J Korean Pediatr Soc.  1999 Jul;42(7):1026-1030.

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

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

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.

Keyword

Isolated right ventricular hypoplasia; Congenital heart disease

MeSH Terms

Cyanosis
Diagnosis
Foramen Ovale, Patent
Heart Defects, Congenital
Heart Septal Defects, Atrial
Heart Ventricles
Humans
Infant, Newborn
Pulmonary Atresia*
Tricuspid Atresia*
United Nations
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