Interrupted aortic arch with transposition of great arteries and hypoplastic right ventricle - A successful repair

Interrupted aortic arch (IAA) is an uncommon anomaly, present in approximately 1.3% of patients with congenital heart disease. The association of IAA with other I conotruncal anomalies carries a substantial mortality rate.(1-3) The combination of IAA with dextro-transposition of the great arteries (d-TGA) is rare: only about 6% of children with IAA have an associated d-TGA.(1) Surgical management of these patients is complex and carries a high mortality rate. Successful surgical repair of IAA in combination with d-TGA has been reported.(4,5) Herein, we describe a patient who, in addition to IAA and d-TGA, had substantial hypoplasia of the right ventricle (RV), which made complete biventricular repair of the anomalies impossible during the neonatal period.