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Semin Respir Crit Care Med 2023; 44(06): 746-761
DOI: 10.1055/s-0043-1770118
Review Article

Management of Pulmonary Arterial Hypertension

Jason Weatherald
1   Division of Pulmonary Medicine, Department of Medicine, University of Alberta, Edmonton, Canada
,
Rhea A. Varughese
1   Division of Pulmonary Medicine, Department of Medicine, University of Alberta, Edmonton, Canada
,
Jonathan Liu
2   Division of Respirology, Department of Medicine, University of Calgary, Calgary, Canada
,
Marc Humbert
3   Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
4   Service de Pneumologie et Soins Intensifs Respiratoires, Assistance Publique Hôpitaux de Paris, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
5   Hôpital Marie Lannelongue, Le Plessis Robinson, INSERM UMR_S 999, France
› Institutsangaben
Funding J.W. is funded by the Canadian Institutes of Health Research and the Heart and Stroke Foundation of Canada.
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Abstract

Pulmonary arterial hypertension (PAH) is a rare pulmonary vascular disease characterized by progressive pulmonary arterial remodeling, increased pulmonary vascular resistance, right ventricular dysfunction, and reduced survival. Effective therapies have been developed that target three pathobiologic pathways in PAH: nitric oxide, endothelin-1, and prostacyclin. Approved therapies for PAH include phosphodiesterase type-5 inhibitors, soluble guanylate cyclase stimulators, endothelin receptor antagonists, prostacyclin analogs, and prostacyclin receptor agonists. Management of PAH in the modern era incorporates multidimensional risk assessment to guide the use of these medications. For patients with PAH and without significant comorbidities, current guidelines recommend two oral medications (phosphodiesterase type-5 inhibitor and endothelin receptor antagonist) for low- and intermediate-risk patients, with triple therapy including a parenteral prostacyclin to be considered in those at high or intermediate-high risk. Combination therapy may be poorly tolerated and less effective in patients with PAH and cardiopulmonary comorbidities. Thus, a single-agent approach with individualized decisions to add-on other PAH therapies is recommended in older patients and those with significant comorbid conditions. Management of PAH is best performed in multidisciplinary teams located in experienced centers. Other core pillars of PAH management include supportive and adjunctive treatments including oxygen, diuretics, rehabilitation, and anticoagulation in certain patients. Patients with PAH who progress despite optimal treatment or who are refractory to best medical care should be referred for lung transplantation, if eligible. Despite considerable progress, PAH is often fatal and new therapies that reverse the disease and improve outcomes are desperately needed.

Keywords

pulmonary arterial hypertension - management - treatment - risk assessment


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Artikel online veröffentlicht:
27. Juni 2023

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