Sustained ventricular tachycardia in a patient with isolated non-compaction cardiomyopathy

Abstract

Isolated non-compaction of the left ventricular myocardium (INVM) was first described in 1984 as an unclassified cardiomyopathy, not being dilated, hypertrophic or restrictive. It is assumed to occur as a result of an arrest in endomyocardial morphogenesis during normal development of the heart. The disease is characterised by heart failure due to systolic and diastolic left ventricular (LV) dysfunction, systemic emboli and ventricular arrhythmias. Echocardiography has been shown to be the method of choice in diagnosis. INVM is a rare congenital cardiomyopathy and only a few cases of this condition have been reported. It is characterised by prominent and excessive trabeculation in a ventricular wall segment, with deep inter-trabecular spaces perfused from the ventricular cavity. We report a case of INVM with ventricular tachycardia induced during electrophysiological study in a 24-year-old female patient with a family history of sudden death.