The Rare Association of Truncus Arteriosus with a Cervical Double Aortic Arch Presenting with Left Main Bronchial Compression

Abstract

Truncus arteriosus, a double aortic arch, and a cervical aortic arch are all rare cardiovascular anomalies. We experienced a unique female newborn with the rare combination of truncus arteriosus with a cervical double aortic arch, which probably resulted from abnormal persistence of the bilateral 2nd or 3rd rather than the 4thembryonic aortic arches and failure of regression of the right 8th somitic segment of the right dorsal aorta. She presented with respiratory distress soon after birth, which was initially attributed to the vascular ring and hypertensive pulmonary arteries. Our inability to relieve her respiratory compromise by surgical division of the vascular ring and main pulmonary artery banding prompted the diagnosis of left main bronchial compression caused by a posteriorly displaced dilated ascending aorta that compressed the right pulmonary artery and left main bronchus against the descending aorta. The patient then underwent successful left main bronchus stent implantation. We speculate the cervical double aortic arch is redundant in nature and is a loose ring that may not cause tracheal compression. Nevertheless, a posteriorly displaced dilated ascending aorta in patients with truncus arteriosus may compress the right pulmonary artery and the main bronchus on the side of the aortic arch against the descending aorta.

Truncus arteriosus with double aortic arch: A rare association

“…Patent foramen ovale is frequent, atrial septal defect is observed with about 10 % of the cases. Also atrioventricular discordance, situs inversus with dextrocardia, double inlet ventricle, atrioventricular valve stenosis and atresia, hypoplastic left ventricle, atrioventricular canal defect, vascular ring may be accompanied 10 .…”

Prenatal diagnosis of the rare association of common arterial trunk and double aortic arch

“…We report the 10th known case of common arterial trunk with double aortic arch, and the first in utero diagnosis in the literature . Other reported cases have presented with respiratory symptoms of a vascular ring, which most commonly include respiratory distress and even respiratory arrest in infants.…”

Common arterial trunk and double aortic arch: a rare association

“…Common arterial trunk (CAT) is a rare congenital malformation characterized by a single arterial trunk arising from the heart, overriding the interventricular septum and gives rise to the systemic, pulmonary, and coronary circulations 1 . The incidence of defect is 6-10/100,000 live births, it is occasionally associated with aortic arch anomaly such as right aortic arch and aortic arch interruption 1,2 . Double aortic arch (DAA) is the most common vascular ring, and results from persistence of both 4 th arches, compressing the trachea or esophagus or both 2 .…”