A Case of Prenatal Presentation with Double Aortic Arch

Introduction

Left and right aortic arch refers to the position of the aortic arch in relation to the trachea. Normal embryological development of the arch is from the primitive pharyngeal arch system. The normal left aortic arch (LAA), descending on the left side of the trachea, is formed from the left fourth arch and the left dorsal aorta with regression of a segment of the embryological right aortic arch (RAA).

Double aortic arch (DAA) is a form of vascular ring in which the trachea and esophagus are completely encircled by the RAA and LAA. This condition causes compression of the trachea and esophagus, leading to various symptoms, including respiratory obstruction, dysphagia, chronic wheezing, vomiting, and aspiration. A late diagnosis is common, and this may aggravate tracheal damage due to persistent compression. DAA is found in approximately 1 in 2000– 4000 pregnancies on fetal echocardiography and in 1.5% of abnormal fetal echocardiography examinations. To our knowledge, there have been only a few reports on prenatal diagnosis of DAA . We recently encountered a case of DAA that was diagnosed prenatally.

Case Report

The Hokkaido University Hospital Institutional Review Board approved this study and the patient provided signed informed consent

A 36-year-old Japanese woman underwent screening echocardiography of spatiotemporal image correlation (STIC)including both grayscale and color Doppler studies at gestational week (GW) 23. The images revealed RAA and left arterial duct, but not LAA . Subsequent repeat echocardiographic examinations at GW 26, 29, 32, and 38 consistently suggested the presence of DAA with the trachea encircled by the RAA and LAA (Figure 2) and normal intracardiac anatomy. A female neonate was born by repeat cesarean section, weighing 2894 g with 1- and 5-minute Apgar scores of 8 and 9, respectively. Within 10 minutes after birth, she exhibited respiratory difficulty, including tachypnea, persistent stridor, and wheezing, and required inhaled oxygen with continuous positive airway pressure. Postnatal computed tomography (CT) angiography of the thorax confirmed the diagnosis of DAA with mild narrowing of the midtrachea in this patient. Surgical treatment, including division of the LAA and left arterial duct approached via the side of the arterial duct, was performed 10 hours after birth. The postoperative course was uneventful, and the infant left hospital at age 15 days. The infant was free from any symptoms when last seen at 1 month old.