Alban-Elouen Baruteau1, Emre Belli2, Younes Boudjemline3, Daniela Laux2, Marilyne Lévy3, Gérald Simonneau4, Adriano Carotti5, Marc Humbert4, Damien Bonnet3. 1. Marie Lannelongue Hospital, Pediatric and Congenital Cardiac Surgery, M3C, Reference Center for Complex Congenital Heart Diseases, Le Plessis Robinson, France Paris Sud University, Le Kremlin Bicêtre, France a.baruteau@gmail.com. 2. Marie Lannelongue Hospital, Pediatric and Congenital Cardiac Surgery, M3C, Reference Center for Complex Congenital Heart Diseases, Le Plessis Robinson, France. 3. AP-HP, Necker Hospital for Sick Children, Pediatric Cardiology, M3C, Reference Center for Complex Congenital Heart Diseases, Paris, France Université Paris Descartes, Sorbonne Paris Cité, Paris, France. 4. Paris Sud University, Le Kremlin Bicêtre, France AP-HP, Bicêtre Hospital, Pneumology, Reference Center for Severe Pulmonary Hypertension, TORINO, Le Kremlin-Bicêtre, France INSERM UMR 999, LabEx-LERMIT, Marie Lannelongue Hospital, Le Plessis Robinson, France. 5. Pediatric Cardiac Surgery, Bambino Gesu Children's Hospital, IRCCS, Rome, Italy.
Abstract
OBJECTIVES: Palliative Potts shunt has been proposed in children with suprasystemic pulmonary arterial hypertension (PAH). METHODS: A retrospective multicentre study was performed to assess short- and long-term outcomes after Potts shunt. RESULTS: From 2003 to 2014, 24 children underwent a Potts shunt [19 surgical, median age: 7.7 years (1.5-17 years), median weight: 19.5 kg (10.2-47 kg) and 5 transcatheter, median age: 8.1 years (2.3-9.7 years), median weight: 22 kg (12.5-31 kg)] for drug-refractory PAH. For the first time in humans, we performed an unidirectional valved Potts anastomosis in a child with infrasystemic PAH on intravenous epoprostenol who experienced repeated central line infections. Severe postoperative complications occurred in 6 patients (25.0%, all from the surgical group) including 3 early deaths (12.5%) related to low cardiac output. After a median follow-up (FU) of 2.1 years (range, 3 months to 14.3 years, ≥8 years in 7 patients), World Health Organization (WHO) functional class was dramatically improved in the 21 survivors, all being in WHO-functional class 1 or 2 (P < 0.05); none experienced syncope during the FU; none had overt right ventricular failure; mean 6-min walk distance improved from 42.3 ± 10.0% to 81.2 ± 9.7% of adjusted values for age and sex (P < 0.001), BNP/NT-proBNP levels normalized in all; and weaning of intravenous epoprostenol was obtained in all patients who received triple combination as pre-Potts anastomosis therapy. Finally, all survivors caught up to normal growth curves. Arterial oxygen saturation gradient between upper and lower limbs persisted at the last FU (94.7 ± 3.6% vs 81.6 ± 5.1%, P < 0.001). One patient required double lung transplantation 6 years after a surgical Potts shunt. CONCLUSIONS: Palliative Potts shunt allows prolonged survival and dramatic, long-lasting improvement in functional capacities in children with severe, drug-refractory PAH. The Potts shunt might be considered as a first surgical or interventional step in the management of children with severe, drug-refractory PAH, leaving the door open for further lung transplantation, if needed.
OBJECTIVES: Palliative Potts shunt has been proposed in children with suprasystemic pulmonary arterial hypertension (PAH). METHODS: A retrospective multicentre study was performed to assess short- and long-term outcomes after Potts shunt. RESULTS: From 2003 to 2014, 24 children underwent a Potts shunt [19 surgical, median age: 7.7 years (1.5-17 years), median weight: 19.5 kg (10.2-47 kg) and 5 transcatheter, median age: 8.1 years (2.3-9.7 years), median weight: 22 kg (12.5-31 kg)] for drug-refractory PAH. For the first time in humans, we performed an unidirectional valved Potts anastomosis in a child with infrasystemic PAH on intravenous epoprostenol who experienced repeated central line infections. Severe postoperative complications occurred in 6 patients (25.0%, all from the surgical group) including 3 early deaths (12.5%) related to low cardiac output. After a median follow-up (FU) of 2.1 years (range, 3 months to 14.3 years, ≥8 years in 7 patients), World Health Organization (WHO) functional class was dramatically improved in the 21 survivors, all being in WHO-functional class 1 or 2 (P < 0.05); none experienced syncope during the FU; none had overt right ventricular failure; mean 6-min walk distance improved from 42.3 ± 10.0% to 81.2 ± 9.7% of adjusted values for age and sex (P < 0.001), BNP/NT-proBNP levels normalized in all; and weaning of intravenous epoprostenol was obtained in all patients who received triple combination as pre-Potts anastomosis therapy. Finally, all survivors caught up to normal growth curves. Arterial oxygen saturation gradient between upper and lower limbs persisted at the last FU (94.7 ± 3.6% vs 81.6 ± 5.1%, P < 0.001). One patient required double lung transplantation 6 years after a surgical Potts shunt. CONCLUSIONS: Palliative Potts shunt allows prolonged survival and dramatic, long-lasting improvement in functional capacities in children with severe, drug-refractory PAH. The Potts shunt might be considered as a first surgical or interventional step in the management of children with severe, drug-refractory PAH, leaving the door open for further lung transplantation, if needed.
Authors: Martin Koestenberger; Mark K Friedberg; Eirik Nestaas; Ina Michel-Behnke; Georg Hansmann Journal: Pulm Circ Date: 2016-03 Impact factor: 3.017
Authors: R Mark Grady; Matthew W Canter; Fei Wan; Anton A Shmalts; Ryan D Coleman; Maurice Beghetti; Rolf M F Berger; Maria J Del Cerro Marin; Scott E Fletcher; Russel Hirsch; Tilman Humpl; D Dunbar Ivy; Edward C Kirkpatrick; Thomas J Kulik; Marilyne Levy; Shahin Moledina; Delphine Yung; Pirooz Eghtesady; Damien Bonnet Journal: J Am Coll Cardiol Date: 2021-08-03 Impact factor: 27.203