ARYA Atherosclerosis Journal

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

Document Type : Case Report

Authors

Zahra Alizadeh Sani 1
Mohammad Vojdanparast 2
Nahid Rezaeian 1
Azin Seifi 3
Sahar Omidvar Tehrani 4
Pouya Nezafati 4

1 ‎Assistant Professor, Rajaie Cardiovascular Medical and Research Center, Iran University of ‎Medical Sciences, Tehran, Iran

2 ‎Cardiologist, Cardiovascular Research Center, School of Medicine, Mashhad University of ‎Medical Sciences, Mashhad, Iran

3 ‎Student of Medicine, Department of Medical Sciences, School of Medicine, Islamic Azad ‎University, Mashhad Branch, Mashhad, Iran

4 ‎Student of Medicine, Cardiac Surgery Research Committee‏ ‏AND Student Research ‎Committee, Mashhad University of Medical Sciences, Mashhad, Iran

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
ARYA Atherosclerosis Journal
Volume 12, Issue 1 - Serial Number 1
January and February 2016
Pages 50-54

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