Simeone Beatrice, Maggio Enrico, Schirone Leonardo, Rocco Erica, Sarto Gianmarco, Spadafora Luigi, Bernardi Marco, D'Ambrosio Luca, Forte Maurizio, Vecchio Daniele, Valenti Valentina, Sciarretta Sebastiano, Vizza Carmine Dario
Department of Cardiology, ICOT Istituto Marco Pasquali, Latina, Italy.
Department of Clinical Internal, Anesthesiological and Cardiovascular Sciences, Sapienza University of Rome, Rome, Italy.
Front Cardiovasc Med. 2024 Jul 25;11:1439411. doi: 10.3389/fcvm.2024.1439411. eCollection 2024.
Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is a severe and complex condition that evolves from unresolved pulmonary embolism, leading to fibrotic obstruction of pulmonary arteries, pulmonary hypertension, and potential right heart failure. The cornerstone of CTEPH management lies in a multifaceted therapeutic approach tailored to individual patient profiles, reflecting the disease's heterogeneity. This review delves into the current therapeutic strategies for CTEPH, including surgical pulmonary endarterectomy (PEA), balloon pulmonary angioplasty (BPA), and targeted pharmacological treatments such as PDE5 inhibitors, endothelin receptor antagonists, sGC stimulators, and prostanoids. Lifelong anticoagulation is also highlighted as a preventive strategy against recurrent thromboembolism. Special emphasis is placed on the interdisciplinary nature of CTEPH care, necessitating collaboration among PEA surgeons, BPA interventionists, PH specialists, and thoracic radiologists to ensure comprehensive treatment planning and execution. The review underscores the importance of selecting an appropriate treatment modality based on the patient's specific disease characteristics and the evolving landscape of CTEPH treatment, aiming to improve patient outcomes through integrated care strategies.
慢性血栓栓塞性肺动脉高压（CTEPH）是一种严重且复杂的疾病，由未解决的肺栓塞发展而来，导致肺动脉纤维化阻塞、肺动脉高压以及潜在的右心衰竭。CTEPH管理的基石在于根据个体患者情况定制的多方面治疗方法，这反映了该疾病的异质性。本综述深入探讨了CTEPH的当前治疗策略，包括外科肺动脉内膜剥脱术（PEA）、球囊肺动脉成形术（BPA）以及靶向药物治疗，如磷酸二酯酶5抑制剂、内皮素受体拮抗剂、可溶性鸟苷酸环化酶刺激剂和前列环素类药物。终身抗凝也被强调为预防复发性血栓栓塞的策略。特别强调了CTEPH护理的跨学科性质，需要PEA外科医生、BPA介入专家、肺动脉高压专家和胸科放射科医生之间的合作，以确保全面的治疗计划和实施。该综述强调了根据患者的特定疾病特征和CTEPH治疗的不断发展的情况选择合适治疗方式的重要性，旨在通过综合护理策略改善患者预后。
