KEGG   DISEASE: Chronic thromboembolic pulmonary hypertensionHelp
Entry
H01622                      Disease                                

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
Brite
Human diseases [BR:br08402]
 Cardiovascular diseases
  Hypertensive diseases
   H01622  Chronic thromboembolic pulmonary hypertension
Human diseases in ICD-11 classification [BR:br08403]
 11 Diseases of the circulatory system
  Pulmonary heart disease or diseases of pulmonary circulation
   BB01  Pulmonary hypertension
    H01622  Chronic thromboembolic pulmonary hypertension
BRITE hierarchy
Drug
Riociguat [DR:D09572]
Other DBs
ICD-11: BB01.3
ICD-10: I27.2
Reference
PMID:26739298 (drug)
  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
Reference
PMID:26585271 (description, drug)
  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
Reference
PMID:27345156 (description, drug)
  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
Reference
PMID:26099554 (description, drug)
  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
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