TITLE: DILATED AND RESTRICTIVE CARDIOMYOPATHIES
AUTHORS: JAMES O. O'NEILL, MD -- Department of Cardiovascular Medicine
   CORRINE BOTT-SILVERMAN, MD -- Department of Cardiovascular Medicine
REVIEWED: DECEMBER 29, 2003
Table 1:
World Health Organization Classification of Cardiomyopathies
Cardiomyopathy
Definition
Sample Etiologies
Dilated Dilated left/both ventricle(s) with impaired contraction Ischemic, idiopathic, familial/genetic, immune, alcoholic, toxic, valvular
Hypertrophic Left and/or right ventricular hypertrophy Familial with autosomal dominant inheritance (see chapter on Hypertrophic Cardiomyopathy)
Restrictive Restrictive filling and reduced diastolic filling of one/both ventricles. Normal/near normal systolic function Idiopathic, amyloidosis, endomyocardial fibrosis
Arrhythmogenic right ventricular cardiomyopathy Fibro-fatty replacement of right ventricular myocardium,Uhl's anomaly ("parchment heart") Unknown. Familial, usually autosomal dominant inheritance, with incomplete penetrance. Autosomal recessive inheritance may also occur. Rare forms associated with typical phenotype, eg, Naxos disease.
Unclassified Not typical for previous 4 groups Fibroelastosis, noncompacted myocardium, systolic dysfunction with minimal dilatation, mitochondrial diseases
Adapted from reference 1.

 

 

 

 

 

 

 

 

 

 

Table 2:
Causes of Cardiomyopathy
Type
Cause
Cardiovascular
  • Ischemic heart disease
  • Hypertension
  • Valvular heart disease
  • Idiopathic dilated cardiomyopathy
  • Idiopathic restrictive cardiomyopathy
  • Cardiac amyloidosis
Metabolic
  • Starvation, vitamin deficiency
  • Diabetes, hypo- and hyperthyroidism, acromegaly, pheochromocytoma
  • Glycogen storage disease
Infectious/
Inflammatory
  • Secondary amyloidosis, sarcoidosis
  • Acute viral (coxsackie B), HIV, hepatitis C
  • Protozoal: Chagas' disease
Toxic
  • Alcohol, cocaine, amphetamines, chemotherapy
Genetic
  • Familial dilated cardiomyopathy
  • Familial cardiac amyloidosis
  • Noncompacted myocardium
  • Systolic dysfunction without dilatation
  • Arrhythmogenic right ventricular cardiomyopathy
  • Hemochromatosis
Tachycardia
  • Tachycardia-induced cardiomyopathy
Pregnancy
  • Peripartum cardiomyopathy
  

 

Table 3:
American Heart Association/American College of Cardiology Staging of Heart Failure
Stage
Description
A
 Patients at risk of heart failure, with no structural heart disease
B
 Patients with structural heart disease, without symptoms of heart failure
C
 Patients with past or present heart failure symptoms
D
 Patients with advanced disease
(eg, inotropic support)
  

 

 

 

 

 

Table 4:
Diagnostic Features of Arrhythmogenic
Right Ventricular Cardiomyopathy
Test
Features
Electrocardiogram Epsilon waves (slurred ST segments) V1-3, inverted T waves V2,V3*) in absence of right bundle branch block
Echocardiography Localized RV aneurysm, isolated RV failure
Magnetic resonance imaging Fatty infiltration of RV
Histology Fatty infiltration of RV
* In patients >12 years old; RV = right ventricle

 

Table 5:
Predictors of Poor Outcome in Dilated Cardiomyopathy
Test
Features
Clinical Increased age, male gender, ischemic heart disease, diabetes, syncope, right heart failure symptoms, symptomatic ventricular arrhythmias, persistent gallop rhythm, persistent jugular venous distention, systemic hypotension, peripheral vascular disease
Laboratory Hyponatremia, persistently elevated B-type natriuretic peptide and A-type natriuretic peptide levels, elevated norepinephrine and renin levels
Electrocardiogram Left bundle branch block, first- and second-degree atrioventricular block
Echocardiogram Increased ventricular dimensions, reduced ejection fraction, restrictive diastolic filling pattern, severe mitral and/or tricuspid regurgitation
Chest radiograph Increased cardiothoracic ratio
Coronary angiography Multivessel obstructive disease
Hemodynamic data Pulmonary capillary wedge pressure >20 mm Hg, cardiac index <2.5 L/min/m2, pulmonary hypertension, elevated central venous pressure
Cardiopulmonary exercise test Maximal systemic oxygen uptake <12 mL/kg/min
Endomyocardial biopsy Loss of intracellular myofilaments
  

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