Results 1 to 10 of about 600,369 (246)

Clinical phenotyping of plasma thrombospondin-2 reveals relationship to right ventricular structure and function in pulmonary hypertension

ERJ Open Research, 2023
Background Converging evidence from proteogenomic analyses prioritises thrombospondin-2 (TSP2) as a potential biomarker for idiopathic or heritable pulmonary arterial hypertension (PAH).
Anna M. Dittrich, Julia Mienert, Julian Pott, Lena Engels, Christoph Sinning, Jan K. Hennigs, Hans Klose, Lars Harbaum   +7 more
doaj   +1 more source

Transcription factors in the pathogenesis of pulmonary arterial hypertension—Current knowledge and therapeutic potential

Frontiers in Cardiovascular Medicine, 2023
Pulmonary arterial hypertension (PAH) is a disease characterized by elevated pulmonary vascular resistance and pulmonary artery pressure. Mortality remains high in severe cases despite significant advances in management and pharmacotherapy.
Jakob Körbelin, Julius Klein, Julius Klein, Christiane Matuszcak, Christiane Matuszcak, Johannes Runge, Johannes Runge, Lars Harbaum, Hans Klose, Jan K. Hennigs, Jan K. Hennigs   +10 more
doaj   +1 more source

Residual risk identified in routine noninvasive follow-up assessments in pulmonary arterial hypertension

ERJ Open Research, 2023
Background The 2022 ESC/ERS guidelines on pulmonary hypertension recommend noninvasive risk assessments based on three clinical variables during follow-up in patients with pulmonary arterial hypertension (PAH).
Jonna Ostermann, Julian Pott, Jan K. Hennigs, Kevin Roedl, Christoph Sinning, Lars Harbaum, Hans Klose   +6 more
doaj   +1 more source

Sotatercept for the Treatment of Pulmonary Arterial Hypertension.

New England Journal of Medicine, 2021
BACKGROUND Pulmonary arterial hypertension is characterized by pulmonary vascular remodeling, cellular proliferation, and poor long-term outcomes.
M. Humbert, V. McLaughlin, J. Gibbs, M. Gomberg-Maitland, M. Hoeper, I. Preston, R. Souza, A. Waxman, Pilar Escribano Subías, J. Feldman, G. Meyer, D. Montani, K. Olsson, S. Manimaran, J. Barnes, P. Linde, J. de Oliveira Pena, D. Badesch   +17 more
semanticscholar   +1 more source

A cluster of heritable pulmonary arterial hypertension cases in a family with all three siblings carrying the same novel AQP1 c.273C>G variant‐a case report

Pulmonary Circulation, 2023
Approximately 25%–30% of patients diagnosed with idiopathic pulmonary arterial hypertension (PAH) have a clustered underlying Mendelian genetic cause and should be classified as heritable PAH (HPAH).
Kae‐Woei Liang, Sheng‐Kai Chang, Yu‐Wei Chen, Wan‐Jane Tsai, Kuo‐Yang Wang   +4 more
doaj   +1 more source

BMPR2 Mutation and Metabolic Reprogramming in Pulmonary Arterial Hypertension

Circulation Research, 2023
: Pulmonary arterial hypertension forms the first and most severe of the 5 categories of pulmonary hypertension. Disease pathogenesis is driven by progressive remodeling of peripheral pulmonary arteries, caused by the excessive proliferation of vascular ...
Iona Cuthbertson, N. Morrell, P. Caruso
semanticscholar   +1 more source

COMPERA 2.0: a refined four-stratum risk assessment model for pulmonary arterial hypertension

European Respiratory Journal, 2021
Background Risk stratification plays an essential role in the management of patients with pulmonary arterial hypertension (PAH). The current European guidelines propose a three-stratum model to categorise risk as low, intermediate or high, based on the ...
M. Hoeper, C. Pausch, K. Olsson, D. Huscher, D. Pittrow, E. Grünig, G. Staehler, C. Vizza, H. Gall, O. Distler, C. Opitz, J. Gibbs, M. Delcroix, H. Ghofrani, D. Park, R. Ewert, H. Kaemmerer, H. Kabitz, D. Skowasch, J. Behr, K. Milger, M. Halank, H. Wilkens, H. Seyfarth, M. Held, D. Dumitrescu, I. Tsangaris, A. Vonk-Noordegraaf, S. Ulrich, H. Klose, M. Claussen, T. Lange, S. Rosenkranz   +32 more
semanticscholar   +1 more source

Mortality in Pulmonary Arterial Hypertension in the Modern Era: Early Insights From the Pulmonary Hypertension Association Registry

Journal of the American Heart Association : Cardiovascular and Cerebrovascular Disease, 2022
Background Current mortality data for pulmonary arterial hypertension (PAH) in the United States are based on registries that enrolled patients prior to 2010.
Kevin Y. Chang, S. Duval, D. Badesch, T. Bull, M. Chakinala, T. De Marco, R. Frantz, A. Hemnes, S. Mathai, E. Rosenzweig, J. Ryan, T. Thenappan   +11 more
semanticscholar   +1 more source

Sotatercept for the treatment of pulmonary arterial hypertension: PULSAR open-label extension

European Respiratory Journal, 2022
Background In participants with pulmonary arterial hypertension, 24 weeks of sotatercept resulted in a significantly greater reduction from baseline in pulmonary vascular resistance than placebo.
M. Humbert, V. McLaughlin, J. Gibbs, M. Gomberg-Maitland, M. Hoeper, I. Preston, Rogério Souza, A. Waxman, H. Ghofrani, Pilar Escribano Subías, J. Feldman, G. Meyer, D. Montani, K. Olsson, S. Manimaran, J. de Oliveira Pena, D. Badesch   +16 more
semanticscholar   +1 more source

Phenotyping of idiopathic pulmonary arterial hypertension: a registry analysis

The Lancet Respiratory Medicine, 2022
Summary Background Among patients meeting diagnostic criteria for idiopathic pulmonary arterial hypertension (IPAH), there is an emerging lung phenotype characterised by a low diffusion capacity for carbon monoxide (DLCO) and a smoking history.
M. Hoeper, K. Dwivedi, C. Pausch, Robert A. Lewis, K. Olsson, D. Huscher, D. Pittrow, E. Grünig, G. Staehler, C. Vizza, H. Gall, O. Distler, C. Opitz, J. Gibbs, M. Delcroix, D. Park, H. Ghofrani, R. Ewert, H. Kaemmerer, H. Kabitz, D. Skowasch, J. Behr, K. Milger, T. Lange, H. Wilkens, H. Seyfarth, M. Held, D. Dumitrescu, I. Tsangaris, A. Vonk-Noordegraaf, S. Ulrich, H. Klose, M. Claussen, S. Eisenmann, Kai-Helge Schmidt, A. Swift, A. Thompson, C. Elliot, S. Rosenkranz, R. Condliffe, D. Kiely, M. Halank   +41 more
semanticscholar   +1 more source