Palliative Potts shunt for the treatment of children with drug-refractory pulmonary arterial hypertension: updated data from the first 24 patients.

Baruteau AE; Belli E; Boudjemline Y; Laux D; Lévy M; Simonneau G; Carotti A; Humbert M; Bonnet D

doi:10.1093/ejcts/ezu445

Palliative Potts shunt for the treatment of children with drug-refractory pulmonary arterial hypertension: updated data from the first 24 patients.

Affiliations

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
Authors
Baruteau AE¹
(1 author)
2. Marie Lannelongue Hospital, Pediatric and Congenital Cardiac Surgery, M3C, Reference Center for Complex Congenital Heart Diseases, Le Plessis Robinson, France.
Authors
Belli E²
Laux D²
(2 authors)
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.
Authors
Boudjemline Y³
Lévy M³
Bonnet D³
(3 authors)
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.
Authors
Simonneau G⁴
Humbert M⁴
(2 authors)
5. Pediatric Cardiac Surgery, Bambino Gesu Children's Hospital, IRCCS, Rome, Italy.
Authors
Carotti A⁵
(1 author)

ORCIDs linked to this article

European Journal of Cardio-thoracic Surgery : Official Journal of the European Association for Cardio-thoracic Surgery, 04 Dec 2014, 47(3):e105-10
https://doi.org/10.1093/ejcts/ezu445 PMID: 25475943

A comment on this article appears in "In search of ideal surgical palliation for drug refractory pulmonary hypertension." Eur J Cardiothorac Surg. 2015 Mar;47(3):e110-2.

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.