276: Potts’ shunt in children with idiopathic pulmonary arterial hypertension: long-term results

Abstract

Idiopathic pulmonary arterial hypertension (IPAH) remains a progressive fatal disease. Palliative Potts'shunt has been proposed in children displaying supra-systemic IPAH. A retrospective multicenter study to evaluate Potts'shunt in pediatric IPAH. Between 2003 and 2010, 8 children with supra-systemic IPAH and in WHO functional class IV despite medical PAH therapy underwent Potts'shunt. Age at IPAH diagnosis ranged from 4 to 180 months (median age: 64 months). Surgical procedure was performed in a mean delay of 41.9±54.3 months (from 4 to 167 months, median delay: 20 months) after IPAH diagnosis. Mean size of the Potts'shunt was 9.25±3.30 mm. Two patients, whose medical PAH therapy had been interrupted just after surgery, died at post-operative day 11 and 13 from acute pulmonary hypertensive crisis. After a mean follow-up of 63.7±16.1 months, the 6 children who were discharged from hospital were alive. Functional status improved markedly in the 6 survivors with a WHO functional class I (n=4) or II (n=2) at last follow-up, consistent with significant improvement of 6 minute-walk distance [302±95 (51±20% of theoretical values) vs 456±91 meters (68±10% of theoretical values), p=0.038] and decrease of brain natriuretic peptid (BNP) levels (608±109 vs 76±45 pg/ml, p=0.035). No Potts'shunt was found restrictive at last echocardiography. Palliative Potts'shunt constitutes a new alternative to lung transplantation in severely ill children with supra-systemic IPAH, carrying a prolonged survival and persistent improvement in functional capacities.