Centro de Investigaciones Biomédicas en Red de enfermedades CardioVasculares (CIBERCV), 28029 Madrid, Spain.
Servicio de Cardiología, Hospital Universitario 12 de Octubre, Instituto de Investigación Sanitaria Hospital 12 de Octubre (imas12), 28041 Madrid, Spain.
Int J Mol Sci. 2020 Apr 4;21(7):2509. doi: 10.3390/ijms21072509.
Pulmonary arterial hypertension (PAH) is a rare disease caused by pulmonary vascular remodeling. Current vasodilator treatments have substantially improved patients' survival. This improved survival has led to the appearance of complications related to conditions previously underdiagnosed or even ignored, such as pulmonary artery aneurysm (PAA). The presence of a dilated pulmonary artery has been shown to be related to an increased risk of sudden cardiac death among PAH patients. This increased risk could be associated to the development of left main coronary artery compression or pulmonary artery dissection. Nevertheless, very little is currently known about the molecular mechanisms related to PAA. Thoracic aortic aneurysm (TAA) is a well-known condition with an increased risk of sudden death caused by acute aortic dissection. TAA may be secondary to chronic exposure to classic cardiovascular risk factors. In addition, a number of genetic variants have been shown to be related to a marked risk of TAA and dissection as part of multisystemic syndromes or isolated familial TAA. The molecular pathways implied in the development of TAA have been widely studied and described. Many of these molecular pathways are involved in the pathogenesis of PAH and could be involved in PAA. This review aims to describe all these common pathways to open new research lines that could help lead to a better understanding of the pathophysiology of PAH and PAA and their clinical implications.
肺动脉高压（PAH）是一种由肺血管重构引起的罕见疾病。目前的血管扩张剂治疗大大提高了患者的生存率。这种生存率的提高导致了与以前诊断不足甚至忽视的疾病相关的并发症的出现，如肺动脉瘤（PAA）。已经表明扩张的肺动脉的存在与 PAH 患者心脏性猝死的风险增加有关。这种风险的增加可能与左主干冠状动脉受压或肺动脉夹层的发展有关。然而，目前对于与 PAA 相关的分子机制知之甚少。胸主动脉瘤（TAA）是一种众所周知的疾病，其急性主动脉夹层导致猝死的风险增加。TAA 可能继发于慢性暴露于经典心血管危险因素。此外，许多遗传变异已被证明与 TAA 和夹层的显著风险相关，作为多系统综合征或孤立性家族性 TAA 的一部分。TAA 发展中涉及的分子途径已被广泛研究和描述。这些分子途径中的许多都参与了 PAH 的发病机制，并且可能与 PAA 有关。本综述旨在描述所有这些常见途径，开辟新的研究路线，有助于更好地理解 PAH 和 PAA 的病理生理学及其临床意义。
