Molecular Pathophysiology of Chronic Thromboembolic Pulmonary Hypertension: A Clinical Update from a Basic Research Perspective
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 withou...
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MDPI AG
2024-11-01
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| Series: | Advances in Respiratory Medicine |
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| author | Leslie Marisol Gonzalez-Hermosillo Guillermo Cueto-Robledo Dulce Iliana Navarro-Vergara Maria Berenice Torres-Rojas Marisol García-Cesar Oscar Pérez-Méndez Galileo Escobedo |
| author_facet | Leslie Marisol Gonzalez-Hermosillo Guillermo Cueto-Robledo Dulce Iliana Navarro-Vergara Maria Berenice Torres-Rojas Marisol García-Cesar Oscar Pérez-Méndez Galileo Escobedo |
| author_sort | Leslie Marisol Gonzalez-Hermosillo |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-77bc4a8bfc994ee5a0a2e9f949a703a8 |
| institution | Kabale University |
| issn | 2543-6031 |
| language | English |
| publishDate | 2024-11-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Advances in Respiratory Medicine |
| spelling | doaj-art-77bc4a8bfc994ee5a0a2e9f949a703a82024-12-27T14:09:24ZengMDPI AGAdvances in Respiratory Medicine2543-60312024-11-0192648550310.3390/arm92060044Molecular Pathophysiology of Chronic Thromboembolic Pulmonary Hypertension: A Clinical Update from a Basic Research PerspectiveLeslie Marisol Gonzalez-Hermosillo0Guillermo Cueto-Robledo1Dulce Iliana Navarro-Vergara2Maria Berenice Torres-Rojas3Marisol García-Cesar4Oscar Pérez-Méndez5Galileo Escobedo6Laboratory of Immunometabolism, Research Division, General Hospital of Mexico “Dr. Eduardo Liceaga”, Mexico City 06720, MexicoCardiorespiratory Emergency Department, Pulmonary Hypertension Clinic, General Hospital of Mexico “Dr. Eduardo Liceaga”, Mexico City 06720, MexicoCardiorespiratory Emergency Department, Pulmonary Hypertension Clinic, General Hospital of Mexico “Dr. Eduardo Liceaga”, Mexico City 06720, MexicoCardiorespiratory Emergency Department, Pulmonary Hypertension Clinic, General Hospital of Mexico “Dr. Eduardo Liceaga”, Mexico City 06720, MexicoCardiorespiratory Emergency Department, Pulmonary Hypertension Clinic, General Hospital of Mexico “Dr. Eduardo Liceaga”, Mexico City 06720, MexicoTecnológico de Monterrey, School of Engineering and Sciences, Mexico City 14380, MexicoLaboratory of Immunometabolism, Research Division, General Hospital of Mexico “Dr. Eduardo Liceaga”, Mexico City 06720, MexicoChronic 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.https://www.mdpi.com/2543-6031/92/6/44chronic thromboembolic pulmonary hypertensionacute pulmonary embolismfibrotic thrombus formationpulmonary vascular remodelingpulmonary hypertensiongene expression |
| spellingShingle | Leslie Marisol Gonzalez-Hermosillo Guillermo Cueto-Robledo Dulce Iliana Navarro-Vergara Maria Berenice Torres-Rojas Marisol García-Cesar Oscar Pérez-Méndez Galileo Escobedo Molecular Pathophysiology of Chronic Thromboembolic Pulmonary Hypertension: A Clinical Update from a Basic Research Perspective Advances in Respiratory Medicine chronic thromboembolic pulmonary hypertension acute pulmonary embolism fibrotic thrombus formation pulmonary vascular remodeling pulmonary hypertension gene expression |
| title | Molecular Pathophysiology of Chronic Thromboembolic Pulmonary Hypertension: A Clinical Update from a Basic Research Perspective |
| title_full | Molecular Pathophysiology of Chronic Thromboembolic Pulmonary Hypertension: A Clinical Update from a Basic Research Perspective |
| title_fullStr | Molecular Pathophysiology of Chronic Thromboembolic Pulmonary Hypertension: A Clinical Update from a Basic Research Perspective |
| title_full_unstemmed | Molecular Pathophysiology of Chronic Thromboembolic Pulmonary Hypertension: A Clinical Update from a Basic Research Perspective |
| title_short | Molecular Pathophysiology of Chronic Thromboembolic Pulmonary Hypertension: A Clinical Update from a Basic Research Perspective |
| title_sort | molecular pathophysiology of chronic thromboembolic pulmonary hypertension a clinical update from a basic research perspective |
| topic | chronic thromboembolic pulmonary hypertension acute pulmonary embolism fibrotic thrombus formation pulmonary vascular remodeling pulmonary hypertension gene expression |
| url | https://www.mdpi.com/2543-6031/92/6/44 |
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