Skip to Main content Skip to Navigation
Journal articles

Beyond the Lungs: Systemic Manifestations of Pulmonary Arterial Hypertension

Abstract : Pulmonary 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.
Complete list of metadatas

Cited literature [93 references]  Display  Hide  Download

https://www.hal.inserm.fr/inserm-02566746
Contributor : Frédéric Perros <>
Submitted on : Thursday, May 7, 2020 - 12:07:14 PM
Last modification on : Monday, July 20, 2020 - 1:06:06 PM

File

 Restricted access
To satisfy the distribution rights of the publisher, the document is embargoed until : jamais

Please log in to resquest access to the document

Identifiers

Collections

Citation

Nils Nickel, Ke Yuan, Peter Dorfmüller, Steeve Provencher, Yen-Chun Lai, et al.. Beyond the Lungs: Systemic Manifestations of Pulmonary Arterial Hypertension. American Journal of Respiratory and Critical Care Medicine, American Thoracic Society, 2020, 201, pp.148 - 157. ⟨10.1164/rccm.201903-0656CI⟩. ⟨inserm-02566746⟩

Share

Metrics

Record views

53