Pulmonary endarterectomy in chronic thromboembolic pulmonary hypertension - Holy Grail or Holy Trinity?

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare pulmonary vascular disease characterized by a heterogeneous combination of intravascular fibrotic clots and secondary microvasculopathy that may lead to right heart failure and death. Traditionally, CTEPH is considered a complication of acute pulmonary embolism (PE), occurring in 0.4% to 9.1% of cases depending on the defining criteria [ [1] Wilkens H. Konstantinides S. Lang I.M. Bunck A.C. Gerges M. Gerhardt F. et al. Chronic thromboembolic pulmonary hypertension (CTEPH): updated recommendations from the Cologne consensus conference 2018. Int. J. Cardiol. 2018; 272s: 69-78 Abstract Full Text Full Text PDF PubMed Scopus (97) Google Scholar ]. Although some risk factors have been previously described, the main catalyst driving the fibrotic transformation of clots is yet to be identified [ [2] Delcroix M. Torbicki A. Gopalan D. Sitbon O. Klok F.A. Lang I. et al. ERS statement on chronic thromboembolic pulmonary hypertension. Eur. Respir. J. 2021; 57 Crossref Scopus (194) Google Scholar ]. Additionally, a significant proportion of patients develops CTEPH without a history of PE, raising the question of different entities that may be currently summed up under the umbrella diagnosis of CTEPH. The damage in the microvasculature, similar to some degree to the one seen in pulmonary arterial hypertension (PAH), is thought to be caused by circulatory redistribution in the pulmonary vascular bed. Firstly, increased blood flow in non-obstructed regions may lead to endothelial sheer stress, secondly pronounced anastomoses between bronchial and pulmonary circulation may promote similar changes distal of occluded vessels [ [2] Delcroix M. Torbicki A. Gopalan D. Sitbon O. Klok F.A. Lang I. et al. ERS statement on chronic thromboembolic pulmonary hypertension. Eur. Respir. J. 2021; 57 Crossref Scopus (194) Google Scholar ]. Comparison of different treatment strategies in patients with chronic thromboembolic pulmonary hypertension: a single centre real-world experienceInternational Journal of CardiologyVol. 391PreviewPulmonary endarterectomy (PEA) has been the most effective therapy for chronic thromboembolic pulmonary hypertension (CTEPH). However, there is a substantial proportion of patients deemed not operable in whom other treatment strategies are available: medical therapy and balloon pulmonary angioplasty (BPA). We aimed to compare different CTEPH treatment strategies effect in a real-world setting. Full-Text PDF