Double aortic arch and tetralogy of fallot with pulmonary atresia

Abstract

Double aortic arch (DAA) is the most common form of complete vascular ring and results in respiratory and feeding problems because the trachea and the esophagus are circumscribed by the ring. It often occurs as an isolated anomaly; however, DAA may be associated with other congenital cardiovascular anomalies such as the tetralogy of Fallot (TOF), transposition of the great arteries, coarctation of the aorta, ventricular septal defect, and patent ductus arteriosus. [1-3] The coexistence of TOF and DAA is a rare entity. [2,4] We herein present our experience with a patient who was diagnosed with symmetrical DAA in conjunction with TOF along with pulmonary atresia, which resulted in tracheoesophageal compression symptoms. CASE REPORT A six-day-old girl weighing 2.37 kg was referred to our hospital with a diagnosis of pulmonary atresia and TOF. She was intubated immediately after birth due to severe respiratory problems. A physical examination showed the typical findings of TOF, and echocardiography confirmed the diagnosis of TOF with pulmonary atresia. Through a median sternotomy, a shunt was inserted between the right innominate artery and the right pulmonary artery using a 3.5 mm polytetrafluoroethylene (PTFE) graft. An intraoperative evaluation detected the presence of DAA, with both arches being patent and about equal in size. Postoperative multislice computed tomography (MSCT) confirmed the diagnosis of symmetrical DAA. The right subclavian and right common carotid arteries originated from the right aortic arch, whereas the left common carotid and left subclavian arteries originated from the left aortic arch (Figure 1). The trachea and esophagus were circumscribed and compressed by the vascular ring, resulting in tracheal stenosis (Figure 2). In the second operation, a left posterolateral thoracotomy was performed in which the right arch