title: Palliative Potts shunt for the treatment of children with drug-refractory pulmonary arterial hypertension: updated data from the first 24 patients.
abstract: OBJECTIVE
  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.affiliation: 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.
authors.forename: Alban-Elouen
authors.initials: AE
authors.lastname: Baruteau
authors.affiliation: Marie Lannelongue Hospital, Pediatric and Congenital Cardiac Surgery, M3C, Reference Center for Complex Congenital Heart Diseases, Le Plessis Robinson, France.
authors.forename: Emre
authors.initials: E
authors.lastname: Belli
authors.affiliation: 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.
authors.forename: Younes
authors.initials: Y
authors.lastname: Boudjemline
authors.affiliation: Marie Lannelongue Hospital, Pediatric and Congenital Cardiac Surgery, M3C, Reference Center for Complex Congenital Heart Diseases, Le Plessis Robinson, France.
authors.forename: Daniela
authors.initials: D
authors.lastname: Laux
authors.affiliation: 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.
authors.forename: Marilyne
authors.initials: M
authors.lastname: Lévy
authors.affiliation: 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.
authors.forename: Gérald
authors.initials: G
authors.lastname: Simonneau
authors.affiliation: Pediatric Cardiac Surgery, Bambino Gesu Children's Hospital, IRCCS, Rome, Italy.
authors.forename: Adriano
authors.initials: A
authors.lastname: Carotti
authors.affiliation: 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.
authors.forename: Marc
authors.initials: M
authors.lastname: Humbert
authors.affiliation: 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.
authors.forename: Damien
authors.initials: D
authors.lastname: Bonnet
mesh_terms: D000293:Adolescent; D000714:Anastomosis, Surgical / Q000009:adverse effects* / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000601:surgery*; D007223:Infant; D008297:Male; D010166:Palliative Care / Q000379:methods*; D012189:Retrospective Studies
keywords: Paediatric; Potts shunt; Pulmonary arterial hypertension