Hemolytic anaemia often challenges congenital heart surgery. Hereditary spherocytosis is a rare familial hemolytic anaemia. When associated with congenital heart disease, the safe performance of cardiopulmonary bypass becomes a priority. The increased risk of hemolysis during cardiopulmonary bypass could potentially lead to significant secondary organ damage. Till now, only very few reports of successful repair of a congenital heart defect in patients with hereditary spherocytosis have been reported. We report the only case of successful repair of a congenital heart defect in an infant with hereditary spherocytosis.

Isolated congenital pulmonary valve regurgitation (IPVR) is very rare. Etiologies of absent pulmonary valve and bicuspid/dysplastic pulmonary valve are among the more common congenital causes of this condition. Here we present a rare cause of IPVR caused by absent single pulmonary cusp. Review of literature to establish timing and management of isolated pulmonary regurgitation is also attempted.