortic stenosis. The Valsalva maneuver
decreases the murmur of aortic stenosis while it increases the murmur of hypertrophic
cardiomyopathy.
The S2 heart sound is often paradoxically split in patients with aortic stenosis due to
the significantly delayed closure of the aortic valve resulting from the increased time
needed to complete LV systole.

As disease progresses and the aortic valve leaflets lose their mobility, the intensity of
S2 decreases. When the S2 sound is no longer audible, it can be concluded that the
aortic stenosis is relatively severe. A S4 heart sound is also often present due to the
severe concentric left ventricular hypertrophy that develops in aortic stenosis. If a S3
heart sound is present, then significant systolic dysfunction has developed which is
common in end stage aortic stenosis.
Perhaps the best bedside method to estimate the severity of aortic stenosis is derived
from evaluation of the carotid arteries. The phenomenon known as "pulsus parvus et
tardus" refers to a weak (parvus) and delayed (tardus) carotid upstroke. To asses for
"parvus", it is often helpful to palpate ones own carotid artery while concurrently
palpating the patient's carotid artery. It is important to note that in some elderly
individuals the carotids may be stiff due to calcification, which may falsely normalize
the carotid upstroke. To assess for "tardus", auscultate the patient's S2 heart sound
while palpating their carotid upstroke. The S2 and carotid upstroke should occur
almost simultaneously. If the carotid upstroke comes significantly after the S2 heart
sound, "tardus" is present indicating severe aortic stenosis. Other physical exam
findings in patients with aortic stenosis include those of both right and left heart
failure.

Clinical Investigations
The gold standard for diagnosing aortic stenosis is noninvasive 2dimensional Doppler echocardiography.
Significance:
Estimation of severity of aortic stenosis, left ventricular size, and ejection fraction.
Estimation of pulmonary pressures, aortic valve gradient, aortic valve area.
Assessment of thickening of aortic valve leaflet, reduced leaflet motion, reduced
valve opening.

Cardiac catheterization
Assessment of coronary arteries to determine need for simultaneous coronary artery
bypass surgery and aortic valve replacement.
Direct measurement of left ventricular and ascending aortic pressures to determine
aortic valve pressure gradient.
Determination of left ventricular systolic pump function quantified by measuring left
ventricular end-diastolic and end-systolic volumes, and ejection fraction.

12-Lead electrocardiography
Evidence of left ventricular hypertrophy: Increased R-wave amplitude of the QRS
complex in lead V6, increased S-wave amplitude in lead V1.

ST-segment depression and T-wave inversion in leads facing the left ventricle: I, aVL,
V5, and V6.

Chest radiography
Determination of heart size
Detection of calcification in the aortic valve (lateral view).
With heart failure, enlarged heart size from dilatation of left atrium and left ventricle,
venous congestion and pulmonary edema.

Stress test
Determination of the degree of exercise tolerance
Distinguish between asymptomatic and symptomatic aortic stenosis.

PanelAshowsalongaxis,twodimensionalechocardiographicviewofanormalaorticvalve,inwhichthe
thinvalveleafletsareseenintheopenposition,paralleltothewallsoftheaorta,inmidsystole.Theleft
ventricleisnormalinsizeandwallthickness,themitralvalveisclosed,andtheleftatriumisnotenlarged.
PanelBshowsthecorrespondingviewofastenoticaorticvalve,inwhichthecalcified,thickened,and
relativelyimmobileleafletsareseeninsystoleasabrightwhitebandthatobstructsleftventricularoutflow.
Themitralvalveisclosedinsystole.Theleftventricleshowsincreasedwallthickness,andtheleftatriumis
enlarged.PanelCshowscolorDopplerimagingofanormalaorticvalvewithunobstructedflowacrossthe
aorticvalveshowninblueduringsystole.PanelDshowsthecorrespondingimageofastenoticaorticvalve
withnormalflowproximaltotheaorticvalve(inred)withamixtureofcolorsintheaorta,reflectingthe
increaseinvelocityandpressuredropacrossthevalve.PanelEshowsacontinuouswaveDopplerrecording
ofnormalantegradeflowacrosstheaorticvalve,obtainedwiththetransducerattheleftventricularapex.
PanelFshowsacorrespondingrecordingofhighvelocityflowacrossastenoticaorticvalve.Thevertical
axisshowsascaleupto6mpersecondforflowdirectedawayfromthetransducer.Theaorticstenosis
velocityprofileshowsahighvelocitypattern(typically4mpersecondorhigher)withapeakinmidsystole
andamoreroundedshapethannormalflow.Thispatienthaslittleaorticregurgitation,whichwouldbeseen
indiastoleifpresent.

Treatment
Currentlynoknownmedicaltherapyisavailabletopreventordelaytheprogression
ofaorticstenosis.Treatmentfocusesonreducingcardiovascularriskfactors,

includinghypertension,diabetesmellitus,smokingtobacco,highcholesterollevels,
overweight,andlackofexercise.
Treatmentisgenerallynotnecessaryinpeoplewithoutsymptoms.Inmoderatecases,
echocardiographyisperformedevery12yearstomonitortheprogression,possibly
complementedwithacardiacstresstest.Inseverecases,echocardiographyis
performedevery36months.Inbothmoderateandmildcases,thepatientshould
immediatelymakearevisitorbeadmittedforinpatientcareifanynewrelated
symptomsappear.Physicalactivityisnotrestrictedinmildaorticstenosis,but
competitivesportsshouldbeavoidedbypatientswithmoderatetosevereaortic
stenosis.Therearenocurrenttherapeuticoptionscurrentlyavailabletotreatpatients
withaorticvalvestenosis;however,studieshaveindicatedthatthediseaseoccursas
aresultofactivecellularprocesses,suggestingthattargetingtheseprocessesmay
leadtoviabletherapeuticapproaches.
Medical Management
The effect of statins on the progression of AS is still unclear. The latest trials do not
show any benefit in slowing AS progression, but did demonstrate a decrease in
ischemic cardiovascular events. Angiotensin-converting enzyme (ACE) and
angiotensin II receptors have been found in stenotic aortic valves. This leads to the
hypothesis that the renin-angiotensin system may play a role in the progression of the
disease. In general, medical therapy has relatively poor efficacy in treating aortic
stenosis. However, it may be useful to manage commonly coexisting conditions that
correlate with aortic stenosis:
Any angina is generally treated with beta-blockers and/or calcium blockers. Nitrates
are contraindicated due to their potential to cause profound hypotension in aortic
stenosis.
Any hypertension is treated aggressively, but caution must be taken in administering
beta-blockers.
Any heart failure is generally treated with digoxin and diuretics, and, if not
contraindicated, cautious inpatient administration of ACE inhibitors.
Since calcific aortic stenosis shares many pathological features and risk factors with
atherosclerosis, and since atherosclerosis may be prevented and/or reversed by
cholesterol lowering, there has been interest in attempting to modify the course of
calcific aortic stenosis by lowering cholesterol levels with statin drugs.

Surgical Treatment
Procedure

Indication

Description

Aortic valve
replacement

Symptomatic severe aortic stenosis

Severe aortic stenosis with ejection
fraction <50%
Severe aortic stenosis and a need for
any other heart surgery

Aorticvalveisremovedanda
newvalve(mechanicalor
biological)issewntothe
annulusofthenativevalve

Balloon aortic
valvuloplasty

Bridge to aortic valve replacement in

patients in unstable condition
Palliative to reduce symptoms when
surgery is high risk

Aballoonisplacedacrossthe
stenoticvalveandinflatedand
deflatedseveraltimesper
secondtowidenthevalve
annulusandreducedegreeof
stenosis

Transcatheter aortic
valve implantation

Seriously ill patients who are not

candidates for conventional aortic
valve replacement surgery

Replacementvalveinsidea
stentthatisdeployedoverthe
diseasedaorticvalveannulus
viaatransapicalor
transcatheterapproach

The only effective treatment for aortic stenosis is removal of the mechanical
obstruction. To this end, only aortic valve replacement (AVR) has been shown to
achieve this while reducing mortality.
Surgical Outcome
The risk of death or serious complications from aortic valve replacement is typically
quoted as being between 1-3%, depending on the health and age of the patient, as
well as the skill of the surgeon. Older patients, as well as those who are frail and/or
have multiple comorbidities (i.e. other health problems), may face significantly
higher surgical risk.
Complications related to the catheter include:
Pain, swelling, and tenderness at the catheter insertion site. Irritation of the vein by
the catheter (superficial thrombophlebitis). This can usually be treated with warm
compresses. Bleeding at the catheter site. A bruise where the catheter was inserted.
This usually goes away in a few days.Trouble urinating after the procedure.
Prognosis
If untreated, severe symptomatic aortic stenosis carries a poor prognosis with a 2-year
mortality rate of 50-60% and a 3-year survival rate of less than 30%.

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2014