Review
Cardiac & Cardiovascular Systems
Dunbar Ivy, Benjamin S. Frank
Summary: Pulmonary arterial hypertension (PAH) in children is associated with high morbidity and mortality. Recent advancements in diagnosis and treatment include updated definitions, FDA approval of the first targeted therapy, and improved risk stratification through advanced imaging and monitoring. New therapies have shown improved outcomes for affected children.
CURRENT OPINION IN CARDIOLOGY
(2021)
Article
Chemistry, Multidisciplinary
Yang-yang He, Yi Yan, Ji-wang Chen, Sheng Liu, Lu Hua, Xin Jiang, Xi-qi Xu, Dan Lu, Zhi-cheng Jing, Fu-xia Yan, Zhi-yan Han
Summary: Plasma metabolites in CHD-PAH patients exhibited significant alterations during the perioperative period of defect repair, with 17 metabolites correlated with the reduction in pulmonary arterial pressure. Changes in certain metabolites were positively linked to clinical measures such as DPAP, arterial bicarbonate, and dissolved CO2 gas, providing valuable insights for disease monitoring and therapeutic interventions.
ACTA PHARMACOLOGICA SINICA
(2022)
Article
Medicine, General & Internal
Susanne J. Maurer, Veronika Habdank, Juergen Horer, Peter Ewert, Oktay Tutarel
Summary: This study aimed to investigate the risk factors for poor outcome in patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD). The results showed that NT-proBNP is an independent predictor of all-cause mortality in PAH-CHD patients. The role of CRP and uric acid should be further studied.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Radiology, Nuclear Medicine & Medical Imaging
Ursula Reiter, Gabor Kovacs, Clemens Reiter, Corina Kraeuter, Volha Nizhnikava, Michael Fuchsjaeger, Horst Olschewski, Gert Reiter
Summary: MR 4D flow imaging allows for accurate non-invasive prediction and quantification of changes in mean pulmonary arterial pressure (mPAP) in adult patients with pulmonary hypertension (PH) or at risk of developing PH, with high accuracy and sensitivity.
EUROPEAN RADIOLOGY
(2021)
Article
Biophysics
Melody L. Dong, Ingrid S. Lan, Weiguang Yang, Marlene Rabinovitch, Jeffrey A. Feinstein, Alison L. Marsden
Summary: This study estimated hemodynamic and biomechanical metrics in the entire pediatric pulmonary artery tree with varying degrees of ventricular septal defect severity, and has implications for future research assessing the effects of abnormal mechanical stimuli on endothelial cells and vascular wall mechanics, particularly during the initiation and progression of PAH associated with congenital heart disease.
BIOMECHANICS AND MODELING IN MECHANOBIOLOGY
(2021)
Article
Critical Care Medicine
Rui-Sheng Wang, Shi Huang, Stephen W. Waldo, Edward Hess, Madhura Gokhale, Shelsey W. Johnson, Katarina Zeder, Gaurav Choudhary, Jane A. Leopold, William M. Oldham, Gabor Kovacs, Matthew S. Freiberg, Ryan J. Tedford, Bradley A. Maron, Evan L. Brittain
Summary: This study aims to identify clinical markers and therapeutic targets that inform survival in patients with pulmonary hypertension (PH). The results suggest that elevated pulmonary arterial compliance (PAC) greater than 3.0 ml/mmHg is associated with improved survival, especially in patients with precapillary PH.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2023)
Review
Cardiac & Cardiovascular Systems
Maolin Zhao, Jian Liu, Mei Xin, Ke Yang, Honghao Huang, Wenxin Zhang, Jinbao Zhang, Siyi He
Summary: Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is a progressive condition with uncertain physiological course. Omics technology, such as genomics, transcriptomics, epigenomics, proteomics, metabolomics, and multi-omics integration, has provided advanced techniques for comprehensive assessment of the disease and identification of treatment strategies. Recent research has made significant progress in understanding PAH-CHD through the application of omics approaches.
FRONTIERS IN CARDIOVASCULAR MEDICINE
(2023)
Article
Cardiac & Cardiovascular Systems
Patrick D. Evers, Patrick Quinn, Paul J. Critser, Benjamin S. Frank, Mohammad Alnoor, Laurie B. Armsby
Summary: Upon diagnosis of pulmonary hypertension in pediatrics, acute vasoreactivity testing (AVT) is commonly performed. This study aimed to examine the prognostic significance of serial AVT results in pediatric patients. The results showed that patients with subsequent AVT positive results had better event-free survival compared to those with initial and subsequent AVT negative results.
PULMONARY CIRCULATION
(2022)
Article
Critical Care Medicine
Rebecca R. Vanderpool, Kendall S. Hunter, Michael Insel, Joe G. N. Garcia, Edward J. Bedrick, Ryan J. Tedford, Franz P. Rischard
Summary: In patients with advanced PAH, RV-pulmonary arterial coupling could not differentiate irreversible RV failure at presentation, but showed an improving trend by follow-up. Early change in Eed and baseline RV ejection fraction were the best predictors of therapeutic response.
Article
Cardiac & Cardiovascular Systems
Raquel Luna-Lopez, Teresa Segura de la Cal, Fernando Sarnago Cebada, Irene Martin de Miguel, Williams Hinojosa, Alejandro Cruz-Utrilla, Maria Teresa Velazquez, Juan F. Delgado, Alberto Mendoza, Fernando Arribas Ynsaurriaga, Pilar Escribano-Subias
Summary: This study assessed the long-term effects of triple therapy with prostanoids on patients with PAH-CHD. The results showed that this treatment approach improved patients' exercise capacity, functional class, and NT-proBNP levels, with manageable side effects. It was particularly effective for patients with pre-tricuspid defects and non-Eisenmenger PAH-CHD.
Review
Peripheral Vascular Disease
Gary F. Mitchell
Summary: Aortic stiffness increases significantly with age and is linked to various adverse clinical outcomes. While evidence of its negative effects is overwhelming, incorporating measures of aortic stiffness into routine clinical practice has been slow.
Article
Cell Biology
Jing-jing Zhou, Huang Li, Li Li, Yue Li, Pei-he Wang, Xian-min Meng, Jian-guo He
Summary: Research has shown that CYLD plays a crucial role in the development of CHD-PAH by modulating HPASMC dysfunction and inflammation, thus influencing the progression of PAH. Inhibiting CYLD expression can attenuate PAH levels in rats and prevent pulmonary vascular remodeling.
JOURNAL OF CELLULAR PHYSIOLOGY
(2021)
Review
Medicine, General & Internal
Amalia Baroutidou, Alexandra Arvanitaki, Adam Hatzidakis, Georgia Pitsiou, Antonios Ziakas, Haralambos Karvounis, George Giannakoulas
Summary: Haemoptysis is a severe bleeding manifestation in the clinical course of pulmonary arterial hypertension associated with congenital heart disease. Dysfunction of the pulmonary vascular bed increases the risk of haemoptysis episodes, and despite treatment strategies like BAE, bleeding recurrences are prevalent. There is currently no clear guideline regarding the use of oral anticoagulation in patients with haemoptysis.
JOURNAL OF CLINICAL MEDICINE
(2022)
Article
Respiratory System
Johannes M. Douwes, Willemijn M. H. Zijlstra, Erika B. Rosenzweig, Mark-Jan Ploegstra, Usha S. Krishnan, Meindina G. Haarman, Marcus T. R. Roofthooft, Douwe Postmus, Hans L. Hillege, D. Dunbar Ivy, Rolf M. F. Berger
Summary: This study retrospectively analyzed data from 275 children with PAH and found that early initiation and higher doses of IV/SC prostanoid therapy, as well as combination with other targeted drugs, were associated with better outcomes. Transition from IV/SC prostanoid therapy to oral or inhaled therapies was found to be safe in selected patients who met specific hemodynamic criteria.
ANNALS OF THE AMERICAN THORACIC SOCIETY
(2022)
Review
Cardiac & Cardiovascular Systems
Gnalini Sathananthan, Robert D. Levy, John Swiston, Nathan W. Brunner, Elisa A. Bradley, Marla Kiess, Jasmine Grewal
Summary: Atrial septal defects with pulmonary arterial hypertension in adults can present a challenging clinical scenario. Literature continues to evolve in determining the best approach for this subset of patients. Clinical presentation, history, medical therapy, and closure options are key aspects to consider in managing these patients.
CARDIOLOGY IN REVIEW
(2021)