Baroutidou Amalia, Arvanitaki Alexandra, Hatzidakis Adam, Pitsiou Georgia, Ziakas Antonios, Karvounis Haralambos, Giannakoulas George
First Department of Cardiology, AHEPA University Hospital, Aristotle University of Thessaloniki, St. Kiriakidi 1, 54636 Thessaloniki, Greece.
Adult Congenital Heart Centre, National Centre for Pulmonary Arterial Hypertension, Royal Brompton Hospital, Guy's and St Thomas' NHS Foundation Trust, Sydney Street, London SW3 6NP, UK.
J Clin Med. 2022 Jan 26;11(3):633. doi: 10.3390/jcm11030633.
Haemoptysis represents one of the most severe major bleeding manifestations in the clinical course of pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD). Accumulating evidence indicates that dysfunction of the pulmonary vascular bed in the setting of PAH predisposes patients to increased hemorrhagic diathesis, resulting in mild to massive and life-threatening episodes of haemoptysis. Despite major advances in PAH targeted treatment strategies, haemoptysis is still correlated with substantial morbidity and impaired quality of life, requiring a multidisciplinary approach by adult CHD experts in tertiary centres. Technological innovations in the field of diagnostic and interventional radiology enabled the application of bronchial artery embolization (BAE), a valuable tool to efficiently control haemoptysis in modern clinical practice. However, bleeding recurrences are still prevalent, implying that the optimum management of haemoptysis and its implications remain obscure. Moreover, regarding the use of oral anticoagulation in patients with haemoptysis, current guidelines do not provide a clear therapeutic strategy due to the lack of evidence. This review aims to discuss the main pathophysiological mechanisms of haemoptysis in PAH-CHD, present the clinical spectrum and the available diagnostic tools, summarize current therapeutic challenges, and propose directions for future research in this group of patients.
咯血是先天性心脏病（CHD）相关肺动脉高压（PAH）临床过程中最严重的主要出血表现之一。越来越多的证据表明，PAH患者肺血管床功能障碍使其出血素质增加，导致轻度至大量且危及生命的咯血发作。尽管PAH靶向治疗策略取得了重大进展，但咯血仍与高发病率和生活质量受损相关，需要三级中心的成人CHD专家采取多学科方法。诊断和介入放射学领域的技术创新使得支气管动脉栓塞术（BAE）得以应用，这是现代临床实践中有效控制咯血的一项重要工具。然而，出血复发仍然普遍存在，这意味着咯血的最佳管理及其影响仍不明确。此外，关于咯血患者口服抗凝药的使用，由于缺乏证据，目前的指南未提供明确的治疗策略。本综述旨在讨论PAH-CHD咯血的主要病理生理机制，介绍临床谱和可用的诊断工具，总结当前的治疗挑战，并为该组患者的未来研究提出方向。
