All issues > Volume 42(7); 1999

Case Report

J Korean Pediatr Soc. 1999;42(7):1026-1030. Published online July 15, 1999.

A Case of Isolated Right Ventricular Hypoplasia without Tricuspid Atresia or Pulmonary Atresia

Eui Jun EJ Yang¹, Sun Young SY Kim¹, Sank Ook SO Nam¹, Hee Ju HJ Park¹, Nam Hee NH Kwak²

¹Department of Pediatrics, College of Medicine, Pusan National University, Pusan, Korea
²Department of Pediatrics, Munwha Hospital, Pusan, Korea

Correspondence Eui Jun EJ Yang ,Email: 1

Abstract

Isolated right ventricular hypoplasia, unassociated with severe pulmonary or tricuspid valvar malformations, is a rare primary congenital cardiac anomaly in which of the trabecular portion of right ventricle fails to develop. An atrial septal defect or a patent foramen ovale serves as an escape valve. We observed a 2-day-old neonate with this disorder who suffered from cyanosis. The diagnosis was made by Doppler-echocardiography which revealed marked reduction in right ventricular size and right-to-left shunt through the atrial septal defect. There was no other cardiac malformation such as pulmonary atresia or tricuspid atresia.

Keywords :Isolated right ventricular hypoplasia, Congenital heart disease