Definisi

Cardiomyopathies are diseases of the heart muscle, characterized by abnormality in chamber

size and wall thickness, or functional contractile dysfunctions mainly systolic or diastolic
dysfunction in the absence of coronary artery disease, hypertension, valvular disease, or
congenital heart disease (Elliott et al., 2008).
The American Heart Association (AHA) expert consensus panel proposed definition of
cardiomyopathies is as follows: Cardiomyopathies are a heterogeneous group of diseases of
the myocardium associated with mechanical and/or electrical dysfunction, which usually (but
not invariably) exhibit inappropriate ventricular hypertrophy or dilatation, due to a variety of
etiologies that frequently are genetic.
Klasifikasi & Etiologi
Cardiomyopathies are classified traditionally according to morphological and functional
criteria into four categories: dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy
(HCM), restrictive cardiomyopathy (RCM)
1. Dilated cardiomiopathy
Kardiomiopati dilatasi adalah jenis kardiomiopati dengan ciri-ciri yaitu
terdapatnya dilatasi ruang ventrikel yang progresif dan disertai disfungsi
dari kontraksi ventrikel saat sistolik. Penyakit ini memiliki banyak etiologi
antara lain: genetik, bahan toksik (alkohol, doxorubicin), peripartum,
miokarditis virus, tetapi pada sebagian besar kasus penyebabnya adalah
idiopatik.
Gejala:
Symptoms of left ventricular failure dominate the clinical picture. Clinical
presentation is variable, as most cases remain asymptomatic for years.
Fatigue and weakness due to diminished cardiac output is often the

presenting symptom. Many patients present with sudden onset of dyspnea

during an episode of chest infection.
Chest pain due to pulmonary embolism or abdominal pain of enlarged liver
may occur during late stage of the disease. Clinical examination will show
dyspnea and persistent tachycardia. Chyene-Stokes breathing indicates
poor prognosis. Pulse pressure is low and during late stages of the disease,
hands and feet may appear cold and clammy. Significant elevation of
jugular venous pressure with prominent a and v waves may be seen. Large
v waves due to tricuspid regurgitation indicate an ominous prognosis.
Dependant edema and ascites are features of right-sided involvement.
Moderate to large cardiomegaly with signs of biventricular enlargement
and palpable shock of third heart sound will be present. Auscultation will
reveal soft first heart sound, loud diastolic gallop and soft pansystolic
murmur of mitral regurgitation. Pulmonary congestion produces bibasal
rales over lung fields. Right heart failure is diagnosed by the presence of
tender hepatomegaly, ascites and dependant edema.

2. Hypertropic cardiomiopathy
Kardiomiopati hipertrofi merupakan kardiomiopati dengan ciri yang
dominan adalah terjadinya hipertrofi otot jantung. Etiologinya adalah
kelainan genetik dengan pola penuruan autosomal dominan.

3. Restrictive cardiomiopathy
Kardiomiopati resktriktif merupakan kardiomiopati dengan ciri kekakuan
ventrikel yang abnormal serta gangguan dalam pengisian ventrikel. Angka
kejadian kardiomiopati jenis ini lebih jarang dibandingkan kedua jenis
kardiomiopati lainnya.3-5 Etiologi dari keadaan ini adalah idiopatik,
genetik, radiasi, infiltrasi (amiloid, sarkoidosis, hemokromatosis,glycogen),
skleroderma.