Potts Shunt in Children With Idiopathic Pulmonary Arterial Hypertension: Long-Term Results

Background. Idiopathic pulmonary arterial hypertension (IPAH) remains a progressive fatal disease. Palliative Potts shunt has been proposed in children displaying suprasystemic IPAH. Methods. A retrospective multicenter study was performed to evaluate Potts shunt in pediatric IPAH. Results. Between 2003 and 2010, 8 children with suprasystemic IPAH and in World Health Organization functional class IV despite medical pulmonary arterial hypertension 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 (range, 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 pulmonary arterial hypertension therapy had been interrupted just after surgery, died at postoperative days 11 and 13 of acute pulmonary hypertensive crisis. After a mean follow-up of 63.7 +/- 16.1 months, the 6 children who were discharged from the hospital were alive. Functional status improved markedly in the 6 survivors, with a World Health Organization functional class I (n = 4) or II (n = 2) at last follow-up, consistent with significant improvement of 6-minute-walk distance (302 +/- 95 m [51% +/- 20% of theoretical values] versus 456 +/- 91m[68% +/- 10% of theoretical values]; p = 0.038) and decrease of brain natriuretic peptide levels (608 +/- 109 pg/mL versus 76 +/- 45 pg/mL; p = 0.035). No Potts shunt was found to be restrictive at last echocardiography. Conclusions. Palliative Potts shunt constitutes a new alternative to lung transplantation in severely ill children with suprasystemic IPAH, carrying a prolonged survival and persistent improvement in functional capacities.