Persistent 5th aortic arch with coexisting discontinuous pulmonary arteries, double-outlet right ventricle, and complete atrioventricular septal defect.

Abstract

A diabetic mother's newborn female infant, delivered at 34 weeks of gestation at a birth weight of 4.8 lb, was transferred to our cardiac intensive care unit for treatment. The neonate had a heart rate of 141 beats/min, a respiratory rate of 33 breaths/min, and an oxygen saturation of 100% on room air. She had been diagnosed prenatally with a complete unbalanced atrioventricular septal defect and double-outlet right ventricle. A transthoracic echocardiogram confirmed the diagnosis of complete unbalanced, right-dominant atrioventricular septal defect, double-outlet right ventricle with the aorta to the right of the pulmonary artery (PA), a subaortic ventricular septal defect, and moderate-to-severe pulmonary stenosis. Additional echocardiographic evaluation revealed a left aortic arch with a persistent 5th aortic arch, and the left PA was found to be discontinuous with the main PA (Figs. 1–4). Results of cardiac catheterization confirmed the diagnosis of a persistent 5th aortic arch and a left PA arising from the ascending aorta (Fig. 5). Fig. 1 Transthoracic echocardiogram (long-axis view from the suprasternal notch) shows the persistent 5th aortic arch (A) and the origin of the left pulmonary artery (B) from the ascending aorta (C). Fig. 5 Angiogram of the ascending aorta (anteroposterior view with ascending aortic injection) shows the persistent 5th aortic arch (A), and the anomalous left pulmonary artery (B) arising from the ascending aorta (C). Fig. 2 Color-flow Doppler echocardiogram (long-axis view from the suprasternal notch) shows the persistent 5th aortic arch (A) and the origin of the left pulmonary artery (B) from the ascending aorta (C). There was turbulent flow from the ascending aorta into ... Fig. 3 Transthoracic echocardiogram (modified long-axis view from the suprasternal notch) reveals the persistent 5th aortic arch (A) and left aortic arch (B). The origin of the left pulmonary artery from the ascending aorta (C) is seen. Fig. 4 Color-flow Doppler echocardiogram (modified long-axis view from the suprasternal notch) reveals the persistent 5th aortic arch (A) and left aortic arch (B). The origin of the left pulmonary artery from the ascending aorta (C) is seen. There was turbulent ...