Multidetector Computed Tomographic Angiography Imaging of Pentalogy of Cantrell

Abstract

Figure. A, Clinical picture of the patient reveals large ventral herniation of the heart into lower chest and upper abdomen (arrow) along with ventral bowel herniation through the umbilicus (arrowhead) suggestive of omphalocele. B, Echocardiogram through the ectopia cordis demonstrates overlap of ascending aorta (aAO) with small-caliber partially visualized main pulmonary artery (mPA) arising from the ventricle. C, Sagittal 3-dimensional volume-rendered computed tomographic image reveals lower sternum defect (arrowhead), ventral ectopia cordis (bold arrow), and an omphalocele (thin arrow). D and E, Maximum-intensity projection (D) and volume-rendered images (E) demonstrate ventrally herniated single ventricle (SV) with rightward apex, giving rise to tetralogy of Fallot-type conotruncus and diffusely hypoplastic main pulmonary artery, which branches into the hypoplastic right pulmonary artery (triangular arrowhead) with absent left pulmonary artery originating from undersurface of aortic arch (thin arrow) by way of patent ductus arteriosus (bold arrow). Note patent ductus arteriosus (bold arrow) extending between the inferior aspect of aortic arch and the left pulmonary artery (thin arrow). Additional findings include aberrant right subclavian artery (short arrowhead) from the proximal descending aorta.by guest on

Images of Extremely Rare Cantrell Phenomenon

“…In the first days of life, treatment involves surgical, palliative repair of the abdominal hernia and diaphragmatic defect, as well as palliative or corrective repair of cardiovascular abnormalities. However, mortality in complex operations performed in very severe cases remains high [5]. Multiple surgical interventions should be avoided due to the high risk of wound infection [6].…”