Purpose or Case Report: Ectopia cordis is a rare congenital condition where the heart is located outside the chest cavity, often associated with midline defects like omphalocele and Pentalogy of Cantrell. This condition presents significant challenges in surgical management and has a high mortality rate due to complications related to cardiac and associated anomalies.

A newborn delivered at 37 weeks gestation was admitted for surgical management of ectopia cordis and omphalocele. Prenatal imaging suggested Pentalogy of Cantrell. An echocardiogram was performed, but visualization was limited due to the heart’s external position, hindering comprehensive assessment. A follow-up CT angiogram confirmed ectopia cordis with complex cardiac and vascular findings. The left-sided superior vena cava drained into the coronary sinus, and the inferior vena cava was dilated, draining into the right atrium. The right atrium was dilated, while the left atrium was elongated with normal pulmonary venous drainage. A 4 mm atrial septal defect and a possible 2 mm perimembranous ventricular septal defect were noted. The right ventricle appeared normal, but the left ventricle was hypoplastic. The aortic root and ascending aorta were hypoplastic, with Z-scores ranging from -3.20 to -4.90, while the aortic isthmus and descending aorta were within normal limits. The pulmonary arteries were normal in size, and a large PDA was observed.
3D modeling was used to assess the feasibility of surgical repair and chest closure. The heart outside the chest measured 30.65 ml, and the total intrathoracic volume was 107.31 ml. The combined volume of the lungs and pleural effusions totaled 77.69 ml, leaving limited space for the heart within the thoracic cavity. This made the prospect of repositioning the heart into the chest highly challenging, with significant risk of compressing the lungs or other critical structures. The baby passed away during the hospital stay.
This case highlights the role of CTA and 3D modeling in congenital heart disease, illustrating how these tools can assist in evaluating spatial constraints and informing surgical decisions in complex conditions like ectopia cordis.
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