Early Prenatal Echocardiographic Diagnosis of Cantrell Pentology with Hypoplastic Right Heart Syndrome


  • The authors wish to acknowledge the use of Saudi ARAMCO Medical Services Organization (SAMSO) facilities for the data and study that resulted in this article. The authors were employed by Saudi Aramco during the time the study was conducted and the article written.

Address for correspondence and reprints: Mohamed A. Seliem, M.D., FACC, Consultant Pediatric Cardiologist, Specialty Pediatric Services Division, Aramco-Dhahran Health Center, Box 76, Dhahran 31311, Saudi Arabia. Fax: 966–3–877–3792.


Pentology of Cantrell is a complex syndrome involving the thoracic and abdominal walls, the diaphragm, and the heart. We report a case of that syndrome diagnosed early in pregnancy (16 weeks gestation) by fetal echocardiogram. The fetus had a severe form of hypoplastic right heart syndrome. Early diagnosis of such complex cardiac anatomy together with other severe anatomical malformations is important in planning the surgical intervention. It also shows the intimate relationship between the developmental anatomy and physiology of the fetal heart.