Type B Interrupted Aortic Arch with a Patent Ductus Arteriosus in an Adult Presenting with Secondary Polycythaemia

Abstract

Interrupted aortic arch (IAA) is an extremely rare congenital cyanotic heart disease characterized by complete disruption between the ascending and descending aorta. A patent ductus arteriosus (PDA) or other collateral pathways provide blood flow to the distal descending aorta. Mortality is extremely high at early infancy, particularly after the closure of ductus arteriosus. Survival and presentation in adulthood are extremely rare. Here we illustrate a rare case of type B interrupted aortic arch in an adult who presented with secondary polycythaemia. The blood supply to descending aorta and beyond is almost solely by a patent ductus arteriosus. The case demonstrates the value of multimodality imaging including CT and MRI for diagnosis and treatment planning in these patients.

Keywords

• interrupted aortic arch
• congenital heart disease
• secondary polycythaemia
• computed tomography
• magnetic resonance imaging