VALVULAR HEART DISEASES Col Samina Waqar VALVULAR HEART

VALVULAR HEART DISEASES Col. Samina Waqar

VALVULAR HEART DISEASES Normal Anatomy:

VALVULAR HEART DISEASES Stenosis/ Regurgitation: intrinsic disease damage to the supporting structure Congenital: Acquired: • Stenosis of aotic or mitral valve • 2/3 - valvular diseases Functional regurgitation: • Abnormality of supporting structures • Dilation- right/left ventricle, dilated- pulmonary artery/aorta

VALVULAR HEART DISEASES Clinical consequences: Depends upon: • Valve involved • Degree of impairment • Rate and quality of compensatory mechanism e. g Infective endocarditis produces severe, rapid and fatal aortic regurgitation. Rheumatic mitral stenosis is indolent and well tolerated. Pregnancy complicates valvular disease by increasing demands on heart.

VALVULAR HEART DISEASES Valve degeneration associated with calcification: • Calcific aortic stenosis • Bicuspid valve stenosis(congenital) • Mitral valve prolapes RHD (Rheumatic Heart Disease) Endocarditis: • IEC (Infective endocarditis) • NBTE (Non bacterial thrombotic endocarditis) • LSE (Libman Sacks endocarditis

VALVULAR HEART DISEASES Aortic stenosis: • Senile calcific aortic stenosis • Bicuspid aortic valve • Mitral annular calcification

CALCIFIC AORTIC STENOSIS Calcific Aortic stenosis: • Age related: 7 th, 8 th decade senile calcific aortic stenosis • Stenotic bicuspid valve: 5 th- 7 th decade • Functional area decreases • Non Rheumatic, Rheumatic • Left ventricular hypertrophy

Calcific aortic stenosis Pathogenesis: • Accumulation of hydroxyapatite • Wear and tear • Chronic injury due to hyperlipidemias, hypertention and/or inflammation • Osteoblast like cells- synthesize –bone matrix protein. • Causes outflow obstruction

MITRAL LEAFLET PROLAPSE Myxomatous degeneration of mitral leaflets Attenuation of outer fibrosa core Increase in inner spongiosa core Etiology? Marfans syndrome: fibrillin mutation leading to dysregulation of TGF-b Valve surgical correction Complication- mitral insufficiency, IE, stroke

VALVULAR HEART DISEASES RHEUMATIC HD:

RHEUMATIC FEVER & RHEUMATIC HEART DISEASE (RHD) • DEFINITION “ACUTE IMMUNOLOGICALLY MEDIATED MULTISYSTEM INFLAMMATORY DISEASE THAT OCCURS A FEW WEEKS AFTER AN EPISODE OF GROUP- A, ß – HAEMOLYTIC STREPTOCOCCAL PHARYNGITIS AND OFTEN INVOLVES HEART”

PATHOLOGY OF RHEUMATIC HEART DISEASE AETIOLOGY 1. INFECTIVE ELEMENT 2. PERSONAL SUSCEPTIBILITY 3. SOCIAL DISTRIBUTION

MAJOR MANIFESTATIONS • MIGRATORY POLYARTHRITIS-LARGE JOINTS • CARDITIS • SUBCUTANEOUS NODDULES • ERYTHEMA MARGINATUM – SKIN • SYDENHAM’S CHOREA

MINOR MANIFESTATIONS • • • FEVER ARTHRALGIA INCREASED ESR LEUKOCYTOSIS C – REACTIVE PROTEIN ECG CHANGES: PROLONGED P- R INTERVAL

JONE’S CRITERIA FOR DIAGNOSIS OF RF • DIAGNOSIS REQUIRES TWO MAJOR FEATURES OR ONE MAJOR AND TWO MINOR FEATURES PLUS RAISED ANTISTREPTOCOCCAL ANTIBODY LEVELS (ANTI-STREPTOLYSIN O TITRE) OR POSITIVE THROAT CULTURE FOR GROUP A, ß-HEMOLYTIC STREPTOCOCCUS

PATHOGENESIS • EXACT- UNKNOWN • IMMUNE REACTION • HYPERSENSITIVITY REACTION TO GROUP A, ß-HEMOLYTIC STREPTOCOCCUS • ABSENCE OF STREPTOCOCCI –LESIONS • ANTIBODIES AGAINST M-PROTEINS • CROSS REACTION WITH TISSUE GLYCOPROTEINS • AUTOIMMUNITY • GENETIC SUSCEPTIBILITY

PATHOGENESIS - RHEUMATIC HEART DISEASE

PATHOLOGY OF RHEUMATTIC HEART DIEASE ACUTE R. H. D – HEART (Pancarditis) 1. PERICARDIUM 2. MYOCARDIUM 3. ENDOCARDIUM

MORPHOLOGY ACUTE RHEUMATIC FEVER • MYOCARDITIS – ASCHOFF BODIES – FIBRINOID NECROSIS, T-LYMPHOS, PLASMA CELLS, MACROPHAGES AND GIANT CELLS. – ANTISCHKOW CELLS (CATERPILLAR) ASCHOFF giant cells • PERICARDITIS – BREAD AND BUTTER

MORPHOLOGY (Contd) • ENDOCARDITIS SMALL VEGETATIONS ALONG LINE OF CLOSURE – VALVES • LEFT ATRIUM Mac. CALLUM PLACQUES

RHEUMATIC HEART DISEASE (RHD) • ACTIVE LESION a) EXUDATIVE LESION i. iii. b) • COLLAGEN DEGENERATION OEDEMA CELLULAR INFILTRATE ASCHOFF BODY HEALED LESION a) b) c) FIBROSIS MYXOMATOUS CHANGES CALCIFICATION

MORPHOLOGY-CHRONIC RHD • SOLITARY MITRAL VALVE (65 -70%) – LEAFLETS THICKENING, COMMISSURAL FUSIONS AND SHORTENING, THICKENING AND FUSION OF TENDINOUS CORDS (FISH-MOUTH APPEARANCE) • AORTIC/MITRAL VALVE (20 -25%) • TRICUSPID & PULM. VALVE (RARE) • DILATATION OF LEFT ATRIUM • MURAL THROMBUS

CLINICAL FEATURES • • 10 -42 DAYS AFTER PHARYNGITIS MOSTLY CHILDREN (5 -15 YEARS) MIDDLE AGED 20% MIGRATORY POLYARTHRITIS TACHYCARDIA, ARRHYTHMIA PERICARDIAL RUB RAISED ASOT

EFFECTS / COMPLICATIONS • • VALVULAR STENOSIS / DEFORMITY LEFT ATRIAL DILATATION / HYPERTROPHY ATRIAL FIBRILLATION MURAL THROMBUS – EMBOLISM CHRONIC CONGESTION OF LUNG RIGHT VENTRICULAR HYPERTROPHY & CHF INCREASED RISK OF IE ADHESIVE PERICARDITIS

CAUSES OF DEATH IN RHEUMATIC HEART DISEASE 1. CARDIAC FAILURE 2. BACTERIAL ENDOCARDITIS 3. EMBOLISM

VALVULAR HEART DISEASES BACTERIAL ENDOCARDITIS:

Figure 12 -27 Diagrammatic comparison of the lesions in the four major forms of vegetative endocarditis. The rheumatic fever phase of RHD (rheumatic heart disease) is marked by a row of small, warty vegetations along the lines of closure of the valve leaflets. IE (infective endocarditis) is characterized by large, irregular masses on the valve cusps that can extend onto the chordae (see Fig. 12 -26). NBTE (nonbacterial thrombotic endocarditis) typically exhibits small, bland vegetations, usually attached at the line of closure. One or many may be present (see Fig. 12 -28). LSE (Libman-Sacks endocarditis) has small or medium-sized vegetations on either or both sides of the valve leaflets. Downloaded from: Robbins & Cotran Pathologic Basis of Disease (on 27 October 2005 04: 48 AM) © 2005 Elsevier

VEGETATIVE ENDOCARDITIS • INFECTIVE ENDOCARDITIS • NON INFECTIVE ENDOCARDITIS NON BACTERIAL THROMBOTIC ENDOCARDITIS ASSOCIATED WITH SYSTEMIC LUPUS ERYTHEMATOSUS

INFECTIVE ENDOCARDITIS (IE) • DEFINITION “ Characterized by colonization or invasion of the heart valves, the mural endocardium or other cardiovascular sites by a microbiologic agent, leading to the formation of bulky, friable vegetations composed of thrombotic debris and organisms, often associated with destruction of the underlying cardiac tissues. ”

INFECTIVE ENDOCARDITIS (IE) (Contd) • TYPES ACUTE INFECTIVE ENDOCARDITIS HIGHLY VIRULENT ORGANISMS, NORMAL HEART SUBACUTE INFECTIVE ENDOCARDITIS LOW VIRULENT ORGANISMS, ABNORMAL HEART

CAUSES AND PATHOGENESIS -IE • PREDISPOSING FACTORS RHEUMATIC HEART DISEASE CONGENITAL HEART DISEASES MYXOMATOUS MITRAL VALVE DEGENERATIVE CALCIFIC VALVULAR STENOSIS BICUSPID AORTIC VALVE PROSTHETIC VALVE VASCULAR GRAFTS

OTHER RISK FACTORS-IE • • • NEUTROPENIA IMMUNODEFICIENCY DIABETES MELLITUS ALCOHOL INTRARENOUS DRUG ABUSE INDWELLING VASCULAR CATHETERS

CAUSATIVE ORGANISMS-IE • STREPTOCOCCUS VIRIDANS (alpha. HAEMOLYTICUS) - 50 to 60 % • STAPHYLOCOCCUS AUREUS -10 to 20 % • STAPHYLOCOCCUS EPIDERMIDUS – PROSTHETIC VALVE • HAEMOPHILUS INFLUNENZAE, ACTINOBACILLUS, CARDIOBACTERIUM, EIKENELLA, KINGELLA - (HACEK) • FUNGI • CHLAMYDIA • RICKETTSIA

SOURCE OF INFECTION-IE • DENTAL/SURGICAL PROCEDURES • CONTAMINATED INJECTIONS • OCCULT SOURCE: GUT, ORAL CAVITY, TRIVIAL INJURIES & INFECTED SITES

CULTURE NEGATIVE ENDOCARDITIS (10%) • PRIOR ANTIBIOTICS THERAPY • IMPROPER TIMINGS – BLOOD SAMPLING • DIFFICULTIES IN ISOLATION • DEEPLY EMBEDDED ORGANISMS

MOROPHOLOGY -IE • ACUTE IE - BULKY, FRIABLE, IRREGULAR VEGETATIONS ON VALVE CUSPS AND CORDS • SUBACUTE IE SMALLER VEGETATIONS

MOROPHOLOGY –IE (Contd) • NONVALVULAR IE VEGETATIONS DOWNSTREAM • WITH PROSTHETIC VALVES - RING ABSCESSES • WITH I/V DRUG ABUSE – RIGHT SIDED VALVE

CLINICAL FEATURES-IE • • FEVER, CHILLS FATIGUE LOSS OF WEIGHT MURMURS(90%) PETECHIAE SUBUNGUAL HAEMORRHAGES ROTH SPOTS-EYES

COMPLICATIONS-IE • CARDIAC COMPLICATIONS v VAVULAR INSUFFICIENCY / STENOSIS v MYOCARDIAL RING ABSCESS v PERFORATION-AORTA & INTERVENTRICULAR SEPTUM v SUPPURATIVE PERICARDITIS v DEHISCENCE WITH PARAVALVULAR LEAKS

EMBOLIC COMPLICATIONS - IE • LEFT SIDED LESIONS CEREBRAL INFARCT, BRAIN ABSCESS, MENINGITIS, MI, SPLENIC ABSCESS & RENAL ABSCESS • RIGHT SIDED LESIONS PULMONARY INFARCT, LUNG ABSCESS AND PNEUMONIA

RENAL COMPLICATIONS -IE • RENAL INFARCT • FOCAL AND DIFFUSE GLOMERULONEPHRITIS • MULTIPLE RENAL ABSCESSES