Clinical & Experimental Cardiology
Open Access
ISSN: 2155-9880
ISSN: 2155-9880
Naba Raj Mainali
Posters: J Clin Exp Cardiolog
Introduction: Non compaction cardiomyopathy (NCCM) is a rare disorder (reported incidence of 3 to 4% among heart failure patients) caused by the arrest of myocardial compaction during embryogenesis, leading to a non-compactedendocardial layer with marked trabeculations. Here we report a case ofa NCCM presenting with left sided heart failure and left ventricular thrombus. Case: 29 years old male with no prior medical history presented with increasing shortness of breath and orthopnea for ten days. He denied alcohol and drug abuse. Family history was negative for any cardiac disease. Examination revealed distended neck veins, bibasilar cracklesand S3. EKG showed normal sinus rhythm with QRS duration of 130ms. An echocardiogram demonstrated non compacted ventricles, global left ventricular hypokinesia (ejection fraction 17%) with left ventricular thrombus. Left and right cardiac catheterization showed clean coronaries and normal hemodynamics respectively. He was started on standard heart failure regimen and anticoagulation. Since 3 months follow up echocardiogram was unchanged, he underwent placement ofbiventricular pacing andintracardiac defibrillator with anticipation of heart transplant in the future. Discussion: Although a rare form ofcardiomyopathy, non-compaction is being recognized more frequently than before due to increased awareness about its natural history, clinical manifestations and improvedmodalities of cardiac imaging. It usually presents with left heart failure. If not recognized early it can present with fatal arrhythmias, sudden cardiac death and systemic embolism which can lead to significant morbidity and mortality. Echocardiography is diagnostic and revealstrabeculated myocardium with thick non-compacted layer to compacted layer ratio> 2. Familial occurrence is frequent with autosomal dominant and X-linked transmissions. Early cardiology referral and genetic testing of the first degree relatives are important. Cardiac transplantation is the treatment of choice