ArticleEuropean heart journal. Cardiovascular Imaging2019
Proximal pulmonary vascular stiffness as a prognostic factor in children with pulmonary arterial hypertension.
Article in European heart journal. Cardiovascular Imaging, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 32 papers.
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Who cites it
32 citing papers in PubMed, 47 citations in OpenAlex.
- Postnatal pulmonary artery development from transcript to tissue.Journal of the Royal Society, Interface · 2026Article
- Article
- Cardiac MRI-derived ventricular-pulmonary arterial coupling predicts outcomes in connective tissue disease-associated pulmonary arterial hypertension patients.European journal of radiology open · 2025Article
- Pulmonary Artery Remodelling Assessed by Four-Dimensional Flow Magnetic Resonance Imaging in Pulmonary Arterial Hypertension and Atrial Septal Defect.CJC pediatric and congenital heart disease · 2025Article
- Pharmacologic Management of Segmental Pulmonary Hypertension in Children After Unifocalization and Pulmonary Artery Reconstruction: Initial Experience.Pulmonary circulation · 2025Article
- Evaluation of pulmonary arterial stiffness in post mild COVID-19 patients: a pilot prospective study.Journal of cardiovascular imaging · 2024Article
- Characterization of pulmonary arterial stiffness using cardiac MRI.The international journal of cardiovascular imaging · 2024Review
- Advanced Imaging Technologies for Assessing Tetralogy of Fallot: Insights Into Flow Dynamics.CJC pediatric and congenital heart disease · 2023Review
- 3D-bioprinted, phototunable hydrogel models for studying adventitial fibroblast activation in pulmonary arterial hypertension.Biofabrication · 2022Article
- Location matters: Offset in tissue-engineered vascular graft implantation location affects wall shear stress in porcine models.JTCVS open · 2022Article
- Computational modelling of multi-temporal ventricular-vascular interactions during the progression of pulmonary arterial hypertension.Journal of the Royal Society, Interface · 2022Article
- Assessment of pulmonary arterial stiffness in patients with systemic sclerosis without overt pulmonary hypertension.The international journal of cardiovascular imaging · 2022Observational
- A structural approach to 3D-printing arterial phantoms with physiologically comparable mechanical characteristics: Preliminary observations.Proceedings of the Institution of Mechanical Engineers. Part H, Journal of engineering in medicine · 2022Article
- Patient-Specific Inverse Modeling of In Vivo Cardiovascular Mechanics with Medical Image-Derived Kinematics as Input Data: Concepts, Methods, and Applications.Applied sciences (Basel, Switzerland) · 2022Article
- Pulmonary hypertension: Linking inflammation and pulmonary arterial stiffening.Frontiers in immunology · 2022Review
- Data-driven computational models of ventricular-arterial hemodynamics in pediatric pulmonary arterial hypertension.Frontiers in physiology · 2022Article
- Abnormal pulmonary flow is associated with impaired right ventricular coupling in patients with COPD.The international journal of cardiovascular imaging · 2021Article
- Role of cardiovascular magnetic resonance in pediatric pulmonary hypertension-novel concepts and imaging biomarkers.Cardiovascular diagnosis and therapy · 2021Review
- Metalloproteinases and their inhibitors are associated with pulmonary arterial stiffness and ventricular function in pediatric pulmonary hypertension.American journal of physiology. Heart and circulatory physiology · 2021Article
- Inverse modeling framework for characterizing patient-specific microstructural changes in the pulmonary arteries.Journal of the mechanical behavior of biomedical materials · 2021Article
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Authors and funding
9 authors at 4 institutions in 1 country.
Funding
Abstract
Aims: Main pulmonary artery (MPA) stiffness and abnormal flow haemodynamics in pulmonary arterial hypertension (PAH) are strongly associated with elevated right ventricular (RV) afterload and associated with disease severity and poor clinical outcomes in adults with PAH. However, the long-term effects of MPA stiffness on RV function in children with PAH remain poorly understood. This study is the first comprehensive evaluation of MPA stiffness in children with PAH, delineating the mechanistic relationship between flow haemodynamics and MPA stiffness as well as the prognostic ability of these measures regarding clinical outcomes. Methods and results: Fifty-six children diagnosed with PAH underwent baseline cardiac magnetic resonance (CMR) acquisition and were compared with 23 control subjects. MPA stiffness and wall shear stress (WSS) were evaluated using phase contrast CMR and were evaluated for prognostic potential along with standard RV volumetric and functional indices. Pulse wave velocity (PWV) was significantly increased (2.8 m/s vs. 1.4 m/s, P < 0.0001) and relative area change (RAC) was decreased (25% vs. 37%, P < 0.0001) in the PAH group, correlating with metrics of RV performance. Decreased WSS was associated with a decrease in RAC over time (r = 0.679, P < 0.001). For each unit increase in PWV, there was approximately a 3.2-fold increase in having a moderate clinical event. Conclusion: MPA stiffness assessed by non-invasive CMR was increased in children with PAH and correlated with RV performance, suggesting that MPA stiffness is a major contribution to RV dysfunction. PWV is predictive of moderate clinical outcomes, and may be a useful prognostic marker of disease activity in children with PAH.
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