Unusual vascular ring in infant with pulmonary atresia and ventricular septal defect

Abstract

A 3-day-old infant investigated for cyanotic heart disease was found to have partial DiGeorge syndrome and pulmonary atresia with ventricular septal defect. The only source of pulmonary blood flow was a right-sided persistent ductus arteriosus which originated from an aberrant right subclavian artery. Such a vascular ring has not been described previously. Surgical treatment included a left-sided prosthetic shunt in infancy followed by resection of the ligamentum arteriosum and right subclavian artery during the second year of life.