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Volume 92
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10.3390/arm92060044
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Review

Molecular Pathophysiology of Chronic Thromboembolic Pulmonary Hypertension: A Clinical Update from a Basic Research Perspective

by
Leslie Marisol Gonzalez-Hermosillo
1,2,3,
Guillermo Cueto-Robledo
2,
Dulce Iliana Navarro-Vergara
2,
Maria Berenice Torres-Rojas
2,
Marisol García-Cesar
2,
Oscar Pérez-Méndez
4,5 and
Galileo Escobedo
1,*
1
Laboratory of Immunometabolism, Research Division, General Hospital of Mexico “Dr. Eduardo Liceaga”, Mexico City 06720, Mexico
2
Cardiorespiratory Emergency Department, Pulmonary Hypertension Clinic, General Hospital of Mexico “Dr. Eduardo Liceaga”, Mexico City 06720, Mexico
3
Doctorate Program in Biomedical Sciences, Postgraduate Unit, National Autonomous University of Mexico, Mexico City 04510, Mexico
4
Tecnológico de Monterrey, School of Engineering and Sciences, Mexico City 14380, Mexico
5
Departamento de Biología Molecular, Instituto Nacional de Cardiología Ignacio Chávez, Mexico City 14080, Mexico
*
Author to whom correspondence should be addressed.
Adv. Respir. Med. 2024, 92(6), 485-503; https://doi.org/10.3390/arm92060044
Submission received: 5 September 2024 / Revised: 19 November 2024 / Accepted: 26 November 2024 / Published: 27 November 2024
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Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but severe condition characterized by persistent obstruction and vascular remodeling in the pulmonary arteries following an acute pulmonary embolism (APE). Although APE is a significant risk factor, up to 25% of CTEPH cases occur without a history of APE or deep vein thrombosis, complicating the understanding of its pathogenesis. Herein, we carried out a narrative review discussing the mechanisms involved in CTEPH development, including fibrotic thrombus formation, pulmonary vascular remodeling, and abnormal angiogenesis, leading to elevated pulmonary vascular resistance and right heart failure. We also outlined how the disease’s pathophysiology reveals both proximal and distal pulmonary artery obstruction, contributing to the development of pulmonary hypertension. We depicted the risk factors predicting CTEPH, including thrombotic history, hemostatic disorders, and certain medical conditions. We finally looked at the molecular mechanisms behind the role of endothelial dysfunction, gene expression alterations, and inflammatory processes in CTEPH progression and detection. Despite these insights, there is still a need for improved diagnostic tools, biomarkers, and therapeutic strategies to enhance early detection and management of CTEPH, ultimately aiming to reduce diagnostic delay and improve patient outcomes.
Keywords: chronic thromboembolic pulmonary hypertension; acute pulmonary embolism; fibrotic thrombus formation; pulmonary vascular remodeling; pulmonary hypertension; gene expression chronic thromboembolic pulmonary hypertension; acute pulmonary embolism; fibrotic thrombus formation; pulmonary vascular remodeling; pulmonary hypertension; gene expression

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MDPI and ACS Style

Gonzalez-Hermosillo, L.M.; Cueto-Robledo, G.; Navarro-Vergara, D.I.; Torres-Rojas, M.B.; García-Cesar, M.; Pérez-Méndez, O.; Escobedo, G. Molecular Pathophysiology of Chronic Thromboembolic Pulmonary Hypertension: A Clinical Update from a Basic Research Perspective. Adv. Respir. Med. 2024, 92, 485-503. https://doi.org/10.3390/arm92060044

AMA Style

Gonzalez-Hermosillo LM, Cueto-Robledo G, Navarro-Vergara DI, Torres-Rojas MB, García-Cesar M, Pérez-Méndez O, Escobedo G. Molecular Pathophysiology of Chronic Thromboembolic Pulmonary Hypertension: A Clinical Update from a Basic Research Perspective. Advances in Respiratory Medicine. 2024; 92(6):485-503. https://doi.org/10.3390/arm92060044

Chicago/Turabian Style

Gonzalez-Hermosillo, Leslie Marisol, Guillermo Cueto-Robledo, Dulce Iliana Navarro-Vergara, Maria Berenice Torres-Rojas, Marisol García-Cesar, Oscar Pérez-Méndez, and Galileo Escobedo. 2024. "Molecular Pathophysiology of Chronic Thromboembolic Pulmonary Hypertension: A Clinical Update from a Basic Research Perspective" Advances in Respiratory Medicine 92, no. 6: 485-503. https://doi.org/10.3390/arm92060044

APA Style

Gonzalez-Hermosillo, L. M., Cueto-Robledo, G., Navarro-Vergara, D. I., Torres-Rojas, M. B., García-Cesar, M., Pérez-Méndez, O., & Escobedo, G. (2024). Molecular Pathophysiology of Chronic Thromboembolic Pulmonary Hypertension: A Clinical Update from a Basic Research Perspective. Advances in Respiratory Medicine, 92(6), 485-503. https://doi.org/10.3390/arm92060044

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