Table 3.
Phenotypic Clues Linking Imaging to Non-HCM Genetic Cardiomyopathies*
| Imaging Phenotype | Additional Clinical Clues† | Genetic Considerations |
|---|---|---|
| Dilated cardiomyopathy with diastolic dysfunction, atrial myopathy |
Conduction system disease | Lamin A/C |
| Dilated cardiomyopathy with circumferential or confluent midwall enhancement by LGE‡ |
Acute myocarditis-like presentation | Left-dominant arrhythmogenic cardiomyopathy |
| Right ventricular dilatation, segmental contraction abnormalities, aneurysms; fibrofatty changes in myocardium |
Left bundle branch-morphology ventricular arrhythmia |
Arrhythmogenic right ventricular cardiomyopathy |
This summary relates to DCM and ARVC, and does not include findings related to hypertrophic cardiomyopathy or genetic cardiomyopathies associated with muscular dystrophies or inborn errors of metabolism.
A valuable clinical clue pointing to genetic cardiomyopathy may be obtained from a meticulous family history.
LGE = late gadolinium enhancement imaging. Note that left-dominant arrhythmogenic cardiomoypathy may present with preserved LV size and systolic function as well.