The extracellular matrix (ECM), a highly organized network of structural and nonstructural proteins, plays a pivotal role in cellular and tissue homeostasis. Changes in the ECM are critical for normal tissue repair, whereas dysregulation contributes to aberrant tissue remodeling. Pulmonary arterial hypertension is a severe disorder of the pulmonary vasculature characterized by pathologic remodeling of the pulmonary vasculature and right ventricle, increased production and deposition of structural and nonstructural proteins, and altered expression of ECM growth factors and proteases. Furthermore, ECM remodeling plays a significant role in disease progression, as several dynamic changes in its composition, quantity, and organization are documented in both humans and animal models of disease. These ECM changes impact vascular cell biology and affect proliferation of resident cells. Furthermore, ECM components determine the tissue architecture of the pulmonary and myocardial vasculature as well as the myocardium itself and provide mechanical stability crucial for tissue homeostasis. However, little is known about the basement membrane (BM), a specialized, self-assembled conglomerate of ECM proteins, during remodeling. In the vasculature, the BM is in close physical association with the vascular endothelium and smooth muscle cells. While in the myocardium, each cardiomyocyte is enclosed by a BM that serves as the interface between cardiomyocytes and the surrounding interstitial matrix. In this review, we provide a brief overview on the current state of knowledge of the BM and its ECM composition and their impact on pulmonary vascular remodeling and right ventricle dysfunction and failure in pulmonary arterial hypertension.

细胞外基质（ECM）是一种高度组织化的结构和非结构蛋白网络，在细胞和组织稳态中发挥着关键作用。 ECM 的变化对于正常组织修复至关重要，而失调则导致异常组织重塑。肺动脉高压是肺血管系统的严重疾病，其特征在于肺血管系统和右心室的病理性重塑、结构和非结构蛋白的产生和沉积增加以及ECM生长因子和蛋白酶的表达改变。此外，ECM 重塑在疾病进展中发挥着重要作用，因为在人类和动物疾病模型中都记录了其组成、数量和组织的一些动态变化。这些 ECM 变化会影响血管细胞生物学并影响常驻细胞的增殖。此外，ECM 成分决定肺和心肌脉管系统以及心肌本身的组织结构，并提供对组织稳态至关重要的机械稳定性。然而，人们对基底膜 (BM) 知之甚少，基底膜是 ECM 蛋白在重塑过程中特化的自组装聚集体。在脉管系统中，BM 与血管内皮和平滑肌细胞有着密切的物理联系。在心肌中，每个心肌细胞都被 BM 包围，BM 充当心肌细胞与周围间质基质之间的界面。在这篇综述中，我们简要概述了 BM 及其 ECM 成分的知识现状及其对肺血管重塑和右心室功能障碍以及肺动脉高压衰竭的影响。