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Relevant issues in the pathology and pathobiology of pulmonary hypertension.

Abstract：

:Knowledge of the pathobiology of pulmonary hypertension (PH) continues to accelerate. However, fundamental gaps remain in our understanding of the underlying pathological changes in pulmonary arteries and veins in the different forms of this syndrome. Although PH primarily affects the arteries, venous disease is increasingly recognized as an important entity. Moreover, prognosis in PH is determined largely by the status of the right ventricle, rather than the levels of pulmonary artery pressures. It is increasingly clear that although vasospasm plays a role, PH is an obstructive lung panvasculopathy. Disordered metabolism and mitochondrial structure, inflammation, and dysregulation of growth factors lead to a proliferative, apoptosis-resistant state. These abnormalities may be acquired, genetically mediated as a result of mutations in bone morphogenetic protein receptor-2 or activin-like kinase-1, or epigenetically inherited (as a result of epigenetic silencing of genes such as superoxide dismutase-2). There is a pressing need to better understand how the pathobiology leads to severe disease in some patients versus mild PH in others. Recent recognition of a potential role of acquired abnormalities of mitochondrial metabolism in the right ventricular myocytes and pulmonary vascular cells suggests new therapeutic approaches, diagnostic modalities, and biomarkers. Finally, dissection of the role of pulmonary inflammation in the initiation and promotion of PH has revealed a complex yet fascinating interplay with pulmonary vascular remodeling, promising to lead to novel therapeutics and diagnostics. Emerging concepts are also relevant to the pathobiology of PH, including a role for bone marrow and circulating progenitor cells and microribonucleic acids. Continued interest in the interface of the genetic basis of PH and cellular and molecular pathogenetic links should further expand our understanding of the disease.

journal_name

J Am Coll Cardiol

journal_title

Journal of the American College of Cardiology

authors

Tuder RM,Archer SL,Dorfmüller P,Erzurum SC,Guignabert C,Michelakis E,Rabinovitch M,Schermuly R,Stenmark KR,Morrell NW

doi

10.1016/j.jacc.2013.10.025

subject

Has Abstract

pub_date

2013-12-24 00:00:00

pages

D4-12

issue

25 Suppl

eissn

0735-1097

issn

1558-3597

pii

S0735-1097(13)05868-3

journal_volume

pub_type

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Relevant issues in the pathology and pathobiology of pulmonary hypertension.