Open Access

REVIEW

Reverse Potts Shunt in Children with Suprasystemic Pulmonary Arterial Hypertension: A Systematic Review and Meta-Analysis

Yong An^1,2, Gang Wang^1,2, Jiangtao Dai^1,2, Zhengxia Pan^1,2, Yuhao Wu^1,2,*

1 Department of Cardiothoracic Surgery, Children’s Hospital of Chongqing Medical University, Chongqing, 400014, China
2 National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing Key Laboratory of Pediatrics, Chongqing, 400014, China

* Corresponding Author: Yuhao Wu. Email:

Congenital Heart Disease 2025, 20(1), 1-12. https://doi.org/10.32604/chd.2025.063152

Received 06 January 2025; Accepted 21 February 2025; Issue published 18 March 2025

Abstract

Background Pulmonary arterial hypertension (PAH) is a progressive condition with a poor prognosis in children. Lung transplantation (Ltx) remains the ultimate option when patients are refractory to PAH-specific therapy. Reverse Potts shunt (RPS) has been introduced to treat suprasystemic PAH. This study aims to investigate the clinical outcomes of suprasystemic PAH in children. Methods Embase, Pubmed, and the Cochrane Library databases were searched for related studies that reported the clinical outcomes of suprasystemic PAH following RPS in children. To investigate the clinical outcomes of RPS, meta-analyses of the early and overall mortalities were performed. Results Nine studies were included in this study. The estimated early mortality was 14.4% (95% CI, 7.1% to 23.1%), and the overall mortality/Ltx was 23.2% (95% CI, 14.4% to 32.9%). The estimated 1-year survival was 86.3% (95% CI, 75.9% to 88.7%). A qualitative review showed that the median value of 5-year survival free from Ltx of patients undergoing RPS was 68.6% (range: 65% to 92.3%). Compared to Ltx, RPS did not significantly increase the early mortality (OR, 2.48, 95% CI 0.75 to 8.24, p = 0.14). RPS also significantly improved the New York Heart Association/World Health Organization functional class, reduced the BNP/NT-pro BNP levels, decreased the PAH-specific therapy, and increased the six-minute-walking distance. Conclusions RPS may serve as an alternative treatment for suprasystemic drug-refractory PAH. Further large-scale and prospective cohort studies are needed to validate these findings.

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Supplementary Material

Supplementary Material File

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