CardiomyopathyIsolated Left Ventricular Noncompaction in Identical Twins
Section snippets
Case Descriptions
A 35-year-old black man was diagnosed and treated for new-onset heart failure 6 weeks before being evaluated at a tertiary academic cardiomyopathy clinic. He was categorized in New York Heart Association functional class I and was receiving heart failure therapy, which included a loop diuretic, carvedilol, an angiotensin-converting enzyme inhibitor, and spironolactone. His cardiac history before the recent admission was unremarkable, with no preceding viral or systemic illnesses. In addition,
Comments
ILVNC is thought to be a result of failure of the myocardium to compact during embryogenesis and is presumed to be a genetic cardiomyopathy.1, 6 Heart failure, arrhythmias (which can be life threatening), and cardioembolism are the most important clinical manifestations.7
Current evidence suggests that familial and sporadic forms of ILVNC occur. Familial occurrence varies from 15% to 64%.8, 9 The mode of inheritance in adults is usually autosomal dominant compared to children, in whom X-linked
Acknowledgment
We gratefully acknowledge Barbara Danek, Joe Grundle, and Katie Klein for editorial preparation of the report and Brian Miller and Brian Schurrer for their help with the figures.
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