Diagnosis and Treatment of Cardiomyopathies

Feature or Method

Pathophysiology

Systolic dysfunction

Diastolic dysfunction ± outflow obstruction

Diastolic dysfunction

Clinical findings

LV and RV failure, sudden death

Cardiomegaly

Functional AV valve regurgitation

S3 and/or S4

Exertional dyspnea, angina, syncope, sudden death

Systolic murmur ± mitral regurgitation murmur, S4

Bifid carotid pulse with a brisk upstroke and rapid downstroke

Exertional dyspnea and fatigue

LV ± RV failure

Functional AV valve regurgitation

ECG

Nonspecific ST- and T-wave abnormalities

Q waves ± BBB

LV hypertrophy and ischemia

Deep septal Q waves

LV hypertrophy or low QRS voltage

Echocardiography

Dilated hypokinetic ventricles ± mural thrombus

Low EF and, frequently, functional AV valve regurgitation

Hypertrophied ventricle, high, normal or low EF ± mitral systolic anterior motion ± asymmetric hypertrophy ± LV gradient

Increased wall thickness ± cavity obliteration

LV diastolic dysfunction

X-ray

Cardiomegaly

Pulmonary venous congestion

No or mild cardiomegaly

No or mild cardiomegaly

Hemodynamics

Normal or high EDP, low EF, diffusely dilated hypokinetic ventricles ± AV valve regurgitation

Low CO

High EDP ± outflow subvalvular gradient ± mitral regurgitation

Normal or low CO

High EDP, dip and plateau diastolic LV pressure curve

Normal or low CO

Prognosis

20% mortality in first year, and about 10%/year thereafter

About 1% annual risk of sudden death

70% 5-year mortality

Treatment

Diuretics, ACE inhibitors, angiotensin II

Beta-blockers, ±

Phlebotomy for hemochromatosis

Endocardial resection

ACE = angiotensin-converting enzyme; ARNI = angiotensin II receptor blocker and neprilysin inhibitor; AV = atrioventricular; BBB = bundle branch block; CO = cardiac output; EDP = end-diastolic pressure; EF = ejection fraction; ICD = implantable cardioverter-defibrillator ; LV = left ventricular; RV = right ventricular; S3 = 3rd heart sound; S4 = 4th heart sound; SGLT2 = sodium glucose cotransporter 2 protein; ± = with or without.