10. RHD.pptx

Rheumatic Heart Disease Acute Rheumatic Fever Incidence and Pathogenesis  affects children (most commonly between 5 and 15 years) triggered by an immune-mediated delayed response to infection with specific strains of group A streptococci  antigens that may cross-react with cardiac myosin and sar...

Rheumatic Heart Disease Acute Rheumatic Fever Incidence and Pathogenesis  affects children (most commonly between 5 and 15 years) triggered by an immune-mediated delayed response to infection with specific strains of group A streptococci  antigens that may cross-react with cardiac myosin and sarcolemmal membrane protein  antibodies cause inflammation in the endocardium, myocardium and pericardium, as well as the joints, skin and central nervous system Clinical Features  multisystem disorder that usually presents with fever, anorexia, lethargy and joint pain  2–3 weeks after an episode of streptococcal pharyngitis but there may be no history of sore throat  the diagnosis, made using the revised Jones criteria  is based upon two or more major manifestations, or one major and two or more minor manifestations, along with evidence of preceding streptococcal infection  Carditis manifests as: ■ new or changed heart murmurs ■development of cardiac enlargement or cardiac failure ■appearance of a pericardial effusion and ECG changes of pericarditis, myocarditis, AV block or other cardiac arrhythmias  Non-cardiac features include the following: ■ Fever ■ Arthritis associated with RF is classically a fleeting migratory polyarthritis affecting large joints such as the knees, elbows, ankles and wrists ■Sydenham’s chorea (or St Vitus’ dance) is involvement of the central nervous system that develops late after a streptococcal infection. Sufferers are noticeably ‘fidgety’ and display spasmodic, unintentional choreiform movements Speech is often affected (explosive and halting)  Erythema marginatum - a transient pink rash with slightly raised edges, which occurs in 20% of cases -found mostly on the trunk and limbs coalesce into Crescent or ring-shaped patches  Subcutaneous nodules- painless, pea-sized, hard nodules beneath the skin Investigations  Evidence of a systemic illness (non-specific) • Leucocytosis, raised ESR and CRP  Evidence of preceding streptococcal infection (specific) •Throat swab culture: group A β-haemolytic streptococci (also from family members and contacts) • Antistreptolysin O antibodies (ASO titres): rising titres, or levels of > 200 U (adults) or > 300 U (children)  Evidence of carditis •Chest X-ray: cardiomegaly; pulmonary congestion •ECG: first- and rarely second-degree AV block; features of pericarditis; T-wave inversion; reduction in QRS voltages •Echocardiography: cardiac dilatation and valve abnormalities Management of the acute attack To eliminate any residual streptococcal infection as well as limiting cardiac damage and relieving symptoms  A single dose of benzyl penicillin (1.2 million U IM) or  oral phenoxymethylpenicillin (250 mg 4 times daily for 10 days)  If pen-allergic, erythromycin or a cephalosporin To lessen joint pain and reduce cardiac workload  Bed rest and supportive therapy  can return to normal physical activity but strenuous exercise should be avoided in those who have had carditis Cardiac failure should be treated as necessary If heart failure with severe MR and concomitant AR does not To relieve the symptoms of arthritis rapidly and a response within 24 hours helps confirm the diagnosis  Aspirin - reasonable starting dose is 60 mg/kg body weight/day, divided into six doses. In adults, 100 mg/kg per day or a maximum of 8 g per day To produce more rapid symptomatic relief than aspirin and are indicated in cases with carditis or severe arthritis  Prednisolone - 1.0–2.0 mg/kg per day in divided doses Secondary prevention  Benzathine penicillin (1.2 million U IM monthly) or  oral phenoxymethylpenicillin (250 mg twice daily)  Sulfadiazine or erythromycin may be used if the patient is allergic to penicillin  until the age of 21 years Valvular heart disease Chronic Rheumatic Heart Disease  develops in at least half of those affected by rheumatic fever with carditis  main pathological process is progressive fibrosis  the heart valves are predominantly affected but involvement of the pericardium and myocardium may contribute to heart failure and conduction disorders  fusion of the mitral valve commissures and shortening of the chordae tendineae may lead to MS with or without regurgitation  similar changes in the aortic and tricuspid valves produce distortion and rigidity of the cusps, leading to stenosis and regurgitation Valvular Heart Disease Causes of valvular heart disease 1.Rheumatic Heart disease 2.Degenerative 3 Congenital 4 Ischaemic 5 Infective endocarditis Rheumatic disease strep throat. The infection can cause scarring of the heart valve. This is the most common cause of valve disease worldwide Endocarditis infection and damage Congenital heart valve disease is malformations of the heart valves, such as missing one of its leaflets. The most commonly affected valve with a congenital defect is a bicuspid aortic valve, which has only two leaflets rather than three. caused by valvular or chordal degeneration Degenerative Ischaemic Restricted leaflet motion is the principal mechanism for ischemic mitral regurgitation. Increased leaflet tethering caused by papillary muscle displacement and left ventricular dilatation, Valve involved Stenotic disease Insufficiency/regurgitation disease Aortic valve Aortic valve stenosis Aortic insufficiency/ regurgitation Mitral valve Mitral valve stenosis Mitral insufficiency/ regurgitation Tricuspid valve Tricuspid valve stenosis Tricuspid insufficiency/ regurgitation Pulmonary valve Pulmonary valve stenosis Pulmonary insufficiency/ regurgitation Rheumatic valvular lesions  Mitral Stenosis, Mitral Regurgitation & Mitral Valve Prolapse 50%  Mitral & Aortic valve lesions  Tricuspid valve is affected infrequently 40%  Mitral, Aortic & Tricuspid Lesions  Aortic Stenosis & Aortic regurgitation 5%  All other combinations 2% 3% Mitral Stenosis Causes of MS  almost always rheumatic in origin  older people can be caused by heavy calcification of the mitral valve apparatus  Congenital (rare) Symptoms  Clinical Features Fatigue (low cardiac output)  Oedema, ascites (right heart failure)  Palpitation (atrial fibrillation)  Breathlessness (pulmonary congestion)  Haemoptysis (pulmonary congestion, pulmonary embolism)  Cough (pulmonary congestion)  Chest pain (pulmonary hypertension)  Thromboembolic complications ( e.g. stroke, ischaemic limb) Signs  Atrial fibrillation  Mitral facies  Auscultation Loud first heart sound, opening snap Mid-diastolic murmur  Crepitations, pulmonary oedema, effusions (raised pulmonary capillary pressure)  Left parasternal heave, loud P2 (RVH ,pulmonary hypertension) Complications of MS  Atrial fibrillation :Systemic embolization  Pulmonary hypertension  Infective endocarditis (rare)  Tricuspid regurgitation  Right ventricular failure Investigations ECG  Right ventricular hypertrophy: tall R waves in V1–V3,  P mitrale or atrial fibrillation Chest X-ray  Enlarged LA and appendage, Signs of pulmonary venous congestion Echo  Thickened immobile cusps, Reduced valve area, Enlarged LA ,Reduced rate of diastolic filling of LV Doppler  Pressure gradient across mitral valve, Pulmonary artery pressure, Left ventricular function Cardiac catheterisation  Coronary artery disease, Pulmonary artery pressure ,Mitral stenosis and regurgitation Management Medical management  anticoagulation- to reduce the risk of systemic embolism  digoxin, β-blockers or rate-limiting calcium antagonists – to control ventricular rate in AF  diuretic therapy – to control pulmonary congestion Surgery Mitral balloon valvuloplasty Mitral valvotomy Valve replacement Mitral Regurgitation Causes of Mitral Regurgitation  Rheumatic disease is the principal cause  Mitral valve prolapse  Dilatation of the LV and mitral valve ring (e.g. coronary artery disease, cardiomyopathy)  Damage to valve cusps and chordae (e.g. rheumatic heart disease, endocarditis)  Ischiaemia or infarction of the papillary muscle (Myocardial infarction) Clinical features (and their causes) in MR Symptoms  Dyspnoea (pulmonary venous congestion)  Fatigue (low cardiac output)  Palpitation (atrial fibrillation, increased stroke volume)  Oedema, ascites (right heart failure) Signs  Atrial fibrillation/flutter  Cardiomegaly: displaced hyperdynamic apex beat  Apical pansystolic murmur ± thrill  Soft S1, apical S3  Signs of pulmonary venous congestion (crepitations, pulmonary oedema, effusions)  Signs of pulmonary hypertension and right heart failure Investigations of MR ECG  Left atrial hypertrophy (if not in atrial fibrillation) Chest X-ray  Enlarged LA, LV  Pulmonary venous congestion  Pulmonary oedema (if acute) Echo  Dilated LA, LV  Dynamic LV (unless myocardial dysfunction predominates)  Structural abnormalities of mitral valve (e.g. prolapse) Doppler  Detects and quantifies regurgitation Cardiac catheterisation  Dilated LA, dilated LV, mitral regurgitation  Pulmonary hypertension  Coexisting coronary artery disease Management of MR Medical Management  Diuretics  Vasodilators, e.g. ACE inhibitors  if atrial fibrillation is present - Digoxin - Anticoagulants Mitral valve replacement  sudden torrential mitral regurgitation, as seen with chordal or papillary muscle rupture or infective endocarditis Aortic regurgitation Causes of AR Congenital  Bicuspid valve or disproportionate cusps Acquired  Rheumatic disease  Infective endocarditis  Trauma  Aortic dilatation (Marfan’s syndrome, aneurysm, dissection, syphilis, ankylosing spondylitis) Clinical features of aortic regurgitation Symptoms Mild to moderate aortic regurgitation  Often asymptomatic Signs Pulses  Large-volume or ‘collapsing’ pulse  Low diastolic and increased pulse pressure  Bounding peripheral pulses  Capillary pulsation in nail beds: Quincke’s sign  Femoral bruit (‘pistol shot’): Duroziez’s sign  Head nodding with pulse: de Musset’s sign  Awareness of heart beat, Murmurs  Early diastolic murmur ‘palpitations’  Systolic murmur (increased stroke volume)  Austin Flint murmur (soft mid-diastolic) Severe aortic regurgitation  Breathlessness Other signs  Displaced, thrusting apex beat (volume overload)  Pre-systolic impulse  Angina  Fourth heart sound  Crepitations (pulmonary venous congestion) Investigations in aortic regurgitation ECG • Initially normal, later left ventricular hypertrophy and T-wave inversion Chest X-ray • Cardiac dilatation, maybe aortic dilatation • Features of left heart failure Echo • Dilated LV • Hyperdynamic LV • Fluttering anterior mitral leaflet Doppler - detects reflux Cardiac catheterisation (may not be required) • Dilated LV • Aortic regurgitation Management of AR  Treat underlying conditions - such as endocarditis or syphilis - Aortic valve replacement (combined with aortic root  Symptomatic AR replacement and coronary bypass surgery)  Asymptomatic patients - followed up annually with echocardiography for evidence of increasing ventricular size  Systolic BP - controlled with vasodilating drugs, such as nifedipine or ACE inhibitors Aortic Stenosis Causes of AS Infants, children, adolescents  Congenital aortic stenosis  Congenital subvalvular aortic stenosis  Congenital supravalvular aortic stenosis Young adults to middle-aged  Calcification and fibrosis of congenitally bicuspid aortic valve  Rheumatic aortic stenosis Middle-aged to elderly  Senile degenerative aortic stenosis  Calcification of bicuspid valve  Rheumatic aortic stenosis Clinical features of Aortic Stenosis Symptoms • Mild or moderate stenosis: usually asymptomatic • Exertional dyspnoea • Angina (↑demand of LVH) Cardinal symptoms CO fails to rise to meet demand • Exertional syncope • Sudden death • Episodes of acute pulmonary oedema Signs • Ejection systolic murmur • Slow-rising carotid pulse • Heaving apex beat (LV pressure overload) • Narrow pulse pressure • Signs of pulmonary venous congestion (e.g.crepitations) Investigations in Aortic stenosis ECG  Left ventricular hypertrophy (usually)  Left bundle branch block Chest X-ray  May be normal; sometimes enlarged LV and dilated ascending aorta on PA view, calcified valve on lateral view Echo  Calcified valve with restricted opening, hypertrophied LV Doppler  Measurement of severity of stenosis  Detection of associated aortic regurgitation Cardiac catheterisation  Mainly to identify associated coronary artery disease  May be used to measure gradient between LV and aorta Management of AS  Conservative management - asymptomatic AS  Doppler echocardiography- moderate or severe stenosis are evaluated every 1–2 years (to detect progression in severity)  aortic valve replacement - symptomatic severe aortic stenosis, old age  Aortic balloon valvuloplasty – congenital AS  Anticoagulants - atrial fibrillation or postvalve replacement with a mechanical prosthesis Transcatheter aortic valve replacement (TAVR) Transcatheter aortic valve replacement (TAVR) Tricuspid regurgitation  common, and is most frequently ‘functional’ as a result of right ventricular dilatation Causes of TR Primary  Rheumatic heart disease  Endocarditis, particularly in injection drug-users  Ebstein’s congenital anomaly Secondary  Right ventricular dilatation due to chronic left heart failure (‘functional tricuspid regurgitation’)  Right ventricular infarction  Pulmonary hypertension (e.g. cor pulmonale) Clinical Features Symptoms  Usually non- specific  Tiredness (reduced forward flow)  Oedema  Hepatic enlargement (venous congestion) Signs  Raised JVP  Pansystolic murmur (left sternal edge)  Pulsatile liver Management  Correction of the cause of right ventricular overload (if TR is due to rt ventricular dilatation) - use of diuretic and vasodilator treatment of congestive cardiac failure  Valve repair  Valve replacement Pulmonary stenosis Symptoms  Fatigue, dyspnea on exertion, cyanosis  Poor weight gain or failure to thrive in infants  Hepatomegaly, ascities, edema Signs  Ejection systolic murmur (loudest at the left upper sternum & radiating towards the left shoulder)  Murmur often preceded by an ejection sound (click)  May be wide splitting of second heart sound (delay in ventricular ejection)  May be a thrill (best felt when patient leans forward and breaths out) Investigation  ECG - right ventricular hypertrophy,  Chest X-ray- post-stenotic dilatation in the pulmonary artery  Doppler echocardiography - definitive investigation Management  Mild to moderate isolated pulmonary stenosis - does not usually progress or require treatment  Severe pulmonary stenosis - percutaneous pulmonary balloon valvuloplasty - surgical valvotomy Pulmonary regurgitation rare in isolation  usually associated with pulmonary artery dilatation due to pulmonary hypertension which may be - secondary to other disease of the left side of the heart - primary pulmonary vascular disease or - Eisenmenger’s syndrome  complication mitral stenosis- producing an early diastolic decrescendo murmur at the left sternal edge that is difficult to distinguish from aortic regurgitation (Graham Steell murmur)  Trivial PR is frequent finding in normal individuals and has no clinical significance THANK YOU