KEGG   DISEASE: 慢性血栓塞栓性肺高血圧症
エントリ  
H01622                                                             

名称    
慢性血栓塞栓性肺高血圧症
概要    
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.
カテゴリ  
循環器疾患
階層分類  
ヒト疾患 [BR:jp08402]
 循環器系疾患
  高血圧性疾患
   H01622  慢性血栓塞栓性肺高血圧症
ICD-11 による疾患分類 [BR:jp08403]
 11 循環器系の疾患
  肺性心疾患または肺循環疾患
   BB01  肺高血圧
    H01622  慢性血栓塞栓性肺高血圧症
特定疾患 (難病) [jp08407.html]
 H01622
治療薬   
セレキシパグ [DR:D09994]
リオシグアト [DR:D09572]
リンク   
ICD-11: BB01.3
ICD-10: I27.2
文献    
PMID:26739298 (drug)
  著者
Poch DS, Auger WR
  タイトル
Chronic thromboembolic pulmonary hypertension: detection, medical and surgical treatment approach, and current outcomes.
  雑誌
Heart Fail Rev 21:309-22 (2016)
DOI:10.1007/s10741-015-9518-3
文献    
PMID:26585271 (description, drug)
  著者
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
  タイトル
Chronic thromboembolic pulmonary hypertension.
  雑誌
Presse Med 44:e409-16 (2015)
DOI:10.1016/j.lpm.2015.10.010
文献    
PMID:27345156 (description, drug)
  著者
Robbins IM, Pugh ME, Hemnes AR
  タイトル
Update on chronic thromboembolic pulmonary hypertension.
  雑誌
Trends Cardiovasc Med 27:29-37 (2017)
DOI:10.1016/j.tcm.2016.05.010
文献    
PMID:26099554 (description, drug)
  著者
Witkin AS, Channick RN
  タイトル
Chronic Thromboembolic Pulmonary Hypertension: the End Result of Pulmonary Embolism.
  雑誌
Curr Cardiol Rep 17:63 (2015)
DOI:10.1007/s11886-015-0621-9
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