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Primary pulmonary hypertension (PPH) is a rare disease characterized by elevated pulmonary artery pressure with no apparent cause. PPH is also termed precapillary pulmonary hypertension or, more recently, idiopathic pulmonary arterial hypertension (IPAH). The term IPAH is now the preferred term for pulmonary arterial hypertension of unknown etiology; thus, IPAH represents pulmonary vascular disease with a spectrum of clinical presentations.
Secondary Pulmonary Hypertension
Pulmonary hypertension (PH) defined as a mean pulmonary arterial (PA) pressure of greater than 25 mm Hg at rest or greater than 30 mm Hg during exercise, is characterized by a progressive and sustained increase in pulmonary vascular resistance that eventually leads to right ventricular (RV) failure. Pulmonary hypertension is a life-threatening condition if untreated; treatment success rates vary based on etiology.
Current Guidelines and Expert Consenus:
A Report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association Developed in Collaboration With the American College of Chest Physicians; American Thoracic Society, Inc.; and the Pulmonary Hypertension Association.
2009 Focused Update Incorporated Into the ACC/AHA 2005 Guidelines for the Diagnosis and Management of Heart Failure in Adults
A Report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines Developed in Collaboration With the International Society for Heart and Lung Transplantation.
European Society of Cardiology Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension
Guidelines published by The Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT).
European Society of Cardiology Heart Failure Association Standards for Delivering Heart Failure Care
This document aims to summarize the key elements which should be involved in, as well as some more desirable features which can improve the delivery of care in a HF management programme, while bearing in mind that the specifics of the service may vary from site to site.
Self-care Management of Heart Failure: Practical Recommendations From the Patient Care Committee of the Heart Failure Association of the European Society of Cardiology
This paper summarizes the available evidence, promotes self-care management, and aims to provide practical advice for health professionals delivering care to HF patients. It also defines avenues of research to optimize self-care strategies in a number of key areas to derive further benefits.
A Report of the American College of Cardiology Foundation Appropriate Use Criteria Task Force, American Society of Echocardiography, American Heart Association, American Society of Nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, Society of Critical Care Medicine, Society of Cardiovascular Computed Tomography, and Society for Cardiovascular Magnetic Resonance Endorsed by the American College of Chest Physicians
The Merck Manual for Healthcare Professionals
Chest, July 2004
Chest, June, 2009
Perspectives on the Optimal Management of Pulmonary Arterial Hypertension
Medscape, April 2011
Management of PAH: When and How to Use IV Agents
Medscape, August 2011
Striving to Optimize Individual Patient Outcomes in Pulmonary Arterial Hypertension
Medscape, April 2011
J Am Coll Cardiol, 2006
IMAJ, February, 2008
Chest, July, 2007
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October 2011 - Page Reviewed and Updated.
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