Table 3.
Definitions of cardiomyopathy phenotypes. Cardiomyopathy is defined as a myocardial disorder in which the heart muscle is structurally and functionally abnormal in the absence of any other disease sufficient to cause the observed myocardial abnormality
Phenotype | Definition |
---|---|
Hypertrophic cardiomyopathy (HCM) | Diffuse or regional increased LV wall thickness with a nondilated LV chamber. |
Restrictive cardiomyopathy (RCM) | |
Endomyocardial form | Characterized macroscopically by prominent endocardial scar that usually bridges the interventricular septum and LV free wall, and may cause fixed, mid‐LV obstruction and often apical LV thinning or aneurysm; LA or biatrial enlargement is generally present. |
Myocardial form | Normal LV dimensions (including wall thickness) with LA or biatrial enlargement |
Dilated cardiomyopathy (DCM) | LV systolic dysfunction characterized by progressive increase in ventricular dimensions, normal or reduced LV wall thickness, and atrial dilatation. |
Arrhythmogenic cardiomyopathy (AC), also known as arrhythmogenic right ventricular cardiomyopathy (ARVC) or dysplasia (ARVD) | Severe RA and RV dilatation and often, RV systolic dysfunction and RV wall thinning. The left heart may also be affected. Arrhythmias and right‐sided congestive heart failure are common. |
Nonspecific phenotype | A cardiomyopathic phenotype that is not adequately described by the other categories; the cardiac morphology and function should be described in detail |