肺高壓
肺高壓又稱肺動脈高壓(Pulmonary hypertension,簡稱PH或PHTN),是描述肺循環內的壓力升高的情形。肺高壓會造成呼吸困難、暈眩、昏厥、下肢水腫,肺高壓患者會因為心臟負荷增加令運動能力急速下降,以及發生其他症狀,可引致心臟衰竭導致病患死亡[1]。此症於1891年由恩斯特·馮·倫伯格首次描述[2]。根據最新的分類,肺高壓共有六種形式[3]。
肺高壓 | |
---|---|
![]() | |
肺高壓患者的血管的顯微切片,圖中可以見到血管內膜和中膜。 | |
类型 | 高血压[*]、高血壓、疾病 |
分类和外部资源 | |
醫學專科 | 胸腔醫學、心臟病學 |
ICD-11 | BB01 |
DiseasesDB | 10998 |
MedlinePlus | 000112 |
eMedicine | 361242、303098 |
Orphanet | 71198 |
症狀及徵象
编辑較罕見的症狀包含乾咳、運動時暈眩和嘔吐[5]。部分患者有咳血的現象,特別是肺高壓的某些亞型,如遺傳性肺動脈高壓、艾森門格症侯群,和慢性栓塞型肺動脈高壓等[7]。肺靜脈高壓的典型症狀為臥姿時呼吸困難(端坐呼吸及陣發性夜間呼吸困難),肺動脈高壓則無此現象[8]。
其他典型症狀包含較強的第二心音。另外右心室肥大也會造成右心室產生第三心音,以及胸骨旁起伏。右心衰竭導致的全身性鬱血則可能合併有頸靜脈屈張、腹水,以及肝頸動脈回流[5][6][9] 。
病因
编辑1973年,世界卫生组织首次嘗試將肺高壓分類,當時分為原發性肺高壓,以及次發性肺高壓兩大類,並將初級肺高壓分為「叢狀動脈型」(arterial plexiform)、「靜脈阻塞型」(veno-occlusive),以及「血栓栓塞型」(thromboembolic)等[11]。1988年埃維昂萊班會議將次級肺高壓按原因分類[12]。隨著新研究的進展,2008年在达纳角召開的第四屆世界肺動脈高壓研討會更新了定義。以下為達納點的分類系統:[3]
治療
编辑肺高壓可利用藥物控制病情發展的程度,若針對第一型肺動脈高目前主要分有三種不同的標靶治療途徑: 1.内皮素1 2前列環素(PGI:途徑) 3一氧化氮途徑 而目前針對此三種不同途徑的藥物如下列所述: 1.內皮素1途徑--Bosentan、Macitentan(雙重ETA-ETB拮抗劑)、Ambrisentan(選擇性ETA受體拮抗劑) 2.前列環素(PG₂I)途徑--Epoprostenol、lloprost、Treprostinil(三者皆為PGl₂類似劑)、Selexipag(PGl受體奮劑) 3.一氧化氮途徑--一氧化氮氣體(刺激可溶性鳥苷酸環化酶)、Sildenafil(PDE5抑利劑)、口服Riociguat(直接sGC刺激劑)[13] 由於肺高壓不能根治,若已引發較嚴重的心臟衰竭,就需要進行心肺移植,由於器官移植輪候需時,所以一旦確診,除藥物控制病情外,醫生都會安排病人排入器官移植的等候名單,以期病人在症狀惡化至嚴重心臟衰竭前,有適合器官進行移植手術[14]。
參考文獻
编辑- ^ Pulmonary hypertension: MedlinePlus Medical Encyclopedia. www.nlm.nih.gov. [2015-12-30]. (原始内容存档于2016-07-05).
- ^ von Romberg, Ernst. Über Sklerose der Lungenarterie. Dtsch Arch Klin Med. 1891–1892, 48: 197–206 (德语).
- ^ 3.0 3.1 Simonneau G, Robbins I, Beghetti M, et al. Updated Clinical classification of pulmonary hypertension. J. Am. Coll. Cardiol. 2009-06-30, 54 (1 Suppl S): S43–S54 [2016-12-07]. PMID 19555858. doi:10.1016/j.jacc.2009.04.012. (原始内容存档于2015-09-24).
- ^ What Are the Signs and Symptoms of Pulmonary Hypertension? - NHLBI, NIH. www.nhlbi.nih.gov. [2015-12-30]. (原始内容存档于2016-01-05).
- ^ 5.0 5.1 5.2 5.3 Galie N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). European Heart Journal. 2016-01-01, 37 (1): 67–119. ISSN 1522-9645. PMID 26320113. doi:10.1093/eurheartj/ehv317.
- ^ 6.0 6.1 6.2 McLaughlin VV, Archer SL, Badesch DB, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association developed in collaboration with the American College of Chest Physicians; American Thoracic Society, Inc.; and the Pulmonary Hypertension Association. Journal of the American College of Cardiology. 2009-04-28, 53 (17): 1573–1619. ISSN 1558-3597. PMID 19389575. doi:10.1016/j.jacc.2009.01.004.
- ^ Diller, Gerhard-Paul; Gatzoulis, Michael A. Pulmonary vascular disease in adults with congenital heart disease. Circulation. 2007-02-27, 115 (8): 1039–1050. ISSN 1524-4539. PMID 17325254. doi:10.1161/CIRCULATIONAHA.105.592386.
- ^ Fang JC, DeMarco T, Givertz MM, et al. World Health Organization Pulmonary Hypertension group 2: pulmonary hypertension due to left heart disease in the adult--a summary statement from the Pulmonary Hypertension Council of the International Society for Heart and Lung Transplantation. The Journal of Heart and Lung Transplantation: The Official Publication of the International Society for Heart Transplantation. 2012-09-01, 31 (9): 913–933. ISSN 1557-3117. PMID 22884380. doi:10.1016/j.healun.2012.06.002.
- ^ Yusuf, Salim; Cairns, John; Camm, John; Fallen, Ernest L.; Gersh, Bernard J. Evidence-Based Cardiology. John Wiley & Sons. 2011-09-07: 70.3(figure) [2016-12-07]. ISBN 9781444359459. (原始内容存档于2020-08-19).
- ^ Primary Pulmonary Hypertension Clinical Presentation: History, Physical Examination, Complications. emedicine.medscape.com. [2015-12-30]. (原始内容存档于2015-11-01).
- ^ Hatano S, Strasser R. Primary pulmonary hypertension. Geneva: World Health Organization. 1975.
- ^ Rich S, Rubin LJ, Abenhail L, et al. Executive summary from the World Symposium on Primary Pulmonary Hypertension (Evian, France, September 6–10, 1998). Geneva: The World Health Organization. 1998. (原始内容存档于2002-04-08).
- ^ Hassoun, Paul M. Pulmonary Arterial Hypertension. New England Journal of Medicine. 2021-12-16, 385 (25): 2361–2376 [2023-05-28]. doi:10.1056/NEJMra2000348. (原始内容存档于2023-05-28).
- ^ 喘到心臟衰竭! 淺談肺動脈高壓. 全民健康基金會. [2018-12-18]. (原始内容存档于2018-12-18).
延伸閱讀
编辑- Rubin LJ, Badesch DB. Evaluation and management of the patient with pulmonary arterial hypertension. Ann. Intern. Med. 2005, 143 (4): 282–92 [2016-12-07]. PMID 16103472. doi:10.7326/0003-4819-143-4-200508160-00009. (原始内容存档于2009-06-17).
- Abman, SH; Hansmann, G; Archer, SL; Ivy, DD; Adatia, I; Chung, WK; Hanna, BD; Rosenzweig, EB; Raj, JU; Cornfield, D; Stenmark, KR; Steinhorn, R; Thébaud, B; Fineman, JR; Kuehne, T; Feinstein, JA; Friedberg, MK; Earing, M; Barst, RJ; Keller, RL; Kinsella, JP; Mullen, M; Deterding, R; Kulik, T; Mallory, G; Humpl, T; Wessel, DL. Pediatric Pulmonary Hypertension: Guidelines From the American Heart Association and American Thoracic Society.. Circulation. 2015-11-03, 132: 2037–99. PMID 26534956. doi:10.1161/CIR.0000000000000329.
參見
编辑外部連結
编辑- Pulmonary Hypertension Association
- European Pulmonary Hypertension Association (页面存档备份,存于互联网档案馆)
- The Merck Manual Home Edition: Pulmonary Hypertension (页面存档备份,存于互联网档案馆)
- Pulmonary Arterial Hypertension database (页面存档备份,存于互联网档案馆)
- PH Central - the internet resource for Pulmonary Arterial Hypertension (页面存档备份,存于互联网档案馆)
- Webcast: The Changing World of Pulmonary Arterial Hypertension Therapies - American College of CHEST Physicians (页面存档备份,存于互联网档案馆)
- OMIM entries on Heritable Pulmonary Arterial Hypertension
- Pulmonary Hypertension Association of Australia (页面存档备份,存于互联网档案馆)