Cho Min-Jung, Kim Soo-Jin, Choi Miyoung, Lee Joo Sung, Oh Kyung Jin, Choi Hee Jung, Choi Gwang-Jun, Na Jae Yoon, Seol Jae Hee, Song Jin Young
Department of Pediatrics, Geoungsang National University Changwon Hospital, Changwon, Korea.
Department of Pediatrics, Bucheon Sejong Hospital, Bucheon, Korea.
Korean Circ J. 2025 Aug;55(8):721-732. doi: 10.4070/kcj.2024.0378. Epub 2025 Apr 11.
The effect of pulmonary vasodilator therapy on patients with Fontan circulation remains unclear. This study aims to assess its impact on exercise capacity and hemodynamic parameters in this population.
We searched PubMed, Embase, and the Cochrane Library for relevant studies up to November 2023. Pooled outcomes were used to evaluate the efficacy of pulmonary vasodilators in Fontan patients.
A total of 18 studies with 667 patients were included. Exercise capacity was assessed in 14 studies (526 patients). Pulmonary vasodilator therapy improved oxygen consumption anaerobic threshold (VO₂AT; MD, 1.12 mL/min/kg; 95% CI, 0.35 to 1.89; p=0.004) and Ve/VCO₂ slope (MD, -1.14; 95% CI, -1.97 to -0.31; p=0.007) during exercise. No significant differences were found among drug classes regarding peak oxygen consumption, Ve/VCO₂, or VO₂AT. Invasive hemodynamics were evaluated in 6 studies (126 patients). Pulmonary vasodilators significantly reduced mean pulmonary arterial pressure (MD, -2.28 mmHg; p=0.02), pulmonary vascular resistance (MD, -0.91 WU*m²; p=0.01), and improved pulmonary flow (MD, 0.46 L/min/m²; p=0.02).
Pulmonary vasodilator therapy appears beneficial for exercise capacity and pulmonary hemodynamics in Fontan patients. More randomized controlled trials are needed to confirm these findings.
肺血管扩张剂治疗对接受Fontan循环手术的患者的影响尚不清楚。本研究旨在评估其对该人群运动能力和血流动力学参数的影响。
我们检索了截至2023年11月的PubMed、Embase和Cochrane图书馆中的相关研究。采用汇总结果评估肺血管扩张剂对Fontan患者的疗效。
共纳入18项研究，涉及667例患者。14项研究（526例患者）评估了运动能力。肺血管扩张剂治疗可改善运动期间的耗氧量无氧阈值（VO₂AT；MD，1.12 mL/分钟/千克；95%CI，0.35至1.89；p=0.004）和Ve/VCO₂斜率（MD，-1.14；95%CI，-1.97至-0.31；p=0.007）。在峰值耗氧量、Ve/VCO₂或VO₂AT方面，不同药物类别之间未发现显著差异。6项研究（126例患者）评估了有创血流动力学。肺血管扩张剂可显著降低平均肺动脉压（MD，-2.28 mmHg；p=0.02）、肺血管阻力（MD，-0.91 WU·m²；p=0.01），并改善肺血流量（MD，0.46 L/分钟/m²；p=0.02）。
肺血管扩张剂治疗似乎对Fontan患者的运动能力和肺血流动力学有益。需要更多随机对照试验来证实这些发现。
