Pathology and pathobiology of pulmonary hypertension: state of the art and research perspectives

…,C Guignabert,S Bonnet, PDorfmüller… - European …, 2019 - Eur Respiratory Soc
临床and translational research has played a major role in advancing our understanding
of pulmonary hypertension (PH), including pulmonary arterial hypertension and other forms …

Relevant issues in the pathology and pathobiology of pulmonary hypertension

RM Tuder,SL Archer, PDorfmüller, SC Erzurum… - Journal of the American …, 2013 - jacc.org
Knowledge of the pathobiology of pulmonary hypertension (PH) continues to accelerate.
However, fundamental gaps remain in our understanding of the underlying pathological …

Platelet-derived growth factor expression and function in idiopathic pulmonary arterial hypertension

F Perros,D Montani, PDorfmüller… - American journal of …, 2008 - atsjournals.org
Rationale: Platelet-derived growth factor (PDGF) promotes the proliferation and migration of
pulmonary artery smooth muscle cells (PASMCs), and may play a role in the progression of …

Inflammation in pulmonary arterial hypertension

LC Price, SJ Wort, F Perros, PDorfmüller, A Huertas… - Chest, 2012 - Elsevier
Pulmonary arterial hypertension (PAH) is characterized by pulmonary vascular remodeling
of the precapillary pulmonary arteries, with excessive proliferation of vascular cells. Although …

Endothelial-to-mesenchymal transition in pulmonary hypertension

…, A Hautefort, C Péchoux, HJ Bogaard, PDorfmüller… - Circulation, 2015 - Am Heart Assoc
Background— The vascular remodeling responsible for pulmonary arterial hypertension (PAH)
involves predominantly the accumulation of α-smooth muscle actin–expressing …

Fibrous remodeling of the pulmonary venous system in pulmonary arterial hypertension associated with connective tissue diseases

PDorfmüller,M Humbert, F Perros, O Sanchez… - Human pathology, 2007 - Elsevier
Pulmonary arterial hypertension is a severe complication of connective tissue diseases. It is
currently well established that pulmonary arterial hypertension associated with connective …

Right ventricular diastolic impairment in patients with pulmonary arterial hypertension

…,WJ Paulus, CAC Ottenheijm, JT Marcus, PDorfmüller… - Circulation, 2013 - Am Heart Assoc
Background— The role of right ventricular (RV) diastolic stiffness in pulmonary arterial
hypertension (PAH) is not well established. Therefore, we investigated the presence and possible …

CX3C Chemokine Fractalkine in Pulmonary Arterial Hypertension

K Balabanian, A Foussat, PDorfmüller… - American journal of …, 2002 - atsjournals.org
Perivascular infiltrates composed of macrophages and lymphocytes have been described in
lung biopsies of patients displaying pulmonary arterial hypertension (PAH), suggesting that …

EIF2AK4 mutations cause pulmonary veno-occlusive disease, a recessive form of pulmonary hypertension

…, N Chelghoum,F Coulet,D Bonnet, PDorfmüller… - Nature …, 2014 - nature.com
Pulmonary veno-occlusive disease (PVOD) is a rare and devastating cause of pulmonary
hypertension that is characterized histologically by widespread fibrous intimal proliferation of …

Chemokine RANTES in severe pulmonary arterial hypertension

PDorfmuller, V Zarka, I Durand-Gasselin… - American journal of …, 2002 - atsjournals.org
The recent discovery that sporadic and familial primary pulmonary hypertension can be
associated with germline mutations of genes encoding receptor members of the transforming …