Slide 1

Cardiomyopathies Dr. Munaf Inamdar (MD Med.)Associate Professor,BHRC, Pune.

DefinitionA primary disorder of the heart muscle that causes abnormal myocardial performance and is not the result of disease or dysfunction of other cardiac structures myocardial infarction, systemic hypertension, valvular stenosis or regurgitation

WHO ClassificationUnknown cause(primary)Dilated HypertrophicRestrictive

Specific heart muscle disease (secondary) due to a known cause:InfectiveMetabolicSystemic diseaseHerediofamilialSensitivityToxic(alcohol)

Functional ClassificationDilated (congestive, DCM, IDC)ventricular enlargement and syst dysfunction

Hypertrophic (IHSS, HCM, HOCM)inappropriate myocardial hypertrophyin the absence of HTN or aortic stenosis

Restrictive (infiltrative)abnormal filling and diastolic function

We are going to cover.1. dilated cardiomyopathy (DCM)2. hypertrophic cardiomyopathy (HCM)3. restrictive cardiomyopathy (RCM)

And in addition

4. arrhythmogenic right ventricular dysplasia (ARVD)5. obliterative cardiomyopathy (OCM)

Diagramatic representation of different cardiomyopathies

Dilated cardiomyopathy

Idiopathic Dilated Cardiomyopathya disease of unknown etiology that principally affects the myocardium

LV dilatation and systolic dysfunction

pathologyincreased heart size and weightventricular dilatation, normal wall thicknessheart dysfunction out of portion to fibrosis

Most common cause of clinical syndrome of Heart Failure

Incidence and Prognosis3-10 cases per 100,00020,000 new cases per year in the U.S.A.death from progressive pump failure1-year25%2-year35-40%5-year40-80%stabilization observed in 20-50% of patientcomplete recovery is rare

Symptoms.Symptoms of heart failureDue to pulmonary congestion (left HF)dyspnea (rest, exertional, nocturnal), orthpnea

Due to systemic congestion (right HF)edema, nausea, abdominal pain, nocturia

Due to low cardiac outputfatigue and weakness

Clinical Signs.Pulsus alternansPulsus tardusSystolic blood pressure normal or less than normal.Raised JVPProminent a and v waves tricuspid regurgitationb/l basal creptsPedal edemaHepatomegalyGallop rhythmSystolic murmursMitral,tricuspid reg.

Findings on ECGSinus tachycardia in presence of heart failure.Atrial and ventricular tachyarryhthmiasPoor r wave progressionAnterior q waves Intaventricular conduction defects mostly LBBBLeft atrial abnormalityHypertensive changes by voltage criteria not evidentST T changes are seen.

Chest X Ray

Findings on 2D Echo.Dilated chambersLeft atrium is usualy enlarged Left ventricle is enlarged.normal 3.85.0cmMitral and tricuspid regurgitation on doppler flow.LV or Global hypokinesia with an LVEF of