Rheumatic fever Rheumatic fever is an acute immunologically mediated multisystem inflammatory disease that occurs a few weeks following an episode of group A streptococcal pharyngitis. Acute rheumatic carditis, during active phase leads to valve regurgitation. May progress to Chronic rheumatic heart disease. usually valve stenosis.

Acute rheumatic fever Incidence and pathogenesis  Acute rheumatic fever usually affects children ( most commonly between 5 and 15 years ) or young adults, and has become very rare in Western Europe and North America. However, it remains endemic in parts of Asia, Africa and South America, and is the most common cause of acquired heart disease in childhood and adolescence.

Etiopathogenesis  Acute rheumatic fever is a hypersensitivity reaction induced by group A streptococci cause inflammation in the endocardium, myocardium and pericardium, as well as the joints and skin.  Immune mediated delayed Antibody response against M proteins of certain strains of streptococci cross react with antigens in heart, joints, skin and other tissues. Genetic susceptibility is suggested  Chronic sequelae are a result of progressive fibrosis ( healing process ) and blood turbulance in valvular areas

Pathogenesis Aschoff nodules are pathognomonic and occur only in the heart vegetations Aschoff body, myocardium Fibrinous pericarditis CROSS REACTIONS

Morphology ACUTE RH. FEVER --  Pancarditis Pericarditis -  serofibrinous / Bread and butter type Myocarditis  Aschoff bodies Endocarditis  Verrucous vegetations (1- 2 mm ) at lines of closure of valves Fibrinoid necrosis along cusps and tendinous cords

vegetations Aschoff body, myocardium Fibrinous pericarditis

Carditis Aschoff bodies Aschoff giant cell

Morphology of chronic RHD Mitral valve is most often affected with rheumatic heart disease, followed by combined involvement of mitral and aortic together, then aortic alone, then mitral, aortic, and tricuspid together. Mitral stenosis (99% cases ) Fish mouth / buttonhole stenosis Microscopy Fibrosis / calcification and scarring

Mitral valve as seen from above in the left atrium. Typical " fish mouth " shape with chronic rheumatic scarring and fibrosis. Mitral valve is most often affected with rheumatic heart disease.

Clinical features of ARF The major clinical manifestations of ARF : migratory polyarthritis carditis, subcutaneous nodules, erythema marginatum, and Sydenham chorea. Minor manifestations of ARF : Fever, arthralgias, Increased blood levels of acute phase reactants etc.

DIAGNOSIS Jones criteria : Evidence of preceding group A strept. Infection Presence of two major or one major and two minor manifestations.

Clinical features of ARF Age : 5-15 but may be in adults Time : 10 days to 6 wks after pharyngitis 3% of pts effected Prognosis of 1 st attack is good.

Clinical features of chronic rheumatic carditis Valvular disease and its sequelae (years later) Murmurs Cardiac hypertrophy, dilatation, heart failure Atrial Arrythmias esp Atrial fibrillation Thromboembolic complications

Chronic disease rheumatic aortic stenosis With fused commisures rheumatic mitral valve,

' pancarditis '  involves the endocardium, myocardium and pericardium to varying degrees. Its incidence declines with increasing age.  It may manifest as breathlessness ( due to heart failure or pericardial effusion ), palpitations or chest pain ( usually due to pericarditis or pancarditis ). tachycardia, cardiac enlargement and new or changed cardiac murmurs.  A soft systolic murmur due to mitral regurgitation is very common.  A soft mid - diastolic murmur ( the Carey Coombs murmur ) is typically due to valvulitis, with nodules forming on the mitral valve leaflets.

 Aortic regurgitation occurs in about 50% of cases but the tricuspid and pulmonary valves are rarely involved.  Pericarditis may cause chest pain, a pericardial friction rub and precordial tenderness.  Cardiac failure may be due to myocardial dysfunction or valvular regurgitation.  ECG changes commonly include ST and T wave changes. Conduction defects sometimes occur and may cause syncope

Arthritis  This is the most common major manifestation and tends to occur early when streptococcal antibody titres are high.  An acute painful asymmetric and migratory inflammation of the large joints typically affects the knees, ankles, elbows and wrists.  The joints are involved in quick succession and are usually red, swollen and tender for between a day and 4 weeks.  The pain characteristically responds to aspirin ; a dramatic response, if not, the diagnosis is in doubt.

Skin lesions  Erythema marginatum occurs in < 5% of patients. The lesions start as red macules ( blotches ) that fade in the centre but remain red at the edges and occur mainly on the trunk and proximal extremities but not the face. The resulting red rings or ' margins ' may coalesce or overlap  Subcutaneous nodules occur in 5-7% of patients. They are small ( cm ), firm and painless, and are best felt over extensor surfaces of bone or tendons. They typically appear more than 3 weeks after the onset of other manifestations and therefore help to confirm rather than make the diagnosis

Sydenham's chorea  ( St Vitus dance )  This is a late neurological manifestation that appears at least 3 months after the episode of acute rheumatic fever, when all the other signs may have disappeared.  It occurs in up to one - third of cases and is more common in females. Emotional lability may be the first feature and is typically followed by purposeless involuntary choreiform movements of the hands, feet or face.   Spontaneous recovery usually occurs within a few months. Approximately one - quarter of affected patients will go on to develop chronic rheumatic valve disease

Management of the acute attack  A single dose of benzyl penicillin 1.2 million U i. m. or oral phenoxymethylpenicillin 250 mg 6- hourly for 10 days should be given on diagnosis to eliminate any residual streptococcal infection.  If the patient is penicillin - allergic, erythromycin or a cephalosporin can be used.  Treatment is then directed towards limiting cardiac damage and relieving symptoms.  Bed rest and supportive therapy Bed rest is important, as it lessens joint pain and reduces cardiac workload. The duration should be guided by symptoms along with temperature, leucocyte count and ESR, and should be continued until these have settled. Patients can then return to normal physical activity but strenuous exercise should be avoided in those who have had carditis.

 Cardiac failure should be treated as necessary. Some patients develop a fulminant form of the disease with severe mitral regurgitation and sometimes concomitant aortic regurgitation. If heart failure in these cases does not respond to medical treatment, valve replacement may be necessary and is often associated with a dramatic decline in rheumatic activity.  AV block is seldom progressive and pacemaker insertion rarely needed.

 Aspirin will usually relieve the symptoms of arthritis rapidly and a response within 24 hours helps to confirm the diagnosis. A reasonable starting dose is 60 mg / kg body weight / day, divided into six doses. In adults, 100 mg / kg per day may be used. Aspirin should be continued until the ESR has fallen and then gradually tailed off.  Corticosteroids produce more rapid symptomatic relief than aspirin and are indicated in cases with carditis or severe arthritis.  There is no evidence that long - term steroids are beneficial. Prednisolone, mg / kg per day in divided doses, should be continued until the ESR is normal then tailed off.

Mitral Stenosis: Etiology  Primarily a result of rheumatic fever ( ~ 99% of MV stenosis at surgery show rheumatic damage ) Rheumatic, Rheumatic, Rheumatic, and Rheumatic  Scarring & fusion of valve apparatus  Rarely congenital  Pure or predominant MS occurs in approximately 40% of all patients with rheumatic heart disease  Two - thirds of all patients with MS are female.

In the dilated atrium with a stenotic mitral valve, the blood stagnates. Hence, stasis is in favor of thrombogen esis..

Mitral Stenosis

Mitral Stenosis: Pathophysiology Right Heart Failure: Hepatic Congestion JVD Tricuspid Regurgitation RA Enlargement  Pulmonary HTN Pulmonary Congestion LA Enlargement Atrial Fib LA Thrombi  LA Pressure RV Pressure Overload RVH RV FailureLV Filling

Normal Mitral valve area: 4-6 cm 2

Mitral Stenosis: Symptoms Fatigue Palpitation s Cough SOB Left sided failure Orthopnea PND Palpitation  Atrial fibrillation  Systemic embolism  Pulmonary infection  Hemoptysis  Right sided failure › Hepatic Congestion › Edema  Worsened by conditions that  cardiac output. › Exertion, fever, anemia, tachycardia, Afib, intercourse, pregnancy, thyrotoxicosi s

Recognizing Mitral Stenosis Palpation : Small volume pulse Tapping apex - palpable S 1 +/- palpable opening snap ( OS ) RV lift Palpable S 2 ECG : LAE, AFIB, RVH, RAD Auscultation : Loud S 1- as loud as S 2 in aortic area A 2 to OS interval inversely proportional to severity Diastolic rumble : length proportional to severity In severe MS with low flow - S 1, OS & rumble may be inaudible

Mitral Stenosis: Physical Exam  First heart sound ( S 1) is accentuated and snapping  Opening snap ( OS ) after aortic valve closure  Low pitch diastolic rumble at the apex  Pre - systolic accentuation ( esp. if in sinus rhythm ) S1 S2 OS S1

Mitral Stenosis: Natural History Progressive, lifelong disease, Usually slow & stable in the early years. Progressive acceleration in the later years year latency from rheumatic fever to symptom onset. Additional 10 years before disabling symptoms

Mitral Stenosis: EKG LAE RVH Premature contractions Atrial flutter and / or fibrillation  freq. in pts with mod - severe MS for several years A fib develops in  30% to 40% of pts w / symptoms

Mitral Stenosis: Complications Atrial dysrrhythmias Systemic embolization (10- 25%) Risk of embolization is related to, age, presence of atrial fibrillation, previous embolic events Congestive heart failure Hemoptysis Massive : 2 0 to ruptured bronchial veins ( pulm HTN ) Streaking / pink froth : pulmonary edema, or infection Pulmonary infections

Mitral Stenosis: Role of Echocardiography  Diagnosis of Mitral Stenosis  Assessment of hemodynamic severity › mean gradient, mitral valve area, pulmonary artery pressure  Assessment of right ventricular size and function.  Assessment of valve morphology to determine suitability for percutaneous mitral balloon valvuloplasty  Diagnosis and assessment of concomitant valvular lesions  Reevaluation of patients with known MS with changing symptoms or signs.  F / U of asymptomatic patients with mod - severe MS

Mitral Stenosis:Therapy Medical Diuretics for LHF / RHF Digitalis / Beta blockers / CCB : Rate control in A Fib Anticoagulation : In A Fib Balloon valvuloplasty Effective long term improvement

Mitral Stenosis:Therapy Surgical Mitral commissurotomy Mitral Valve Replacement Mechanical Bioprosthetic. Give lifelong warfarin after mechanical prosthetic MV replacement Maintain target INR 2,5-3,5 TIMES control.

Criteria for mitral valvuloplasty  Significant symptoms  Isolated mitral stenosis  No ( or trivial ) mitral regurgitation  Pliable, Mobile, non - calcified valve / subvalve apparatus on echo  LA free of thrombus  Sinus rhythm  minimal fibrosis, thin leaflet  Criteria for mitral Valve replacement  is indicated if there is substantial mitral reflux or if the valve is rigid and calcified  Significant concomittant MR.

Aortic regurgitation This condition is due to disease of the aortic valve cusps or dilatation of the aortic root. The LV dilates and hypertrophies to compensate for the regurgitation. The stroke volume of the LV may eventually be doubled or trebled, and the major arteries are then conspicuously pulsatile

Symptoms _ Dyspnea, orthopnea, PND _Chest pain. Nocturnal angina >> exertional angina {  diastolic aortic pressure and increased LVEDP thus  coronary artery diastolic flow} *With extreme reductions in diastolic pressures {e.g. < 40} may see angina Mild to moderate AR : * Often asymptomatic * Palpitation Severe AR :

_ Quincke’s sign: capillary pulsation in nail beds _Corrigan’s sign: _Water hammer pulse Large- volume or 'collapsing' pulse _Bisferiens pulse (AS/AR > AR) _De Musset’s sign: systolic head bobbing or Head nodding with pulse: de Musset's sign _Mueller’s sign: systolic pulsation of uvula Low diastolic and increased pulse pressure _ Durosier’s sign: femoral retrograde bruits _Traube’s sign: pistol shot femorals _Hill’s sign: BP Lower extremity >BP Upper extremity by _> 20 mm Hg - mild AR _> 40 mm Hg – moderate AR _> 60 mm Hg – severe AR Peripheral Signs

 Cardiac catheterisation and aortography can help in assessing the severity of regurgitation, and dilatation of the aorta and the presence of coexisting coronary artery disease.  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  Dilated aortic root

Causes of aortic regurgitation  Congenital  Bicuspid valve or disproportionate cusps  Acquired  Rheumatic disease  Infective endocarditis  Trauma  Aortic dilatation (Marfan's syndrome, aneurysm, dissection, syphilis, ankylosing spondylitis)

Natural history : * Asymptomatic+Normal LV function {--good prognosis} _Progression to symptoms or LV dysfunction < 6 % _Progression to asymptomatic LV dysfunction < 3.5 % _ 5-year survival 75% _Sudden death <0.2 % Abnormal LV function _Progression to cardiac symptoms 25 % * Symptomatic {poor prognosis _Mortality >10% Bonow RO, et al, JACC. 1998;32:1486.

Treatment Medical  Surgery BEFORE LV dysfunction. Surgery BEFORE LV dilatation. Once LVD occur you can do nothing,As you left with a last choice of heart transplantation

Chest XR PA view

Assessing severity of AR * Assess severity by impact on peripheral signs and LV  peripheral signs =  severity  LV =  severity S 3 Austin – Flint murmer LVH radiological cardiomegaly

Management : * Medical : _Vasodilator { ACEIs } _ Diuretics for pulmonary congestion __Treatment of underline cause e.g. IE, Syphilis *Surgical : _AV replacement Mechanical or Bioprosthesis _Aortic root replacement for dilated Aortic root, {e.g. Syphilis, Marfan`s syndrome, Dissecting aneurysm } *ECHO indications for AVR : _LVEDD >55 _EF > 55%

Criteria for Aortic Valve Replacement in Chronic Aortic Regurgitation Symptoms Congestive heart failure. Declining exercise tolerance on exercise testing. Angina Anatomy, regardless of symptoms: ie EVEN ASYMPTOMATIC Left ventricular dysfunction: EF <50% Progressive left ventricular dilation or decline in EF on serial studies Severe dilation (echo): - Left ventricular diastolic dimension >75 mm - Left ventricular systolic dimension >55 mm -Aortic root dimension >50 mm