التفاصيل البيبلوغرافية
| العنوان: |
Pulmonary arterial hypertension. (English) |
| العنوان البديل: |
Hypertension artérielle pulmonaire (French) |
| المؤلفون: |
Montani, D., Sitbon, O., Fadel, E., Dartevelle, P., Nunes, H., Lebrec, D., Simonneau, G., Humbert, M. |
| المصدر: |
EMC-Pneumologie; Apr2004, Vol. 1 Issue 2, p46-68, 23p |
| مصطلحات موضوعية: |
PULMONARY hypertension, DRUG therapy, HEART failure, HIV infections |
| الملخص (بالإنجليزية): |
Pulmonary arterial hypertension (PAH) is characterised by elevated pulmonary arterial resistance leading to right heart failure. PAH has been described as either sporadic or clustered in families. Defects within BMPR2, a receptor member of the TGF-β superfamily, underlie at least half of familial cases and more than 10 % of so-called sporadic PAH. Additional genetic or environmental determinants presumably play a key role in the pathogenesis of this disorder. PAH rarely complicates the course of human immunodeficiency infection, as well as portal hypertension. In both situation PAH is not linked to the severity of the underlying medical conditions, but it constitutes an important prognosis factor. Chronic thromboembolic hypertension is the consequence of acute pulmonary embolism in a rare subset of patients. Pulmonary endarterectomy is the treatment of choice for cases with proximal obstruction of pulmonary arteries. PAH treatment is based on simple measures (exercise limitation, anticoagulation, diuretics, oxygen…) and more specific drugs (calcium channel antagonists, prostacyclin, endothelin receptor antagonists). Medical advances over the last decade, and particularly the availability of novel drugs (prostaglandins and endothelin receptor antagonists) have resulted in significant improvements in prognosis. Interestingly, these drugs have both vasodilator and antiproliferative properties and the long-term effects of treatment may therefore extend beyond simple acute vasodilator activity. The evolution of therapy from vasodilators to antiproliferative agents reflects the advancement in our understanding of the mechanisms mediating PAH. [Copyright &y& Elsevier] |
| Abstract (French): |
Sous le terme d''hypertension arte´rielle pulmonaire (HTAP) sont regroupe´es diffe´rentes maladies touchant les arte`res pulmonaires de petit calibre, entraiˆnant une augmentation progressive des re´sistances arte´rielles pulmonaires et une de´faillance ventriculaire droite. L''HTAP peut survenir de fac¸on sporadique ou dans un contexte familial. Des mutations de BMPR2, un membre de la famille des re´cepteurs du transforming growth factor (TGF)-β, sont responsables de plus de la moitie´ des cas familiaux et de plus de 10 % des formes apparemment sporadiques. D''autres facteurs ge´ne´tiques ou environnementaux sont certainement implique´s dans le de´veloppement de cette maladie. Ainsi, l''infection par le virus de l''immunode´ficience humaine et l''hypertension portale peuvent rarement se compliquer d''HTAP. La survenue de cette complication n''est pas lie´e a` la se´ve´rite´ de l''infection virale ou de la maladie he´patique, mais l''HTAP constitue un facteur pronostique majeur. L''HTAP postembolique correspond a` l''organisation fibreuse d''embolies pulmonaires aigue¨s chez de rares patients. L''endarte´riectomie pulmonaire sous circulation extracorporelle repre´sente le traitement de re´fe´rence des obstructions proximales des arte`res pulmonaires. Le traitement de l''HTAP repose sur des mesures simples (limitation des efforts, anticoagulation, diure´tiques, oxyge´nothe´rapie…) associe´es a` des the´rapeutiques plus spe´cifiques (antagonistes calciques, prostacycline, antagonistes des re´cepteurs de l''endothe´line). L''arte´riopathie plexiforme associe des le´sions de fibrose et de prolife´ration endothe´liale et musculaire lisse vasculaire pulmonaire qui pre´dominent en ge´ne´ral sur les phe´nome`nes de vasoconstriction. Les avance´es me´dicales de ces dernie`res anne´es, et en particulier l''apparition de traitements aux proprie´te´s mixtes vasodilatatrices et antiprolife´rantes (prostacycline et antagonistes des re´cepteurs de l''endothe´line), ont permis une ame´lioration significative du pronostic. [Copyright 2004 Elsevier] |
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