VALVULAR DISEASES OF HEART
 Dr. Bindu.K  BHMS, MD(Hom)

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CHRONIC RHEUMATIC HEART DISEASE
Chronic valvular heart disease develops subsequently in at least half of those affected by rheumatic fever with carditis. The predominantly affected valve is the mitral (in more than 90%) and then less commonly the aortic, tricuspid and pulmonary valves. The lesions develop after 10 -20 years in western countries but much earlier in developing countries.

Pathology
The main pathological process in chronic rheumatic heart disease is a progressive fibrosis particularly affecting heart valve. This is in contrast to the destructive lytic process in acute rheumatic fever. The condition also affects the pericardium and myocardium and may contribute heart failure and conduction disorders. For the mitral valve the result is shortening of chordae tendinae , fusion of the commisures and a reduction in size of the valve orifice. Similar disorders of the aortic and tricuspid valves produce distortion and rigidity of the cusps, and in consequence , stenosis and incompetence. Once damage has developed on a valve altered haemodyanmic stresses on the valve perpetuate and extends the damage even in the absence of a continuing rheumatic process .

MITRAL VALVE STENOSIS
Aetiology and pathophysiology
Isolated mitral stenosis accounts for about 25% of all cases of rheumatic heart disease , and an additional 40 % have mixed mitral stenosis and regurgitation. Two third of the cases occur in women. Acquired mitral stenosis is almost entirely rheumatic in origin but there is evidence that some cases may follow a viral carditis. Some cases of rheumatic fever may be unrecognized and it is only possible to elicit a history of rheumatic fever or chorea in about half of the patients.

The mitral valve orifice is normally about 5cm2 in diastole ,and is reduced to 1cm or less in severe mitral stenosis .Patients usually remain asymptomatic until the stenosis is moderate (approximately 2cm2 ). With the reduction in size of the valve orifice , cardiac output can be maintained only by a rise in left atrial ,pulmonary venous and pulmonary capillary pressures ,with resulting loss of lung compliance and the development of exertional dyspnoea. With mild stenosis atrial pressure rises only on exercise , but as the stenosis becomes more severe (1 -1.5 cm2), raised left atrial pressure is required to maintain cardiac output even at rest, with further increases in pressure on exercise. The raised atrial pressure precipitates atrial fibrillation, which may be the first sign of clinical deterioration. Loss of co-ordinated atrial contraction and shortened diastole in uncontrolled atrial fibrillation. Both contribute to diminished ventricular filling. Conditions associated with an increase in cardiac output (eg: pregnancy) are tolerated poorly.
A sudden increase in pulmonary venous pressure, caused perhaps by the onset of atrial fibrillation may precipitate pulmonary oedema. The onset of atrial fibrillation may be accompanied by a 20% fall in cardiac output. With a more gradual rise in pressure, there tends to be an increase in pulmonary vascular resistance which protects against pulmonary oedema.
In about 80% of cases left atrial dilatation is prominent and is usually accompanied by atrial fibrillation. A minority of patients remain in sinus rhythm; this is usually associated with a small fibrotic left atrium and severe pulmonary hypertension .All cases may eventually develop pulmonary hypertension results and right ventricular hypertrophy . The pulmonary hypertension results from a combination of passive back - pressure , arteriolar constriction , and obliterative changes in the pulmonary vasculature .
All patients with mitral stenosis are at risk from left atrial thrombsis and systemic thromboembolism ,particularly those with atrial fibrillation.

CLINICAL FEATURES
Symptoms
The gradual reduction in the mitral valve orifice usually produces insidious onset of breathlessness, and pulmonary congestion may cause cough. The extra demands of pregnancy or the impairment of function brought about by tachycardia or atrial fibrillation,may precipitate deterioration, and may bring on breathlessness even at rest. Acute pulmonary oedema or pulmonary hypertension can lead to haemoptysis. Systemic embolism is sometimes a presenting feature.
 Exertional dyspnoea, Nocturnal dyspnoea, cough
 Ankle and leg edema, abdominal swelling ( right heart failure)
 Symptoms of acute pulmonary edema ( especially with pregnancy or AF).
 Symptoms secondary to Arterial or Venous emboli - hemoptysis , chest pain.
 Symptoms of diminished cardiac output. Eg: fatigue, tiredness.

Asymptomatic mitral stenosis; the physical signs of mitral stenosis are often found before symptoms develop, and their recognition is of particular importance in pregnancy.
Signs
The stenotic valve prolongs atrial emptying, resulting in the leaflets remaining open at the onset of systole and closing with an unusually loud sound [S1] ,which may be palpable - the tapping apex beat. The turbulent flow, which is heralded by the opening snap, causes the characteristic low pitched diastolic murmur and often a thrill.
 Atrial fibrillation
 Mitral facies
 Auscultation Loud first heart sound, Opening snap, Mitral - diastolic murmur
 Signs of increased pulmonary pressure.
Crepitations,Pulmonary oedema, effusions
 Signs of pulmonary hypertension
The murmur is accentuated by exercise and during atrial systole in early or asymptomatic patients a presystolic murmur may be the only auscultatory abnormality. In patients with symptoms, the murmur usually extends from the opening snap to the first heart sound. The opening snap gets closer to the second sound (S2) as the stenosis becomes more severe, but may be inaudible if the valve is heavily calcified. Accompanying mitral regurgitation causes a pansystolic murmur which radiates towards the axilla. The development of pulmonary hypertension may be demonstrated by an abnormal pulsation felt to the left of the sternum, due either to right ventricular hypertrophy or to forward displacement of the heart by a dilated left atrium. Pulmonary hypertension may cause loud pulmonary component of the second heart sound, and right atrial hypertrophy may produce a prominent 'a' wave in the jugular venous pulse [unless AF is present].Tricuspid regurgitation secondary to right ventricular dilatation causes a systolic murmur and systolic waves in the venous pulse.

Investigations
The ECG may show either the bifid ‘p’ wave [p mitrale] associated with left atrial hypertrophy or atrial fibrillation. There may be evidence of right atrial and right ventricular hypertrophy: one of the earliest signs is a reduction in the size of the usual QS complex in lead VI. Enlargement of the left atrium and its appendage and of the main pulmonary artery may be seen on the chest radiograph. There may be enlargement of the upper pulmonary veins and horizontal linear shadows in the costophrenic angles as indications of high left atrial and pulmonary venous pressure.
 ECG: Left atrial hypertrophy [If not in AF] , RVH
 Chest x-ray- Enlarged left atrium. signs of pulmonary venous hypertension.
 ECHO- thickened immobile cusps, Reduced rate of diastolic filling. Reduced valve area.
 Cardiac Catheterisation- Pressure gradients between Left atrium [pulmonary wedge] & left ventricle.

Echo cardiography can provide the definitive evaluation of MS; apart from confirming diagnosis it allows an assessment of its severity and it also provides information on the rigidity and state of calcification of the valve cusps, the size of the left atrium and the state of left ventricular function.

Management
Patients with minor symptoms should be treated medically, but the definite treatment of mitral stenosis is by mitral valvotomy, balloon valvuloplasty or mitral valve replacement. If the patient remains symptomatic despite medical treatment, if pulmonary congestion persists, or if pulmonary hypertension develops, valvuloplasty or surgery is indicated.

Criteria for mitral valvuloplasty
 Significant symptoms
 Isolated Mitral Stenosis.
 No [or trivial] MR
 Mobile, non-calcified valves/sub valve apparatus on echo.
 Left atrium free of thrombus

MITRAL REGURGITATION
Aetiology and Pathophysiology
Chronic, gradual -onset mitral regurgitation produces volume over load of the left atrium. Left atrial dilatation occurs and there is little increase in pressure until heart failure ensues. In contrast, acute regurgitation results in a rapid rise in left atrial pressure because atrial compliance is normal. The raised atrial and pulmonary venous pressure leads to pulmonary oedema and can be measured invasively. As part of the left ventricular stroke volume regurgitates into the atrium, the stroke volume has to increase to maintain cardiac output, and this results in ventricular enlargement.

CAUSES OF MITRAL REGURGITATIONS
 Mitral valve prolapse.
 Dilatation of the mitral valve ring
 eg:- Rheumatic fever, myocarditis, cardiomyopathy.
 Damage to valve cusps and chordae,
 eg:- Rheumatic heart disease , Endocarditis.
 Damage to papillary muscle
 Myocardial infarction.

CLINICAL FEATURES
The symptoms depend on how suddenly the regurgitation develops. When the valve damage is a slow process the symptoms are similar to those in mitral stenosis. After acute myocardial infarction, the mitral regurgitation may be severe and should be differentiated from ventricular septal rupture.

Symptoms Of Mitral Regurgitation.
I, Acute Mitral Regurgitation
Symptoms of acute pulmonary oedema and reduced cardiac output.

II,Chronic Progressive Mitral Regurgitation
 Exertional dyspnoea,nocturnal dyspnoea, palpitations [AF,Atrial flutter, Increased stroke volume]
 Symptoms of pulmonary oedema [esp with pregnancy or Atrial Flutter]
 Symptoms of diminished cardiac output.
 eg:fatigue,tiredness.
 Ankle,leg oedema,abdominal swelling [right heart failure]

Signs of Mitral Regurgitation
• Atrial fibrillation or Atrial flutter
• Cardiomegaly -Displaced Hyperdynamic apex beat.
• Apical pansystolic murmur with thrill.
• Soft S1,apical S3
• Signs of raised pulmonary capillary pressure- crepitations,pulmonary oedema, effusions.
• Signs of pulmonary hypertension may be present.
The physical signs arise from the regurgitant jet which causes an apical systolic murmur. This often radiates into the axilla, and may be accompanied by a thrill. The apex beat is usually displaced to the left as a result of dilatation of the left ventricle. The abnormal valve closure is often associated with a quiet heart sound and the increased forward flow through the mitral valve may give rise to a loud third heart sound and even a short mid-diastolic murmur.

INVESTIGATIONS
• ECG Left atrial hypertrophy (if not in AF)
Left ventricular hypertrophy
• Chest radiograph Enlarged left atrium ventricle.
Enlarged left ventricle
Signs of pulmonary venous hypertension.
Signs of of pulmonary sion oedema cif a/c.
• Echo Dilated LA, LV
Dynamic LV (unless L V F predominates)
Regurgitation defeatable on doppler or colour doppler.

• Cardic
Catheterisation Dilated LA, LV, mitral reguragitation.
Pulmonary hypertension may be present (chronic MR)

Management
If mitral regurgitation is due to myocardial disease, treatment when available is directed to the latter. When the valve disease is predominant and symptoms severe, mitral valve replacement is indicated, Infective endocarditis should be treated if possible before surgery.

Patients who are being managed medically should be reviewed at regular intervals; worsening symptoms of progressive radiological cardiac enlargement are indications for surgical intervention. Similarly, diminished LV function or markers of raised LV end diastolic pressure on echocardiography are indications for intervention.

Aortic valve disease
Aortic stenosis
Aetiology and pathophysiology
The likely etiology of aortic stenosis varies with the age of the patients
Causes of aortic stenosis

Infants, children,adolescents
• Congenital aortic stenosis
• Congenital subvalvular aortic stenosis
• Congenital supravalvular aortic stenosis

Young adults to middle aged
• Calcification of bicuspid valve
• Senile degenerative aortic stenosis
• Rheumatic aortic stenosis.


Except in the congenital forms, aortic stenosis develops slowly; the cardiac output is maintained at the cost of steadily increasing gradient across the aortic valve. The left ventricle becomes increasingly hypertrohied, and coronary blood flow may become inadequate. The fixed out flow obstruction limits the increase in cardiac output required on exercise. Patients may develop angina, left ventricular failure and arrheythmias even in the absence of concomitant coronary disease. In elderly patients aortic stenosos and coronary atheroma frequently co exist.

Clinical features
Mild or moderate aortic stenosis is asymptomatic.
Symptoms
• extertional dyspnoea
• Angina
• Pulroonary oeduna
• Exertional syncope.
• Sudden death

Signs
• Ejection systolic murmur
• Slow rising carotial pulse, reduced pulse pressure.
• Left ventricular hypertophy.
• Thrusting left ventricle.
• Signs of left ventricular failure
• Signs of left ventricular failure
(creptitations, pulmonary oedema)

Investigations
The ECG may show left atrial and ventricular hypertophy and ST changes and in advanced cases features of hypetrophy are gross. L B B B is common. Down slop ST segments and T inversion ( Strain patters) may be seen in leads reflecting the left ventrocle. However esp in the elderly, the ECG may be normal despite normal stenosis.
The postero - anterior chest radiograph is frequently normal but may show left ventriular enlargement and post - stenotic dilatation of the ascending aorta. A lateral radiograph or magnetic resonance image, may show valve classification, but the diagnosis is more readily made on echocardiography.
Echo cardiography will show an abnormal aortic valve, which may be heavily classified and disorganised and an hypertrophied left ventricle. Doppler cardiography permits calculation of the systolic gradient across the aortic valve from the velocity of the ejected jet of blood, and detects the presence or absence of aortic regurgitation. Cardiac catheterisation is indicated if the ultra sound studies are unsatisfactory or if it is necessary to assess the state of the coronay arteries.
• ECG - LVH (usually)
• Chest X ray - May be normal sometimes enlarged left vetricle and dilated ascending aorta on PA view, clacified valve on lateral view.
• Echo - Clacified valve, hyprotrphied LV Doppler estimate of gradient.
• Cardiac Catheterisation - Systolic gradient between LV and aorta.
Post stenotic dilatation of aorta, Regurgitation of AV may be present.

Management
Patients with symptomatic aortic stenosis and a valve gradient indicative of moderate or sever stenosis (ie) 60 mm mercury in the presence of normal cardiac output at rest) should have aortic valve replacement. To wait too long exposes the patient to the risk of sudden death or irreversible deterioration in ventricular function. However, prospective reviews of asymptomatic stenosis in the elderly have revealed a relatively benign prognosis without surgery, and in some patients conservative management may be appropriate. Patients should be kept under review as the development of angina, symptoms of low cardiac output, or heart failure are indications for surgery. Old age, per se, is not a contraindication to valve replacement and results remain very good in experienced centres even into the ninth decade.
Aortic balloon valvuloplasty may produce transient improvement in patients with severe heart failure or intercurrent illness, but doesnot substitute for valve replacement in this condition. It is useful in congential aortic stenosis. Patients with mild aortic stenosis should be followed up with regular ultrasound examination to detect progression of stenosis.
Aortic Regurgitation

Aetiology and patho physiology
Aortic Regurgitation may be congentital or acquired

Causes of AR
I congential
• Bicuspid valve or diproportionate cusps
II. Acquired aortic regurgitation.
• Rheumatic disease.
• Infective endocarditis
• Trauma
• Aortic dilatation : Marfan syndrome, atheroma, syphilis, ankylosing spondylitis.

Patho physiology
When regurgitation is marked, the stroke output of the left ventricle may be doubled or trebled. The major arteries are then conspicuously pulsatile - the left ventricle dilates and hypertrophies and initially compensates for the regurgitation. The left ventiricular diastolic pressure rises, at first only with exercise the pulmonary vasuclar pressures then also increase and breathlessness develops. In contract to chronic gradual onset regurgitation, acute regurgitation may result from damage to the aortic leaflets endocarditis, trauma) and auscultatory signs may be masked by the abrupt rise in LV end diastolic pressure (shortening or even abolishing the typical murmur).

Clinical features
Until the onset of breathlessness the only symptom may be an awareness of the heart beat, particularly when lying on the left side. This results from the increased stroke volume. Paroxysmal nocturnal dyspnoea may be the first symptom and peripheral oedema or angina may occur. The characteristic murmur is usually best heard to the left of the sternum it is sometimes louder to the right. The early diastolic murmur is best heard at the left sternal edge and may be accompanied by an ejection systolic murmur due to increased blood flow with the enlarged stroke volume. The murmur is best heard with the patient leaning forward and the breath held in expiration. When the leake is large, the diagnosis is usually easy, with gross pulsation in the large arteries, a collapsing pulse, a low diastolic and an increased pulse pressure. There is usually a thrusting apical impulse and often a presystolic impulse and a fourth heart sound the regurgitation jet produces fluttering of the mitral leaflets and a soft mid-diastolic murmur called an Austin Flint murmur. In acute severe regurgitation, the features of heart failure may predominate, the murmur, may be short (or even absent) and there may be insufficient time for the ventricle to dilate this possibility must be considered, especially in the context of endocarditis.

Symptoms of AR
I Mild to moderate AR
• Often asymptomatic
• Awareness of heart beats - palpitations.
severe AR
• Symptoms of heart failure
• Angina

Signs of Aortic Regurgitation
Pulses
• Large volume or collapsing pulse.
• Bounding peripheral pulses.
• Capillary pulsation in nail beds - Quincke’s sign.
• Femoral bruit (pistol shot) - Duroziers signs.
• Head nodding with pulse - de Musset’s sign.
Murmurs
• Early diastolic murmur
• Systolic murmur of increased stroke volume.
• Austin flint murmur (soft mid-diastolic)
Other signs
• Thrusting apex, 4th heart sound, enlarged LV
• Signs of heart failure.
• Hill’s sign - systolic blood pressure in lower limb is greater than that of upper limb.

Investigations in AR
ECG
• Initially normal, later LV hypertophy and T wave inversion
Chest radiograph
• Cardiac dilatation, may be aortic dilatation.
• Features of LVF.
Echo
• Dilated left ventricle
• Hyper dynamic ventricle
• Fluttering anterior mitral leaflet, doppler detects reflux.

Cardiac catheterisation (may not be requried)
• Dilated LV, aortic regurgitation
• Dilated aortic root.

The chest radiograph characteristically shows cardiac and aortic dilatation, together with signs of left heart failure. When regurgitation is marked the ECG may show left ventricular hypertrophy and ST changes. Echo cardiography in aortic regurgitation shows a dilated ventricle with vigorous contraction (until heart failure ensues)

There may be fluterring of the anteriar mitral leaflet in the regurgitant jet. Regurgitation is readily detected by doppler or colour doppler echo cardiography. In severe aortic regurgitation the rise in diastolic pressrue may cause mitral valve closure before the onset of ventiricular systole. The echocardio gram may reveal vegetations in infective endo carditis, and gives information about left ventricular function. Cardiac cathterisation and aortography can be helpful in assessing severity and dilatation of the aorta.

Management
Aortic valve replacement under cardiopulmonary bypass is indicated when aortic regurgitation is begining to cause symptoms or when an enlarging heart or progressive ECG changes gives evidence of increasing left ventricular overload. Treatment is required for underlying conditons (endocanditis, syphilis)

Tricuspid valve disease
Tricuspid stenosis
It is usually rheumatic in origin, and nearly always occurs in association with mitral and aortic valve disease. It is uncommon, with clinically evident tricuspid stenosis occuring in < 5% of rheumatic heart disease. Isolated rheumatic tricuspid stenosis is very rare. T S and regurgitation are also associated with carcinoid syndrome.

Clinical features
Usually the symptoms of the associated mitral and aortic valve disease predominate, but tricuspid stenosis causes symptoms of right heart failure including hepatic discomifort and ascites and peripheral oedema.
The main clinical feature is a raised jugular venous pressure with a prominent a wave and a slow descent on account of the loss of the normal rapid RV filling there may be presystolic hepatic pulsation, which represents a palpable a wave. There is a mid-diastolic murmur best heard at the lower left or right sternal edge; this is usually higher pitched than the murmur of MS and increased by inspiration.

Symptoms of TS
• Symptoms of mitral or aortic disease.
• Symptoms of right heart failure.
Abdominal swelling
Hepatic discomfort
Peripheral oedema
Tiredness fatigue
Signs of tricuspid stenosis
• Raised JVP, prominent ‘a’ wave.
• Mid diastolic murmur - Increased by inspiration.
• Right heart failure - Ascites, peripheral oedema.
Investigations
1 Chest radiograph
Enlarged RA (not specific for TS)
2 Echo
Fused, thickened tricuspid valve, Dilated RA, doppler features of TS.

Tricuspid Regurgitation
Aetiology and Pathophysiology

Tricuspid regurgitation is common. The most frequent cause is described as functional, as the valve is not stucturally abnormal but is stretched as a result of reight ventricular dilatation (eg: follwing pulmonary hypertension or cor pulmonale).

Causes of Tricuspid Regurgitation
I. Primary
• Rheumatic heart disease
• Endo carditis, particularly in intravenous drug abusers.
• Ebstein’s congenital anomaly.
II Secondary
• Following right ventricular dilatation (functional TR)
• Right ventricular infarction.

Symptoms
Are usually non specific and relate to reduced forward flow and venous congestion (tiredness, oedema, hepatic enlargement). The most prominent clinical feature is a large systolic wave in the jugular venous pulse (a ‘cv’ wave replaces the normal ‘x’ descent)
Signs
• Raised JVP
• Large systolic wave in JVP (CV wave)
• Systolic hepatic pulsattion.
• Pansystolic murmur (left sternal edge) - louder on inspiration).
Investigations
Chest radiograph
• Dilated RA, RV
Echo
• RV dilatation
• TV may be structurally abnormal (rheumatic disease, endocarditis, Ebstein’s congenital anomaly).

Management
Tricuspid regurgitation, which is due to right ventricular dilatation gets better when the cause of right ventricular overload is corrected. Patients with a normal pulmonary artery pressure tolerate isolated tricuspid refline without ill effects, and valves damaged by endocarditis do not always need to be replaced. A few patients with organic TV damage and elevated pulmonary artery pressure may need tricuspid valve repair (annuloplasty) or valve replacement.

Pulmonary Valve Disease
Pulmonary stenosis
The condition is virutally always congerital. It may be islated or associated with other abnormalities such as Fallot’s tetralogy.

Clinical Features and Management
Symptoms of PS
• Symptoms of right heart failure
• Symptoms of the underlying cause of pulmonary stenosis - eg: carcinoid syndrome.
Signs of pulmonary stenosis
• Giant a wave in the JVP
• RV hypertophy and dilatation.
• Systolic murmur (upper left sternum) increases on inspiration with increased pulmonary flow.
• Systolic thrill over pulmonary outflow
• P2 soft and delayed.
• Valvular PS may have an ejection click.

Investigations
Chest radiograph
• Prominent atrial and RV hypertrophy
ECHO
• Abnormal PV
• Out flow gradient on Doppler.

The principal finding on examination is the ejection systolic murmur, loudest to the left of the upper sternum, and radiating towards the left shoulder. There may be a thrill, best felt when the patient learns forward and breaths out. The murmur is often preceded by an ejection sound. Delay in right ventriculon ejection may cause wide splitting of the second heart sound. Severe PS is characterised clinically by : a loud harsh murmur, an inaudible pulmonary closure sound (P2); an increased right ventricular thrust prominent a waves in the jugular pulse; ECG evidence of right ventricular hypertrophy; and poststenotic dilatation in the pulmonary artery on the chest radiograph.
Mild to moderate isolated pulmonary stenosis is relatively common, does not usually progress and does not require treatment. It is a low risk lesion for refectively evdocanditis. Severe pulmonary stenosis (resting gradient > 50 mm Hg with a normal caridace output) is treated by percutaneous pulmonary ballon valvulopalasty or if unavailable, by surgical balloon valvotomy. Long term results are very good. Post operative pulmonary regurgitation is common but enough.

Pulmonary regurgitation
It is rare as an isolated phenomenon. Usually assoicated with pulmonary artery dilatation which is due to pulmonary hypertension, and may follow us. An early decrescendo murmur is heard at the left eternal edge and may be difficult to distinguish from AR (Graham steel murmur).
The pulmonary hypertension may also be secondary to other diseases of the left side of the heart, to primary pulmonary vascular disease or to Eisenmger’s syndrome. Trivial pulmonary regurgitation is frequent echo cardio graphic Doppler finding in normal individuals and is not of clinical significance.

REFERENCES
1. Textbook of Medicine – K.V. Krishna Das
2. Clinical Medicine – K.V. Krishna Das
3. Harrison’s internal Medicine
4. Gray’s anatomy
5. Davidson’s principles and practice of medicine

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