Journal
JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY
Volume 65, Issue 18, Pages 1976-1997Publisher
ELSEVIER SCIENCE INC
DOI: 10.1016/j.jacc.2015.03.540
Keywords
echocardiography; endothelin receptor antagonists; hemodynamics; phosphodiesterase type 5 inhibitors; prostacyclins; pulmonary arterial hypertension
Categories
Funding
- Actelion Bayer
- Gilead
- United Therapeutics
- Actelion
- Bayer
- National Institutes of Health [R24 HL123767, R01 HL107577]
- Actelion Pharmaceuticals
- American Board of Internal Medicine
- Novartis
- DC Devices
- AstraZeneca
- Alnylam Pharmaceuticals
- Pulmonary Hypertension Association
- American Society of Echocardiography
- GlaxoSmithKline
- Bristol-Myers Squibb
Ask authors/readers for more resources
Pulmonary hypertension (PH) is common and may result from a number of disorders, including left heart disease, lung disease, and chronic thromboembolic disease. Pulmonary arterial hypertension (PAH) is an uncommon disease characterized by progressive remodeling of the distal pulmonary arteries, resulting in elevated pulmonary vascular resistance and, eventually, in right ventricular failure. Over the past decades, knowledge of the basic pathobiology of PAH and its natural history, prognostic indicators, and therapeutic options has exploded. A thorough evaluation of a patient is critical to correctly characterize the PH. Cardiac studies, including echocardiography and right heart catheterization, are key elements in the assessment. Given the multitude of treatment options currently available for PAH, assessment of risk and response to therapy is critical in long-term management. This review also underscores unique situations, including perioperative management, intensive care unit management, and pregnancy, and highlights the importance of collaborative care of the PAH patient through a multidisciplinary approach. (C) 2015 by the American College of Cardiology Foundation.
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