Prenatal Diagnosis of Double Aortic Arch

Fetal echocardiography is feasible and effective for the prenatal diagnosis of congenital heart disease. Even though a vascular ring is potentially lethal, it can be difficult to diagnose in early infancy because symptoms may be absent or non-specific. We report a vascular ring with a double aortic arch (DAA) associated with a double outlet right ventricle (DORV) that was diagnosed in a male fetus at 25 weeks gestational age. Tracheal compression was noted at 36 weeks. After delivery at 39 weeks, the patient gradually developed respiratory distress and mild dysphagia with postprandial vomiting. At age one month, the double arch was surgically divided. Postoperative tracheomalacia was present. After 6 months of supportive care, biventricular total correction of the DORV was performed and a tracheal stent was implanted. Relief of respiratory distress followed. Early fetal diagnosis of vascular rings will reduce preoperative and postoperative morbidity and mortality.