摘要
Background Pulmonary arterial hypertension (PAH) is a progressive condition with a poorprognosis in children. Lung transplantation (Ltx) remains the ultimate option when patients are refractory toPAH-speciffc therapy. Reverse Potts shunt (RPS) has been introduced to treat suprasystemic PAH. This studyaims 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 ofsuprasystemic PAH following RPS in children. To investigate the clinical outcomes of RPS, meta-analyses ofthe early and overall mortalities were performed. Results Nine studies were included in this study. Theestimated 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 reviewshowed 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 signiffcantly increase the early mortality (OR, 2.48, 95% CI0.75 to 8.24, p = 0.14). RPS also signiffcantly improved the New York Heart Association/World HealthOrganization functional class, reduced the BNP/NT-pro BNP levels, decreased the PAH-speciffc therapy,and increased the six-minute-walking distance. Conclusions RPS may serve as an alternative treatmentfor suprasystemic drug-refractory PAH. Further large-scale and prospective cohort studies are needed tovalidate these ffndings.
基金
Chongqing Medical University Program for Youth Innovation in Future Medicine(W0204)
Natural Science Foundation Project of Chongqing,Chongqing Science and Technology Commission(CSTB2023NSCQ-BHX0010)
Chongqing Postdoctoral Research Project Special Support(2023CQBSHTB3074)
Science and Technology Research Program of Chongqing Municipal Education Commission(KJQN202400421).
