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High Shear Stress Reduces ERG Causing Endothelial-Mesenchymal Transition and Pulmonary Arterial Hypertension.
Arterioscler Thromb Vasc Biol
December 2024
Department of Pediatrics (T.S., J.-R.M., Y.H.C., J.M.S., J. Kaplan, A.C., L.W., D.G., S.T., S.I., M.D., W.Y., A.L.M., M.R.).
Background: Computational modeling indicated that pathological high shear stress (HSS; 100 dyn/cm) is generated in pulmonary arteries (PAs; 100-500 µm) in congenital heart defects causing PA hypertension (PAH) and in idiopathic PAH with occlusive vascular remodeling. Endothelial-to-mesenchymal transition (EndMT) is a feature of PAH. We hypothesize that HSS induces EndMT, contributing to the initiation and progression of PAH.
View Article and Find Full Text PDFThe Minimally Important Difference in the Six-minute Walk Test Predicts Clinical Worsening in Pulmonary Arterial Hypertension.
Respir Med
December 2024
Department of Pulmonary Medicine, Respiratory Institute, Cleveland Clinic, Cleveland, Ohio.
Background: The minimally important difference (MID) in the six-minute walk test (6MWT) for pulmonary arterial hypertension (PAH) is estimated to be 33 meters using distributional and anchor-based methods. Quality of life was used as the anchor. Here, we sought to determine whether the MID is predictive of clinical worsening.
View Article and Find Full Text PDFRecent developments in connective tissue disease associated pulmonary arterial hypertension.
Int J Cardiol Congenit Heart Dis
June 2024
Centre for Rheumatology and Connective Tissue Diseases, University College London Medical School, London, UK.
Connective tissue disease associated pulmonary arterial hypertension (CTD-PAH) has benefited from the major treatment advances that have occurred within pulmonary hypertension over the past three decades. Inclusion of CTD-PAH cases in pivotal clinical trials led to regulatory approval and drug availability. This has improved outcomes but there are additional challenges for management.
View Article and Find Full Text PDFWhat can we learn from pathophysiology and therapeutic targetable pathways from all genetic causes and associations in PH?
Int J Cardiol Congenit Heart Dis
September 2024
VPD Heart and Lung Research Institute, University of Cambridge, United Kingdom.
Pulmonary hypertension (PH) encompasses a group of conditions which ultimately lead to elevated pulmonary arterial pressure. PH is classified into five subgroups, of which Group 1 pulmonary arterial hypertension (PAH), is the most extensively studied. Numerous causal genes have been identified in PAH, most notably germline mutations in bone morphogenetic protein receptor type 2 () and the wider BMP pathway.
View Article and Find Full Text PDFRecurrent internal carotid artery dissection due to missed diagnosis of Eagle's Syndrome.
World Neurosurg
December 2024
Department of Neurosurgery, Kantonsspital Aarau, Aarau, Switzerland.
The clinical image illustrates an unusual case of a patient with bilateral carotid artery dissection caused by compression from elongated styloid processes. In this case, the diagnosis was overlooked eight years earlier. Eagle's syndrome, marked by an elongated styloid process, can result in cervical artery dissection, highlighting the significance of recognizing this correlation in recurrent cases, which occur more frequently than idiopathic ICA dissections.
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