0071643192.pdf

COMPLICATIONS

■ Sequelae of CHF

■ Dysrhythmias

■ Pulmonary and peripheral embolization from mural thrombus

■ Sudden death

Restrictive Cardiomyopathy

Restrictive cardiomyopathy is characterized by heart muscle disease that results

in restricted ventricular filling while systolic function remains normal.

Systemic disorders associated with restrictive cardiomyopathy include amy-

loidosis, sarcoidosis, hemochromatosis, and tropical endomyocardial fibrosis

(most common cause worldwide).

PATHOPHYSIOLOGY

■ Fibrotic process reduces the size of the left ventricle →reduced filling of

L ventricle → diastolic pressures and ↓diastolic volumes.

SYMPTOMS

■ R-sided heart failure symptoms may predominate

■ Dyspnea

■ Fatigue

■ Lower extremity swelling

EXAM

■ Kussmaul’s sign:Increase in jugular venous pressure during inspiration

■ Rales

■ Jugular venous distention

■ Hepatomegaly

■ Edema

DIFFERENTIAL

■ It is important to exclude constrictive pericarditisand diastolic LV dys-

function (eg, HTN) as these are both treatable.

DIAGNOSIS

■ ECG: Nonspecific, decreased voltages, conduction abnormalities

■ CXR: Mild (if any) cardiomegaly

■ Echocardiogram: Normal left ventricular size and systolic function, dilated

atria

■ Characteristic“dip and plateau” (also seen with constrictive pericarditis)

of LV pressures on catheterization

■ Biopsy is definitive.

TREATMENT

■ Limited treatment modalities

■ Diuretics

■ Treat underlying disease process, if present.

COMPLICATIONS

■ CHF

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CARDIOVASCULAR EMERGENCIES

R-sided heart failure

symptoms (JVD,

hepatomegaly) predominate

in restrictive cardiomyopathy.

Consider restrictive

cardiomyopathy in the patient

presenting with CHF without

cardiomegaly or systolic

dysfunction.