| H01622 | |
| H number | H01622 |
| Name | Chronic thromboembolic pulmonary hypertension |
| Description | Chronic thromboembolic pulmonary hypertension (CTEPH) is classified as group 4 pulmonary hypertension (PH) by the World Health Organization. It is a rare, but underdiagnosed, complication of acute pulmonary embolism (PE) resulting from occlusion of large pulmonary arteries with a fibrothrombotic material and in many cases, the development of a distal vessel arteriopathy that closely mimics pulmonary arterial hypertension. While the pathogenesis of CTEPH is not entirely understood, the primary pathophysiology appears to be related to an initial thrombotic event followed by both inadequate thrombus resolution and secondary arteriopathy. Several risk factors for development of CTEPH have been found, including the size of the initial PE, splenectomy, chronic inflammatory conditions, and indwelling catheters and cardiac pacemaker leads. There is however an increased incidence of elevated factor VIII levels in CTEPH, and this finding persists after treatment. However, none of these findings explain the development of CTEPH in the majority of patients affected by this disease. |
| Category | Cardiovascular disease |
| Network | - |
| Gene | - |
| Pathogen | - |
| Env factor | - |
| Carcinogen | - |
| Drug | Riociguat [DR:D09572] |
| Comment | - |
| Other DBs | ICD-11: BB01.3 ICD-10: I27.2 |
| Reference | PMID:26739298 AUTHORS Poch DS, Auger WR TITLE Chronic thromboembolic pulmonary hypertension: detection, medical and surgical treatment approach, and current outcomes. JOURNAL Heart Fail Rev 21:309-22 (2016) DOI:10.1007/s10741-015-9518-3 PMID:26585271 AUTHORS O'Connell C, Montani D, Savale L, Sitbon O, Parent F, Seferian A, Bulifon S, Fadel E, Mercier O, Mussot S, Fabre D, Dartevelle P, Humbert M, Simonneau G, Jais X TITLE Chronic thromboembolic pulmonary hypertension. JOURNAL Presse Med 44:e409-16 (2015) DOI:10.1016/j.lpm.2015.10.010 PMID:27345156 AUTHORS Robbins IM, Pugh ME, Hemnes AR TITLE Update on chronic thromboembolic pulmonary hypertension. JOURNAL Trends Cardiovasc Med 27:29-37 (2017) DOI:10.1016/j.tcm.2016.05.010 PMID:26099554 AUTHORS Witkin AS, Channick RN TITLE Chronic Thromboembolic Pulmonary Hypertension: the End Result of Pulmonary Embolism. JOURNAL Curr Cardiol Rep 17:63 (2015) DOI:10.1007/s11886-015-0621-9 |