Pulmonary arterial hypertension: pathogenesis and clinical management

T Thenappan, ML Ormiston, JJ Ryan, SL Archer - Bmj, 2018 - bmj.com
Pulmonary hypertension is defined as a resting mean pulmonary artery pressure of 25 mm
Hg or above. This review deals with pulmonary arterial hypertension (PAH), a type of …

Sotatercept for the treatment of pulmonary arterial hypertension

M Humbert, V McLaughlin, JSR Gibbs… - … England Journal of …, 2021 - Mass Medical Soc
Background Pulmonary arterial hypertension is characterized by pulmonary vascular
remodeling, cellular proliferation, and poor long-term outcomes. Dysfunctional bone …

Inflammation and immunity in the pathogenesis of pulmonary arterial hypertension

M Rabinovitch, C Guignabert, M Humbert… - Circulation …, 2014 - Am Heart Assoc
This review summarizes an expanding body of knowledge indicating that failure to resolve
inflammation and altered immune processes underlie the development of pulmonary arterial …

Cell death in the lung: the apoptosis–necroptosis axis

M Sauler, IS Bazan, PJ Lee - Annual review of physiology, 2019 - annualreviews.org
Regulated cell death is a major mechanism to eliminate damaged, infected, or superfluous
cells. Previously, apoptosis was thought to be the only regulated cell death mechanism; …

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 …

Pulmonary arterial hypertension: the clinical syndrome

YC Lai, KC Potoka, HC Champion, AL Mora… - Circulation …, 2014 - Am Heart Assoc
Pulmonary arterial hypertension is a progressive disorder in which endothelial dysfunction
and vascular remodeling obstruct small pulmonary arteries, resulting in increased …

A novel channelopathy in pulmonary arterial hypertension

L Ma, D Roman-Campos, ED Austin… - … England Journal of …, 2013 - Mass Medical Soc
Background Pulmonary arterial hypertension is a devastating disease with high mortality.
Familial cases of pulmonary arterial hypertension are usually characterized by autosomal …

Modern age pathology of pulmonary arterial hypertension

E Stacher, BB Graham, JM Hunt… - American journal of …, 2012 - atsjournals.org
Rationale: The impact of modern treatments of pulmonary arterial hypertension (PAH) on
pulmonary vascular pathology remains unknown. Objectives: To assess the spectrum of …

Advances in therapeutic interventions for patients with pulmonary arterial hypertension

M Humbert, EMT Lau, D Montani, X Jaïs, O Sitbon… - Circulation, 2014 - Am Heart Assoc
Pulmonary arterial hypertension (PAH) is a disease characterized by progressive
remodeling of the distal pulmonary arteries, resulting in the loss of vascular cross-sectional …

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 …