Authors' objectives: Pulmonary arterial hypertension (PAH) is a rare and progressive disease associated with increased pulmonary vascular resistance that, if unrelieved, progresses to right ventricular pressure overload, dysfunction, right heart failure, and premature death. PAH is more prevalent in some populations, thereby warranting screening of asymptomatic individuals. This review seeks to evaluate the comparative validity, reliability, and feasibility of echocardiography and biomarker testing for the screening, diagnosis, and management of PAH; to clarify whether the use of echocardiography or biomarkers affects decisionmaking and clinical outcomes; and to determine which medications are effective for treating PAH and whether combination therapy is more effective than monotherapy.

Authors' recommendations: Further confirmation is needed to determine if the combination of echocardiography and the biomarker NT-proBNP is sufficiently accurate to rule out PAH when testing symptomatic patients. In asymptomatic populations, more research is needed to permit conclusions regarding their effectiveness for screening. BNP, RA size, presence of pericardial effusion, and uric acid had prognostic value in patients with PAH, but other echocardiographic parameters and biomarkers either were not predictive or had insufficient data. Although no studies were powered to detect a mortality reduction, monotherapy was associated with improved 6MWD and reduced hospitalization rates. Comparisons of different drug combinations were inconclusive regarding a mortality reduction but suggested an improvement in 6MWD when a second drug was added to existing monotherapy.

Project Status: Completed

Year Published: 2013

URL for published report: http://www.effectivehealthcare.ahrq.gov/ehc/products/370/1414/hypertension-pulmonary-arterial-report-130524.pdf

URL for additional information: http://www.effectivehealthcare.ahrq.gov/search-for-guides-reviews-and-reports/?pageaction=displayproduct&productid=1479

English language abstract: An English language summary is available

Publication Type: Not Assigned

Country: United States

Organisation Name: Agency for Healthcare Research and Quality

Contact Address: Center for Outcomes and Evidence Technology Assessment Program, 540 Gaither Road, Rockville, MD 20850, USA. Tel: +1 301 427 1610; Fax: +1 301 427 1639;

Contact Name: martin.erlichman@ahrq.hhs.gov

Contact Email: martin.erlichman@ahrq.hhs.gov

Copyright: Agency for Healthcare Research and Quality (AHRQ)