Aborted sudden cardiac death in a child with left ventricular non‐compaction

Abstract

Left ventricular non-compaction is a rare form of cardiomyopathy believed to be the result of intrauterine arrest of compaction of the endomyocardial morphogenesis, leading to persistence of the embryonic myocardium. Clinical manifestations are highly variable, ranging from no symptoms to a progressive deterioration in cardiac function that results in congestive heart failure, systemic thromboemboli, arrhythmias, and sudden cardiac death. Presented here is the case of a 4-year-old child with a history of aborted sudden cardiac death. Following resuscitation, he was admitted to the intensive care unit with neurologic sequelae that regressed later on. Transthoracic echocardiography and magnetic resonance imaging showed numerous prominent trabeculations and deep intertrabecular recesses at the apical and anterolateral region of the left ventricle. Electrophysiologic study showed polymorphic ventricular tachycardia. An implantable cardioverter-defibrillator (ICD) was implanted following clinical recovery. Five months after implantation, appropriate ICD shock due to ventricular fibrillation was documented.

Spectrum of Cardiac Arrhythmias in Isolated Ventricular Non-Compaction

“…Five months after discharge, he experienced VF, for which he received an appropriate shock. 34 Cohen and Prahlow describe SCD in a 14-year-old AfricanAmerican girl who was found to have LVNC. She had a history of ''episodic tachycardia,'' which had been treated with ablation eight years prior.…”