• Nils P Nickel, Ke Yuan, Peter Dorfmuller, Steeve Provencher, Yen-Chun Lai, Sebastien Bonnet, Eric D Austin, Carl D Koch, Alison Morris, Frédéric Perros, David Montani, Roham T Zamanian, and Vinicio A de Jesus Perez.
• Division of Pulmonary and Critical Care Medicine, Stanford University, Stanford, California.
• Am. J. Respir. Crit. Care Med. 2020 Jan 15; 201 (2): 148-157.

AbstractPulmonary arterial hypertension (PAH) is a disease characterized by progressive loss and remodeling of the pulmonary arteries, resulting in right heart failure and death. Until recently, PAH was seen as a disease restricted to the pulmonary circulation. However, there is growing evidence that patients with PAH also exhibit systemic vascular dysfunction, as evidenced by impaired brachial artery flow-mediated dilation, abnormal cerebral blood flow, skeletal myopathy, and intrinsic kidney disease. Although some of these anomalies are partially due to right ventricular insufficiency, recent data support a mechanistic link to the genetic and molecular events behind PAH pathogenesis. This review serves as an introduction to the major systemic findings in PAH and the evidence that supports a common mechanistic link with PAH pathophysiology. In addition, it discusses recent studies describing morphological changes in systemic vessels and the possible role of bronchopulmonary anastomoses in the development of plexogenic arteriopathy. On the basis of available evidence, we propose a paradigm in which metabolic abnormalities, genetic injury, and systemic vascular dysfunction contribute to systemic manifestations in PAH. This concept not only opens exciting research possibilities but also encourages clinicians to consider extrapulmonary manifestations in their management of patients with PAH.

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