第1组肺动脉高压高心输出量心力衰竭的病理生理与患病率
基于PVDOMICS队列449例第1组肺动脉高压患者，9%存在高输出状态，与更密集的血管扩张剂使用、更低的肺血管阻力和体循环血管阻力相关，并伴有左心扩大及运动时右室做功增加。高输出患者校正基线风险后死亡/移植风险仍升高（校正HR 2.1，95%CI 1.2-3.7；P=0.007），但无功能获益。
要点
- 第1组肺动脉高压中约9%存在高输出状态，多见于 prevalent PH 且与血管扩张剂强度相关。
- 高输出与更低肺血管阻力、体循环血管阻力及左心室/左心房扩大相关。
- 高输出患者校正后死亡/移植风险升高（aHR 2.1，95%CI 1.2-3.7），无功能或生存获益。
核心结论
- 约十分之一第1组肺动脉高压患者存在高输出状态，与血管扩张剂强度相关并伴不良心脏重构和心肌做功增加。
研究设计与样本
- PVDOMICS队列中449例第1组肺动脉高压参与者的临床特征比较分析，另设37例验证队列。
主要发现
- 449例中低输出占23%（n=103），正常输出占68%（n=304），高输出占9%（n=42）。
- 随心输出量增加，三联治疗比例分别为11%、19%、33%（P=0.0008），肺血管阻力进行性降低（P<0.0001）。
- 高输出与最低体循环血管阻力（P<0.0001）及更明显的左心室和左心房扩大相关（均P<0.001）。
- 高输出患者静息左室和右室做功及运动时绝对/相对右室做功增加（均P<0.0001），但氧摄取率（P=0.001）和动静脉氧差（P=0.005）提示外周氧利用受损。
- 校正基线风险后，高输出较正常输出死亡/移植风险升高（校正HR 2.1，95%CI 1.2-3.7；P=0.007）。
关键数值与推荐
- 低输出定义为心脏指数<2.2 L·min-1·m-2，高输出定义为心输出量≥8 L·min-1或心脏指数≥4 L·min-1·m-2。
临床怎么用
- 对第1组肺动脉高压患者应关注高输出状态，尤其在强化血管扩张剂治疗时。
- 高输出状态与不良预后相关，需进一步研究优化血管扩张剂剂量及与sotatercept等保血管扩张剂治疗的相互作用。
局限性
- 验证队列样本量较小（n=37）。
- 高输出状态的诊断时机多在血管扩张剂启动后的随访中出现。
以上解读依据论文摘要（全文未开放获取或许可不允许翻译转载）。
原文
BACKGROUND: Pulmonary vasodilators increase cardiac output (CO) in group 1 pulmonary hypertension (PH) and can cause high CO with unclear implications. Our objective was to describe the pathophysiology of high CO in group 1 PH. METHODS: Clinical characteristics were compared among PVDOMICS group 1 PH participants by low (cardiac index (CI) <2.2 L·min-1·m-2), normal or high output (CO ≥8 L·min-1 or CI ≥4 L·min-1·m-2). RESULTS: Of 449 group 1 PH participants, 23% (n=103) had low output, 68% (n=304) had normal CO and 9% (n=42) had high output. Increasing CO was associated with more intensive vasodilator use (triple therapy 11% versus 19% versus 33%, respectively; p=0.0008), with progressively lower pulmonary vascular resistance (p<0.0001). High output was associated with the lowest systemic vascular resistance (p<0.0001), with greater left ventricular (LV) and left atrial enlargement (p<0.001 for all). High flow resulted in an increase in LV and right ventricular (RV) work at rest, and absolute/relative RV work during exercise (p<0.0001 for all). Despite greater exercise oxygen delivery (p<0.0001), peripheral oxygen utilisation was impaired by oxygen extraction ratio (p=0.001) and arteriovenous oxygen difference (p=0.005), without incremental functional or survival benefit compared to normal output PH. After adjusting for baseline risk, high output had increased risk of death/transplantation compared to normal output (adjusted hazard ratio 2.1, 95% CI 1.2-3.7; p=0.007). In a validation cohort (n=37), 93% had normal CO at diagnosis, with the high output state developing in follow-up after vasodilator initiation. CONCLUSIONS: Around one in 10 patients with group 1 PH has a high output state, which is most common in prevalent PH and related to vasodilator intensity, with adverse cardiac remodelling and myocardial workload. Further studies are needed to determine optimal vasodilator dosing with high output and therapeutic interaction with vasodilator-sparing therapies such as sotatercept.
引用
- Reddy YNV, Frantz RP, Miranda WR, et al. Pathophysiology and prevalence of high output heart failure in group 1 pulmonary hypertension. Eur Respir J. 2026. PMID: 41713952. doi:10.1183/13993003.01871-2025
供执业医师工作参考，不替代临床判断；数值与分级以指南原文为准。