RHEUMATIC FEVER

1
RHEUMATIC FEVER

• VALVULAR HEART DISEASE
• GEORGE BRODERICK, M.D.
• GOOD SAMARITAN HOSPITAL

2
INTRODUCTION

• AN ACUTE IMMUNOLOGIC RESPONSE TO ACUTE
  STREPTCOCCAL PHARYNGITIS
• LEADS LATER IN LIFE TO CHRONIC RHEUMATIC VALVULAR
  HEART DISEASE
• MUCH LESS COMMON NOW THAN EARLIER IN 20TH CENTURY

3
INTRODUCTION

• STILL OCCASIONAL OUTBREAKS
• VERY SIGNIFICANT PUBLIC HEALTH PROBLEM IN THIRD
  WORLD
• HAS DECLINED REMARKABLY IN INDUSTRIALIZED WORLD

4
EPIDEMIOLOGY

• IDENTICAL TO GROUP A STREPTOCOCCAL UPPER
  RESPIRATORY TRACT INFECTIONS
• MOST OFTEN IN CHILDREN
• AGE 5-15 YEARS
• LOWER STANDARDS OF LIVING
• OVERCROWDING

5
PATHOGENESIS

• NOT INFECTION OF OTHER SITES
• DIRECT EFFECT OF INFECTION
• TOXIC EFFECT OF STREPTOCOCCAL EXTRACELLULAR
  PRODUCTS
• DYSFUNCTIONAL IMMUNE RESPONSE
• STILL NOT CLEAR

6
DIAGNOSIS

• NO SPECIFIC LABORATORY TEST
• CLINICAL ONE SUPPORTED BY MICROBIOLOGICAL AND
  IMMUNOLOGIC TESTS
• JONES CRITERIA
• 2 MAJOR OR 1 MAJOR AND 2 MINOR WITH DOCUMENTED
  EVIDENCE OF ANTECEDENT INFECTION

7
JONES CRITERIA
MAJOR
MINOR

• CARDITIS
• MIGRATORY POLYARTHRITIS
• SYDENHAMS CHOREA
• SUBCUTANEOUS NODULES
• ERYTHEM MARGINATUM

• CLINICAL
• FEVER
• ARTHRALGIA
• LABORATORY
• ELEVATED ACUTE PHASE REACTANTS
• PROLONGED PR INTERVAL

8
TREATMENT

• STREPTOCCAL THERAPY
• ALL PATIENTS SHOULD BE TREATED AS IS THEY HAVE
  ACUTE INFECTION
• PENICILLIN OR ERYTHROMYCIN FOR 10 DAYS
• SECONDARY PROPHYLAXIS
• PENICILLIN DAILY

9
TREATMENT

• MEDICAL TREATMENT OF ARF
• DEPEND ON PATIENTS CLINICAL MANIFESTATIONS
• CONGESTIVE HEART FAILURE
• DIGOXIN, DIURETICS
• ARTHRITIS
• HIGH DOSE SALICYLATES
• BED REST

10
VALVULAR HEART DISEASE

• MITRAL STENOSIS
• GEORGE BRODERICK, MD

11
ETIOLOGYRHEUMATIC 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

12
ETIOLOGYOTHER CAUSES

• CONGENITAL
• MALIGNANT CARCINOID
• SLE OR RHEUMATOID ARTHRITIS
• AMYLOID
• METHYLSERGIDE THERAPY

13
PATHOLOGYSYMPTOMATIC MITRAL STENOSIS

• THICKENED MITRAL CUSPS
• /- CALCIFIC DEPOSITS
• FUSION OF VALVE COMMISSURES
• SHORTENING OF CHORDAE WITH FUSION
• FISH MOUTH OR FUNNEL ORIFICE

14
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

15
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

16
PHYSICAL EXAMINATION

• ARTERIAL PULSE NORMAL OR DIMINISHED
• JUGULAR PRESSURE PROMINENT a WAVE
• PALPATION
• INCONSPICUOUS LV, RV HEAVE IN PULMONARY HTN

17
PHYSICAL EXAMINATIONAUSCULTATION

• ACCENTUATED S1
• PROLONGED Q-S1 INTERVAL
• OPENING SNAP
• SUDDEN TENSING OF VALVE LEAFLETS
• A2-OS INTERVAL SHORTENS WITH SEVERITY
• DIASTOLIC MURMUR

18
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

19
MANAGEMENTNATURAL 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

20
MANAGEMENTMEDICAL 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 gt5.5cm, EMBOLISM OR
  ATRIAL FIBRILLATION

21
MANAGEMENTSURGICAL TREATMENT

• OPERATE FOR SEVERE SYMPTOMS
• CLASS III OR GREATER (SYMPTOMS WITH LESS THAN
  USUAL ACTIVITY)
• PULMONARY HTN DEMANDS OPERATION
• ROUTINE CATHETERIZATION MENgt45
• MILDY SYMPTOMATIC PATIENTS
• CONSIDER SIZE OF MV ORIFICE, LIFESTYLE AND
  HISTORY OF COMPLICATIONS

22
MANAGEMENTBALLOON 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

23
VALVULAR HEART DISEASE

• MITRAL INSUFFICIENCTY

24
ETIOLOGYACUTE VS CHRONIC

• INFLAMMATORY
• DEGENERATIVE
• INFECTIVE
• STRUCTURAL
• CONGENITAL

25
ETIOLOGYDEGENERATIVE

• MYXOMATOUS DEGENERATION OF LEAFLETS
• MITRAL VALVE PROLAPSE
• MOST COMMON CAUSE OF ACUTE MR IN US ADULTS
• MARFAN SYNDROME
• CALCIFICATION OF MV ANNULUS

26
ETIOLOGYINFLAMMATORY

• RHEUMATIC HEART DISEASE
• ACUTE RHEUMATIC FEVER VS CHRONIC
• SYSTEMIC LUPUS ERYTHEMATOSUS
• SCLERODERMA

27
ETIOLOGYSTRUCTURAL

• RUPTURED CHORDAE TENDINAE
• RUPTURE OR DYSFUNCTION OF PAPILLARY MUSCLES
• DILATATION OF MITRAL VALVE ANNULUS
• PARAVALVULAR PROSTHETIC LEAK

28
ANATOMY OF MITRAL VALVE

• VALVE LEAFLETS
• ANTERIOR AND POSTERIOR
• MITRAL ANNULUS
• DILATATION
• CHORDAE TENDINAE
• PAPILLARY MUSCLES

29
PATHOPHYSIOLOGYVOLUME 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

30
PATHOPHYSIOLOGYHEMODYNAMICS

• 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

31
CLINICAL MANIFESTATIONSNATURAL 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

32
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

33
PHYSICAL EXAMINATION

• CAROTID UPSTROKE SHARP, RAPID FALLOFF
• S1 USUALLY SOFT, WIDELY SPLIT S2
• HOLOSYSTOLIC MURMUR
• APEX TO AXILLA
• SYSTOLIC EJECTION MURMUR
• ISCHEMIC MR

34
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

35
LABORATORY EXAMINATION

• CHEST XRAY
• CARDIOMEGALY (ECCENTRIC HYPERTROPHY)
• LEFT ATRIAL ENLARGEMENT
• REGURGITANT VOLUME
• MILD 25, MODERATE 40, SEVERE 75

36
ECHOCARDIOGRAPHY

• GOOD ANATOMICAL DETAIL
• LA SIZE, THROMBUS, LV FUNCTION
• UNDERLYING ETIOLOGY OF MR
• INFECTIVE ENDOCARDITIS
• DOPPLER
• SEVERITY OF MR, SIZE OF MR JET

37
MANAGEMENTMEDICAL 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

38
SURGICAL TREATMENT

• OPERATE FOR SYMPTOMS
• ENLARGING LEFT VENTRICULAR SYSTOLIC DIMENSION
  (gt5.5CM), EJECTION FRACTION lt55 ARE PREDICTORS
  OF BAD OUTCOME
• OPERATIVE MORTALITY 2 TO 7 IN CLASS II TO III
  PATIENTS
• RECONSTRUCTION IS BETTER THAN REPLACEMENT IF
  POSSIBLE

39
VALVULAR HEART DISEASE

• AORTIC STENOSIS

40
ETIOLOGYOBSTRUCTION TO LV OUTFLOW

• HYPERTROPHIC CARDIOMYOPATHY
• SUPRAVALVULAR
• SUBVALVULAR
• CONGENITAL
• ACQUIRED

41
ETIOLOGYCONGENITAL 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

42
ETIOLOGYACQUIRED AORTIC STENOSIS

• RHEUMATIC HEART DISEASE
• DEGENERATIVE
• ATHEROSCLEROTIC
• CALCIFIC DUE TO PAGETS DISEASE
• RHEUMATOID

43
ETIOLOGYDEGENERATIVE 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

44
ETIOLOGYRHEUMATIC 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

45
HISTORY

• ANGINA
• MEDIAN SURVIVAL 5 YEARS
• SYNCOPE
• MEDIAN SURVIVAL 3 YEARS
• CONGESTIVE HEART FAILURE
• MEDIAN SURVIVAL 2 YEARS

46
PHYSICAL EXAMINATION

• PULSUS PARVUS AND TARDUS
• IN CAROTID PULSE
• REDUCED PULSE PRESSURE
• SUSTAINED CARDIAC IMPULSE
• DELAYED A2 OR DIMINISHED
• HARSH SYSTOLIC EJECTION MURMUR

47
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

48
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

49
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

50
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

51
MEDICAL HISTORY

• EDUCATION IN SYMPTOMS AND REPORTING
• OPERATE FOR SYMPTOMS WHEN VALVE IS SEVERLY
  NARROWED
• lt1CM2 IN AREA
• DO NOT OPERATE ON SEVERE NARROWING IF
  ASYMPTOMATIC
• ENDOCARDITIS PROPHYLAXIS

52
SURGICAL MANAGEMENTRESULTS

• 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 gt 70

53
SURGICAL MANAGEMENT

• ASYMPTOMATIC PATIENTS
• MORTALITY WITHOUT OPERATION IS lt5 PER YEAR
• FOLLOW EVERY 6 MONTHS IN OFFICE
• COUNSEL ON DEVELOPMENT OF SYMPTOMS OF ANGINA,
  CHF, SYNCOPE

54
AORTIC STENOSIS IN THE ELDERLY

• OPERATIVE MORTALITY IN THE ELDERLY
• 1.8 AGE lt 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

55
AORTIC INSUFFICIENCY

• VALVULAR HEART DISEASE

56
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

57
ETIOLOGY

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

58
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)

59
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

60
HISTORY

• FAMILY HISTORY WITH MARFAN SYNDROME
• INFECTIVE ENDOCARDITIS
• SYPHYLIS
• AWARENESS OF HEARTBEAT
• ORTHOPNEA, DOE LATE OR IN ACUTE
• ANGINA
• EDEMA

61
PHYSICAL FINDINGS

• INSPECTION
• BOBBING HEAD OR JARRING OF BODY
• PALPATION
• ARTERIAL JACK HAMMER PULSE
• CAPILLARY PULSATIONS
• VARIOUS SIGNS
• WIDENED PULSE PRESSURE

62
PHYSICAL FINDINGS

• MURMURS
• DIASTOLIC HIGH PITCHED BLOW
• LOUD SYSTOLIC AORTIC EJECTION FLOW MURMUR
• DIASTOLIC RUMBLE AUSTIN FLINT MURMUR
• MISTAKEN FOR MITRAL STENOSIS

63
LABORATORY

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

64
TREATMENT

• CONGESTIVE HEART FAILURE TREATMENT
• DIGOXIN, DIURETICS, AFTERLOAD REDUCTION
• IV NITROPRUSSIDE MAY BE LIFESAVING

65
TREATMENT

• SURGERY
• SYMPTOMATIC PATIENTS SHOULD BE OPERATED UPON
• ASYMPTOMATIC PATIENTS FOLLOWED FOR LEFT
  VENTRICULAR ENLARGEMENT AND SYSTOLIC DIMENSIONS
  ON ECHOCARDIOGRAM
• YEARLY ECHOCARDIOLOGY
• MORTALITY lt5 IF GOOD LV
• MORTALITY 5-10 IF POOR LV FUNCTION