scholarly journals Letter by Hernandez-Gonzalez et al Regarding Article, “Phenotypic Characterization of EIF2AK4 Mutation Carriers in a Large Cohort of Patients Diagnosed Clinically With Pulmonary Arterial Hypertension”

Circulation ◽  
2018 ◽  
Vol 137 (22) ◽  
pp. 2411-2412 ◽  
Author(s):  
Ignacio Hernandez-Gonzalez ◽  
Paula Navas ◽  
Pilar Escribano-Subias
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Large Cohort ◽  
Phenotypic Characterization ◽  
Mutation Carriers ◽  
Pulmonary Arterial

scholarly journals Phenotypic Characterization of EIF2AK4 Mutation Carriers in a Large Cohort of Patients Diagnosed Clinically With Pulmonary Arterial Hypertension

Circulation ◽  
2017 ◽  
Vol 136 (21) ◽  
pp. 2022-2033 ◽  
Author(s):  
Charaka Hadinnapola ◽  
Marta Bleda ◽  
Matthias Haimel ◽  
Nicholas Screaton ◽  
Andrew Swift ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Large Cohort ◽  
Phenotypic Characterization ◽  
Mutation Carriers ◽  
Pulmonary Arterial

Model Characterization of Pulmonary Arterial Hypertension: Analysis of Lung Pathology with Respect to Human Disease

2020 ◽  
Author(s):  
◽  
Eptisam lambu
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Human Disease ◽  
Vascular Remodeling ◽  
Vascular Wall ◽  
Lung Pathology ◽  
Pulmonary Arterial ◽  
Lung Pathogenesis ◽  
Arterial Endothelial Cells

Pulmonary arterial hypertension (PAH) is a rare multifactorial disease characterized by abnormal high blood pressure in the pulmonary artery, or increased pulmonary vascular resistance (PVR), caused by obstruction in the small arteries of the lung. Increased PVR is also thought to be caused by abnormal vascular remodeling, due to thickening of the pulmonary vascular wall resulting from significant hypertrophy of pulmonary arterial smooth-muscle cells (PASMCs) and increased proliferation/impaired apoptosis of pulmonary arterial endothelial cells (PAECs). Herein, we investigated the mechanisms and explored molecular pathways mediating the lung pathogenesis in two PAH rat models: Monocrotaline (MCT) and Sugen5416/Hypoxia (SuHx). We analyzed these disease models to determine where the vasculature shows the most severe PAH pathology and which model best recapitulates the human disease. We investigated the role vascular remodeling, hypoxia, cell proliferation, apoptosis, DNA damage and inflammation play in the pathogenesis of PAH. Neither model recapitulated all features of the human disease, however each model presented with some of the pathology seen in PAH patients.

scholarly journals Low-grade albuminuria in pulmonary arterial hypertension

2019 ◽  
Vol 9 (2) ◽  
pp. 204589401882456 ◽  
Author(s):  
Nils P. Nickel ◽  
Vinicio A. de Jesus Perez ◽  
Roham T. Zamanian ◽  
Joshua P. Fessel ◽  
Joy D. Cogan ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Vascular Dysfunction ◽  
Urinary Albumin ◽  
Low Grade ◽  
Healthy Controls ◽  
Creatinine Ratio ◽  
Poor Outcome ◽  
Mutation Carriers ◽  
Pulmonary Arterial

Low-grade albuminuria, determined by the urinary albumin to creatinine ratio, has been linked to systemic vascular dysfunction and is associated with cardiovascular mortality. Pulmonary arterial hypertension is related to mutations in the bone morphogenetic protein receptor type 2, pulmonary vascular dysfunction and is increasingly recognized as a systemic disease. In a total of 283 patients (two independent cohorts) diagnosed with pulmonary arterial hypertension, 18 unaffected BMPR2 mutation carriers and 68 healthy controls, spot urinary albumin to creatinine ratio and its relationship to demographic, functional, hemodynamic and outcome data were analyzed. Pulmonary arterial hypertension patients and unaffected BMPR2 mutation carriers had significantly elevated urinary albumin to creatinine ratios compared with healthy controls ( P < 0.01; P = 0.04). In pulmonary arterial hypertension patients, the urinary albumin to creatinine ratio was associated with older age, lower six-minute walking distance, elevated levels of C-reactive protein and hemoglobin A1c, but there was no correlation between the urinary albumin to creatinine ratio and hemodynamic variables. Pulmonary arterial hypertension patients with a urinary albumin to creatinine ratio above 10 µg/mg had significantly higher rates of poor outcome ( P < 0.001). This study shows that low-grade albuminuria is prevalent in pulmonary arterial hypertension patients and is associated with poor outcome. This study shows that albuminuria in pulmonary arterial hypertension is associated with systemic inflammation and insulin resistance.

scholarly journals Characterization of First-Time Hospitalizations in Patients With Newly Diagnosed Pulmonary Arterial Hypertension in the REVEAL Registry

CHEST Journal ◽  
2014 ◽  
Vol 146 (5) ◽  
pp. 1263-1273 ◽  
Author(s):  
Charles D Burger ◽  
Pamela K Long ◽  
Monarch R Shah ◽  
Michael D McGoon ◽  
Dave P Miller ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Newly Diagnosed ◽  
Pulmonary Arterial ◽  
First Time

scholarly journals Characterization of a caveolin-1 mutation associated with both pulmonary arterial hypertension and congenital generalized lipodystrophy

Traffic ◽  
2016 ◽  
Vol 17 (12) ◽  
pp. 1297-1312 ◽  
Author(s):  
Bing Han ◽  
Courtney A. Copeland ◽  
Yumeko Kawano ◽  
Erika Berman Rosenzweig ◽  
Eric D. Austin ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Caveolin 1 ◽  
Congenital Generalized Lipodystrophy ◽  
Pulmonary Arterial

scholarly journals BMPR2 mutation status influences bronchial vascular changes in pulmonary arterial hypertension

2016 ◽  
Vol 48 (6) ◽  
pp. 1668-1681 ◽  
Author(s):  
Maria-Rosa Ghigna ◽  
Christophe Guignabert ◽  
David Montani ◽  
Barbara Girerd ◽  
Xavier Jaïs ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Bronchial Artery ◽  
Gene Mutations ◽  
Systemic Circulation ◽  
Systematic Analysis ◽  
Mutation Status ◽  
Mutation Carriers ◽  
Pulmonary Arterial ◽  
The Impact

The impact of bone morphogenetic protein receptor 2 (BMPR2) gene mutations on vascular remodelling in pulmonary arterial hypertension (PAH) is unknown. We sought to identify a histological profile of BMPR2 mutation carriers.Clinical data and lung histology from 44 PAH patients were subjected to systematic analysis and morphometry.Bronchial artery hypertrophy/dilatation and bronchial angiogenesis, as well as muscular remodelling of septal veins were significantly increased in PAH lungs carrying BMPR2 mutations. We found that patients displaying increased bronchial artery remodelling and bronchial microvessel density, irrespective of the mutation status, were more likely to suffer from severe haemoptysis. History of substantial haemoptysis (>50 mL) was significantly more frequent in BMPR2 mutation carriers. 43.5% of BMPR2 mutation carriers, as opposed to 9.5% of noncarriers, displayed singular large fibrovascular lesions, which appear to be closely related to the systemic lung vasculature.Our analysis provides evidence for the involvement of the pulmonary systemic circulation in BMPR2 mutation-related PAH. We show that BMPR2 mutation carriers are more prone to haemoptysis and that haemoptysis is closely correlated to bronchial arterial remodelling and angiogenesis; in turn, pronounced changes in the systemic vasculature correlate with increased pulmonary venous remodelling, creating a distinctive profile in PAH patients harbouring a BMPR2 mutation.

Characterization of Pulmonary Arterial Hypertension Patients Walking More Than 450 m in 6 Min at Diagnosis

CHEST Journal ◽  
2010 ◽  
Vol 137 (6) ◽  
pp. 1297-1303 ◽  
Author(s):  
Bruno Degano ◽  
Olivier Sitbon ◽  
Laurent Savale ◽  
Gilles Garcia ◽  
Dermot S. O'Callaghan ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Pulmonary Arterial
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