Rheumatic valvular Heart Disease

1. Rheumatic valvular Heart Disease By Prof. Magdy Abou Al-Khair Pediatric Cardiology Unit Mansoura University Children’s Hospital

2. Rheumatic valvular Heart Disease • Mitral valve involvement is the commenst • (75%). • Aortic valve involvement in (25%). • Combined stenosis and regurgitation of the • same valve may occur. • Involvement of tricuspid valve is very rare. • Involvement of pulmonic valve almost never • occurs.

3. Rheumatic valvular Heart Disease • MITRAL STENOSIS. • MITRAL REGURGITATION. • AORTIC REGURGITATION.

4. Rheumatic valvular Heart Disease Mitral stenosis Prevalence It is the most common valvular involvement in adult rheumatic patients. In countries where rheumatic fever is more prevalent on in Egypt, severe MS occurs in children under age of 15 years.

5. Rheumatic valvular Heart Disease Mitral stenosis Pathology - Thickening of the leaflets and fusion of the commissures dominate the pathologic findings. Calcification with immobility of the valve results over time. - The left atrium (LA) and right-sided heart chambers become dilated and hypertrophied. - In patients with severe pulmonary venous hypertension, pulmonary congestion and edema, and fibrosis of the alveolar walls, hypertrophy of the pulmonary arterioles and loss of lung compliance result.

6. Rheumatic valvular Heart Disease Mitral stenosis Clinical manifestations • History • - Patients with mild MS are asymptomatic. • - Dyspnea with or without exertion is the most common symptom. • - Orthopnea, nocturnal dyspnea, or palpitation is present in more severe cases.

7. Rheumatic valvular Heart Disease Mitral stenosis Clinical manifestations Physical examination - An increased RV impulse is palpable along the LSB. - Peripheral pulses may be weak with narrow pulse pressure. - Neck veins are distended if right-sided heart failure supervenes. - A loud SI at the apex and a narrowly split S2 with accentuated P2 are audible if pulmonary hyptertension is present. - An opening snap (a short snapping sound accompanying the opening of the mitral valve) is followed by a low-frequency mitral diastolic rumble at the apex. - A crescendo presystolic murmur may be audible at the apex. - A high-frequency diastolic murmur of PR (Graham Steel’s murmur) is present at the ULSB.

8. Rheumatic valvular Heart Disease Mitral stenosis

9. Rheumatic valvular Heart Disease Mitral stenosis Laboratory Examination Electrocardiography RAD,LAH, and RVH (caused by pulmonary hypertension) are common. Atrial fibrillation is rare in children. X-ray study -The LA and RV usually are enlarged and the MPA segment usually is prominent. -Lung fields show pulmonary venous congestion, interstitial edema shown as Kerley’s B lines (dense, short, horizontal lines most commonly seen in the costopherenic angles), and redistribution of PBF (with increased pulmonoary vascularity) to the upper lobes.

10. Rheumatic valvular Heart Disease Mitral stenosis Laboratory Examination Echocardiography - Echo is the most accurate noninvasive tool for the detection of MS. -Two-dimensional echo shows doming of thick mitral leaflets, a small mitral valve orifice inscribed by the thickened valve, and a dilated LA. -Doppler studies can estimate the pressure gradient across the mitral valve and the level of PA pressure.

11. Rheumatic valvular Heart Disease Mitral stenosis Complications - Recurrence of rheumatic fever. - Atrial flutter or fibrillation. -Thromboembolism (related to the chronic atrial arrhythmias) are rare in children. - SBE can occur, but it is rare. - Hemoptysis can develop from the rupture small vessels in the bronchi as a result of long-standing pulmonary venous hypertension.

12. Rheumatic valvular Heart Disease Mitral stenosis Management A- Medical: -Good dental hygiene and antibiotic prophylaxis against SBE. -Recurrence of rheumatic fever is prevented with penicillin or sulfonamid. -Varying degrees of restriction of activity may be indicated. -If atrial fibrillation develops (rare in children), digoxin should be used to control ventricular response. -Ballon valvuloplasty is an alternative to surgical closed commissurotomy and may delay surgical intervention.

13. Rheumatic valvular Heart Disease Mitral stenosis Management B- Surgical Indications: -Symptomatic patients (dyspnea on exertion, pulmonary edema, paroxysmal dyspnea). -Recurrent atrial fibrillation, thromboembolic phenomenon, and hemoptysis.

14. Rheumatic valvular Heart Disease Mitral stenosis Management Procedures -Closed mitral commissurotomy remains the procedure of choice for those with a pliable mitral valve without calcification or MR. -Valve replacement may be indicated in patients with calcified valves and those with MR.

15. Rheumatic valvular Heart Disease Mitral Regurgitation Prevalence - MR the most common valvular involvement in children with rheumatic heart disease. Pathology - Mitral valve leaflets are shortened because of fibrosis. - When the degree of MR increases, dilatation of the LA, LV and the mitral valve ring may occur.

16. Rheumatic valvular Heart Disease Mitral Regurgitation Clinical Manifestations History - Patients usually are asymptomatic during childhood (because MR does not produce pulmonary congestion in the early phase).. - Fatigue (caused by reduced forward cardiac output). - Palpitation (caused by atrial fibrillation) develop.

17. Rheumatic valvular Heart Disease Mitral Regurgitation Clinical Manifestations Physical examination -The jugular venous pulse is normal in the absence of CHF. - A heaving, hyperdynamic apical impulse is palpable in severe MR. -The SI is normal or diminished. The S2 may split widely as a result of shortening of the LV ejection and early aortic closure. - The S3 commonly is present and loud. - The hallmark of MR is a regurgitant systolic murmur starting with S1, grade II-IV/VI, at the apex, with good transmission to the left axilla (best demonstrated on left decubitus position). - A short, low-frequency diastolic rumble may be present at the apex.

18. Rheumatic valvular Heart Disease Mitral Regurgitation

19. Rheumatic valvular Heart Disease Mitral Regurgitation Laboratory Examination Electrocardiograph -ECG is normal in mild cases. -LVH or LV dominance, with or without LAH, is usually present. -Atrial fibrillation is rare in children but often develops in the adult. X-ray study -The LA and LV are enlarged in varying degrees. -Pulmonary vascularity usually is within normal limits, but a pulmonary venous congestion pattern may develop if CHF supervenes.

20. Rheumatic valvular Heart Disease Mitral Regurgitation Laboratory Examination Echocardiography: -Two-dimensional echo shows dilated LA and LV, and the degree of dilatation is related to the severity of MR. -Colour-flow mapping of the regurgitant jet into the LA and Doppler studies can assess the severity of the regurgitation.

21. Rheumatic valvular Heart Disease Mitral Regurgitation Management Medical: -Preventive measures against SBE and prophylaxis against recurrence of rheumatic fever are important. -Activity need not be restricted in most mild cases. -Afterload-reducing agents are useful in maintaining the forward stroke volume. -Anticongestive measures (with diuretics and digoxin) are provided if CHF develops. -If atrial fibrillation develops (rare in children), digoxin is indicated to slow the ventricular response.

22. Rheumatic valvular Heart Disease Mitral Regurgitation Management Surgical: Indications. Intractable CHF, progressive cardiomegaly with symptoms, and pulmonary hypertension. Procedures. Mitral valve repair or valve replacement is performed under cardiopulmonary bypass.Valve repair surgery is preferred over valve replacement in children as long as the valve is pliable. Valve replacement is necessary if the valve is thick, scarred, and grossly deformed.

23. Rheumatic valvular Heart Disease Aortic Regurgitation Prevalence - AR is less common than MR . Most patients with AR have associated mitral valve disease. Pathology - Semilunar cusps are deformed and shortened. - The valve ring is dilated so that the cusps fail to oppose tightly. - The commissures usually are fused to a varying degree.

24. Rheumatic valvular Heart Disease Aortic Regurgitation Clinical Manifestations History - Patients with mild regurgitation are asymptomatic. - Exercise tolerance is reduced with more severe AR or CHF.

25. Rheumatic valvular Heart Disease Aortic Regurgitation Clinical Manifestations Physical examination - The precordium may be hyperdynamic with a laterally displaced apical impulse. - A diastolic thrill occasionally is present at the 3rd or 4th left intercostol space. - A wide pulse pressure and a bounding water-hammer pulse may be present with severe AR. - The SI is decreased in intensity. The S2 may be normal or single.

26. Rheumatic valvular Heart Disease Aortic Regurgitation Clinical Manifestations Physical examination - A high –pitched diastolic decrescendo murmur, best audible at the 3LICS or 4LICS, is the auscultatory hallmark. This murmur is more easily audible with the patient sitting and leaning forward. - A systolic murmur of varying intensity may be present at the 2RICS because of relative AS caused by an increased stroke volume. - The combination of the diastolic and systolic murmurs gives rise to a to- and- fro murmur. - A mid-diastolic mitral rumble(Austin Flint murmur) occasionally is present at the apex when the AR is severe.

27. Rheumatic valvular Heart Disease Aortic Regurgitation

28. Rheumatic valvular Heart Disease Aortic Regurgitation Laboratory Examination Electrocardiography - The ECG may be normal in mild cases. - In severe cases,LVH usually is present. - LAH may be present in long- standing cases.

29. Rheumatic valvular Heart Disease Aortic Regurgitation Laboratory Examination X-ray study - Cardiomegaly involving the LV is present. - A dilated ascending aorta and/or a prominent aortic knob frequently are present. - Pulmonary venous congestion develops if LV failure supervenes.

30. Rheumatic valvular Heart Disease Aortic Regurgitation Laboratory Examination Echocardiography. - The LV dimension is increased, but the LA remains normal in size. - Color-flow and Doppler examination can be used to estimate the severity of the regurgitation.

31. Rheumatic valvular Heart Disease Aortic Regurgitation Clinical Manifestations Natural History - The patient remains asymptomatic for a long time, but if symptoms begin to develop, many patients deteriorate rapidly. - Anginal pain and CHF are unfavorable symptoms. - Infective endocardits is a rare complication.

32. Rheumatic valvular Heart Disease Aortic Regurgitation Management Medical. - Good oral hygiene and antibiotic prophylaxis against SBE are important. - Prophylaxis should be continued against the recurrence of rheumatic fever with penicillin or sulfonamides. - Activity need not be restricted in mild cases, but varying degrees of restriction are indicated in more severe cases. - If CHF develops, digoxin, diuretics, and after load-reducing agents may be beneficial.

33. Rheumatic valvular Heart Disease Aortic Regurgitation Management Surgical Indications. A major clinical decision in AR is the timing of aortic valve replacement .Ideally,it should be performed before irreversible dilatation of the LV develops. - Symptomatic patients with anginal pain or dyspnea on exertion. - In asymptomatic patients * significant cardiomegaly (cardiothoracic ratio greater than 55% on chest x-ray films). * Ejection fraction less than 40%. * Stress-test- induced symptoms Procedure. Aortic valve replacement is performed under cardiopulmonary bypass.