Department of Medical Biotechnologies, Division of Cardiology, Le Scotte Hospital, 53100 Siena, Italy.
Cardio-Thoracic and Vascular Department, Division of Cardiology, Le Scotte Hospital, 53100 Siena, Italy.
Int J Mol Sci. 2022 Apr 20;23(9):4554. doi: 10.3390/ijms23094554.
One of the most important diagnostic challenges in clinical practice is the distinction between pulmonary hypertension (PH) due to primitive pulmonary arterial hypertension (PAH) and PH due to left heart diseases. Both conditions share some common characteristics and pathophysiological pathways, making the two processes similar in several aspects. Their diagnostic differentiation is based on hemodynamic data on right heart catheterization, cardiac structural modifications, and therapeutic response. More specifically, PH secondary to heart failure with preserved ejection fraction (HFpEF) shares features with type 1 PH (PAH), especially when the combined pre- and post-capillary form (CpcPH) takes place in advanced stages of the disease. Right ventricular (RV) dysfunction is a common consequence related to worse prognosis and lower survival. This condition has recently been identified with a new classification based on clinical signs and progression markers. The role and prevalence of PH and RV dysfunction in HFpEF remain poorly identified, with wide variability in the literature reported from the largest clinical trials. Different parenchymal and vascular alterations affect the two diseases. Capillaries and arteriole vasoconstriction, vascular obliteration, and pulmonary blood fluid redistribution from the basal to the apical district are typical manifestations of type 1 PH. Conversely, PH related to HFpEF is primarily due to an increase of venules/capillaries parietal fibrosis, extracellular matrix deposition, and myocyte hypertrophy with a secondary "arteriolarization" of the vessels. Since the development of structural changes and the therapeutic target substantially differ, a better understanding of pathobiological processes underneath PH-HFpEF, and the identification of potential maladaptive RV mechanisms with an appropriate diagnostic tool, become mandatory in order to distinguish and manage these two similar forms of pulmonary hypertension.
在临床实践中，最重要的诊断挑战之一是区分由原发性肺动脉高压（PAH）引起的肺动脉高压（PH）和由左心疾病引起的 PH。这两种情况有一些共同的特征和病理生理途径，使得这两个过程在几个方面相似。它们的诊断区分基于右心导管检查的血流动力学数据、心脏结构改变和治疗反应。更具体地说，射血分数保留的心力衰竭（HFpEF）继发的 PH 与 1 型 PH（PAH）具有相似的特征，特别是当复合前毛细血管和后毛细血管形式（CpcPH）发生在疾病的晚期时。右心室（RV）功能障碍是与预后较差和生存率降低相关的常见后果。这种情况最近根据临床症状和进展标志物被确定为一种新的分类。HFpEF 中 PH 和 RV 功能障碍的作用和流行率仍然未被充分确定，从最大的临床试验报告的文献中存在很大的差异。不同的实质和血管改变影响这两种疾病。毛细血管和小动脉收缩、血管闭塞以及从基底到顶部区域的肺血流再分布是 1 型 PH 的典型表现。相反，HFpEF 相关的 PH 主要是由于静脉/毛细血管壁纤维化、细胞外基质沉积和心肌细胞肥大的增加，以及血管的继发性“动脉化”。由于结构变化的发展和治疗靶点有很大的不同，因此更好地了解 PH-HFpEF 下的病理生物学过程，并确定潜在的适应性不良 RV 机制，以及使用适当的诊断工具，对于区分和管理这两种相似形式的肺动脉高压是强制性的。
