title: Current clinical management of pulmonary arterial hypertension.
abstract: During the past 2 decades, there has been a tremendous evolution in the evaluation and care of patients with pulmonary arterial hypertension (PAH). The introduction of targeted PAH therapy consisting of prostacyclin and its analogs, endothelin antagonists, phosphodiesterase-5 inhibitors, and now a soluble guanylate cyclase activator have increased therapeutic options and potentially reduced morbidity and mortality; yet, none of the current therapies have been curative. Current clinical management of PAH has become more complex given the focus on early diagnosis, an increased number of available therapeutics within each mechanistic class, and the emergence of clinically challenging scenarios such as perioperative care. Efforts to standardize the clinical care of patients with PAH have led to the formation of multidisciplinary PAH tertiary care programs that strive to offer medical care based on peer-reviewed evidence-based, and expert consensus guidelines. Furthermore, these tertiary PAH centers often support clinical and basic science research programs to gain novel insights into the pathogenesis of PAH with the goal to improve the clinical management of this devastating disease. In this article, we discuss the clinical approach and management of PAH from the perspective of a single US-based academic institution. We provide an overview of currently available clinical guidelines and offer some insight into how we approach current controversies in clinical management of certain patient subsets. We conclude with an overview of our program structure and a perspective on research and the role of a tertiary PAH center in contributing new knowledge to the field.
authors.affiliation: Division of Pulmonary & Critical Care Medicine, Stanford University School of Medicine.|Vera Moulton Wall Center for Pulmonary Vascular Disease, Stanford University School of Medicine.
authors.forename: Roham T
authors.initials: RT
authors.lastname: Zamanian
authors.affiliation: Division of Pulmonary & Critical Care Medicine, Stanford University School of Medicine.|Vera Moulton Wall Center for Pulmonary Vascular Disease, Stanford University School of Medicine.
authors.forename: Kristina T
authors.initials: KT
authors.lastname: Kudelko
authors.affiliation: Division of Pulmonary & Critical Care Medicine, Stanford University School of Medicine.|Vera Moulton Wall Center for Pulmonary Vascular Disease, Stanford University School of Medicine.
authors.forename: Yon K
authors.initials: YK
authors.lastname: Sung
authors.affiliation: Division of Pulmonary & Critical Care Medicine, Stanford University School of Medicine.|Vera Moulton Wall Center for Pulmonary Vascular Disease, Stanford University School of Medicine.
authors.forename: Vinicio de Jesus
authors.initials: VJ
authors.lastname: Perez
authors.affiliation: Division of Pulmonary & Critical Care Medicine, Stanford University School of Medicine.|Vera Moulton Wall Center for Pulmonary Vascular Disease, Stanford University School of Medicine.
authors.forename: Juliana
authors.initials: J
authors.lastname: Liu
authors.affiliation: Division of Pulmonary & Critical Care Medicine, Stanford University School of Medicine.|Vera Moulton Wall Center for Pulmonary Vascular Disease, Stanford University School of Medicine.
authors.forename: Edda
authors.initials: E
authors.lastname: Spiekerkoetter
mesh_terms: D003422:Critical Care; D015199:Extracorporeal Membrane Oxygenation; D065627:Familial Primary Pulmonary Hypertension; D006353:Heart-Assist Devices; D006801:Humans; D006976:Hypertension, Pulmonary / Q000145:classification / Q000175:diagnosis / Q000628:therapy*; D016040:Lung Transplantation; D017410:Practice Guidelines as Topic; D012017:Referral and Consultation
keywords: pulmonary arterial hypertension