Left Ventricular Non-compaction Cardiomyopathy

Abstract

Introduction: Noncompaction cardiomyopathy (NCCM) is a rare and considerably young entity characterized by a two-layered left ventricular myocardium composed of a thin compacted epicardium and a thick trabeculated endocardium with deep intertrabecular recesses, as a result of morphogenetic abnormalities in embryological development. Its clinical manifestation is widely variable, from asymptomatic to arrhythmias, thromboembolic events and heart failure. Materials and Methods: Several echocardiographic criteria have been proposed and were taken into consideration for the diagnosis of the two patients with NCCM we present. Cardiac magnetic resonance, for its superiority in imaging of the apex and endocardial fibrosis, and 24-hour Holter ECG monitoring, to determine any possible clinically significant arrhythmias, were also used. Results: Our patient A is a 47-year-old woman, who presented with acute left-sided heart failure (EDV 113ml; ESV 75ml; EF 33`5%), precipitated by a hypertensive crisis (BP 220/120), while our patient B is a 68-year-old man with congestive heart failure, NYHA class III (EDV 124ml; ESV 67ml; EF 45`8%), who had history of complete AV block, subendocardial infarction and several comorbidities. Echocardiography showed in both cases systolic and diastolic dysfunction, moderate mitral regurgitation, mild tricuspid regurgitation and the characteristic myocardial structure of NCCM, which was further confirmed by cardiac magnetic resonance. Additionally, the ECG of patient A demonstrated intraventricular conduction abnormalities, LA enlargement and nonspecific ST-T changes. Conclusion: NCCM still waits for unification of diagnostic criteria and guidelines, which would not only have an impact on its prevalence, but also allow better management and prevention of its complications. Until then, the need for a comprehensive approach, including several diagnostic criteria and investigations, is emphasized.