Multidetector Computed Tomographic Angiography Imaging of Pentalogy of Cantrell

A 4-day-old female mildly cyanotic neonate presented clinically with ventral herniation of the heart to the anterior abdominal wall and umbilical defect containing bowel loop (Figure [A] and Movie I in the online-only Data Supplement), raising suspicion of pentalogy of Cantrell. Echocardiogram of the ectopic heart, although technically difficult, revealed overlap of the ascending aorta with small-caliber partially visualized main pulmonary artery arising from the ventricle (Figure [B]). Multidetector computed tomographic angiography (MDCTA) confirmed the diagnosis of pentalogy of Cantrell, which consisted of ectopia cordis with rightward apex containing ventrally herniated single ventricle with right- sided atrioventricular valve atresia protruding through the defective anterior diaphragm anterior to the liver, omphalocele, lower sternal and ventral thoracoabdominal wall defects, and multiple cardiac anomalies (Figure [C through E] and Movie II in the online-only Data Supplement). Cardiovascular anomalies included single ventricle with right-sided atrioventricular valve atresia giving rise to tetralogy of Fallot–type conotruncus with diffusely narrowed main pulmonary artery. Additional anomalies included large secundum atrial septal defect, absent left pulmonary artery originating from the undersurface of the aortic arch …