Left ventricular apical hypoplasia: Case report on cardiomyopathy and a history of sudden cardiac death

Background: isolated left ventricular apical hypoplasia with several different unrecognized dimensions is a newly discovered congenital anomaly of the heart. case report: in this report,we describe a case of cardiomyopathy of this type occurring in a 13-year-old male with a history of mental retardation and sudden cardiac death (scd) of second-degree relatives. the patient was referred for an evaluation of cardiac status. an echocardiography analysis demonstrated a spherical left ventricle (lv) appearance with mild mitral regurgitation. cardiac magnetic resonance imaging (mri) confirmed a spherical and truncated lv appearance. the right ventricle was found to have elongated and wrapped around the lv,and diverticulum was also seen in the cardiac mri.conclusion: to the best of our knowledge,this is to present the first case of lv apical hypoplasia combined with lv diverticulum and a family history of scd. as more cases featuring this cardiomyopathy type are recognized,it will be easier to elucidate the natural history and management of such cardiac anomalies. © 2016,isfahan university of medical sciences(iums). all rights reserved.