|Treatment of sarcoidosis-associated pulmonary hypertension. A two-center experience.|
|PMID: 19118270 Owner: NLM Status: MEDLINE|
|BACKGROUND: Pulmonary hypertension (PH) is a common complication of sarcoidosis that is associated with increased mortality. The pathogenesis of PH in sarcoidosis is uncertain, and the role of pulmonary arterial hypertension (PAH)-specific therapies remains to be determined.
METHODS: We conducted a retrospective study of patients with sarcoidosis and PH at two referral centers. New York Heart Association (NYHA) functional class, exercise capacity, hemodynamic data, pulmonary function tests, and survival were collected and analyzed.
RESULTS: Twenty-two sarcoidosis patients treated with PAH-specific therapies were identified. After a median of 11 months of follow-up, NYHA class was improved in nine subjects. Mean 6-min walk distance (n = 18) increased by 59 m (p = 0.032). Patients with a higher FVC experienced a greater increment in exercise capacity. Among 12 patients with follow-up hemodynamic data, mean pulmonary artery pressure was reduced from 48.5 +/- 4.3 to 39.4 +/- 2.8 mm Hg (p = 0.008). The 1- and 3-year transplant-free survival rates were 90% and 74%, respectively.
CONCLUSIONS: PAH-specific therapy may improve functional class, exercise capacity, and hemodynamics in PH associated with sarcoidosis. Prospective, controlled trials of PAH therapies for sarcoidosis are warranted to verify this apparent benefit. Mortality among the study population was high, highlighting the need for urgent evaluation at a lung transplant center.
|Christopher F Barnett; Eric J Bonura; Steven D Nathan; Shahzad Ahmad; Oksana A Shlobin; Kwabena Osei; Ari L Zaiman; Paul M Hassoun; David R Moller; Scott D Barnett; Reda E Girgis|
|Type: Journal Article; Multicenter Study; Research Support, N.I.H., Intramural Date: 2008-12-31|
|Title: Chest Volume: 135 ISSN: 1931-3543 ISO Abbreviation: Chest Publication Date: 2009 Jun|
|Created Date: 2009-06-05 Completed Date: 2009-07-07 Revised Date: 2013-06-18|
Medline Journal Info:
|Nlm Unique ID: 0231335 Medline TA: Chest Country: United States|
|Languages: eng Pagination: 1455-61 Citation Subset: AIM; IM|
|Critical Care Medicine Department, Clinical Center, National Institutes of Health, Bethesda, MD, USA. firstname.lastname@example.org|
|APA/MLA Format Download EndNote Download BibTex|
Antihypertensive Agents / administration & dosage*
Drug Therapy, Combination
Epoprostenol / administration & dosage
Hemodynamics / drug effects*, physiology
Hypertension, Pulmonary / drug therapy*, etiology*, mortality
Iloprost / administration & dosage
Piperazines / administration & dosage
Purines / administration & dosage
Respiratory Function Tests
Sarcoidosis / complications*, diagnosis, mortality
Severity of Illness Index
Sulfonamides / administration & dosage
Sulfones / administration & dosage
|0/Antihypertensive Agents; 0/Piperazines; 0/Purines; 0/Sulfonamides; 0/Sulfones; 35121-78-9/Epoprostenol; 3M7OB98Y7H/sildenafil; 78919-13-8/Iloprost; Q326023R30/bosentan|
|Chest. 2009 Jun;135(6):1410-2
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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