Management of Pulmonary Arterial Hypertension
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SECONDARY PREVENTION AND INTERVENTION (D. STEINBERG)
Management of Pulmonary Arterial Hypertension Jennalyn D. Mayeux 1 & Irene Z. Pan 2 & John Dechand 2 & Joshua A. Jacobs 2 & Tara L. Jones 3 & Stephen H. McKellar 4 & Emily Beck 1 & Nathan D. Hatton 1 & John J. Ryan 3 Accepted: 12 August 2020 # Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Purpose of Review This review focuses on the therapeutic management and individualized approach to Group 1 pulmonary arterial hypertension (PAH), utilizing Food and Drug Administration-approved PAH-specific therapies and various interventional and surgical options for PAH. Recent Findings The paradigm for the optimal management of PAH has shifted in recent years. Upfront combination therapy with an endothelin receptor antagonist and a phosphodiesterase 5 inhibitor is now widely accepted as standard of care. In addition, there is increasing emphasis on starting prostanoids early in order to delay time to clinical worsening. However, less is known regarding which prostanoid agent to initiate and the optimum time to do so. In order to facilitate shared decisionmaking, there is an increasing need for decision tools based on guidelines and collective clinical experiences to navigate between pharmacologic and interventional treatments, as well as explore innovative, therapeutic pathways for PAH. Summary The management of PAH has become increasingly complex. With a growing number of PAH-specific therapies, intimate knowledge of the therapeutics and the potential barriers to adherence are integral to providing optimal care for this highrisk patient population. While current PAH-specific therapies largely mediate their effects through pulmonary vasodilation, ongoing research efforts are focused on ways to disrupt the mechanisms leading to pulmonary vascular remodeling. By targeting aberrations identified in the metabolism and proliferative state of pulmonary vascular cells, novel PAH treatment pathways may be just on the horizon. Keywords Pulmonary hypertension . Therapeutics . Right heart failure . Pharmacology . Prostaglandin . Prostacyclin
Introduction Pulmonary hypertension (PH) describes an abnormal elevation in pulmonary arterial blood pressure [1]. The World Jennalyn D. Mayeux and Irene Z. Pan contributed equally to this work. This article is part of the Topical Collection on Secondary Prevention and Intervention * John J. Ryan [email protected] 1
Division of Pulmonary and Critical Care Medicine, Department of Medicine, University of Utah, Salt Lake City, UT 84132, USA
2
Department of Pharmacy, University of Utah Health, Salt Lake City, UT 84132, USA
3
Division of Cardiovascular Medicine, Department of Medicine, University of Utah, 30 North 1900 East, Room 4A100, Salt Lake City, UT 84132, USA
4
Division of Cardiothoracic Surgery, Department of Surgery, University of Utah, Salt Lake City, UT 84132, USA
Health Organization (WHO) classifies PH into five groups based on shared histology and pathophysiology. Group 1 pulmonary arterial hyper
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