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Multicenter Study
. 2021 Aug 3;78(5):468-477.
doi: 10.1016/j.jacc.2021.05.039.

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

R Mark Grady  1 , Matthew W Canter  2 , Fei Wan  2 , Anton A Shmalts  3 , Ryan D Coleman  4 , Maurice Beghetti  5 , Rolf M F Berger  6 , Maria J Del Cerro Marin  7 , Scott E Fletcher  8 , Russel Hirsch  9 , Tilman Humpl  10 , D Dunbar Ivy  11 , Edward C Kirkpatrick  12 , Thomas J Kulik  13 , Marilyne Levy  14 , Shahin Moledina  15 , Delphine Yung  16 , Pirooz Eghtesady  2 , Damien Bonnet  14 ; International Registry Potts Shunt
Collaborators, Affiliations
Multicenter Study

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

R Mark Grady et al. J Am Coll Cardiol. .

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.

Keywords: Potts shunt; pediatric pulmonary hypertension.

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Conflict of interest statement

Funding Support and Author Disclosures Supported by the St. Louis Children’s Hospital Foundation. The authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Figures

Figure 1:
Figure 1:. Freedom from Death or Lung Transplant Post-Shunt Procedure.
Kaplan-Meier survival curve for all children post-shunt procedure (N=110). One and 5-year rates of freedom from death or lung transplant were 77% and 58% respectively. Median survival was 12.5 years.
Figure 2:
Figure 2:
Freedom from Death or Lung Transplant Post-Shunt Procedure for Children Successfully Discharged Home.
Central Illustration:
Central Illustration:. Potts Shunt and Pulmonary Hypertension.
Creation of an anastomosis between the left pulmonary artery and the descending aorta (Potts shunt) in a patient with severe pulmonary hypertension and suprasystemic pulmonary artery pressures. Desaturated pulmonary blood can flow into the systemic circulation, decreasing right ventricular afterload.
See this image and copyright information in PMC

Comment in

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