Does late gadolinium enhanced CMR accurately determine cardiac involvement in patients with lamin A/C gene mutation related dilated cardiomyopathy?
CMR can accurately identify typical cardiac involvement in lamin A/C cardiomyopathy, potentially aiding in early treatment initiation.
Cardiac conduction abnormalities, mildly dilated LV and depressed systolic dysfunction are common in DCM caused by a lamin A/C gene mutation. However, other cardiac diseases may produce similar symptoms. CMR is an accurate tool to determine the typical cardiac involvement in lamin A/C cardiomyopathy and may help to initiate early treatment in this malignant familiar form of DCM.
Holmström et al. (Sat,) studied this question.