RHEUMATIC HEART DISEASERHD COMMONEST HEART DISEASE IN YOUNG

RHEUMATIC HEART DISEASE(RHD) COMMONEST HEART DISEASE IN YOUNG POPULATION IN DEVELOPING COUNTRIES RHEUMATIC HEART DISEASE(RHD) IS SEQUELAE TO ACUTE RHEUMATIC FEVER(ARF).

RHEUMATIC FEVER(RF) DEFINITION – IS A SYSTEMIC, POSTSTREPTOCOCCAL, NONSUPPURATIVE INFLAMMATORY DISEASE, PRINCIPALLY AFFECTING THE HEART, JOINTS, CNS, SKIN & SUBCUTANEOUS TISSUE

RHEUMATIC FEVER(RF) RHEUMATIC FEVER LICKS THE JOINTS , BUT BITES THE HEART

RHEUMATIC HEART DISEASE RHD IS NONSUPPURATIVE DISEASE OF THE HEART INVOLVING ALLTHE 3 LAYRERS OF THE HEART WITH DOMINANT LESIONS OF CARDIAC VALVE LEADING TO PROGRESSIVE VALVULAR SCLEROSIS & CHRONIC CONGESTIVE CARDIAC FAILURE.

ACUTE RHEUMATIC FEVER(ARF) 1. PAINFUL SWELLING OF LARGER JOINTS. 2. PANCARDITIS

CLINICAL FEATURES AGE- 5 – 15 YRS POOR SOCIOECONOMIC BACKGROUND OVERCROWDING BREEDS THE DISEASE

CLINICAL FEATURES REPEATED ATTACKS OF SORE THROAT – PHARYNGITIS , TONSILLITIS DUE TO GROUP A HAEMOLYTIC STREPTOCOCCI

CLINICAL FEATURES AFTER A LATENT PERIOD OF 3 WEEKS OR SO FEATURES OF ARF APPEARPAINFUL SWELLING OF THE LARGER JOINTS SHIFTING FROM JOINT TO JOINT- MIGRATORY POLYARTHRITIS.

CLINICAL FEATURES HEART- CARDIAC MURMURS, PERICARDITIS- PERICARDIAL RUB CNS- CHOREA. SKIN- SUBCUTANEOUS PAINFUL NODULES ON THE EXTENSOR SURFACES OF THE LIMBS, ERYTHEMA MARGINATUM

ACUTE RHEUMATIC FEVER(ARF) LESIONS OF CENTRAL NERVOUS SYSTEM -CHOREA.

REVISED JONES’ CRITERIA FOR DIAGNOSIS OF ARF MAJOR CRITERIA 1. CARDITIS 2. POLYARTHRITIS 3. CHOREA(SYDENHAM’S) 4. ERYTHEMA MARGINATUM 5. SUBCUTANEOUS NODULES

JONES’ MINOR CRITERIA 1. FEVER 2. ARTHRALGIA 3. PREVIOUS HISTORY OF RF 4. ELEVATED ESR

JONES’ MINOR CRITERIA 5. RAISED C REACTIVE PROTEIN & LEUKOCYTES 6. ECG FINDINGS OF PROLONGED PR INTERVAL

REVISED JONES’ CRITERIA FOR DIAGNOSIS OF ARF FOR DIAGNOSIS OF RHD 2 MAJOR CRITERIA OR ONE MAJOR CRITERIA AND 2 MINOR CRITERIA ARE NEEDED

NORMAL ECG

SUPPORTIVE EVIDENCE 1. POSITIVE THROAT CULTURE 2. RAISED TITRES OF STREPTOCOCCAL ANTIBODIESANTISTREPTOLYSIN O & S, ANTISTREPTOKINASE, ANTISTREPTOHYALURONIDASE , ANTI DNAase B

ETIOPATHOGENESIS OF RHEUMATIC FEVER & RHEUMATIC CARDITIS THE ORGANISM HAS NUMBER OF PROTEINS & ENZYMES AGAINST WHICH THE BODY PRODUCES ANTIBODIES. 1. M PROTEIN 2. HYALURONIDASE ENZYME

ETIOPATHOGENESIS OF RHEUMATIC FEVER & RHEUMATIC CARDITIS 3. STREPTOLYSINS O & S (HAEMOLYSINS) 4. NADASE IS A MITOCHONDRIAL TOXIN 5. LIPOTECHOEIC ACID AN ADHESION FACTOR

LABORATORY INVESTIGATIONS 1. LEUKOCYTOSIS 2. RAISED ESR. 3. HIGH TITRE OF C REACTIVE PROTEIN

LABORATORY INVESTIGATIONS 4. SPECIFIC TYPES OF ANTIBODIES TO STEPTOCOCCAL ANTIGENS IN THE SERUM.

ETIOPATHOGENESIS ESTABLISHED FACTORS 1. IT IS RARE TO FIND A PATIENT OF ARF WHOSE THROAT DOES NOT HARBOUR THE STREPTOCOCCUS.

ETIOPATHOGENESIS 2. THE STREPTOCOCCI REMAIN RESTRICTED TO THE PHARYNX. NO ORGANISMS ARE FOUND IN THE LESIONS.

PATHOGENESIS 3 VIEWS ARE PREVALENT TO EXPLAIN THE DEVELOPMENT OF THE DISEASE. 1. CROSS REACTING ANTIBODIES- ANTIGENIC MIMICRY

PATHOGENESIS 2. STREPTOCOCCAL ANTIGENS DAMAGE TISSUE , DAMAGED TISSUE ACT AS AUTOANTIGENS. 3. FORMATION OF ANTIGEN COMPLEXES CONTAINING STREPTOCOCCAL COMPONENT & A COLLAGEN OR CARDIAC ANTIGEN.

PATHOLOGY OF RHD 1. HEART- PANCARDITIS THE ASCHOFF GRANULOMA (ASCHOFF BODY)-ONE TO TWO mm SIZE FOUND ANYWHERE MAINLY ENDOCARDIUM & MYOCARDIUM OF

PATHOLOGY OF RHD POSTERIOR WALL OF THE LEFT VENTRICLE & THE ENDOCARDIUM OF LEFT ATRIUM AT THE SITE OF Mc. CALLUM PATCH , THE ADVENTITIA OF SMALL VESSELS IN THE MYOCARDIUM

3 STAGES OF EVOLUTION OF ASCHOFF BODY 1. EXUDATIVE STAGE- CENTRAL NECROSIS WITH LYMPHOCYTIC , PLASMACYTIC REACTION & OEDEMA 2. PROLIFERATIVE OR GRANULOMATOUS STAGE- MONONUCLEAR HISTIOCYTES, MULTINUCLEAR ASCHOFF GIANT CELLS IN ADDITION TO LYMPHOCYTES & PLASMA CELLS and FIBRINOID

ASCHOFF GRANULOMA THE CELLS OF THE ASCHOFF BODY A. MACROPHAGES – FROM BLOOD MONOCYTES & CARDIAC HISTIOCYTES OR MYOCYTE (ANITSCHKOW’S HISTIOCYTES OR MYOCYTE- CATERPILLAR CELL ON LONGITUDINAL SECTION- OWL EYED CELL ON CROSS SECTION)

ASCHOFF GRANULOMA (ASCHOFF BODY)

ANITSCHKOW’S HISTIOCYTES

ASCHOFF GRANULOMA B. ASCHOFF GIANT CELLMULTINUCLEATED GIANT CELLS ARE DERIVED FROM ANITSCHKOW’S CELL OR CARDIAC MUSCLE CELL

ASCHOFF GRANULOMA 3. FIBROTIC STAGE- GRANULOMA IS GRADUALLY REPLACED BY FIBROUS SCAR TISSUE.

ASCHOFF BODY-PROLIFERATIVE OR GRANULOMATOUS STAGE

ASCHOFF GRANULOMA

ANITSCHKOW’S HISTIOCYTES-

RHEUMATIC PANCARDITIS 1. RHEUMATIC ENDOCARDITIS VALVULAR ENDOCARDITIS MURAL ENDOCARDITIS

RHEUMATIC VALVULAR ENDOCARDITIS THICKENING, LOSS OF TRANSLUCENCY OF LEAFLETS OR CUSPS, FOLLOWED BY FORMATION OF VEGETATIONSSMALL 1 -3 mm

RHEUMATIC VALVULAR ENDOCARDITIS MULTIPLE WARTY VEGETATIONS OR VERRUCAE ALONG THE LINE OF CLOSURE OF THE LEAFLETS OR CUSPS ( FREE MARGINS OF THE CUSPS OR LEAFLETS). GREY BROWN (TAWNEY, FIRMLY ATTACHED TO THE CUSPS) - SO EMBOLISM IS RARE

VALVULITIS IN RHEUMATIC HEART DISEASE CONSTANT FRICTION BETWEEN THE OEDEMATOUS VALVE LEAFLETSDENUDATION OF MARGINS OF CONTACT – PLATELETS & FIBRIN DEPOSITS- RESPONSIBLE FORMATION OF VEGETATIONS

RHEUMATIC VALVULITIS

RHEUMATIC VALVULAR ENDOCARDITIS VALVE INVOLVEMENT IN THE DECREASING ORDER OF FREQUENCYMITRAL, COMBINED MITRAL & AORTIC TRICUSPID & PULMONARY ARE INFREQUENTLY INVOLVED.

RHEUMATIC VALVULAR ENDOCARDITIS GREATER STRESS IS RESPONSIBLE FOR HIGHER INVOLVEMENT ON LEFT SIDE VALVES

RHEUMATIC VALVULAR ENDOCARDITIS VEGETATIONS ARE SEEN ON THE ATRIAL SURFACE OF ATRIOVENTRICULAR VALVE, ON VENTRICULAR SURFACE OF THE SEMILUNAR VALVES(AORTIC & PULMONARY) MECHANICAL PRESSURE ON THESE SURFACES IS RESPONSIBLE FOR THE PATHOGENESIS OF VEGETATIONS.

MITRAL VALVE -2 LEAFLETS

VEGETATIONS IN RHEUMATIC HEART DISEASE

RHD - MITRAL VALVULITIS VERRUCAE BODIES

RHEUMATIC VALVULITIS CHRONIC STAGE PERMANENT DEFORMITY OF ONE OR MORE VALVES MITRAL ALONE – 37% OF CASES MITRAL + AORTIC – 27% OF CASES MITRAL, AORTIC& TRICUSPID – 22% OF CASES AORTIC ALONE – 2% OF CASES ALL 4 VALVES- LESS THAN 1% OF CASES

MICROSCOPY OF RHEUMATIC VALVULAR ENDOCARDITIS EARLY STAGE – CONGESTION , OEDEMA OF LEAFLETS & INFILTRATION WITH LYMPHOCYTES , PLASMA CELLS, HISTIOCYTES WITH MANY ANITSCHKOW CELLS WITH FEW POLYMORPHS

MICROSCOPY OF RHEUMATIC VALVULAR ENDOCARDITIS DIAGNOSTIC LESION UNDER MICROSCOPY IS ASCHOFF GRANULOMAS. VEGETATIONS PRESENT ON THE FREE MARGINS OF CUSPS CONSIST OF FIBRIN & SUPERIMPOSED PLATELET THROMBI

MICROSCOPY OF RHEUMATIC VALVULAR ENDOCARDITIS HEALED CHRONIC STAGE VEGETATIONS UNDERGO ORGANISATION – RESULT IN DIFFUSE VALVULAR THICKENING DUE TO FIBROSIS, HYALINISATION & CALCIFICATION WITH THICK WALLED BLOOD VESSELS VALVE ORIFICE LOOKS LIKE A BUTTON HOLE OR FISH MOUTH DUE TO FIBROUS ADHESSION OF CUSP LEAFLETS

RHD - MITRAL STENOSIS

RHEUMATIC MURAL ENDOCARDITIS LESS CONSPICUOUS THAN VALVULAR ENDOCARDITIS -COMMONLY SEEN AS Mac. Callum’S PATCH – A MAP LIKE AREA OF THICKENNED ROUGHENED WRINKELED PART OF ENDOCADIUM ON THE POSTERIOR WALL OF LEFT ATRIUM ABOVE THE POSTERIOR LEAFLET OF MITRAL VALVE.

Mac. Callum’S PATCH IN THE POSTERIOR WALL OF LEFT ATRIUM

RHEUMATIC MURAL ENDOCARDITIS MICROSCOPICALLY Mac. Callum’S PATCH SHOWS FIBRINOID CHANGE IN COLLAGEN, LYMPHOCYTES, PLASMA CELLS & MACROPHAGES WITH MANY ANITSCHKOW’S HISTIOCYTES TYPICAL ASCHOFF BODIES ARE SEEN

RHEUMATIC VALVULITIS CHRONIC STAGE VALVULAR DEFORMITY IS DUE TO DIFFUSE FIBROCOLLAGENOUS THICKENING & CALCIFICATION OF VALVE CUSPS OR LEAFLETS WHICH CAUSE ADHESIONS BETWEEN LATERAL PORTIONS IN THE REGION OF THE COMMISSURES.

RHEUMATIC VALVULITIS CHRONIC STAGE THICKENING , SHORTENING & FUSION OF THE CHORDAE TENDINEAE FURTHER CONTRIBUTE TO THE DEFORMITY

2. RHEUMATIC MYOCARDITIS EARLY STAGE MYOCARDIUM OF MAINLY LEFT VENTRICAL BECOMES SOFT, FLABBY IN THE INTERMEDIATE STAGE SMALL FOCI OF NECROSIS APPEAR

RHEUMATIC MYOCARDITIS LATER PALE FOCI OF ASCHOFF BODIES ARE SEEN THROUGHOUT THE MYOCARDIUM OF INTERVENTRICULAR SEPTUM, LEFT VENTRICAL & LEFT ATRIUM IN THE VICINITY OF BLOOD VESSELS.

RHEUMATIC MYOCARDITIS THIS LEADS TO DERANGEMENT OF CONDUCTION SYSTEM MICROSCOPICALLY –ASCHOFF BODIES IN DIFFERENT STAGES ARE SEEN, LATER REPLACED BY SMALL FOCI OF FIBROUS SCAR

ACUTE SEROFIBRINOUS PERICARDITIS WITH EFFUSION SEROFIBRINOUS EXUDATE FIBRIN RICH GRANULAR MATERIAL WITH LEUKOCYTES- BREAD & BUTTER PERICARDITIS. ASCHOFF BODIES ARE RARE – HEALED STAGE - CHRONIC ADHESIVE OR CONSTRICTIVE PERICARDITIS.

EXTRACARDIAC LESIONS IN ACUTE STAGE LESIONS DEVELOP IN CONNECTIVE TISSUE ELSEWHERE IN THE BODY CHIEFLY THE JOINTS, SUBCUTANEOUS TISSUE, ARTERIES BRAIN & LUNGS

POLYARTHRITIS SEROUS EFFUSION WITH HYPERAEMIA, OEDEMA, FIBRINOID CHANGE & INFLAMMATORY CELL INFILTRATION LESIONS MAY RESEMBLE ASCHOFF BODIES

RHEUMATIC SUBCUTANEOUS NODULES MORE OFTEN IN CHILDREN THAN IN ADULTS 0. 5 TO 2 cm , OVOID, PAINLESS ATTACHED TO DEEPER STRUCTURES LIKE TENDONS, LIGAMENTS, FASCIA OR PERIOSTEUM

RHEUMATIC SUBCUTANEOUS NODULE

RHEUMATIC SUBCUTANEOUS NODULES LOCATED ON EXTENSOR SURFACES OF WRISTS, ELBOWS, ANKLES & KNEES MICROSCOPIC FEATURES – RESEMBLE GIANT ASCHOFF BODIES

ERYTHEMA MARGINATUM NONPRURITIC ERYTHEMATOUS RASH MAINLY IN THE TRUNK & PROXIMAL PARTS OF EXTREMITIES TRANSIENT & MIGRATORY

ERYTHEMA MARGINATUM

RHEUMATIC ARTERITIS ANY ARTERIES –INCLUDING CORONARY, AORTA, ARTERIES OF ANY ORGAN HISTOLOGICALLY – HYPERSENSITIVITY ANGITIS

RHEUMATIC FEVER HYPERSENSITIVITY ANGITIS

CHOREA MINOR SYDENHAM’S CHOREA OR SAINT VITUS DANCE DANCING MANIA- VICTIMS OF DANCING MANIA OFTEN ENDED THEIR PROCESSIONS AT PLACES DEDICATED TO THAT SAINT DISORDERED & INVOLUNTARY JERKY MOVEMENTS OF THE TRUNK & EXTREMITIES

CHOREA MINOR OCCURS IN YOUNG GIRLS CEREBRAL HEMISPHERES, BASAL GANGLIA, BRAINSTEM ARE INVOLVED SMALL HAEMORRHAGES, OEDEMA & PERIVASCULAR INFILTRATE OF LYMPHOCYTES

RHEUMATIC PNEUMONITIS & PLEURITIS LUNGS – LARGE, FIRM & RUBBERY. HAEMORRHAGES & FIBRINOUS EXUDATE IN THE ALVEOLI ASCHOFF BODIES ARE NOT SEEN

RHEUMATIC PLEURITIS SEROFIBRINOUS PLEURAL EFFUSION ASCHOFF BODIES ARE NOT SEEN

FATE OF ARF 1. COMPLETE RESOLUTION IN MAJORITY OF CASES 2. RECOVERY FOLLOWED BY REPEAT ATTACK. 3. NO RECOVERY BUT PROGRESSIVE DISEASE. 4. RARE CASES DIE IN ACUTE STAGE OF ARRHYTHMIA

CHRONIC RHEUMATIC HEART DISEASE 3% OF CASES. CHRONIC MYOCARDITISGRANULOMAS , HEAL WITH VARIABLE AMOUNT OF FIBROSIS

CAUSES OF DEATH IN RHD 1. CARDIAC FAILURE – CHRONIC VALVULAR DEFORMITIES 2. SUPERIMPOSED BACTERIAL ENDOCARDITIS

CAUSES OF DEATH IN RHD 3. EMBOLISM FROM MURAL THROMBI IN THE LEFT ATRIUM AND IT’S APPENDAGES TO BRAIN , KIDNEY, SPLEEN & LUNGS 4. SUDDEN DEATH DUE TO BALL THROMBUS IN THE LEFT ATRIUM OR DUE TO ACUTE CORONARY INSUFFICIENCY DUE TO AORTIC STENOSIS.