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

Zahra Alizadeh Sani; Mohammad Vojdanparast; Nahid Rezaeian; Azin Seifi; Sahar Omidvar Tehrani; Pouya Nezafati

Vol 12, No 1 (2016):50-54

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

Zahra Alizadeh Sani, Mohammad Vojdanparast, Nahid Rezaeian, Azin Seifi, Sahar Omidvar Tehrani, Pouya Nezafati

Abstract

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.

DISCUSSION: 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.

Keywords

Cardiomyopathy; Hypoplasia; Magnetic Resonance Imaging Scan; Sudden Cardiac Death

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