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The diagnosis of heart disease must be The diagnosis of heart disease must be * Etiological: → (Congenital - Rheumatic) * Anatomical: → (VSD - TOF - MR - MS) * Functional: → HF {decompensated} OR no HF {compensated} * Complication: → rheumatic activity - infective endocarditis - PH - arrhythmia - chest infection

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RHEUMATIC HEART DISEASE Occurs in severe cardiac involvement during initial or recurrent attacks of ARF Left - sided heart valves are most often affected, (mitral followed by the aortic valves) Mitral regurge is the commonest lesion in children and adolescent with RHD

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MITRAL REGURGE (MR, Insufficiency, Regurgitation, Incompetence) The mitral valve consists of: an annulus 2 leaflets ( anterior & posterior ) - chordae tendinea - 2 papillary muscles

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Pathophysiology Healing of ARF results in Fibrosis & contracture of leaflets Shortening & thickening of chordea tendinea. Leaflets cannot coapt and separated LA and LV volume overload and enlargement. Pulmonary venous congestion, PH, RVH

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Clinical Manifestations: Symptoms Mild MR → no symptoms Severe MR → Symptoms of HF, pulmonary congestion, pulmonary edema dyspnea - orthopnea - paroxysmal nocturnal dyspnea

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Signs: Signs: Apex → (LV apex), shifted downward, localized, forcible, hyperdynamic (ill sustained) with systolic thrill. S1 is usually normal S2 is usually normal except in PH Pansystolic murmur maximal intensity at the apex, radiating to the axilla. Short middiastolic murmur over the apex may be heard (functional MS) Ejection systolic murmur on 2nd Lt ics (PH)

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Mitral regurge CXR

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Differential Diagnosis of MR 1- VSD: -maximal intensity over the 3rd & 4th left intercostal spaces -propagated in fan manner 2- Tricuspid regurge: - maximal intensity on lower left sternal border - increases in intensity during inspiration. 3- Mitral regurge of Carditis: - maximal intensity on the apex - soft, musical, not associated with thrill, changeable

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Management Prophylaxis → Against rheumatic recurrences (LONG ACTING PENECILLIN) → Against infective endocarditis Medical treatment of heart failure arrhythmia infective endocarditis Captoprile ( After load reducing agent) Surgical treatment (Annuloplasty or valve replacement ) is indicated in severe mitral regurge with: Recurrent heart failure cardiomegaly with pulmonary hypertension.

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MITRAL STENOSIS (MS) Pathophysiology -Thickening of valve leaflets - Fusion of commissures - Shortening & thickening of chordae tendineae. - Funnel shaped valve apparatus → marked obstruction to blood flow from LA to LV LA enlargement (Not LV), pulmonary venous congestion, PH, RV & RA dilation Right side HF

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Clinical manifestations: The clinical course depends on the severity of MS. Symptoms: Dyspnea on exertion. Orthopnea & paroxysmal nocturnal dyspnea. Poor growth and development. Tachycardia and atrial fibrillation. Congestive heart failure may be present.

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Signs Signs Signs of RV hypertrophy: a- The apex is diffuse and shifted outward (RV apex), diastolic thrill b- Left parasternal pulsations c- Epigastric pulsations loud S1 Apical, rumbling mid-diastolic murmur.

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Mitral stenosis CXR

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Differential Diagnosis of MS Mitral flow murmur (functional MS) associated with large VSD, PDA, MR, AR (Austin flint murmur) - Normal S1 - No presysolic accentuation or opening snap - Original lesion Carditis (Carey Coombs murmur). Soft , low pitched Changeable Not associated with thrill. Normal or muffled S1

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Management: Prophylaxis → Against rheumatic recurrences (LONG ACTING PENECILLIN) → Against infective endocarditis Medical treatment: Heart failure and atrial fibrillation (AF). Surgical or baloon trans-catheter valvotomy .

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Combined MS and MR Combined MS and MR Dilatation, scaring and narrowing → stenosis & leakage Obstruction and leakage of mitral valve → LA , RV & LV hypertrophy LV enlargement is going with MR and against pure MS RV enlargement is going with MS and unusual with MR

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AORTIC REGURGE (AR, Insufficiency, Regurgitation, Incompetence) Rheumatic AR is the result of fibrosis and contracture of the aortic valve structure Hemodynamically AR → LV volume overload Rheumatic AR is almost always associated with mitral valve disease.

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Clinical manifestations: Symptoms Depend on the severity. In moderate and severe cases: Effort intolerance, palpitation, dyspnea, orthopnea & paroxysmal nocturnal dyspnea, excessive sweating. Manifestations of pulmonary congestion and edema.

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Signs The rapid run off of the blood from aorta during diastole causes the signs of hyperdynamic circulation: - The pulse is collapsing (water hammer) - BP: wide pulse pressure (high systolic & low diastole) - Corrigan’s sign prominent carotid pulsation in the neck. - Capillary pulsation is visible (alternative systolic flushing and diastolic blanching as pressure is applied to finger nails ) - Pistol shots heard over the femoral arteries due opening of collapsed arteries during systole Duroziez’s murmur: a systolic and diastolic murmur detected by applying mild pressure by the stethoscope over the femoral artery. - Musset’s sign: Head movement in time with heart beat.

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* Manifestations of LV enlargement. The apex is shifted downword, forcible, localized and hyperdynamic (ill sustained) - The S1 & S2 are normal * Early diastolic murmur begins immediately after the S2. maximum intensity at the 2nd aortic area, the patient sitting and leaning forward & the breath held in expiration. * Austin flint murmur Apical (mid diastolic), rumbling in Character (functional mitral stenosis)

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Aortic regurge CXR Aortic regurge Echocardiography Aortic regurge CXR Aortic regurge Echocardiography

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Management Prophylaxis → Against rheumatic recurrences (LONG ACTING PENECILLIN) → Against infective endocarditis Surgery: Aortic valve replacement. It could be recommended at earlier stages of the disease.

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AORTIC STENOSIS - Commissural adhesions occur slowly and progressive → narrowing and calcification of the orifice leads to significant aortic stenosis. - Obstruction of LV emptying results in LV hypertrophy.

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Clinical manifestations: Symptoms: In cases with severe stenosis: Chest pain, exercise intolerance, dyspnea, syncope.

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Signs - The apex: Localized, forceful & sustained (pressure overload). - Systolic thrill is common on Rt sternal border radiates to the neck. - Normal S1 - Normal or single S2 Beyond childhood, scarring & calcification decrease mobility of the valve and thus the intensity of aortic component decreases (single) - Ejection systolic murmur maximally on 2nd Rt or 3rd Lt ics radiates to the neck.

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Aortic stenosis CXR Aortic stenosis CXR Aortic stenosis Echocardiography shows morphology of the valve and degree of stenosis.

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Management → Against rheumatic recurrences (LONG ACTING PENECILLIN) → Against infective endocarditis Surgical intervention by valve replacement. Trans-catheter balloon dilatation is considered in some cases.