Rheumatic heart disease (RHD)

1. Rheumatic heart disease (RHD) By : Dr. Sanjeev

2. Rheumatic heart disease • The sequelae of rheumatic fever consist of mitral, aortic and tricuspid valve disease • The mitral valve involvement manifests predominantly as mitral regurgitation and less common as mitral stenosis • The aortic and tricuspid valve involvement presents exclusively as aortic and tricuspid regurgitation • Rheumatic aortic stenosis has never been described below the age of 15 years.

3. Terms • Regurgitation : results from failure of a valve to close completely, thereby allowing reversed flow • Stenosis : failure of a valve to open completely, thereby impeding forward flow • Pure : only stenosis or regurgitation is present • Mixed : both stenosis and regurgitation coexist in the same valve, but one of these defects usually predominates

4. Heart sounds • First heart sound : when AV valve closed (mitral and tricuspid) • Second heart sound : pulmonary and aortic valve closed • Third heart sound: increase volume of blood within the ventricle • Fourth heart sound : just after atrial contraction at the end of diastole and immediately before S1.

5. Mitral regurgitation • Is the commonest manifestation of acute as well as previous rheumatic carditis

6. Hemodynamics • When mitral regurgitation is present ----- blood leaks backwards through the mitral valve and into the left atrium when the heart contracts (systolic phase) --- regurgitant volume of blood reaches the left atrium during ventricular systole, however, during diastole it can pass freely across the mitral valve ---- thus, mean atrial pressure = normal or is only slightly increased (because left atrial pressure increases during systole, it drops during diastole) ---- there is thus no increase in pulmonary venous pressure and no pulmonary congestion --------

7. Cont… • ----the increased volume of blood handled by the left atrium and left ventricle results in an increase in the size of both these chambers --Mitral regurgitation provides two exits for the left ventricular blood flow -- the forward flow through the aortic valve into the systemic circulation and the backward leak into the left atrium -- the forward output becomes insufficient during exertion-- this decrease in the systemic output results in fatigue, the commonest symptom of significant MR -- absence of pulmonary congestion prevents occurrence of dyspnea unless the MR is severe or the left ventricular myocardium is failing ----

8. Cont… • With failing left ventricle, the left ventricular diastolic pressure increases, the left atrial and pulmonary venous pressure increases and pulmonary congestion appears -- there is an increase in pulmonary arterial pressure and features of pulmonary arterial hypertension appear. • Presence of features of pulmonary arterial hypertension in a patient having pure MR suggests : • 1. severe MR or • 2. failing left ventricular myocardium, or • 3. acute MR

9. Cont… • MR developing during acute RF is of sudden onset. In addition there is active myocarditis resulting in poorly functioning left ventricular myocardium. Thus the left ventricular failure can occur even with relatively moderate leaks during the acute illness. • The size of the left atrium also plays significant role in MR • With acute MR the left atrial size is normal and the increased volume reaching the left atrium increases the left atrial and the pulmonary venous pressure, resulting in pulmonary congestion and feature of left ventricular failure

10. Cont…. • In long standing MR the left atrium increases in size to accommodate the regurgitant volume without increasing the left atrial pressure and features of LVF are absent. • Another adjustment consists of decrease in the systemic vascular resistance to help increase the forward flow. R = P/Q • where R is the vascular resistance (fluid resistance), P is the pressure difference, and Q is the rate of blood flow through it.

11. Cont… • The maximum ejection of blood into the aorta takes place during early systole. The combination of these two factors results in an increased systolic and decreased diastolic pressure in the systemic circuit . The pulse pressure is, therefore, increased resulting in the small water hammer pulse of MR

12. Aetiology • Dilatation of valve ring (Acute rheumatic fever, Cardiomyopathy) • Damage to the valve cusp and chordae (Rheumatic heart disease, Infective Endocarditis) • Damage to the papillary muscle (myocardial ischaemia , infarction) • Mitral valve prolapse (congenital, degenerative, connective tissue disease such as Marfan’s syndrome). • Trauma — Chest trauma can rarely cause breakage of the chords that hold the mitral leaflets in their normal position. Untethered leaflets swing widely, allowing valve leakage.

13. Clinical features • 1. Fatigue : when cardiac output starts to fall • 2. Dysponea : when pulmonary venous hypertension occurs, dysponea on exertion, orthopnea and paroxysmal nocturnal dysponea (PND) may ocuur • 3. Pulse rate increased to maintain an adequate cardiac output • 4. Features of left ventricular failure are absent and appear late unless the mitral regurgitation is acute, severe or left ventricular myocardium is failing

14. Cont.. • 5. Heart size is dependent on the severity of MR as well as the status of the left ventricular myocardium. • 6. Apex beat is shifted down and out, farther than the normal position, due to ventricular dilatation • 7. Systolic thrill (<10 %) due to the direction of the regurgitant stream which is backwards into the left atrium • 8. Systolic murmur is heard over the cardiac apex (mitral area) with following characteristic : - • 1. Pansystolic murmur extending from s1 to s2 • 2.High frequency murmur (diaphragm) • 3. murmur radiates towards the left axilla and to the back below the scapula

15. Cont… • 9. First heart sound may be normal or diminished in intensity • 10. Severe MR, when a large amount of blood flows downs suddenly from the left atrium to the left ventricle during diastole, a third sound (s3) or ventricular gallop is produced. Immediately after such a third sound, a short mid diastolic murmur may also be heard.

16. Investigations Chest X-Ray: ECG Echocardiography Doppler Cardiac catheterization

17. Chest X-Ray Backflow of blood due to incompetent mitral valve • Heart is enlarged transversely • The pulmonary vascular markings are typically normal, since pulmonary venous pressures are usually not significantly elevated. • Pulmonary vascular markings prominent (marked pul. HTN)

18. Cont… • ECG: Atrial fibrillation, left atrial enlargement (if patient is in sinus rhythm). left ventricular hypertrophy can be seen • Echocardiography: Images mitral valve, left ventricular function and left atrial size. LA and LV will be dilated. • Doppler will quantify regurgitation • Cardiac catheterization can be done for pressure measurements

19. Differential diagnosis :- • Atrial septal defect • Coarctation of aorta with MR (congenital) • Left ventricular fibroelastosis • Myocarditis

20. Management : - Medical Management : • Low sodium diet • Diuretics (patient with orthopnoea and PND) • Vasodilator: Sodium Nitroprusside or Nitroglycerine may be used in acute and/or severe MR. • ACE inhibitors are used for treatment of chronic MR (decreased the after load).

21. Cont… • Digoxin is used for patients with atrial fibrillation or associated left ventricular failure. • Anticoagulant for patients with atrial fibrillation, for prevention of thromboembolism and who already developed features of systemic embolization to prevent further embolization. • Infective Endocarditis prophylaxis. • Prophylaxis for Rheumatic fever if MR is of rheumatic origin.

22. Cont… Surgical Management : • Symptomatic patients despite optimal medical therapy • Asymptomatic or mildly symptomatic patient in presence of progressive LV dysfunction. • Mitral valve repair (Annuloplasty with valve Reconstruction) can be done if valvular cusps and basic architecture is preserved. • Otherwise markedly deformed, with shrunken, calcified leaflets requires mitral valve replacement with a prosthesis.

23. Complications : • Atrial fibrillation (in case of severe MR and chronic long standing MR) • Systemic embolization • Infective endocarditis • Congestive heart failure • Pulmonary hypertension

24. Mitral stenosis Normal size: 5 sq. cm Cardiac symptoms due to mitral stenosis start to be appear only when the valve is reduced to 2 sq.cm Severe stenosis < 1 cm2 Aetiology : • Acute RF with rheumatic endocarditis (99%) • Some due to calcification of senile mitral valve apparatus • Congenital (very rare)

25. Pathophysiology : - • Blood cannot flow freely from the left atrium to the left ventricle during diastole -- left atrial pressure as well as volume increases --- increase in pressure and volume occurs in the pulmonary veins and capillaries --- when the pulmonary venous pressure exceeds the plasma oncotic pressure, fluid from the vessels flow out into the interstitial space and alveoli of the lungs --- leads to pulmonary arterial hypertension--- right ventricle has to work more during systole to push the blood into the pulmonary artery --- leads to right ventricular hypertrophy and later on to right ventricular dilatation -- if pulmonary HTN becomes severe, the amount of blood going to the left atrium from the right ventricle and pulmonary congestion tends to become less.

26. Clinical features : - • Symptoms : • 1. Dyspnoea (commonest symptom) : due to pulmonary venous congestion. • Mild stenosis-dyspnoea occurs on exertion or when the heart rate increases due to any reason. • Severe stenosis-dyspnoea at rest • May develop orthopnoea and PND • 2. Cold extremities, with or without peripheral cyanosis and a smaller volume pulse -- decreased cadiac output in severe MS (recognized on the bed side)

27. Cont.. • 3. Fatigue (due to low cardiac output) • 4. Palpitation (Atrial fibrillation, Sinus tachycardia) • 5. Haemoptysis (Pulmonary congestion, Pulmonary embolism) • 6. Cough, chest pain • 7. Symptoms of Thromboembolism • 8. Oedema, ascites (right heart failure)

28. Signs • Irregularly irregular pulse (atrial fibrillation) • Mitral facies (bluish pink hue over the malar prominences) • Auscultation: Loud S1 , opening snap, mid diastolic murmur • Signs of raised pulmonary capillary pressure: Basal crepitation, pulmonary oedema, and pleural effusion • Signs of pulmonary hypertension: RV heave, loud P2 • Signs of right heart failure : E.g. Raised JVP, Hepatomegaly • Signs of systemic Thromboembolism : E.g. Stroke, Acute limb ischaemia

29. Mitral Facies

30. Investigations : ECG : Atrial fibrillation,Left Atrial abnormality, Right ventricular enlargement • Echocardiogram : Structural imaging of mitral valve, valve area, left atrial dimension, presence of thrombus in LA, pulmonary arterial pressure, RV dilatation.

31. 31 Chest X-Ray: Straightening of the left border with fullness & outwards bulging of the pulmonary conus There is double border on the right side Pulmonary vasculature increases Normally, Heart is normal in transverse diameter Cardiomegaly (rt. Ventricular enlargement) Kerley B line Dr S Chakradhar

32. Management 1 . Medical management : • Penicillin prophylaxis for rheumatic fever. • Prophylaxis for infective endocarditis. • Low sodium intake, diuretics. • If patient is in Atrial fibrillation ---- use digoxin  low dose B-blocker. • Anticoagulation for at least 1 year for patients who suffered Thromboembolism and permanently to those with AF.

33. 2. Surgical management : a. Mitral valvotomy : • Symptomatic patients whose valve area is less than 1.0 cm2/m2 body surface area. • Two methods : 1. Percutaneous ballon mitral valvotomy and 2. Surgical valvotomy : Indicated in Re-stenosis , unsuccessful balloon valvotomy,. • Restenosis is frequent. This procedure cannot be done if there is significant regurgitation, calcification of the mitral valve or thrombus in left atrium.

34. b. Mitral valve replacement : • This is procedure of choice in : • Critical mitral stenosis i.e. < 0.6 cm2/m2 body surface area • Significant mitral regurgitation • Calcified mitral valve leaflets

35. Complications • Atrial fibrillation • Systemic emboli • Pulmonary hypertension and • Heart failure

36. AORTIC STENOSIS • Aortic valve area : 3 square cm

37. Aetiology : Infants ,children, adolescents • Congenital • Valvular aortic stenosis • Subvalvular aortic stenosis • Supravalvular aortic stenosis

38. Cont… Young adults to middle aged • Calcification and fibrosis of bicuspid aortic valve • Acute rheumatic fever with endocarditis

39. Pathophysiology : - • When it gets narrowed, left ventricle has to pump harder to send blood across the narrowed aortic valve into the aorta - increased work load -left ventricular hypertrophy - hypertrophied ventricle manages to maintain the cardiac output inspite of stenosis - during atrial systole, plenty of blood comes to the left ventricle (atrial kick) - left ventricle becomes more stretched due to such atrial kicks and as per Frank Starling`s law, it now contracts more vigorously and thus more blood goes out of the ventricle into the aorta - gradually , the oxygen demand of the left ventricle increases -cause angina and sudden death - if left ventricle is overworked for prolonged period - LVF - aorta blood will be less -left ventricular end diastolic pressure and diastolic volume start to rise -left arterial and pulmonary venous pressure increases and the patient starts to feel dyspnoeic ( pulmonary congestion and hypertension)

40. Clinical features : - • Mild or moderate ----- Asymptomatic • Cardinal symptoms like (1, 2, and 3) • Exertional dyspnoea (signs of LVF):- initially exertional dyspnoea later PND. • Angina • Syncope : due to inadequate blood flow through the stenosed aortic valve and arrhythmia. • Fatigue and palpitation • Apex beat : heaving or forceful and sustained type (finger lifted up during systole, remains up for sometime and then falls down

41. Cont… 6. Auscultation : three main signs : • Aortic ejection sound or click : heard over the cardiac apex by the diaphragm, in early systole, immediately after the first sound. • Aortic ejection murmur : mid systolic murmur, heard over the right 2nd intercostal space by the side of the sternum, radiates to the neck towards both the carotids, and also called diamond shaped ejection systolic murmur. • Aortic component of the second sound is either late or soft

42. Cont… • 7. Fourth heart sound : due to increased stiffness of the left ventricle, the atrium contracts vigorously during atrial systole and pushes the a large amount of blood into the left ventricle, due to such strong ` atrial kick`, S4 becomes audible. It is a soft and low pitched sound and is heard just before S1. best heard over the cardiac apex by using the bell of the stethoscope.

43. Investigations : - • ECG: may show LV hypertrophy and ST depression and T wave inversion; left bundle branch block is common; • Chest X-Ray : may show LV enlargement in PA view and calcification of aortic valve in lateral view. • Echocardiography : will show abnormal aortic valve with left ventricular hypertrophy or dilatation. • Doppler echocardiography : will estimate the pressure gradient

44. enlargement of the ascending aorta(white arrow). left ventricle is enlarged (red arrow) and the heart is mildly enlarged overall. The lateral view on the right demonstrates calcifications in the region of the aortic valve leaflets (circle). generally, the aortic valve lies above a line drawn from the carina to the junction of the diaphragm with the anterior chest wall. The mitral valve lies below the line.

45. Management • Strenuous physical activity should be avoided • Sodium restriction, digitalis and diuretics are used if there is heart failure. • Vasodilators should be avoided or used with extreme caution. • Asymptomatic stenosis in elderly conservative management is appropriate

46. Valve replacement in : • Patients with calcified AS with critical obstruction (valve area <0.5 cm2/m2 BSA). • Patients with symptomatic aortic stenosis (moderate to severe stenosis) even with normal cardiac output at rest. • Patients who exhibit LV dysfunction even they are asymptomatic.

47. Cont… • Penicillin prophylaxis for rheumatic fever. • Prophylaxis for infective endocarditis.

48. Complications • Endocarditis • Cardiac arrhythmias : atrial fibrillation, ventricular arrhythmias, complete heart block • Left ventricular failure

49. Differential diagnosis • Hypertrophic cardiomyopathy • Innocent systolic murmur eg. In anemia, thyrotoxicosis • Hypertension

50. AORTIC REGURGITATION