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. Author manuscript; available in PMC: 2011 Nov 1.
Published in final edited form as: Circ Cardiovasc Imaging. 2010 Nov 1;3(6):753–765. doi: 10.1161/CIRCIMAGING.110.957563

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.