特发性肺动脉高压伴肺动脉广泛扩张患者的长期生存。
Long-term survival in idiopathic pulmonary arterial hypertension associated with massive pulmonary artery dilation.
机构信息
Department of Medicine, University of British Columbia, Vancouver.
出版信息
Can Respir J. 2011 May-Jun;18(3):e50-1. doi: 10.1155/2011/710489.
Abstract
The present report describes two patients with long-term survival after being diagnosed with idiopathic pulmonary arterial hypertension more than 20 years earlier. Both patients were treated with calcium channel blockers for several years and are currently maintained on bosentan, an oral endothelin receptor antagonist. Severe dilation of the main pulmonary arteries is present in both patients and may be related to long-term survival with idiopathic pulmonary arterial hypertension.
摘要
本报告描述了两名患者，他们在被诊断为特发性肺动脉高压超过 20 年后长期存活。两名患者均接受了数年的钙通道阻滞剂治疗，目前正在使用波生坦（一种口服内皮素受体拮抗剂）进行维持治疗。两名患者的主肺动脉均存在严重扩张，这可能与特发性肺动脉高压的长期生存有关。
相似文献
1
Long-term survival in idiopathic pulmonary arterial hypertension associated with massive pulmonary artery dilation.特发性肺动脉高压伴肺动脉广泛扩张患者的长期生存。
Can Respir J. 2011 May-Jun;18(3):e50-1. doi: 10.1155/2011/710489.
2
[Pulmonary arterial hypertension: changing approaches to management].[肺动脉高压：管理方法的转变]
Kardiologiia. 2011;51(1):100-8.
3
Successful bosentan therapy for pulmonary arterial hypertension associated with hereditary hemorrhagic telangiectasia.波生坦成功治疗与遗传性出血性毛细血管扩张症相关的肺动脉高压。
Heart Vessels. 2011 Mar;26(2):231-4. doi: 10.1007/s00380-010-0079-z. Epub 2010 Dec 4.
4
Effects of bosentan on cellular processes involved in pulmonary arterial hypertension: do they explain the long-term benefit?波生坦对肺动脉高压相关细胞过程的影响：这些影响能否解释其长期益处？
Ann Med. 2003;35(8):605-13. doi: 10.1080/07853890310017477.
5
[Impact of bosentan therapy on stress-induced pulmonary hypertension in patients with systemic sclerosis].波生坦治疗对系统性硬化症患者应激性肺动脉高压的影响
Ter Arkh. 2015;87(1):49-56. doi: 10.17116/terarkh201587149-56.
6
Role of iloprost and bosentan in pulmonary arterial hypertension.伊洛前列素和波生坦在肺动脉高压中的作用。
Niger J Med. 2008 Jan-Mar;17(1):13-9. doi: 10.4314/njm.v17i1.37347.
7
Bosentan for the prevention of overcirculation-induced experimental pulmonary arterial hypertension.波生坦用于预防过度循环诱导的实验性肺动脉高压。
Circulation. 2003 Mar 11;107(9):1329-35. doi: 10.1161/01.cir.0000053443.27512.33.
8
Potential benefit of bosentan therapy in borderline or less severe pulmonary hypertension secondary to idiopathic pulmonary fibrosis-an interim analysis of results from a prospective, single-center, randomized, parallel-group study.波生坦治疗特发性肺纤维化相关性边界性或轻度肺动脉高压的潜在获益：一项前瞻性、单中心、随机、平行分组研究的中期分析结果。
BMC Pulm Med. 2017 Dec 13;17(1):200. doi: 10.1186/s12890-017-0523-2.
9
Congenital right pulmonary artery agenesis with atrial septal defect and pulmonary hypertension.先天性右肺动脉缺如合并房间隔缺损及肺动脉高压。
Congenit Heart Dis. 2012 May-Jun;7(3):E6-9. doi: 10.1111/j.1747-0803.2011.00552.x. Epub 2011 Jul 31.
10
Endothelin mediates pulmonary vascular remodelling in a canine model of chronic embolic pulmonary hypertension.内皮素在慢性栓塞性肺动脉高压犬模型中介导肺血管重塑。
Eur Respir J. 2000 Apr;15(4):640-8. doi: 10.1034/j.1399-3003.2000.15d04.x.
引用本文的文献
1
Pulmonary artery enlargement in schistosomiasis associated pulmonary arterial hypertension.血吸虫病相关性肺动脉高压中的肺动脉扩大
BMC Pulm Med. 2015 Oct 12;15:118. doi: 10.1186/s12890-015-0115-y.
本文引用的文献
1
Genetics of pulmonary arterial hypertension.肺动脉高压的遗传学
Semin Respir Crit Care Med. 2009 Aug;30(4):386-98. doi: 10.1055/s-0029-1233308. Epub 2009 Jul 24.
2
Updated clinical classification of pulmonary hypertension.肺动脉高压的更新临床分类。
J Am Coll Cardiol. 2009 Jun 30;54(1 Suppl):S43-S54. doi: 10.1016/j.jacc.2009.04.012.
3
Relationship of pulmonary arterial capacitance and mortality in idiopathic pulmonary arterial hypertension.特发性肺动脉高压中肺动脉容量与死亡率的关系
J Am Coll Cardiol. 2006 Feb 21;47(4):799-803. doi: 10.1016/j.jacc.2005.09.054. Epub 2006 Jan 26.
4
Long term survival in primary pulmonary hypertension.原发性肺动脉高压的长期生存
Heart. 2004 Jul;90(7):e40. doi: 10.1136/hrt.2004.035725.
5
Primary pulmonary hypertension. Case series from the United Kingdom.原发性肺动脉高压。来自英国的病例系列。
Chest. 1994 Feb;105(2 Suppl):29S-32S. doi: 10.1378/chest.105.2_supplement.29s.
6
Benign, idiopathic pulmonary hypertension? Two further cases of unusually long duration.良性特发性肺动脉高压？另外两例病程异常漫长的病例。
Acta Med Scand. 1973 Jan-Feb;193(1-2):137-43.
7
Primary pulmonary hypertension. A national prospective study.原发性肺动脉高压。一项全国性前瞻性研究。
Ann Intern Med. 1987 Aug;107(2):216-23. doi: 10.7326/0003-4819-107-2-216.
8
Survival in patients with primary pulmonary hypertension. Results from a national prospective registry.原发性肺动脉高压患者的生存情况。一项全国性前瞻性登记研究的结果。
Ann Intern Med. 1991 Sep 1;115(5):343-9. doi: 10.7326/0003-4819-115-5-343.
9
The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension.高剂量钙通道阻滞剂对原发性肺动脉高压患者生存率的影响。
N Engl J Med. 1992 Jul 9;327(2):76-81. doi: 10.1056/NEJM199207093270203.
Zotero 插件