Why the study?
Does late gadolinium enhanced CMR accurately determine cardiac involvement in patients with lamin A/C gene mutation related dilated cardiomyopathy?
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.
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May aid noninvasive assessment in lamin A/C cardiomyopathy; leaves open prospective validation before routine adoption.
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.
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Holmström et al. (2011) studied this question.
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