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Scientific article
Open access
English

Pulmonary-to-Systemic Arterial Shunt to Treat Children With Severe Pulmonary Hypertension

Published inJournal of the American College of Cardiology, vol. 78, no. 5, p. 468-477
Publication date2021-08-03
First online date2021-07-26
Abstract

Background: The placement of a pulmonary-to-systemic arterial shunt in children with severe pulmonary hypertension (PH) has been demonstrated, in relatively small studies, to be an effective palliation for their disease.

Objectives: The aim of this study was to expand upon these earlier findings using an international registry for children with PH who have undergone a shunt procedure.

Methods: Retrospective data were obtained from 110 children with PH who underwent a shunt procedure collected from 13 institutions in Europe and the United States.

Results: Seventeen children died in-hospital postprocedure (15%). Of the 93 children successfully discharged home, 18 subsequently died or underwent lung transplantation (20%); the mean follow-up was 3.1 years (range: 25 days to 17 years). The overall 1- and 5-year freedom from death or transplant rates were 77% and 58%, respectively, and 92% and 68% for those discharged home, respectively. Children discharged home had significantly improved World Health Organization functional class (P < 0.001), 6-minute walk distances (P = 0.047) and lower brain natriuretic peptide levels (P < 0.001). Postprocedure, 59% of children were weaned completely from their prostacyclin infusion (P < 0.001). Preprocedural risk factors for dying in-hospital postprocedure included intensive care unit admission (hazard ratio [HR]: 3.2; P = 0.02), mechanical ventilation (HR: 8.3; P < 0.001) and extracorporeal membrane oxygenation (HR: 10.7; P < 0.001).

Conclusions: A pulmonary-to-systemic arterial shunt can provide a child with severe PH significant clinical improvement that is both durable and potentially free from continuous prostacyclin infusion. Five-year survival is comparable to children undergoing lung transplantation for PH. Children with severely decompensated disease requiring aggressive intensive care are not good candidates for the shunt procedure.

eng
Keywords
  • Potts shunt
  • Pediatric pulmonary hypertension
  • Adolescent
  • Anastomosis, Surgical
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Hypertension, Pulmonary / surgery
  • Infant
  • Male
  • Pulmonary Artery / surgery
  • Retrospective Studies
  • Severity of Illness Index
  • Vascular Surgical Procedures / methods
  • Young Adult
Citation (ISO format)
GRADY, R Mark et al. Pulmonary-to-Systemic Arterial Shunt to Treat Children With Severe Pulmonary Hypertension. In: Journal of the American College of Cardiology, 2021, vol. 78, n° 5, p. 468–477. doi: 10.1016/j.jacc.2021.05.039
Main files (2)
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Secondary files (1)
Identifiers
ISSN of the journal0735-1097
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Technical informations

Creation09/09/2022 12:39:48 PM
First validation05/02/2023 4:16:55 PM
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