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.
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.
Authors: Kenneth Guo; David Langleben; Jonathan Afilalo; Avi Shimony; Richard Leask; Ariane Marelli; Giuseppe Martucci; Judith Therrien Journal: Pulm Circ Date: 2013-11-18 Impact factor: 3.017
Authors: Dale A Burkett; Cameron Slorach; Sonali S Patel; Andrew N Redington; D Dunbar Ivy; Luc Mertens; Adel K Younoszai; Mark K Friedberg Journal: Circ Cardiovasc Imaging Date: 2016-09 Impact factor: 7.792
Authors: Roland Fiszer; Blandyna Karwot; Beata Chodór; Małgorzata Szkutnik; Krzysztof Kobylarz; Janusz Skalski; Jacek Białkowski Journal: Kardiochir Torakochirurgia Pol Date: 2015-06-30