Ann Thorac Surg. 2012 Sep;94(3):817-24. Epub 2012 Jun 15.

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

Baruteau AE, Serraf A, Lévy M, Petit J, Bonnet D, Jais X, Vouhé P, Simonneau G, Belli E, Humbert M.

Centre Chirurgical Marie Lannelongue, Département de Chirurgie cardiaque des cardiopathies congénitales, Le Plessis-Robinson, France; Centre de Référence Malformations Cardiaques Congénitales Complexes-M3C, Paris, France; Université Paris-Sud, Le Kremlin-Bicêtre, France.

Abstract

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 ± 91 m [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.

PMID:22704329