1. Faculty of Medicine Universitas Brawijaya Faculty of Medicine Universitas Brawijaya

2. 2 Objectives EtiologyEpidemiologyPathogenesis Pathologic lesions Clinical manifestations & Laboratory findings Diagnosis & Differential diagnosis Treatment & Prevention PrognosisReferences

3. 3 Etiology Acute rheumatic fever is a systemic disease of childhood,often recurrent that follows group A beta hemolytic streptococcal infection It is a delayed non-suppurative sequelae to URTI with GABH streptococci. It is a diffuse inflammatory disease of connective tissue,primarily involving heart,blood vessels,joints, subcut.tissue and CNS

4. 4 Epidemiology Ages 5-15 yrs are most susceptible Rare <3 yrs Girls>boys Common in 3rd world countries Environmental factors-- over crowding, poor sanitation, poverty, Incidence more during fall,winter & early spring

5. 5 Pathogenesis Delayed immune response to infection with group.A beta hemolytic streptococci. After a latent period of 1-3 weeks, antibody induced immunological damage occur to heart valves,joints, subcutaneous tissue & basal ganglia of brain

6. 6 Strains that produces rheumatic fever - M types l, 3, 5, 6,18 & 24 Pharyngitis- produced by GABHS can lead to- acute rheumatic fever, rheumatic heart disease & post strept. Glomerulonepritis Skin infection- produced by GABHS leads to post streptococcal glomerulo nephritis only. It will not result in Rh.Fever or carditis as skin lipid cholesterol inhibit antigenicity Group A Beta Hemolytic Streptococcus

7. 7 Diagrammatic structure of the group A beta hemolytic streptococcus Capsule Cell wall Protein antigens Group carbohydrate Peptidoglycan Cyto.membrane Cytoplasm …………………………………………… ……... Antigen of outer protein cell wall of GABHS induces antibody response in victim which result in autoimmune damage to heart valves, sub cutaneous tissue,tendons, joints & basal ganglia of brain

8. 8 Pathologic Lesions Fibrinoid degeneration of connective tissue,inflammatory edema, inflammatory cell infiltration & proliferation of specific cells resulting in formation of A shcoff nodules, resulting in- -Pancarditis in the heart -Arthritis in the joints -Ashcoff nodules in the subcutaneous tissue -Basal gangliar lesions resulting in chorea

9. 9 Rheumatic Carditis Histology (40X)

10. 10 Histology of Myocardium in Rheumatic Carditis ( 200X)

11. 11 Clinical Features Flitting & fleeting migratory polyarthritis, involving major joints Commonly involved joints- knee,ankle,elbow & wrist Occur in 80%,involved joints are exquisitely tender In children below 5 yrs arthritis usually mild but carditis more prominent Arthritis do not progress to chronic disease 1.Arthritis

12. 12 Clinical Features (Contd) Manifest as pancarditis (endocarditis, myocarditis and pericarditis),occur in 40- 50% of cases Carditis is the only manifestation of rheumatic fever that leaves a sequelae & permanent damage to the organ Valvulitis occur in acute phase Chronic phase- fibrosis,calcification & stenosis of heart valves(fishmouth valves) 2.Carditis

13. 13 Rheumatic heart disease. Abnormal mitral valve. Thick, fused chordae

14. 14 Another view of thick and fused mitral valves in Rheumatic heart disease

15. 15 Clinical Features (Contd) Occur in 5-10% of cases Mainly in girls of 1-15 yrs age May appear even 6/12 after the attack of rheumatic fever Clinically manifest as-clumsiness, deterioration of handwriting,emotional lability or grimacing of face Clinical signs- pronator sign, jack in the box sign, milking sign of hands 3.Sydenham Chorea

16. 16 Clinical Features (Contd) Occur in <5%. Unique,transient,serpiginous-looking lesions of 1-2 inches in size Pale center with red irregular margin More on trunks & limbs & non-itchy Worsens with application of heat Often associated with chronic carditis 4.Erythema Marginatum

17. 17 Clinical Features (Contd) Occur in 10% Painless,pea-sized,palpable nodules Mainly over extensor surfaces of joints,spine,scapulae & scalp Associated with strong seropositivity Always associated with severe carditis 5.Subcutaneous nodules

18. 18 Clinical Features (Contd) Other features (Minor features) Fever-(up to 101 degree F) ArthralgiaPallorAnorexia Loss of weight

19. 19 Laboratory Findings High ESR Anemia, leucocytosis Elevated C-reactive protien ASO titre >200 Todd units. (Peak value attained at 3 weeks,then comes down to normal by 6 weeks) Anti-DNAse B test Anti-DNAse B test Throat culture-GABHstreptococci Throat culture-GABHstreptococci

20. 20 Laboratory Findings (Contd) ECG- prolonged PR interval, 2nd or 3rd degree blocks,ST depression, T inversion 2D Echo cardiography- valve edema,mitral regurgitation, LA & LV dilatation,pericardial effusion,decreased contractility

21. 21 Diagnosis Rheumatic fever is mainly a clinical diagnosis No single diagnostic sign or specific laboratory test available for diagnosis Diagnosis based on MODIFIED JONES CRITERIA

22. 22 Recommendations of the American Heart Association

23. 23 Exceptions to Jones Criteria  Chorea alone, if other causes have been excluded  Insidious or late-onset carditis with no other explanation  Patients with documented RHD or prior rheumatic fever,one major criterion,or of fever,arthralgia or high CRP suggests recurrence

24. 24 Differential Diagnosis Juvenile rheumatiod arthritis Septic arthritis Sickle-cell arthropathy Kawasaki disease Myocarditis Scarlet fever Leukemia

25. 25 Treatment Step I - primary prevention (eradication of streptococci) Step II - anti inflammatory treatment (aspirin,steroids) Step III- supportive management & management of complications Step IV- secondary prevention (prevention of recurrent attacks)

26. 05/05/1999 Dr.Said Alavi 26 STEP I: Primary Prevention of Rheumatic Fever (Treatment of Streptococcal Tonsillopharyngitis) Agent Dose Mode Duration Benzathine penicillin G600 000 U for patients Intramuscular Once 27 kg (60 lb) 1 200 000 U for patients >27 kg or Penicillin VChildren: 250 mg 2-3 times daily Oral 10 d (phenoxymethyl penicillin) Adolescents and adults: 500 mg 2-3 times daily For individuals allergic to penicillin Erythromycin: 20-40 mg/kg/d 2-4 times daily Oral 10 d Estolate (maximum 1 g/d) or Ethylsuccinate40 mg/kg/d 2-4 times daily Oral 10 d (maximum 1 g/d) Recommendations of American Heart Association

27. 27 Step II: Anti inflammatory treatment Clinical condition Drugs

28. 28 Bed rest Treatment of congestive cardiac failure: -digitalis,diuretics Treatment of chorea: -diazepam or haloperidol Rest to joints & supportive splinting 3.Step III: Supportive management & management of complications

29. Dr.Said Alavi 29 STEP IV : Secondary Prevention of Rheumatic Fever (Prevention of Recurrent Attacks) Agent DoseMode Benzathine penicillin G1 200 000 U every 4 weeks*Intramuscular or Penicillin V250 mg twice daily Oral or Sulfadiazine 0.5 g once daily for patients 27 kg (60 lb Oral 1.0 g once daily for patients >27 kg (60 lb) For individuals allergic to penicillin and sulfadiazine Erythromycin250 mg twice daily Oral *In high-risk situations, administration every 3 weeks is justified and recommended Recommendations of American Heart Association

30. 30 Duration of Secondary Rheumatic Fever Prophylaxis CategoryDuration Rheumatic fever with carditis and At least 10 y since last residual heart disease episode and at least until (persistent valvar disease*) age 40 y, sometimes lifelong prophylaxis Rheumatic fever with carditis10 y or well into adulthood, but no residual heart disease whichever is longer (no valvar disease*) Rheumatic fever without carditis5 y or until age 21 y, whichever is longer *Clinical or echocardiographic evidence. Recommendations of American Heart Association

31. 31 Prognosis Rheumatic fever can recur whenever the individual experience new GABH streptococcal infection,if not on prophylactic medicines Good prognosis for older age group & if no carditis during the initial attack Bad prognosis for younger children & those with carditis with valvar lesions

32. 32

33. VALVULAR HEART DISEASE MITRAL STENOSIS G

34. ETIOLOGY RHEUMATIC VALVULAR DISEASE MOST COMMON CAUSE OF M ITRAL STENOSIS –Pure mitral stenosis 25% –Pure mitral regurgitation 35% –Combined MS and MR 40% 15 TO 20 YEAR LATENCY PERIOD

35. ETIOLOGY OTHER CAUSES CONGENITAL MALIGNANT CARCINOID SLE OR RHEUMATOID ARTHRITIS AMYLOID METHYLSERGIDE THERAPY

36. PATHOLOGY SYMPTOMATIC MITRAL STENOSIS THICKENED MITRAL CUSPS –+/- CALCIFIC DEPOSITS FUSION OF VALVE COMMISSURES SHORTENING OF CHORDAE WITH FUSION “FISH MOUTH” OR FUNNEL ORIFICE

37. HISTORY PRINCIPLE SYMPTOM IS DYSPNEA –Reduces compliance of the lung PULMONARY EDEMA –Effort, emotional stress, infection, fever, pregnancy ATRIAL FIBRILLATION –Increased rate causes increased LA to LV gradient

38. HISTORY CHEST PAIN –15% DUE TO RV HTN, EMBOLIZATION THROMBOEMBOLISM –20% HISTORICALLY INVOLVED –CORRELATES INVERSELY WITH CARDIAC OUTPUT –CORRELATES DIRECTLY WITH LA SIZE AND AGE OF PATIENT

39. PHYSICAL EXAMINATION ARTERIAL PULSE NORMAL OR DIMINISHED JUGULAR PRESSURE PROMINENT a WAVE PALPATION –INCONSPICUOUS LV, RV HEAVE IN PULMONARY HTN

40. PHYSICAL EXAMINATION AUSCULTATION ACCENTUATED S1 –PROLONGED Q-S1 INTERVAL OPENING SNAP –SUDDEN TENSING OF VALVE LEAFLETS –A2-OS INTERVAL SHORTENS WITH SEVERITY DIASTOLIC MURMUR

41. PATHOPHYSIOLOGY NORMAL VALVE AREA 4 TO 6cm2 NORMAL MEAN LA TO LV PRESSURE GRADIENT 2 TO 4mmHg MILD MITRAL STENOSIS 2cm2 CRITICAL MITRAL STENOSIS 1cm2 or less –20MMhg GRADIENT REQUIRED FOR FLOW

42. MANAGEMENT NATURAL HISTORY 20 TO 25 YEAR ASYMPTOMATIC PERIOD 5 YEARS FOR PROGRESSION CLASS II-IV SURVIVAL –CLASS III 62% 5 YR SURVIVAL –CLASS IV 15% 5 YR SURVIVAL ASYMPTOMATIC CLASS 1 40% WORSENED OR DIED IN 10 YEARS

43. MANAGEMENT MEDICAL TREATMENT RHD PCN AND SBE PROPHYLAXIS SYMPTOMATIC PATIENTS –ORAL DIURETICS AND ACTIVITY RESTRICTION –BETA BLOCKERS AND LOW HEART RATE –DIGOXIN IN AF AND WITH PULM HTN ANTICOAGULATION FOR LA SIZE >5.5cm, EMBOLISM OR ATRIAL FIBRILLATION

44. MANAGEMENT SURGICAL TREATMENT OPERATE FOR SEVERE SYMPTOMS –CLASS III OR GREATER (SYMPTOMS WITH LESS THAN USUAL ACTIVITY) –PULMONARY HTN DEMANDS OPERATION ROUTINE CATHETERIZATION MEN>45 MILDY SYMPTOMATIC PATIENTS –CONSIDER SIZE OF MV ORIFICE, LIFESTYLE AND HISTORY OF COMPLICATIONS

45. MANAGEMENT BALLOON VALVULOPLASTY PROCEDURE OF CHOICE IN RIGHT PT –TRANSESOPHAGEAL ECHO HELPFUL IN SORTING OUT WHICH PATIENT –ECHO SCALE OF PREDICTORS RELATES TO THICKENING AND CALCIFICATION OF VALVE RESULTS COMPARABLE TO SURGERY MORTALITY 2-3%, MORBIDITY 8-12%

46. VALVULAR HEART DISEASE MITRAL INSUFFICIENCTY

47. ETIOLOGY ACUTE VS CHRONIC INFLAMMATORYDEGENERATIVEINFECTIVESTRUCTURALCONGENITAL

48. ETIOLOGY DEGENERATIVE MYXOMATOUS DEGENERATION OF LEAFLETS –MITRAL VALVE PROLAPSE –MOST COMMON CAUSE OF ACUTE MR IN US ADULTS MARFAN SYNDROME CALCIFICATION OF MV ANNULUS

49. ETIOLOGY INFLAMMATORY RHEUMATIC HEART DISEASE –ACUTE RHEUMATIC FEVER VS CHRONIC SYSTEMIC LUPUS ERYTHEMATOSUS SCLERODERMA

50. ETIOLOGY STRUCTURAL RUPTURED CHORDAE TENDINAE RUPTURE OR DYSFUNCTION OF PAPILLARY MUSCLES DILATATION OF MITRAL VALVE ANNULUS PARAVALVULAR PROSTHETIC LEAK

51. ANATOMY OF MITRAL VALVE VALVE LEAFLETS –ANTERIOR AND POSTERIOR MITRAL ANNULUS –DILATATION CHORDAE TENDINAE PAPILLARY MUSCLES

52. PATHOPHYSIOLOGY VOLUME OVERLOAD IMPEDENCE TO VENTRICULAR EMPTYING IS REDUCED –LV DECOMPRESSES INTO LA VOLUME OF REGURGITANT FLOW –DEPENDENT ON SIZE OF REGURGITANT ORIFICE AND LV TO LA PRESSURE GRADIENT

53. PATHOPHYSIOLOGY HEMODYNAMICS FORWARD CARDIAC OUTPUT USUALLY DEPRESSED –TOTAL LV OUTPUT (FORWARD AND BACKWARD) INCREASED NORMAL LA COMPLIANCE (ACUTE MR) –LITTLE ENLARGEMENT OF LA, HIGH LA PRESSURE LOW LA COMPLIANCE (CHRONIC MR) –ENLARGED LA, MINIMALLY INCREASED LA PRESSURE

54. CLINICAL MANIFESTATIONS NATURAL HISTORY VARIABLE AND DEPENDS ON MR VOLUME MILD MR STABLE IN MAJORITY FOR YEARS –MINORITY DEVELOP SEVERE MR MORE RAPIDLY WITH DEGENERATIVE DISEASE THAN RHEUMATIC

55. CLINICAL MANIFESTATIONS SYMPTOMS USUALLY NOT UNTIL LV STARTS TO FAIL LONGER TIME INTERVAL IN MR THAN MITRAL STENOSIS RIGHT HEART FAILURE IN END STAGE MR

56. PHYSICAL EXAMINATION CAROTID UPSTROKE SHARP, RAPID FALLOFF S1 USUALLY SOFT, WIDELY SPLIT S2 HOLOSYSTOLIC MURMUR –APEX TO AXILLA SYSTOLIC EJECTION MURMUR –ISCHEMIC MR

57. PHYSICAL EXAMINATION MITRAL VALVE PROLAPSE –MID TO LATE SYSTOLIC EJECTION MURMUR –MID SYSTOLIC NON-EJECTION CLICK –VALSALVA PROLONGS MURMUR AND BRINGS IT TO START CLOSER TO S1

58. LABORATORY EXAMINATION CHEST XRAY –CARDIOMEGALY (ECCENTRIC HYPERTROPHY) –LEFT ATRIAL ENLARGEMENT REGURGITANT VOLUME –MILD 25%, MODERATE 40%, SEVERE 75%

59. ECHOCARDIOGRAPHY GOOD ANATOMICAL DETAIL LA SIZE, THROMBUS, LV FUNCTION UNDERLYING ETIOLOGY OF MR INFECTIVE ENDOCARDITIS DOPPLER –SEVERITY OF MR, SIZE OF MR JET

60. MANAGEMENT MEDICAL MANAGEMENT AFTERLOAD REDUCTION –REDUCES IMPEDENCE TO EJECTION IN AORTA –ACE INHIBITORS AND HYDRALAZINE ACUTE MR –IV NITROPRUSSIDE CAN BE LIFESAVING DIGOXIN, DIURETICS IN CHRONIC MR FOLLOW LV SIZE AND FUNCTION

61. SURGICAL TREATMENT OPERATE FOR SYMPTOMS ENLARGING LEFT VENTRICULAR SYSTOLIC DIMENSION (>5.5CM), EJECTION FRACTION 5.5CM), EJECTION FRACTION <55% ARE PREDICTORS OF BAD OUTCOME OPERATIVE MORTALITY 2 TO 7% IN CLASS II TO III PATIENTS RECONSTRUCTION IS BETTER THAN REPLACEMENT IF POSSIBLE

62. VALVULAR HEART DISEASE AORTIC STENOSIS

63. ETIOLOGY OBSTRUCTION TO LV OUTFLOW HYPERTROPHIC CARDIOMYOPATHY SUPRAVALVULARSUBVALVULARCONGENITALACQUIRED

64. ETIOLOGY CONGENITAL AORTIC STENOSIS UNICUSPID –SEVERE AND DEADLY IN INFANCY BICUSPID –MANIFESTED LATER IN LIFE –MOST COMMON CONGENITAL CARDIAC ANOMALY IN LIVE BIRTHS (1%) TRICUSPID –CUSPS OF UNEQUAL SIZE

65. ETIOLOGY ACQUIRED AORTIC STENOSIS RHEUMATIC HEART DISEASE DEGENERATIVEATHEROSCLEROTIC CALCIFIC DUE TO PAGET’S DISEASE RHEUMATOID

66. ETIOLOGY DEGENERATIVE CALCIFIC AORTIC STENOSIS PRIMARY CAUSE OF ADULT AORTIC STENOSIS YEARS OF MECHANICAL STRESS DEPOSITION OF CALCIUM AT CUPAL BASE PRESERVED COMMISSURES RISK FACTORS –DIABETES AND HYPERLIPIDEMIA

67. ETIOLOGY RHEUMATIC AORTIC STENOSIS FUSION OF COMMISSURES AND CUSPS RETRACTION OF CUSPAL BORDERS REDUCE ORIFICE TO TRIANGULAR OPENING ASSOCIATED WITH AORTIC INSUFFICENCY MITRAL DISEASE COMMON ISOLATED AORTIC STENOSIS RARE

68. HISTORY ANGINA –MEDIAN SURVIVAL 5 YEARS SYNCOPE –MEDIAN SURVIVAL 3 YEARS CONGESTIVE HEART FAILURE –MEDIAN SURVIVAL 2 YEARS

69. PHYSICAL EXAMINATION PULSUS PARVUS AND TARDUS –IN CAROTID PULSE REDUCED PULSE PRESSURE SUSTAINED CARDIAC IMPULSE DELAYED A2 OR DIMINISHED HARSH SYSTOLIC EJECTION MURMUR

70. PATHOPHYSIOLOGY GRADUAL DEVELOPMENT OF OBSTRUCTION TO LV OUTFLOW LV OUTPUT MAINTAINED BY LV HYPERTROPHY LV HYPERTROPHY MAY SUSTAIN A LARGE PRESSURE GRADIENT FROM THE LV TO AORTA OVER YEARS ATRIAL CONTRACTION IMPORTANT –ATRIAL FIBRILLATION MAY CAUSE ABRUPT AND SEVERE SYMPTOMS

71. PATHOPHYSIOLOGY INCREASE IN AFTERLOAD INCREASED LV WALL STRESS COMPENSATED BY THE INCREASED LV HYPERTROPHY ULTIMATELY LOSS IN CONTRACTILITY OF LV MASS AND DEVELOPMENT OF HEART FAILURE

72. LABORATORY EKG –LEFT VENTRICULAR HYPERTROPHY IS PROMINENT FINDING CHEST XRAY –MAY BE ENTIRELY NORMAL BECAUSE THE HYPERTROPHY OF LV IS CONCENTRIC (CENTRAL) NOT ECCENTRIC LIKE MR OR AORTIC INSUFFICIENCY –CALCIFICATION OF AORTIC VALVE MAY BE SEEN

73. ECHOCARDIOGRAPHY CALCIFIED VALVE WITH THICKENED LEAFLETS OR COMMISSURES DECREASED OPENING OF AORTIC VALVE SEEN LEFT VENTRICULAR HYPERTROPHY DOPPLER –VALVE PRESSURE GRADIENT CALCULATED –VALVE AREA FROM THIS MEASUREMENT

74. MEDICAL HISTORY EDUCATION IN SYMPTOMS AND REPORTING OPERATE FOR SYMPTOMS WHEN VALVE IS SEVERLY NARROWED –<1CM2 IN AREA DO NOT OPERATE ON SEVERE NARROWING IF ASYMPTOMATIC ENDOCARDITIS PROPHYLAXIS

75. SURGICAL MANAGEMENT RESULTS 5 YEAR ACTUARIAL SURVIVAL 85% REDUCTION IN LV MASS IF PATIENTS HAVE CONGESTIVE HEART FAILURE THEN VALVE REPLACEMENT HAS 10-25% MORTALITY NORMAL 3-5% MORTALITY IN OR PORCINE VALVE FOR AGE > 70

76. SURGICAL MANAGEMENT ASYMPTOMATIC PATIENTS –MORTALITY WITHOUT OPERATION IS <5% PER YEAR –FOLLOW EVERY 6 MONTHS IN OFFICE –COUNSEL ON DEVELOPMENT OF SYMPTOMS OF ANGINA, CHF, SYNCOPE

77. AORTIC STENOSIS IN THE ELDERLY OPERATIVE MORTALITY IN THE ELDERLY –1.8% AGE < 50 –5.1% AGE 50 - 60 –7.1% AGE 60 – 70 ISOLATED AV REPLACEMENT IN PTS AGE 80 TO 89 –94% HAD GOOD RESULTS –APPROPRIATE SELECTION

78. AORTIC INSUFFICIENCY VALVULAR HEART DISEASE

79. ETIOLOGY ¾ OF PATIENTS WITH PURE AI ARE MALES 2/3 OF PATIENTS FROM RHEUMATIC FEVER –THICKENING AND DEFORMATION OF INDIVIDUAL VALVE CUSPS INFECTIVE ENDOCARDITIS –VARIOUS PREVIOUSLY DAMAGING ETIOLOGIES

80. ETIOLOGY PROLAPSE OF AN AORTIC CUSP CONGENITAL FENESTRATIONS OF CUSP TRAUMATIC RUPTURE ASCENDING THORACIC AORTA DISSECTION MARKED AORTIC ROOT DILATATION SYPHILIS, ANKYLOSING SPONDYLITIS

81. PATHOPHYSIOLOGY MARKED INCREASE IN STROKE VOLUME OF LEFT VENTRICLE –EXTRA BLOOD FROM LEAKING BACK INTO LV TO EJECT CONTRAST TO MITRAL INSUFFICIENCY –AI: EJECTING BLOOD INTO HIGH AFTERLOAD (AORTA) –MI: EJECTING BLOOD INTO LOW AFTERLOAD (LEFT ATRIUM)

82. PATHOPHYSIOLOGY DILATATION OF LEFT VENTRICLE –TO MAINTAIN ADEQUATE FORWARD CARDIAC OUTPUT –COR BOVINUM REVERSE PRESSURE GRADIENT FROM AORTA TO LV IN DIASTOLE CAUSES BACK FLOW ACUTE VS CHRONIC INSUFFICIENCY

83. HISTORY FAMILY HISTORY WITH MARFAN SYNDROME INFECTIVE ENDOCARDITIS SYPHYLIS AWARENESS OF HEARTBEAT ORTHOPNEA, DOE LATE OR IN ACUTE ANGINAEDEMA

84. PHYSICAL FINDINGS INSPECTION –BOBBING HEAD OR JARRING OF BODY PALPATION –ARTERIAL JACK HAMMER PULSE –CAPILLARY PULSATIONS –VARIOUS SIGNS –WIDENED PULSE PRESSURE

85. PHYSICAL FINDINGS MURMURS –DIASTOLIC HIGH PITCHED BLOW –LOUD SYSTOLIC AORTIC EJECTION FLOW MURMUR –DIASTOLIC RUMBLE AUSTIN FLINT MURMUR MISTAKEN FOR MITRAL STENOSIS

86. LABORATORY EKG –LEFT VENTRICULAR HYPERTROPHY WITH STRAIN –ECHOCARDIOGRAM FLOW INTO LV FROM AORTIC VALVE LV SIZE FLUTTERING OF MITRAL LEAFLET –BLOOD CULTURES IN ENDOCARDITIS

87. TREATMENT CONGESTIVE HEART FAILURE TREATMENT –DIGOXIN, DIURETICS, AFTERLOAD REDUCTION IV NITROPRUSSIDE MAY BE LIFESAVING

88. TREATMENT SURGERY –SYMPTOMATIC PATIENTS SHOULD BE OPERATED UPON –ASYMPTOMATIC PATIENTS FOLLOWED FOR LEFT VENTRICULAR ENLARGEMENT AND SYSTOLIC DIMENSIONS ON ECHOCARDIOGRAM –YEARLY ECHOCARDIOLOGY –MORTALITY <5% IF GOOD LV –MORTALITY 5-10% IF POOR LV FUNCTION