Molecular genetic framework underlying pulmonary arterial hypertension

L Southgate, RD Machado, S Gräf… - Nature Reviews …, 2020 - nature.com
Pulmonary arterial hypertension (PAH) is a rare, progressive disorder typified by occlusion
of the pulmonary arterioles owing to endothelial dysfunction and uncontrolled proliferation of …

Definitions and diagnosis of pulmonary hypertension

MM Hoeper, HJ Bogaard, R Condliffe, R Frantz… - Journal of the American …, 2013 - jacc.org
Pulmonary hypertension (PH) is defined by a mean pulmonary artery pressure≥ 25 mm Hg
at rest, measured during right heart catheterization. There is still insufficient evidence to add …

Selective enhancement of endothelial BMPR-II with BMP9 reverses pulmonary arterial hypertension

L Long, ML Ormiston, X Yang, M Southwood, S Gräf… - Nature medicine, 2015 - nature.com
Genetic evidence implicates the loss of bone morphogenetic protein type II receptor (BMPR-
II) signaling in the endothelium as an initiating factor in pulmonary arterial hypertension …

Management of pulmonary arterial hypertension

VV McLaughlin, SJ Shah, R Souza… - Journal of the American …, 2015 - jacc.org
Pulmonary hypertension (PH) is common and may result from a number of disorders,
including left heart disease, lung disease, and chronic thromboembolic disease. Pulmonary …

Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era

M Humbert, O Sitbon, A Chaouat, M Bertocchi… - Circulation, 2010 - Am Heart Assoc
Background—Novel therapies have recently become available for pulmonary arterial
hypertension. We conducted a study to characterize mortality in a multicenter prospective …

ACCF/AHA 2009 expert consensus document on pulmonary hypertension: a report of the American College of Cardiology Foundation task force on expert consensus …

Writing Committee Members, VV McLaughlin… - Circulation, 2009 - Am Heart Assoc
ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension | Circulation Science
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Updated clinical classification of pulmonary hypertension

G Simonneau, IM Robbins, M Beghetti… - Journal of the American …, 2009 - jacc.org
The aim of a clinical classification of pulmonary hypertension (PH) is to group together
different manifestations of disease sharing similarities in pathophysiologic mechanisms …

FK506 activates BMPR2, rescues endothelial dysfunction, and reverses pulmonary hypertension

E Spiekerkoetter, X Tian, J Cai… - The Journal of …, 2013 - Am Soc Clin Investig
Dysfunctional bone morphogenetic protein receptor-2 (BMPR2) signaling is implicated in the
pathogenesis of pulmonary arterial hypertension (PAH). We used a transcriptional high …

Cellular and molecular pathobiology of pulmonary arterial hypertension

M Humbert, NW Morrell, SL Archer… - Journal of the American …, 2004 - jacc.org
Pulmonary arterial hypertension (PAH) has a multifactorial pathobiology. Vasoconstriction,
remodeling of the pulmonary vessel wall, and thrombosis contribute to increased pulmonary …

Clinical classification of pulmonary hypertension

G Simonneau, N Galiè, LJ Rubin, D Langleben… - Journal of the American …, 2004 - jacc.org
Abstract In 1998, during the Second World Symposium on Pulmonary Hypertension (PH)
held in Evian, France, a clinical classification of PH was proposed. The aim of the Evian …