A Case of Antenatal Diagnosis of Ectopia Cordis with Cardiac Disease

Fariba Rashidighader; Mohammadrafie  Khorgami; Elio Caruso; Farruggio Silvia; Reza Masihi

doi:10.18502/crcp.v8i1.13098

Fariba Rashidighader Rajaie Cardiovascular Medical and Research Center, Iran University of Medical Sciences, Tehran, Iran.
Mohammadrafie Khorgami Rajaie Cardiovascular Medical and Research Center, Iran University of Medical Sciences, Tehran, Iran.
Elio Caruso Department of Pediatric Cardiology,Mediterranean Pediatric Cardiology Center "Bambin Gesù", San Vincenzo Hospital, Taormina, Italy
Farruggio Silvia Department of Pediatric Cardiology,Mediterranean Pediatric Cardiology Center "Bambin Gesù", San Vincenzo Hospital, Taormina, Italy
Reza Masihi Rajaie Cardiovascular Medical and Research Center, Iran University of Medical Sciences, Tehran, Iran.

DOI: https://doi.org/10.18502/crcp.v8i1.13098

Keywords: Ectopia cordis; Pregnancy; Prenatal diagnosis; Heartdisease

Abstract

Ectopic cordis is a rare anomaly with incidence of 5.5 to 7.9 per one million. In this anomaly, fetal heart is displayed towards outside of the thoracic cavity partially or completely. We describe a case of antenatal diagnosis of Thoracic Ectopia Cordis (EC) with CongenitalHeartDisease (CHD) without any other extracardiac malformations. The mother was referred to our center at 18 weeks of gestation due to abnormal sonography. Fetal Echocardiographic examination showed isolated thoracic ectopia cordis with Double Outlet Right Ventricle (DORV), large inlet to outlet Ventricular Septal Defect (VSD), malposition of Aorta, Pulmonary Atresia with retrograde flow, narrow Pulmonary Artery (PA) and PA branches. Amniocentesis and sonography revealed no other anomaly or chromosomal derangement. Because the diagnosis of Ectopia cordis may be difficult in the fetal period due to multiple factors, meticulous attention should be paid for true diagnosis.