Department of Cardiology, Istituto Auxologico Italiano, IRCCS, Ospedale San Luca, Milano, Italy.
Department of Management, Information and Production Engineering, University of Bergamo, Dalmine, Bergamo, Italy.
Semin Respir Crit Care Med. 2023 Dec;44(6):810-825. doi: 10.1055/s-0043-1772754. Epub 2023 Sep 14.
Pulmonary hypertension (PH) is a common complication of diseases affecting the left heart, mostly found in patients suffering from heart failure, with or without preserved left ventricular ejection fraction. Initially driven by a passive increase in left atrial pressure (postcapillary PH), several mechanisms may lead in a subset of patient to significant structural changes of the pulmonary vessels or a precapillary component. In addition, the right ventricle may be independently affected, which results in right ventricular to pulmonary artery uncoupling and right ventricular failure, all being associated with a worse outcome. The differential diagnosis of PH associated with left heart disease versus pulmonary arterial hypertension (PAH) is especially challenging in patients with cardiovascular comorbidities and/or heart failure with preserved ejection fraction (HFpEF). A stepwise approach to diagnosis is proposed, starting with a proper clinical multidimensional phenotyping to identify patients in whom hemodynamic confirmation is deemed necessary. Provocative testing (exercise testing, fluid loading, or simple leg raising) is useful in the cath laboratory to identify patients with abnormal response who are more likely to suffer from HFpEF. In contrast with group 1 PH, management of PH associated with left heart disease must focus on the treatment of the underlying condition. Some PAH-approved targets have been unsuccessfully tried in clinical studies in a heterogeneous group of patients, some even leading to an increase in adverse events. There is currently no approved therapy for PH associated with left heart disease.
肺动脉高压（PH）是影响左心疾病的常见并发症，主要发生在心力衰竭患者中，无论是否保留左心室射血分数。最初由左心房压力被动增加（后毛细血管 PH）驱动，在一部分患者中，可能会导致肺血管的显著结构改变或前毛细血管成分。此外，右心室可能会受到独立影响，导致右心室与肺动脉解偶联和右心室衰竭，所有这些都与预后较差相关。在伴有心血管合并症和/或射血分数保留的心力衰竭（HFpEF）的患者中，左心疾病相关 PH 与肺动脉高压（PAH）的鉴别诊断尤其具有挑战性。建议采用逐步诊断方法，首先对患者进行适当的临床多维表型分析，以确定需要进行血流动力学确认的患者。在导管室中，有创性检查（运动试验、液体负荷或简单抬腿）可用于识别反应异常的患者，这些患者更可能患有 HFpEF。与 1 型 PH 不同，左心疾病相关 PH 的治疗必须侧重于基础疾病的治疗。一些已被批准用于 PAH 的靶点在异质性患者群体的临床研究中均未成功，有些甚至导致不良事件增加。目前尚无针对左心疾病相关 PH 的获批治疗方法。
