Volume 33, Nº 1, January and february 2020
DOI: http://www.dx.doi.org/10.5935/2359-4802.20190045
CASE REPORT
Complete Interruption of Aortic Arch and Non-Immune Hydrops Fetalis: A Case Report with Autopsy
Cesar Cilento Ponce
Abstract
Interrupted aortic arch (IAA) occurs in three births per million, and accounts for approximately 1% of congenital heart diseases (CHDs).1-3 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 uncommon2,4 and it is considered incompatible with life.3 To understand the pathogenesis of IAA, we must remember the embryological development of the main vessels. At approximately the third week of embryonic life, two aortas, a dorsal and a ventral, are connected by six paired arterial branches (aortic arches). In the course of development, a series of changes occur leading to the disappearance of several aortic arches, beginning with the first, the second and the fifth pairs. The third pair of arches persists to form the common carotid and external carotid arteries, and the fourth arches persist to form the permanent arch of the aorta on the left side and the proximal segment of the subclavian artery on the right side. The proximal portions of the sixth arches become the pulmonary arteries. On the right side, the distal segment disappears, while on the left the corresponding distal segment remains as the ductus arteriosus.1 In IAA, there is a failure of development of the fourth left aortic arch, the pulmonary artery that transports the blood from the heart to the descending aorta via the ductus arteriosus. This absence of the aortic isthmus causes a discontinuity between the ascending and descending aorta.5
Keywords: Vascular Ring; Aorta/abnormalities; Aorta/surgery; Aortic Coarctation; Ductus Arteriosus; Perinatal Mortality; Autopsy.
