Surgical outcome of isolated congenital supravalvular pulmonary stenosis: a case series

Supravalvular stenosis of main pulmonary artery is a rare anomaly characterized by the presence of constriction band just above the pulmonary valve. It is mostly acquired after intervention on the pulmonary trunk or less commonly is congenital in origin associated with complex congenital cardiac malformations and very rarely can present as an isolated native congenital supravalvular pulmonary stenosis (SPS).

We present a series of four cases of isolated congenital SPS who underwent surgical correction at our tertiary care institute over 8 years. Mean age of the patients was 2.25 ± 0.96 years with all of them being males. Mean peak systolic gradient across the stenosis was 82 ± 21.48 mmHg ranging from 60 mmHg to 110 mmHg. There was no early and medium-term mortality with 100% survival at mean follow-up of 31 months (range 7-85 months).

Surgical correction of congenital SPS carries excellent early and mid-term results with almost no mortality and very low risk of re-intervention for restenosis.