Double Aortic Arch Presenting with Neonatal Respiratory Distress But not Due to Airway Obstruction: Anatomic and Embryologic Explanation

Abstract

Double aortic arch resulting from embryonic persistence of bilateral fourth branchial arches often leads to neonatal stridor due to tracheal compression. Two neonates presented with dyspnea without evidence of tracheal compression. An aneurysmal ductal aneurysm from the left-sided arch in the first neonate led to symptoms. Spontaneous duct closure and regression of ductal aneurysm led to relief of symptoms. Second neonate had severe neonatal pulmonary hypertension due to aortic origin of left pulmonary artery (LPA) that was disconnected from main PA. Surgical reanastomosis of the LPA and division of the smaller right arch corrected the anatomy. These two associations show that airway obstruction alone is not the cause of respiratory symptoms in neonates with double aortic arch.