Author/Authors :
Alizadeh Sani, Zahra Iran University of Medical Sciences, Tehran , Vojdanparast, Mohammad School of Medicine - Mashhad University of Medical Sciences, Mashhad , Rezaeian, Nahid Iran University of Medical Sciences, Tehran , Seifi, Azin Department of Medical Sciences - School of Medicine - Islamic Azad University - Mashhad Branch, Mashhad , Omidvar Tehrani, Sahar Mashhad University of Medical Sciences, Mashhad , Nezafati, Pouya Mashhad University of Medical Sciences, Mashhad
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.
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.
Keywords :
Cardiomyopathy , Hypoplasia , Magnetic Resonance Imaging Scan , Sudden Cardiac Death
