Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota, USA.
Division of Pulmonary and Critical Care Medicine, Johns Hopkins University, Baltimore, Maryland, USA.
J Am Coll Cardiol. 2024 Nov 26;84(22):2196-2210. doi: 10.1016/j.jacc.2024.08.061. Epub 2024 Oct 23.
Patients with group 1 pulmonary hypertension (PH) and risk factors for heart failure with preserved ejection fraction (HFpEF) demonstrate worse response to pulmonary vasodilator therapy. The mechanisms and optimal diagnostic approach to identify such patients remain unclear.
The purpose of this study was to compare exercise capacity, cardiac function, and hemodynamic responses to provocative maneuvers among patients with group 1 PH based upon pretest probability of HFpEF.
Pretest probability for HFpEF was determined using the validated HFpEF-ABA algorithm based on age, body mass index, and history of atrial fibrillation among group 1 PH patients recruited to the multicenter PVDOMICS (Redefining Pulmonary Hypertension through Pulmonary Vascular Disease Phenomics) study. Functional capacity, quality of life, and dynamic pulmonary capillary wedge pressure (PCWP) responses were compared between those with low (<25%), intermediate (25%-74%), and high (≥75%) ABA score-based HFpEF probability.
Among 424 patients with group 1 PH, 54% (n = 228) had intermediate HFpEF probability and 15% (n = 64) had high HFpEF probability. Resting PCWP increased progressively with higher HFpEF probability (P < 0.0001), and patients with group 1 PH and high HFpEF probability had the greatest increases in PCWP with nitric oxide, fluid challenge, and exercise (P < 0.001 for all), changes that were comparable to patients with HFpEF with no pulmonary vascular disease (n = 194), but lower than those with HFpEF and combined precapillary and postcapillary PH. Left ventricular/atrial size, diastolic function, quality of life, 6-minute walk distance, and peak VO were most abnormal in patients with group 1 PH and high HFpEF probability compared with those with low or intermediate HFpEF probability (P < 0.0001 for all). Increasing HFpEF probability in group 1 PH was associated with greater risk of death (HR per decile of HFpEF probability 1.09; 95% CI: 1.05-1.13; P < 0.0001).
Quantifying pretest probability for HFpEF in patients with group 1 PH identifies a subset of patients with worse dynamic PCWP response indicative of subclinical left heart disease, with poorer functional status, quality of life, and survival. Further study in this group 1 PH subgroup is indicated to determine whether PH therapies are effective and safe, and also whether HFpEF-specific therapies can improve functional status and outcomes.
患有 1 型肺动脉高压（PH）和射血分数保留型心力衰竭（HFpEF）风险因素的患者对肺动脉扩张剂治疗的反应较差。其机制和最佳诊断方法仍不清楚。
本研究旨在比较基于 1 型 PH 患者预先测试 HFpEF 概率的情况下，运动能力、心功能和血流动力学反应。
使用基于年龄、体重指数和 1 型 PH 患者中房颤病史的经过验证的 HFpEF-ABA 算法来确定 HFpEF 的预先测试概率，这些患者被纳入多中心 PVDOMICS（通过肺血管疾病表型重新定义肺动脉高压）研究。比较低（<25%）、中（25%-74%）和高（≥75%）ABA 评分 HFpEF 概率的患者之间的功能能力、生活质量和动态肺毛细血管楔压（PCWP）反应。
在 424 例 1 型 PH 患者中，54%（n=228）具有中等 HFpEF 概率，15%（n=64）具有高 HFpEF 概率。静息 PCWP 随 HFpEF 概率的增加而逐渐增加（P<0.0001），HFpEF 概率高的 1 型 PH 患者在接受一氧化氮、液体冲击和运动时 PCWP 增加最大（所有 P<0.001），这些变化与没有肺血管疾病的 HFpEF 患者相当（n=194），但低于同时患有毛细血管前和毛细血管后 PH 的 HFpEF 患者。与 HFpEF 概率低或中值的患者相比，1 型 PH 患者中 HFpEF 概率高的患者左心室/心房大小、舒张功能、生活质量、6 分钟步行距离和峰值 VO 最异常（所有 P<0.0001）。1 型 PH 中 HFpEF 概率的增加与死亡风险增加相关（HFpEF 概率每增加十分位数，HR 为 1.09；95%CI：1.05-1.13；P<0.0001）。
在 1 型 PH 患者中量化 HFpEF 的预先测试概率可确定一组对血流动力学 PCWP 反应较差的患者，这些患者具有亚临床左心疾病的迹象，其功能状态、生活质量和生存率较差。需要在这一组 1 型 PH 亚组中进一步研究，以确定 PH 治疗是否有效和安全，以及 HFpEF 特异性治疗是否可以改善功能状态和结局。
