Affiliation:
1. Department of Internal Medicine II, Division of Cardiology, Medical University of Vienna, Vienna, Austria
Abstract
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare major vessel pulmonary vascular disease that is characterised by fibrotic obstructions deriving from an organised clot. Recent advances in treatments for CTEPH have significantly improved outcomes. Apart from classical surgical pulmonary endarterectomy, balloon pulmonary angioplasty (BPA) and vasodilator drugs that were tested in randomised controlled trials of non-operable patients are now available. In Europe, CTEPH affects males and females equally. In the first European CTEPH Registry, women with CTEPH underwent pulmonary endarterectomy less frequently than men, especially at low-volume centres. In Japan, CTEPH is more common in females and is predominantly treated by BPA. More data on gender-specific outcomes are expected from the results of the International BPA Registry (NCT03245268).
Publisher
Radcliffe Media Media Ltd
Subject
Cardiology and Cardiovascular Medicine
Reference62 articles.
1. Gerges C, Gerges M, Friewald R, et al. Microvascular disease in chronic thromboembolic pulmonary hypertension: hemodynamic phenotyping and histomorphometric assessment. Circulation 2020;141:376–86. https://doi.org/10.1161/CIRCULATIONAHA.119.041515; PMID: 31896275.
2. Braams NJ, van Leeuwen JW, Vonk Noordegraaf A, et al. Right ventricular adaptation to pressure-overload: differences between chronic thromboembolic pulmonary hypertension and idiopathic pulmonary arterial hypertension. J Heart Lung Transplant 2021;40:458–66. https://doi.org/10.1016/j.healun.2021.02.018; PMID: 33745783.
3. Riedel M, Stanek V, Widimsky J, Prerovsky I. Longterm follow-up of patients with pulmonary thromboembolism. Late prognosis and evolution of hemodynamic and respiratory data. Chest 1982;81:151–8. https://doi.org/10.1378/chest.81.2.151; PMID: 7056079.
4. Delcroix M, Lang I, Pepke-Zaba J, et al. Long-term outcome of patients with chronic thromboembolic pulmonary hypertension: results from an international prospective registry. Circulation 2016;133:859–71. https://doi.org/10.1161/CIRCULATIONAHA.115.016522; PMID: 26826181.
5. Guth S, D’Armini AM, Delcroix M, et al. Current strategies for managing chronic thromboembolic pulmonary hypertension: results of the worldwide prospective CTEPH registry. ERJ Open Res 2021;7:00850-2020. https://doi.org/10.1183/23120541.00850-2020; PMID: 34409094.