| H01622 | |
| H番号 | 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 | 循環器系疾患 |
| ネットワーク | - |
| 病因遺伝子 | - |
| 病原体 | - |
| 環境要因 | - |
| 発癌物質 | - |
| 治療薬 | セレキシパグ [DR:D09994] リオシグアト [DR:D09572] |
| コメント | - |
| リンク | ICD-11: BB01.3 ICD-10: I27.2 |
| 文献 | PMID:26739298 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 PMID:26585271 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 PMID:27345156 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 PMID:26099554 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 |