Faculty of Faculty of MedicineMedicine

Universitas Universitas BrawijayaBrawijaya

Faculty of Faculty of MedicineMedicine

Universitas Universitas BrawijayaBrawijaya

22

ObjectivesObjectivesEtiologyEtiology

EpidemiologyEpidemiology

PathogenesisPathogenesis

Pathologic lesionsPathologic lesions

Clinical manifestations & Laboratory Clinical manifestations & Laboratory findingsfindings

Diagnosis & Differential diagnosisDiagnosis & Differential diagnosis

Treatment & PreventionTreatment & Prevention

PrognosisPrognosis

ReferencesReferences

33

EtiologyEtiologyAcute rheumatic fever is a systemic Acute rheumatic fever is a systemic disease of childhood,often recurrent that disease of childhood,often recurrent that follows follows group A beta hemolytic group A beta hemolytic streptococcal infectionstreptococcal infection

It is a delayed non-suppurative sequelae It is a delayed non-suppurative sequelae to URTI with to URTI with GABH streptococci.GABH streptococci.

It is a diffuse inflammatory disease of It is a diffuse inflammatory disease of connective tissue,primarily involving connective tissue,primarily involving heart,blood vessels,joints, subcut.tissue heart,blood vessels,joints, subcut.tissue and CNSand CNS

44

EpidemiologyEpidemiology

Ages Ages 5-15 yrs 5-15 yrs are most susceptibleare most susceptible

Rare Rare <3 yrs<3 yrs

Girls>boysGirls>boys

Common in Common in 3rd world countries3rd world countries

Environmental factors--Environmental factors-- over crowding, over crowding, poor sanitation, poverty,poor sanitation, poverty,

Incidence more during Incidence more during fall ,winter & fall ,winter & early springearly spring

55

PathogenesisPathogenesis

Delayed immune response to infection Delayed immune response to infection with with group.A beta hemolytic group.A beta hemolytic streptococci.streptococci.

After a latent period of After a latent period of 1-3 weeks, 1-3 weeks, antibody induced immunological antibody induced immunological damage occur todamage occur to heart valves,joints, heart valves,joints, subcutaneous tissue & basal subcutaneous tissue & basal ganglia of brainganglia of brain

66

Strains that produces rheumatic fever - Strains that produces rheumatic fever - M types l, 3, 5, 6,18 & 24M types l, 3, 5, 6,18 & 24

PharyngitisPharyngitis- - produced by GABHS can produced by GABHS can lead to- lead to- acute rheumatic fever ,acute rheumatic fever ,

rheumatic heart disease & rheumatic heart disease & post strept. Glomerulonepritis post strept. Glomerulonepritis

Skin infection-Skin infection- produced by GABHS leads produced by GABHS leads to to post streptococcal glomerulo nephritispost streptococcal glomerulo nephritis only. It will not result in Rh.Fever or only. It will not result in Rh.Fever or carditis as skin lipid cholesterol inhibit carditis as skin lipid cholesterol inhibit antigenicityantigenicity

Group A Beta Hemolytic Streptococcus

77

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

88

Pathologic LesionsPathologic LesionsFibrinoid degeneration of connective Fibrinoid degeneration of connective tissue,inflammatory edema, inflammatory cell tissue,inflammatory edema, inflammatory cell infiltration & proliferation of specific cells infiltration & proliferation of specific cells resulting in formation of resulting in formation of AAshcoff nodulesshcoff nodules, , resulting in-resulting in-

--PancarditisPancarditis in the heart in the heart

--Arthritis Arthritis in thein the joints joints

--Ashcoff nodulesAshcoff nodules inin the subcutaneous the subcutaneous tissue tissue

-Basal gangliar lesions resulting in -Basal gangliar lesions resulting in choreachorea

99

Rheumatic Carditis Histology (40X)Rheumatic Carditis Histology (40X)

1010

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

1111

Clinical FeaturesClinical Features

Flitting & fleeting migratory polyarthritis, Flitting & fleeting migratory polyarthritis, involving major jointsinvolving major joints

Commonly involved joints-Commonly involved joints-knee,ankle,elbow & wristknee,ankle,elbow & wrist

Occur in 80%,involved joints are Occur in 80%,involved joints are exquisitely tenderexquisitely tender

In children below 5 yrs arthritis usually In children below 5 yrs arthritis usually mild but carditis more prominentmild but carditis more prominent

Arthritis do not progress to chronic diseaseArthritis do not progress to chronic disease

1.Arthritis

1212

Clinical Features (Contd)Clinical Features (Contd)

Manifest as Manifest as pancarditispancarditis(endocarditis, (endocarditis, myocarditis and pericarditis),occur in 40-myocarditis and pericarditis),occur in 40-50% of cases50% of cases

Carditis is the only manifestation of Carditis is the only manifestation of rheumatic fever that leaves a sequelae & rheumatic fever that leaves a sequelae & permanent damage to the organpermanent damage to the organ

Valvulitis occur in acute phaseValvulitis occur in acute phase

Chronic phase- fibrosis,calcification & Chronic phase- fibrosis,calcification & stenosis of heart valvesstenosis of heart valves(fishmouth valves)(fishmouth valves)

2.Carditis

1313

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

1414

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

1515

Clinical Features (Contd)Clinical Features (Contd)

Occur in 5-10% of casesOccur in 5-10% of cases

Mainly in girls of 1-15 yrs ageMainly in girls of 1-15 yrs age

May appear even 6/12 after the attack of May appear even 6/12 after the attack of rheumatic feverrheumatic fever

Clinically manifest as-clumsiness, Clinically manifest as-clumsiness, deterioration of handwriting,emotional deterioration of handwriting,emotional lability or grimacing of facelability or grimacing of face

Clinical signs- Clinical signs- pronator sign, jack in the pronator sign, jack in the box sign , milking sign of handsbox sign , milking sign of hands

3.Sydenham Chorea

1616

Clinical Features (Contd)Clinical Features (Contd)

Occur in <5%.Occur in <5%.

Unique,transient,serpiginous-looking Unique,transient,serpiginous-looking lesions of 1-2 inches in sizelesions of 1-2 inches in size

Pale center with red irregular marginPale center with red irregular margin

More on trunks & limbs & non-itchyMore on trunks & limbs & non-itchy

Worsens with application of heatWorsens with application of heat

Often associated with chronic carditisOften associated with chronic carditis

4.Erythema Marginatum

1717

Clinical Features (Contd)Clinical Features (Contd)

Occur in 10%Occur in 10%

Painless,pea-sized,palpable nodulesPainless,pea-sized,palpable nodules

Mainly over extensor surfaces of Mainly over extensor surfaces of joints,spine,scapulae & scalpjoints,spine,scapulae & scalp

Associated with strong seropositivityAssociated with strong seropositivity

Always associated with severe carditisAlways associated with severe carditis

5.Subcutaneous nodules

1818

Clinical Features (Contd)Clinical Features (Contd)

Other features (Minor features)

Fever-(up to 101 degree F)Fever-(up to 101 degree F)

ArthralgiaArthralgia

PallorPallor

AnorexiaAnorexia

Loss of weightLoss of weight

1919

Laboratory FindingsLaboratory Findings

High ESRHigh ESR

Anemia, leucocytosisAnemia, leucocytosis

Elevated C-reactive protienElevated C-reactive protien

ASO titre >200 Todd units.ASO titre >200 Todd units.(Peak value attained at 3 (Peak value attained at 3

weeks,then weeks,then comes down to normal by comes down to normal by 6 weeks)6 weeks)

Anti-DNAse B testAnti-DNAse B test

Throat culture-GABHstreptococciThroat culture-GABHstreptococci

2020

Laboratory Findings (Contd)Laboratory Findings (Contd)ECG-ECG- prolonged PR interval, 2nd or 3rd prolonged PR interval, 2nd or 3rd degree blocks,ST depression, degree blocks,ST depression, T T inversioninversion

2D Echo cardiography-2D Echo cardiography- valve edema,mitral valve edema,mitral regurgitation, LA & LV dilatation,pericardial regurgitation, LA & LV dilatation,pericardial effusion,decreased contractilityeffusion,decreased contractility

2121

DiagnosisDiagnosisRheumatic fever is mainly a clinical Rheumatic fever is mainly a clinical diagnosisdiagnosis

No single diagnostic sign or specific No single diagnostic sign or specific laboratory test available for diagnosislaboratory test available for diagnosis

Diagnosis based on Diagnosis based on MODIFIED JONES MODIFIED JONES CRITERIACRITERIA

2222

Jones Criteria (Revised) for Guidance in theDiagnosis of Rheumatic Fever*

Major Manifestation MinorManifestations

Supporting Evidence of Streptococal Infection

Clinical LaboratoryCarditisPolyarthritis

ChoreaErythema Marginatum

Subcutaneous Nodules

Previousrheumaticfever orrheumaticheart diseaseArthralgiaFever

Acute phasereactants:Erythrocytesedimentationrate, C-reactiveprotein,leukocytosis Prolonged P-R interval

Increased Titer of Anti-Streptococcal Antibodies ASO (anti-streptolysin O),othersPositive Throat Culture for Group A StreptococcusRecent Scarlet Fever

*The presence of two major criteria, or of one major and two minor criteria,indicates a high probability of acute rheumatic fever, if supported by evidence ofGroup A streptococcal nfection.

Recommendations of the American Heart Association

2323

Exceptions to Jones CriteriaExceptions to Jones Criteria

Chorea alone, if other causes have been Chorea alone, if other causes have been excludedexcluded

Insidious or late-onset carditis with no Insidious or late-onset carditis with no other explanationother explanation

Patients with documented RHD or prior Patients with documented RHD or prior rheumatic fever,one major criterion,or of rheumatic fever,one major criterion,or of fever,arthralgia or high CRP suggests fever,arthralgia or high CRP suggests recurrencerecurrence

2424

Differential DiagnosisDifferential Diagnosis

Juvenile rheumatiod arthritisJuvenile rheumatiod arthritis

Septic arthritisSeptic arthritis

Sickle-cell arthropathySickle-cell arthropathy

Kawasaki diseaseKawasaki disease

MyocarditisMyocarditis

Scarlet feverScarlet fever

LeukemiaLeukemia

2525

TreatmentTreatmentStep IStep I - primary prevention - primary prevention

(eradication of streptococci)(eradication of streptococci)

Step IIStep II - anti inflammatory treatment - anti inflammatory treatment (aspirin,steroids)(aspirin,steroids)

Step IIIStep III- supportive management & - supportive management & management of complications management of complications

Step IVStep IV- secondary prevention - secondary prevention (prevention of recurrent (prevention of recurrent

attacks)attacks)

05/05/199905/05/1999 Dr.Said AlaviDr.Said Alavi 2626

STEP I: Primary Prevention of Rheumatic Fever (Treatment of Streptococcal Tonsillopharyngitis)

Agent Dose Mode Duration

Benzathine penicillin G 600 000 U for patients Intramuscular Once

27 kg (60 lb) 1 200 000 U for patients >27 kg

or Penicillin V Children: 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 Ethylsuccinate 40 mg/kg/d 2-4 times daily Oral 10 d

(maximum 1 g/d)Recommendations of American Heart Association

2727

Arthritis only Aspirin 75-100mg/kg/day,give as 4divided doses for 6weeks(Attain a blood level 20-30 mg/dl)

Carditis Prednisolone 2-2.5mg/kg/day, give as twodivided doses for 2weeksTaper over 2 weeks &while tapering addAspirin 75 mg/kg/dayfor 2 weeks.Continue aspirin alone100 mg/kg/day foranother 4 weeks

Step II: Anti inflammatory treatmentClinical condition Drugs

2828

Bed rest Bed rest

Treatment of congestive cardiac failure: Treatment of congestive cardiac failure: --digitalis,diureticsdigitalis,diuretics

Treatment of chorea:Treatment of chorea: - -diazepam or haloperidoldiazepam or haloperidol

Rest to joints & supportive splintingRest to joints & supportive splinting

3.Step III: Supportive management & management of complications

Dr.Said AlaviDr.Said Alavi 2929

STEP IV : Secondary Prevention of Rheumatic Fever (Prevention of Recurrent Attacks)

Agent Dose Mode

Benzathine penicillin G 1 200 000 U every 4 weeks* Intramuscular

orPenicillin V 250 mg twice daily Oral

orSulfadiazine 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

Erythromycin 250 mg twice daily Oral

*In high-risk situations, administration every 3 weeks is justified and recommended

Recommendations of American Heart Association

3030

Duration of Secondary Rheumatic Fever Prophylaxis

Category Duration

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 carditis 10 y or well into adulthood, but no residual heart disease whichever is longer (no valvar disease*)

Rheumatic fever without carditis 5 y or until age 21 y,

whichever is longer

*Clinical or echocardiographic evidence.Recommendations of American Heart Association

3131

PrognosisPrognosis

Rheumatic fever can recur whenever the Rheumatic fever can recur whenever the individual experience new GABH individual experience new GABH streptococcal infection,if not on streptococcal infection,if not on prophylactic medicinesprophylactic medicines

Good prognosis for older age group & if no Good prognosis for older age group & if no carditis during the initial attackcarditis during the initial attack

Bad prognosis for younger children & Bad prognosis for younger children & those with carditis with valvar lesionsthose with carditis with valvar lesions

3232

VALVULAR HEART VALVULAR HEART DISEASEDISEASE

MITRAL STENOSISMITRAL STENOSIS

GG

ETIOLOGYETIOLOGYRHEUMATIC VALVULAR DISEASERHEUMATIC VALVULAR DISEASE

MOST COMMON CAUSE OF M ITRAL MOST COMMON CAUSE OF M ITRAL STENOSISSTENOSIS– Pure mitral stenosis 25%Pure mitral stenosis 25%– Pure mitral regurgitation 35%Pure mitral regurgitation 35%– Combined MS and MR 40%Combined MS and MR 40%

15 TO 20 YEAR LATENCY PERIOD15 TO 20 YEAR LATENCY PERIOD

ETIOLOGYETIOLOGYOTHER CAUSESOTHER CAUSES

CONGENITALCONGENITAL

MALIGNANT CARCINOIDMALIGNANT CARCINOID

SLE OR RHEUMATOID ARTHRITISSLE OR RHEUMATOID ARTHRITIS

AMYLOIDAMYLOID

METHYLSERGIDE THERAPYMETHYLSERGIDE THERAPY

PATHOLOGYPATHOLOGYSYMPTOMATIC MITRAL STENOSISSYMPTOMATIC MITRAL STENOSIS

THICKENED MITRAL CUSPSTHICKENED MITRAL CUSPS– +/- CALCIFIC DEPOSITS+/- CALCIFIC DEPOSITS

FUSION OF VALVE COMMISSURESFUSION OF VALVE COMMISSURES

SHORTENING OF CHORDAE WITH SHORTENING OF CHORDAE WITH FUSIONFUSION

““FISH MOUTH” OR FUNNEL ORIFICEFISH MOUTH” OR FUNNEL ORIFICE

HISTORYHISTORY

PRINCIPLE SYMPTOM IS DYSPNEAPRINCIPLE SYMPTOM IS DYSPNEA

– Reduces compliance of the lungReduces compliance of the lung

PULMONARY EDEMAPULMONARY EDEMA

– Effort, emotional stress, infection, fever, Effort, emotional stress, infection, fever, pregnancypregnancy

ATRIAL FIBRILLATIONATRIAL FIBRILLATION

– Increased rate causes increased LA to LV Increased rate causes increased LA to LV gradientgradient

HISTORYHISTORY

CHEST PAINCHEST PAIN– 15% DUE TO RV HTN, EMBOLIZATION15% DUE TO RV HTN, EMBOLIZATION

THROMBOEMBOLISMTHROMBOEMBOLISM– 20% HISTORICALLY INVOLVED20% HISTORICALLY INVOLVED– CORRELATES INVERSELY WITH CARDIAC CORRELATES INVERSELY WITH CARDIAC

OUTPUTOUTPUT– CORRELATES DIRECTLY WITH LA SIZE CORRELATES DIRECTLY WITH LA SIZE

AND AGE OF PATIENTAND AGE OF PATIENT

PHYSICAL EXAMINATIONPHYSICAL EXAMINATION

ARTERIAL PULSE NORMAL OR ARTERIAL PULSE NORMAL OR DIMINISHEDDIMINISHED

JUGULAR PRESSURE PROMINENT a JUGULAR PRESSURE PROMINENT a WAVEWAVE

PALPATIONPALPATION– INCONSPICUOUS LV, RV HEAVE IN INCONSPICUOUS LV, RV HEAVE IN

PULMONARY HTNPULMONARY HTN

PHYSICAL EXAMINATIONPHYSICAL EXAMINATIONAUSCULTATIONAUSCULTATION

ACCENTUATED S1ACCENTUATED S1– PROLONGED Q-S1 INTERVALPROLONGED Q-S1 INTERVAL

OPENING SNAPOPENING SNAP– SUDDEN TENSING OF VALVE LEAFLETSSUDDEN TENSING OF VALVE LEAFLETS– A2-OS INTERVAL SHORTENS WITH A2-OS INTERVAL SHORTENS WITH

SEVERITYSEVERITY

DIASTOLIC MURMURDIASTOLIC MURMUR

PATHOPHYSIOLOGYPATHOPHYSIOLOGY

NORMAL VALVE AREA 4 TO 6cm2NORMAL VALVE AREA 4 TO 6cm2

NORMAL MEAN LA TO LV PRESSURE NORMAL MEAN LA TO LV PRESSURE GRADIENT 2 TO 4mmHgGRADIENT 2 TO 4mmHg

MILD MITRAL STENOSIS 2cm2MILD MITRAL STENOSIS 2cm2

CRITICAL MITRAL STENOSIS 1cm2 or CRITICAL MITRAL STENOSIS 1cm2 or lessless– 20MMhg GRADIENT REQUIRED FOR FLOW20MMhg GRADIENT REQUIRED FOR FLOW

MANAGEMENTMANAGEMENTNATURAL HISTORYNATURAL HISTORY

20 TO 25 YEAR ASYMPTOMATIC 20 TO 25 YEAR ASYMPTOMATIC PERIODPERIOD5 YEARS FOR PROGRESSION CLASS 5 YEARS FOR PROGRESSION CLASS II-IVII-IVSURVIVALSURVIVAL– CLASS III 62% 5 YR SURVIVALCLASS III 62% 5 YR SURVIVAL– CLASS IV 15% 5 YR SURVIVALCLASS IV 15% 5 YR SURVIVAL

ASYMPTOMATIC CLASS 1 40% ASYMPTOMATIC CLASS 1 40% WORSENED OR DIED IN 10 YEARSWORSENED OR DIED IN 10 YEARS

MANAGEMENTMANAGEMENTMEDICAL TREATMENTMEDICAL TREATMENT

RHD PCN AND SBE PROPHYLAXISRHD PCN AND SBE PROPHYLAXIS

SYMPTOMATIC PATIENTSSYMPTOMATIC PATIENTS

– ORAL DIURETICS AND ACTIVITY ORAL DIURETICS AND ACTIVITY RESTRICTIONRESTRICTION

– BETA BLOCKERS AND LOW HEART RATEBETA BLOCKERS AND LOW HEART RATE

– DIGOXIN IN AF AND WITH PULM HTNDIGOXIN IN AF AND WITH PULM HTN

ANTICOAGULATION FOR LA SIZE >5.5cm, ANTICOAGULATION FOR LA SIZE >5.5cm, EMBOLISM OR ATRIAL FIBRILLATIONEMBOLISM OR ATRIAL FIBRILLATION

MANAGEMENTMANAGEMENTSURGICAL TREATMENTSURGICAL TREATMENT

OPERATE FOR SEVERE SYMPTOMSOPERATE FOR SEVERE SYMPTOMS

– CLASS III OR GREATER (SYMPTOMS WITH CLASS III OR GREATER (SYMPTOMS WITH LESS THAN USUAL ACTIVITY)LESS THAN USUAL ACTIVITY)

– PULMONARY HTN DEMANDS OPERATIONPULMONARY HTN DEMANDS OPERATION

ROUTINE CATHETERIZATION MEN>45ROUTINE CATHETERIZATION MEN>45

MILDY SYMPTOMATIC PATIENTSMILDY SYMPTOMATIC PATIENTS

– CONSIDER SIZE OF MV ORIFICE, CONSIDER SIZE OF MV ORIFICE, LIFESTYLE AND HISTORY OF LIFESTYLE AND HISTORY OF COMPLICATIONSCOMPLICATIONS

MANAGEMENTMANAGEMENTBALLOON VALVULOPLASTYBALLOON VALVULOPLASTY

PROCEDURE OF CHOICE IN RIGHT PTPROCEDURE OF CHOICE IN RIGHT PT– TRANSESOPHAGEAL ECHO HELPFUL IN TRANSESOPHAGEAL ECHO HELPFUL IN

SORTING OUT WHICH PATIENTSORTING OUT WHICH PATIENT– ECHO SCALE OF PREDICTORS RELATES ECHO SCALE OF PREDICTORS RELATES

TO THICKENING AND CALCIFICATION OF TO THICKENING AND CALCIFICATION OF VALVEVALVE

RESULTS COMPARABLE TO SURGERYRESULTS COMPARABLE TO SURGERYMORTALITY 2-3%, MORBIDITY 8-12%MORTALITY 2-3%, MORBIDITY 8-12%

VALVULAR HEART VALVULAR HEART DISEASEDISEASE

MITRAL MITRAL INSUFFICIENCTYINSUFFICIENCTY

ETIOLOGYETIOLOGYACUTE VS CHRONICACUTE VS CHRONIC

INFLAMMATORYINFLAMMATORY

DEGENERATIVEDEGENERATIVE

INFECTIVEINFECTIVE

STRUCTURALSTRUCTURAL

CONGENITALCONGENITAL

ETIOLOGYETIOLOGYDEGENERATIVEDEGENERATIVE

MYXOMATOUS DEGENERATION OF MYXOMATOUS DEGENERATION OF LEAFLETSLEAFLETS– MITRAL VALVE PROLAPSEMITRAL VALVE PROLAPSE– MOST COMMON CAUSE OF ACUTE MR IN MOST COMMON CAUSE OF ACUTE MR IN

US ADULTSUS ADULTS

MARFAN SYNDROMEMARFAN SYNDROME

CALCIFICATION OF MV ANNULUSCALCIFICATION OF MV ANNULUS

ETIOLOGYETIOLOGYINFLAMMATORYINFLAMMATORY

RHEUMATIC HEART DISEASERHEUMATIC HEART DISEASE– ACUTE RHEUMATIC FEVER VS ACUTE RHEUMATIC FEVER VS

CHRONICCHRONIC

SYSTEMIC LUPUS SYSTEMIC LUPUS ERYTHEMATOSUSERYTHEMATOSUS

SCLERODERMASCLERODERMA

ETIOLOGYETIOLOGYSTRUCTURALSTRUCTURAL

RUPTURED CHORDAE TENDINAERUPTURED CHORDAE TENDINAE

RUPTURE OR DYSFUNCTION OF RUPTURE OR DYSFUNCTION OF PAPILLARY MUSCLESPAPILLARY MUSCLES

DILATATION OF MITRAL VALVE DILATATION OF MITRAL VALVE ANNULUSANNULUS

PARAVALVULAR PROSTHETIC LEAKPARAVALVULAR PROSTHETIC LEAK

ANATOMY OF MITRAL VALVEANATOMY OF MITRAL VALVE

VALVE LEAFLETSVALVE LEAFLETS– ANTERIOR AND POSTERIORANTERIOR AND POSTERIOR

MITRAL ANNULUSMITRAL ANNULUS– DILATATIONDILATATION

CHORDAE TENDINAECHORDAE TENDINAE

PAPILLARY MUSCLESPAPILLARY MUSCLES

PATHOPHYSIOLOGYPATHOPHYSIOLOGYVOLUME OVERLOADVOLUME OVERLOAD

IMPEDENCE TO VENTRICULAR IMPEDENCE TO VENTRICULAR EMPTYING IS EMPTYING IS REDUCEDREDUCED– LV DECOMPRESSES INTO LALV DECOMPRESSES INTO LA

VOLUME OF REGURGITANT FLOWVOLUME OF REGURGITANT FLOW– DEPENDENT ON SIZE OF REGURGITANT DEPENDENT ON SIZE OF REGURGITANT

ORIFICEORIFICEAND LV TO LA PRESSURE GRADIENTAND LV TO LA PRESSURE GRADIENT

PATHOPHYSIOLOGYPATHOPHYSIOLOGYHEMODYNAMICSHEMODYNAMICS

FORWARD CARDIAC OUTPUT USUALLY FORWARD CARDIAC OUTPUT USUALLY DEPRESSEDDEPRESSED– TOTAL LV OUTPUT (FORWARD AND TOTAL LV OUTPUT (FORWARD AND

BACKWARD) INCREASEDBACKWARD) INCREASEDNORMAL LA COMPLIANCE (ACUTE MR)NORMAL LA COMPLIANCE (ACUTE MR)– LITTLE ENLARGEMENT OF LA, HIGH LA LITTLE ENLARGEMENT OF LA, HIGH LA

PRESSUREPRESSURELOW LA COMPLIANCE (CHRONIC MR)LOW LA COMPLIANCE (CHRONIC MR)– ENLARGED LA, MINIMALLY INCREASED LA ENLARGED LA, MINIMALLY INCREASED LA

PRESSUREPRESSURE

CLINICAL MANIFESTATIONSCLINICAL MANIFESTATIONSNATURAL HISTORYNATURAL HISTORY

VARIABLE AND DEPENDS ON MR VARIABLE AND DEPENDS ON MR VOLUMEVOLUME

MILD MR STABLE IN MAJORITY FOR MILD MR STABLE IN MAJORITY FOR YEARSYEARS– MINORITY DEVELOP SEVERE MRMINORITY DEVELOP SEVERE MR

MORE RAPIDLY WITH DEGENERATIVE MORE RAPIDLY WITH DEGENERATIVE DISEASE THAN RHEUMATICDISEASE THAN RHEUMATIC

CLINICAL MANIFESTATIONSCLINICAL MANIFESTATIONS

SYMPTOMS USUALLY NOT UNTIL LV SYMPTOMS USUALLY NOT UNTIL LV STARTS TO FAILSTARTS TO FAIL

LONGER TIME INTERVAL IN MR THAN LONGER TIME INTERVAL IN MR THAN MITRAL STENOSISMITRAL STENOSIS

RIGHT HEART FAILURE IN END STAGE RIGHT HEART FAILURE IN END STAGE MRMR

PHYSICAL EXAMINATIONPHYSICAL EXAMINATION

CAROTID UPSTROKE SHARP, RAPID CAROTID UPSTROKE SHARP, RAPID FALLOFFFALLOFF

S1 USUALLY SOFT, WIDELY SPLIT S2S1 USUALLY SOFT, WIDELY SPLIT S2

HOLOSYSTOLIC MURMURHOLOSYSTOLIC MURMUR– APEX TO AXILLAAPEX TO AXILLA

SYSTOLIC EJECTION MURMURSYSTOLIC EJECTION MURMUR– ISCHEMIC MR ISCHEMIC MR

PHYSICAL EXAMINATIONPHYSICAL EXAMINATION

MITRAL VALVE PROLAPSEMITRAL VALVE PROLAPSE– MID TO LATE SYSTOLIC EJECTION MID TO LATE SYSTOLIC EJECTION

MURMURMURMUR– MID SYSTOLIC NON-EJECTION CLICKMID SYSTOLIC NON-EJECTION CLICK– VALSALVA PROLONGS MURMUR AND VALSALVA PROLONGS MURMUR AND

BRINGS IT TO START CLOSER TO S1BRINGS IT TO START CLOSER TO S1

LABORATORY EXAMINATIONLABORATORY EXAMINATION

CHEST XRAYCHEST XRAY– CARDIOMEGALY (ECCENTRIC CARDIOMEGALY (ECCENTRIC

HYPERTROPHY)HYPERTROPHY)– LEFT ATRIAL ENLARGEMENTLEFT ATRIAL ENLARGEMENT

REGURGITANT VOLUMEREGURGITANT VOLUME– MILD 25%, MODERATE 40%, SEVERE MILD 25%, MODERATE 40%, SEVERE

75%75%

ECHOCARDIOGRAPHYECHOCARDIOGRAPHY

GOOD ANATOMICAL DETAILGOOD ANATOMICAL DETAIL

LA SIZE, THROMBUS, LV FUNCTIONLA SIZE, THROMBUS, LV FUNCTION

UNDERLYING ETIOLOGY OF MRUNDERLYING ETIOLOGY OF MR

INFECTIVE ENDOCARDITISINFECTIVE ENDOCARDITIS

DOPPLERDOPPLER– SEVERITY OF MR, SIZE OF MR JETSEVERITY OF MR, SIZE OF MR JET

MANAGEMENTMANAGEMENTMEDICAL MANAGEMENTMEDICAL MANAGEMENT

AFTERLOAD REDUCTIONAFTERLOAD REDUCTION– REDUCES IMPEDENCE TO EJECTION IN REDUCES IMPEDENCE TO EJECTION IN

AORTAAORTA– ACE INHIBITORS AND HYDRALAZINEACE INHIBITORS AND HYDRALAZINE

ACUTE MRACUTE MR– IV NITROPRUSSIDE CAN BE LIFESAVINGIV NITROPRUSSIDE CAN BE LIFESAVING

DIGOXIN, DIURETICS IN CHRONIC MRDIGOXIN, DIURETICS IN CHRONIC MRFOLLOW LV SIZE AND FUNCTIONFOLLOW LV SIZE AND FUNCTION

SURGICAL TREATMENTSURGICAL TREATMENT

OPERATE FOR SYMPTOMSOPERATE FOR SYMPTOMSENLARGING LEFT VENTRICULAR ENLARGING LEFT VENTRICULAR SYSTOLIC DIMENSION (>5.5CM), SYSTOLIC DIMENSION (>5.5CM), EJECTION FRACTION <55% ARE EJECTION FRACTION <55% ARE PREDICTORS OF BAD OUTCOMEPREDICTORS OF BAD OUTCOMEOPERATIVE MORTALITY 2 TO 7% IN OPERATIVE MORTALITY 2 TO 7% IN CLASS II TO III PATIENTSCLASS II TO III PATIENTSRECONSTRUCTION IS BETTER THAN RECONSTRUCTION IS BETTER THAN REPLACEMENT IF POSSIBLEREPLACEMENT IF POSSIBLE

VALVULAR HEART VALVULAR HEART DISEASEDISEASE

AORTIC STENOSISAORTIC STENOSIS

ETIOLOGYETIOLOGYOBSTRUCTION TO LV OUTFLOWOBSTRUCTION TO LV OUTFLOW

HYPERTROPHIC CARDIOMYOPATHYHYPERTROPHIC CARDIOMYOPATHY

SUPRAVALVULARSUPRAVALVULAR

SUBVALVULARSUBVALVULAR

CONGENITALCONGENITAL

ACQUIREDACQUIRED

ETIOLOGYETIOLOGYCONGENITAL AORTIC STENOSISCONGENITAL AORTIC STENOSIS

UNICUSPIDUNICUSPID– SEVERE AND DEADLY IN INFANCYSEVERE AND DEADLY IN INFANCY

BICUSPIDBICUSPID– MANIFESTED LATER IN LIFEMANIFESTED LATER IN LIFE– MOST COMMON CONGENITAL CARDIAC MOST COMMON CONGENITAL CARDIAC

ANOMALY IN LIVE BIRTHS (1%)ANOMALY IN LIVE BIRTHS (1%)

TRICUSPIDTRICUSPID– CUSPS OF UNEQUAL SIZE CUSPS OF UNEQUAL SIZE

ETIOLOGYETIOLOGYACQUIRED AORTIC STENOSISACQUIRED AORTIC STENOSIS

RHEUMATIC HEART DISEASERHEUMATIC HEART DISEASE

DEGENERATIVE DEGENERATIVE

ATHEROSCLEROTICATHEROSCLEROTIC

CALCIFIC DUE TO PAGET’S DISEASECALCIFIC DUE TO PAGET’S DISEASE

RHEUMATOIDRHEUMATOID

ETIOLOGYETIOLOGYDEGENERATIVE CALCIFIC AORTIC STENOSISDEGENERATIVE CALCIFIC AORTIC STENOSIS

PRIMARY CAUSE OF ADULT AORTIC PRIMARY CAUSE OF ADULT AORTIC STENOSISSTENOSIS

YEARS OF MECHANICAL STRESSYEARS OF MECHANICAL STRESS

DEPOSITION OF CALCIUM AT CUPAL DEPOSITION OF CALCIUM AT CUPAL BASE BASE

PRESERVED COMMISSURESPRESERVED COMMISSURES

RISK FACTORSRISK FACTORS– DIABETES AND HYPERLIPIDEMIADIABETES AND HYPERLIPIDEMIA

ETIOLOGYETIOLOGYRHEUMATIC AORTIC STENOSISRHEUMATIC AORTIC STENOSIS

FUSION OF COMMISSURES AND FUSION OF COMMISSURES AND CUSPSCUSPSRETRACTION OF CUSPAL BORDERSRETRACTION OF CUSPAL BORDERSREDUCE ORIFICE TO TRIANGULAR REDUCE ORIFICE TO TRIANGULAR OPENINGOPENINGASSOCIATED WITH AORTIC ASSOCIATED WITH AORTIC INSUFFICENCYINSUFFICENCYMITRAL DISEASE COMMONMITRAL DISEASE COMMONISOLATED AORTIC STENOSIS RAREISOLATED AORTIC STENOSIS RARE

HISTORYHISTORY

ANGINAANGINA– MEDIAN SURVIVAL 5 YEARSMEDIAN SURVIVAL 5 YEARS

SYNCOPESYNCOPE– MEDIAN SURVIVAL 3 YEARSMEDIAN SURVIVAL 3 YEARS

CONGESTIVE HEART FAILURECONGESTIVE HEART FAILURE– MEDIAN SURVIVAL 2 YEARSMEDIAN SURVIVAL 2 YEARS

PHYSICAL EXAMINATIONPHYSICAL EXAMINATION

PULSUS PARVUS AND TARDUSPULSUS PARVUS AND TARDUS– IN CAROTID PULSEIN CAROTID PULSE

REDUCED PULSE PRESSUREREDUCED PULSE PRESSURE

SUSTAINED CARDIAC IMPULSESUSTAINED CARDIAC IMPULSE

DELAYED A2 OR DIMINISHEDDELAYED A2 OR DIMINISHED

HARSH SYSTOLIC EJECTION MURMURHARSH SYSTOLIC EJECTION MURMUR

PATHOPHYSIOLOGYPATHOPHYSIOLOGY

GRADUAL DEVELOPMENT OF OBSTRUCTION GRADUAL DEVELOPMENT OF OBSTRUCTION TO LV OUTFLOWTO LV OUTFLOWLV OUTPUT MAINTAINED BY LV HYPERTROPHYLV OUTPUT MAINTAINED BY LV HYPERTROPHYLV HYPERTROPHY MAY SUSTAIN A LARGE LV HYPERTROPHY MAY SUSTAIN A LARGE PRESSURE GRADIENT FROM THE LV TO PRESSURE GRADIENT FROM THE LV TO AORTA OVER YEARSAORTA OVER YEARSATRIAL CONTRACTION IMPORTANTATRIAL CONTRACTION IMPORTANT– ATRIAL FIBRILLATION MAY CAUSE ABRUPT ATRIAL FIBRILLATION MAY CAUSE ABRUPT

AND SEVERE SYMPTOMSAND SEVERE SYMPTOMS

PATHOPHYSIOLOGYPATHOPHYSIOLOGY

INCREASE IN AFTERLOADINCREASE IN AFTERLOAD

INCREASED LV WALL STRESS INCREASED LV WALL STRESS COMPENSATED BY THE INCREASED COMPENSATED BY THE INCREASED LV HYPERTROPHYLV HYPERTROPHY

ULTIMATELY LOSS IN CONTRACTILITY ULTIMATELY LOSS IN CONTRACTILITY OF LV MASS AND DEVELOPMENT OF OF LV MASS AND DEVELOPMENT OF HEART FAILUREHEART FAILURE

LABORATORYLABORATORY

EKGEKG– LEFT VENTRICULAR HYPERTROPHY IS LEFT VENTRICULAR HYPERTROPHY IS

PROMINENT FINDINGPROMINENT FINDINGCHEST XRAYCHEST XRAY– MAY BE ENTIRELY NORMAL BECAUSE THE MAY BE ENTIRELY NORMAL BECAUSE THE

HYPERTROPHY OF LV IS CONCENTRIC HYPERTROPHY OF LV IS CONCENTRIC (CENTRAL) NOT ECCENTRIC LIKE MR OR (CENTRAL) NOT ECCENTRIC LIKE MR OR AORTIC INSUFFICIENCYAORTIC INSUFFICIENCY

– CALCIFICATION OF AORTIC VALVE MAY BE CALCIFICATION OF AORTIC VALVE MAY BE SEENSEEN

ECHOCARDIOGRAPHYECHOCARDIOGRAPHY

CALCIFIED VALVE WITH THICKENED LEAFLETS CALCIFIED VALVE WITH THICKENED LEAFLETS OR COMMISSURESOR COMMISSURES

DECREASED OPENING OF AORTIC VALVE DECREASED OPENING OF AORTIC VALVE SEENSEEN

LEFT VENTRICULAR HYPERTROPHYLEFT VENTRICULAR HYPERTROPHY

DOPPLERDOPPLER

– VALVE PRESSURE GRADIENT CALCULATEDVALVE PRESSURE GRADIENT CALCULATED

– VALVE AREA FROM THIS MEASUREMENTVALVE AREA FROM THIS MEASUREMENT

MEDICAL HISTORYMEDICAL HISTORY

EDUCATION IN SYMPTOMS AND EDUCATION IN SYMPTOMS AND REPORTINGREPORTING

OPERATE FOR SYMPTOMS WHEN OPERATE FOR SYMPTOMS WHEN VALVE IS SEVERLY NARROWED VALVE IS SEVERLY NARROWED – <1CM2 IN AREA<1CM2 IN AREA

DO NOT OPERATE ON SEVERE DO NOT OPERATE ON SEVERE NARROWING IF ASYMPTOMATICNARROWING IF ASYMPTOMATIC

ENDOCARDITIS PROPHYLAXISENDOCARDITIS PROPHYLAXIS

SURGICAL MANAGEMENTSURGICAL MANAGEMENTRESULTSRESULTS

5 YEAR ACTUARIAL SURVIVAL 85%5 YEAR ACTUARIAL SURVIVAL 85%

REDUCTION IN LV MASSREDUCTION IN LV MASS

IF PATIENTS HAVE CONGESTIVE IF PATIENTS HAVE CONGESTIVE HEART FAILURE THEN VALVE HEART FAILURE THEN VALVE REPLACEMENT HAS 10-25% REPLACEMENT HAS 10-25% MORTALITYMORTALITY

NORMAL 3-5% MORTALITY IN ORNORMAL 3-5% MORTALITY IN OR

PORCINE VALVE FOR AGE > 70PORCINE VALVE FOR AGE > 70

SURGICAL MANAGEMENTSURGICAL MANAGEMENT

ASYMPTOMATIC PATIENTSASYMPTOMATIC PATIENTS– MORTALITY WITHOUT OPERATION IS <5% MORTALITY WITHOUT OPERATION IS <5%

PER YEARPER YEAR– FOLLOW EVERY 6 MONTHS IN OFFICEFOLLOW EVERY 6 MONTHS IN OFFICE– COUNSEL ON DEVELOPMENT OF COUNSEL ON DEVELOPMENT OF

SYMPTOMS OF ANGINA, CHF, SYNCOPESYMPTOMS OF ANGINA, CHF, SYNCOPE

AORTIC STENOSIS IN THE AORTIC STENOSIS IN THE ELDERLYELDERLY

OPERATIVE MORTALITY IN THE OPERATIVE MORTALITY IN THE ELDERLYELDERLY– 1.8% AGE < 501.8% AGE < 50– 5.1% AGE 50 - 605.1% AGE 50 - 60– 7.1% AGE 60 – 707.1% AGE 60 – 70

ISOLATED AV REPLACEMENT IN PTS ISOLATED AV REPLACEMENT IN PTS AGE 80 TO 89AGE 80 TO 89– 94% HAD GOOD RESULTS94% HAD GOOD RESULTS– APPROPRIATE SELECTIONAPPROPRIATE SELECTION

AORTIC INSUFFICIENCYAORTIC INSUFFICIENCY

VALVULAR HEART DISEASEVALVULAR HEART DISEASE

ETIOLOGYETIOLOGY

¾ OF PATIENTS WITH PURE AI ARE ¾ OF PATIENTS WITH PURE AI ARE MALESMALES2/3 OF PATIENTS FROM RHEUMATIC 2/3 OF PATIENTS FROM RHEUMATIC FEVERFEVER– THICKENING AND DEFORMATION OF THICKENING AND DEFORMATION OF

INDIVIDUAL VALVE CUSPSINDIVIDUAL VALVE CUSPS

INFECTIVE ENDOCARDITISINFECTIVE ENDOCARDITIS– VARIOUS PREVIOUSLY DAMAGING VARIOUS PREVIOUSLY DAMAGING

ETIOLOGIESETIOLOGIES

ETIOLOGYETIOLOGY

PROLAPSE OF AN AORTIC CUSPPROLAPSE OF AN AORTIC CUSP

CONGENITAL FENESTRATIONS OF CONGENITAL FENESTRATIONS OF CUSPCUSP

TRAUMATIC RUPTURETRAUMATIC RUPTURE

ASCENDING THORACIC AORTA ASCENDING THORACIC AORTA DISSECTIONDISSECTION

MARKED AORTIC ROOT DILATATIONMARKED AORTIC ROOT DILATATION

SYPHILIS, ANKYLOSING SPONDYLITISSYPHILIS, ANKYLOSING SPONDYLITIS

PATHOPHYSIOLOGYPATHOPHYSIOLOGY

MARKED INCREASE IN STROKE MARKED INCREASE IN STROKE VOLUME OF LEFT VENTRICLEVOLUME OF LEFT VENTRICLE– EXTRA BLOOD FROM LEAKING BACK EXTRA BLOOD FROM LEAKING BACK

INTO LV TO EJECTINTO LV TO EJECT

CONTRAST TO MITRAL CONTRAST TO MITRAL INSUFFICIENCYINSUFFICIENCY– AI: EJECTING BLOOD INTO HIGH AI: EJECTING BLOOD INTO HIGH

AFTERLOAD (AORTA)AFTERLOAD (AORTA)– MI: EJECTING BLOOD INTO LOW MI: EJECTING BLOOD INTO LOW

AFTERLOAD (LEFT ATRIUM)AFTERLOAD (LEFT ATRIUM)

PATHOPHYSIOLOGYPATHOPHYSIOLOGY

DILATATION OF LEFT VENTRICLEDILATATION OF LEFT VENTRICLE– TO MAINTAIN ADEQUATE FORWARD TO MAINTAIN ADEQUATE FORWARD

CARDIAC OUTPUTCARDIAC OUTPUT– COR BOVINUMCOR BOVINUM

REVERSE PRESSURE GRADIENT REVERSE PRESSURE GRADIENT FROM AORTA TO LV IN DIASTOLE FROM AORTA TO LV IN DIASTOLE CAUSES BACK FLOWCAUSES BACK FLOW

ACUTE VS CHRONIC INSUFFICIENCYACUTE VS CHRONIC INSUFFICIENCY

HISTORYHISTORY

FAMILY HISTORY WITH MARFAN FAMILY HISTORY WITH MARFAN SYNDROMESYNDROME

INFECTIVE ENDOCARDITISINFECTIVE ENDOCARDITIS

SYPHYLISSYPHYLIS

AWARENESS OF HEARTBEATAWARENESS OF HEARTBEAT

ORTHOPNEA, DOE LATE OR IN ACUTEORTHOPNEA, DOE LATE OR IN ACUTE

ANGINAANGINA

EDEMAEDEMA

PHYSICAL FINDINGSPHYSICAL FINDINGS

INSPECTIONINSPECTION– BOBBING HEAD OR JARRING OF BODYBOBBING HEAD OR JARRING OF BODY

PALPATIONPALPATION– ARTERIAL JACK HAMMER PULSEARTERIAL JACK HAMMER PULSE– CAPILLARY PULSATIONSCAPILLARY PULSATIONS– VARIOUS SIGNS VARIOUS SIGNS – WIDENED PULSE PRESSUREWIDENED PULSE PRESSURE

PHYSICAL FINDINGSPHYSICAL FINDINGS

MURMURSMURMURS– DIASTOLIC HIGH PITCHED BLOWDIASTOLIC HIGH PITCHED BLOW– LOUD SYSTOLIC AORTIC EJECTION FLOW LOUD SYSTOLIC AORTIC EJECTION FLOW

MURMURMURMUR– DIASTOLIC RUMBLE DIASTOLIC RUMBLE AUSTIN FLINT AUSTIN FLINT

MURMURMURMURMISTAKEN FOR MITRAL STENOSISMISTAKEN FOR MITRAL STENOSIS

LABORATORYLABORATORY

EKGEKG

– LEFT VENTRICULAR HYPERTROPHYLEFT VENTRICULAR HYPERTROPHY

WITH STRAINWITH STRAIN

– ECHOCARDIOGRAMECHOCARDIOGRAM

FLOW INTO LV FROM AORTIC VALVEFLOW INTO LV FROM AORTIC VALVE

LV SIZE LV SIZE

FLUTTERING OF MITRAL LEAFLETFLUTTERING OF MITRAL LEAFLET

– BLOOD CULTURES IN ENDOCARDITISBLOOD CULTURES IN ENDOCARDITIS

TREATMENTTREATMENT

CONGESTIVE HEART FAILURE CONGESTIVE HEART FAILURE TREATMENTTREATMENT– DIGOXIN, DIURETICS, AFTERLOAD DIGOXIN, DIURETICS, AFTERLOAD

REDUCTIONREDUCTIONIV NITROPRUSSIDE MAY BE LIFESAVINGIV NITROPRUSSIDE MAY BE LIFESAVING

TREATMENTTREATMENTSURGERYSURGERY– SYMPTOMATIC PATIENTS SHOULD BE SYMPTOMATIC PATIENTS SHOULD BE

OPERATED UPONOPERATED UPON– ASYMPTOMATIC PATIENTS FOLLOWED ASYMPTOMATIC PATIENTS FOLLOWED

FOR LEFT VENTRICULAR ENLARGEMENT FOR LEFT VENTRICULAR ENLARGEMENT AND SYSTOLIC DIMENSIONS ON AND SYSTOLIC DIMENSIONS ON ECHOCARDIOGRAMECHOCARDIOGRAM

– YEARLY ECHOCARDIOLOGYYEARLY ECHOCARDIOLOGY– MORTALITY <5% IF GOOD LVMORTALITY <5% IF GOOD LV– MORTALITY 5-10% IF POOR LV FUNCTIONMORTALITY 5-10% IF POOR LV FUNCTION