A Rare Case Report of Thoracic Ectopia Cordis: An Obstetrician’s Point of View in Multidisciplinary Approach

Abstract

Ectopia cordis is a rare congenital anomaly associated with the heart positioned outside of the thoracic cavity either partially or completely. It can be associated with other congenital abnormalities. Overall, the prognosis for infants with ectopia cordis is very poor but depends greatly on the type and severity of ectopia cordis and intracardiac and associated malformations. We present one case of a fetus with prenatally diagnosed thoracic ectopia cordis with intracardiac defects and omphalocele, all the abnormalities seen in pentalogy of Cantrell except a diaphragmatic defect. Considering poor prognosis for fetus, conservative management of prenatal care has been chosen. At the 42nd gestational week, during the active stage of labor, due to fetal distress, cesarean section was performed at a tertiary level hospital. The condition of the infant was impairing rapidly and the newborn succumbed within 24 hours. We discuss the perinatal care concerning this rare anomaly.

Cantrell Syndrome—A Rare Complex Congenital Anomaly: A Case Report and Literature Review

“…The surgical management of EC primarily involves 4 steps: soft tissue coverage of the heart, replacement of the heart into the thoracic cavity, repair of the intracardiac defects, and reconstruction of the chest wall ( 21 ). Therefore, optimal management of these patients requires coordination between a multidisciplinary team involving a neonatologist, a radiologist, a pediatric surgeon, a cardiologist, a pediatric cardiac surgeon, a plastic surgeon, and experienced nurses ( 25 ). Nevertheless, the general prognosis depends upon the promptitude of surgical intervention, which in our case was postponed owing to the patient's unstable status.…”

Ectopia cordis: A case report of pre-surgical care in resource-limited setting

“…Cervical type is said to be incompatible with life. Thoracic EC has poor prognosis compared to other non-cervical types, but some survive [ 5 ]. Many succumb to the malformations with in the first few hours of life [ 1 ].…”

Ectopia cordis in an adult patient with COVID-19: A case report and literature review

“…The prognosis of thoracic EC is generally poor, and those who survive usually had fewer cardiac defects 18 . The morbidity and mortality of EC are reduced when there is no concurrent omphalocele, such as in the case of our patient.…”