A cardiomyopathy is a primary disorder of the heart muscle. It is distinct from structural cardiac disorders such as coronary artery disease, valvular disorders, and congenital heart disorders. Cardiomyopathies are divided into 3 main types based on the pathologic features (see Figure: Forms of cardiomyopathy.):
The term ischemic cardiomyopathy refers to the dilated, poorly contracting myocardium that can occur in patients with severe coronary artery disease (with or without areas of infarction). It is not classically considered to be in the above-listed categories because it does not describe a primary myocardial disorder.
Manifestations of cardiomyopathies are usually those of heart failure and vary depending on whether there is systolic dysfunction, diastolic dysfunction, or both. Some cardiomyopathies may also cause chest pain, syncope, arrhythmias, or sudden death.
Evaluation typically includes family history, blood tests, ECG, chest x-ray, echocardiography, and often cardiac MRI if available. Some patients require endomyocardial biopsy (transvenous right ventricular or retrograde left ventricular). Other tests are done as needed to determine the cause. Treatment depends on the specific type and cause of cardiomyopathy (see Table: Diagnosis and Treatment of Cardiomyopathies).
Diastolic dysfunction outflow obstruction
LV and RV failure, sudden death,
Functional AV valve regurgitation
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
Functional AV valve regurgitation
Nonspecific ST- and T-wave abnormalities
LV hypertrophy and ischemia
LV hypertrophy or low QRS voltage
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
Pulmonary venous congestion
Normal or high EDP, low EF, diffusely dilated hypokinetic ventricles AV valve regurgitation
High EDP outflow subvalvular gradient mitral regurgitation
High EDP, dip and plateau diastolic LV pressure curve
20% mortality in first year, and about 10%/yr thereafter
About 1% annual risk of sudden death
Diuretics, ACE inhibitors, angiotensin II receptor blockers, beta-blockers, spironolactone or eplerenone, digoxin, ICD, cardiac resynchronization therapy, anticoagulants
Beta-blockers verapamil disopyramide septal myotomy catheter alcohol ablation
Phlebotomy for hemochromatosis
Hydroxyurea for hypereosinophilia
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; = with or without.
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