Biondi Filippo, Alberti Mattia, Montemaggi Elisa, D'Alleva Alberto, Madonna Rosalinda
Cardiology Division, Department of Surgical, Medical and Molecular Pathology and Critical Area, University of Pisa, Pisa, Italy.
Cardiac Intensive Care and Interventional Cardiology Unit, Santo Spirito Hospital, Pescara, Italy.
Thromb Haemost. 2025 Jul;125(7):634-642. doi: 10.1055/a-2418-7895. Epub 2024 Sep 19.
Three mutually exclusive entities can underlie a postpulmonary embolism syndrome (PPES): not obstructed postpulmonary embolism syndrome (post-PE dyspnea), chronic thromboembolic pulmonary disease (CTEPD), and chronic thromboembolic pulmonary hypertension (CTEPH). Cardiorespiratory impairment in CTEPH and CTEPD underlies respiratory and hemodynamic mechanisms, either at rest or at exercise. Gas exchange is affected by the space effect, the increased blood velocity, and, possibly, intracardiac right to left shunts. As for hemodynamic effects, after a period of compensation, the right ventricle dilates and fails, which results in retrograde and anterograde right heart failure. Little is known on the pathophysiology of post-PE dyspnea, which has been reported in highly comorbid with lung and heart diseases, so that a "two-hit" hypothesis can be put forward: it might be caused by the acute myocardial damage caused by pulmonary embolism in the context of preexisting cardiac and/or respiratory diseases. More than one-third of PE survivors develops PPES, with only a small fraction (3-4%) represented by CTEPH. A value of ≈3% is a plausible estimate for the incidence of CTEPD. Growing evidence supports the role of CTEPD as a hemodynamic phenotype intermediate between post-PE dyspnea and CTEPH, but it still remains to be ascertained whether it constantly underlies exercise-induced pulmonary hypertension and if it is a precursor of CTEPH. Further research is needed to improve the understanding and the management of CTEPD and post-PE dyspnea.
三种相互排斥的情况可能是肺栓塞后综合征（PPES）的基础：未阻塞性肺栓塞后综合征（肺栓塞后呼吸困难）、慢性血栓栓塞性肺疾病（CTEPD）和慢性血栓栓塞性肺动脉高压（CTEPH）。CTEPH和CTEPD中的心肺功能损害是呼吸和血流动力学机制的基础，无论是在静息状态还是运动状态下。气体交换受空间效应、血流速度增加以及可能的心内右向左分流的影响。至于血流动力学效应，经过一段时间的代偿后，右心室扩张并衰竭，导致逆行和顺行性右心衰竭。关于肺栓塞后呼吸困难的病理生理学知之甚少，据报道其与肺部和心脏疾病高度共病，因此可以提出“两次打击”假说：它可能是由肺栓塞在已有心脏和/或呼吸系统疾病背景下引起的急性心肌损伤所致。超过三分之一的肺栓塞幸存者会发生PPES，其中只有一小部分（3-4%）由CTEPH代表。CTEPD的发病率约为3%是一个合理的估计。越来越多的证据支持CTEPD作为肺栓塞后呼吸困难和CTEPH之间的血流动力学表型的作用，但仍有待确定它是否始终是运动性肺动脉高压的基础，以及它是否是CTEPH的前驱。需要进一步研究以提高对CTEPD和肺栓塞后呼吸困难的理解和管理。
