Interrupted Aortic Arch in an adult

Complete Interruption of Aortic Arch and Non-Immune Hydrops Fetalis: A Case Report with Autopsy

“…Interrupted aortic arch (IAA) occurs in three births per million, and accounts for approximately 1% of congenital heart diseases (CHDs). [1][2][3] The first case reported with these anomalies appears to be the one by Seidel in 1818. 1 IAA is a severe form of CHD characterized by a lack of luminal continuity between the ascending and the descending thoracic aorta.…”

“…4 The two most common associated cardiac anomalies are ventricular septal defect (90%) and patent ductus arteriosus (98%). 4 Isolated IAA is very uncommon 2,4 and it is considered incompatible with life. 3 To understand the pathogenesis of IAA, we must remember the embryological development of the main vessels.…”

“…The median age at death in untreated IAA with associated cardiac anomalies is 10 days. 2 In the embryo, the superior vena cava returns venous blood from the upper body. This less oxygenated blood leaves the heart via the pulmonary artery.…”

“…The umbilical vein transports oxygenated blood to the right auricle via the hepatic veins, ductus venosus and inferior vena cava; the main part flows through the ventricular septal defect into the left side of the heart, 2 and from there it is distributed to the head and arms. 1,2 In this type of circulation, in case of complete loss of continuity between the ascending and descending aorta it is important the re-establishment of a communication that allows the oxygenated blood that reaches the left heart passes into the right side for adequate supply of the lower portion of body. 2 The absence of a septal defect and a patent ductus arteriosus is incompatible with extra-uterine life.…”

“…1,2 In this type of circulation, in case of complete loss of continuity between the ascending and descending aorta it is important the re-establishment of a communication that allows the oxygenated blood that reaches the left heart passes into the right side for adequate supply of the lower portion of body. 2 The absence of a septal defect and a patent ductus arteriosus is incompatible with extra-uterine life.…”