Review Articles Concerning Pulmonary Vascular Disease

A systematic review of clinical study evidence for pulmonary vasodilator therapy following surgery with cardiopulmonary bypass in children with CHD

Henry P. Foote, Christoph P. Hornik, Kevin D. Hill, Alexandre T. Rotta, Karan R. Kumar, Elizabeth J. ThompsonDuke University School of MedicineUnited States Cardiology in the YoungCardiol Young 2022; 20: DOI: 10.1017/S1047951122002293 AbstractObjectives: Complications from pulmonary hypertension are one of the leading contributors to morbidity and mortality post-cardiopulmonary bypass surgery in children with CHD. Pulmonary vasodilator therapies […]

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Channelopathy Genes in Pulmonary Arterial Hypertension

Carrie L. Welch, Wendy K. ChungColumbia University Irving Medical Center.United States BiomoleculesBiomolecules 2022; 12: DOI: 10.3390/biom12020265 AbstractPulmonary arterial hypertension (PAH) is a rare, progressive vasculopathy with significant cardiopulmonary morbidity and mortality. The underlying pathogenetic mechanisms are heterogeneous and current therapies aim to decrease pulmonary vascular resistance but no curative treatments are available. Causal genetic variants can

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Innovations in Pediatric Therapeutics Development: Principles for the Use of Bridging Biomarkers in Pediatric Extrapolation

Thomas R. Fleming, Christine E. Garnett, Laurie S. Conklin, Solange Corriol‑Rohou, Sudharshan Hariharan, Daphne Hsu, Guenther Mueller‑Velten, Yeruk Mulugeta, Ronald Portman, Mark D. Rothmann, Norman L. Stockbridge, Simon Wandel, Jialu Zhang, Lynne YaoUniversity of Washington. U.S. Food and Drug Administration. Janssen Research and Development. AstraZeneca. Children’s Hospital at Montefiore and Albert Einstein College of Medicine. Novartis Pharma AG and Novartis Pharmaceuticals.United States Therapeutic Innovation and Regulatory ScienceTher Innov Regul Sci 2023; 57: 109-120DOI: 10.1007/s43441-022-00445-6 AbstractEven with recent substantive improvements in health care in pediatric populations, considerable need remains

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BMPR2 Mutation and Metabolic Reprogramming in Pulmonary Arterial Hypertension

Iona Cuthbertson, Nicholas W. Morrel, Paola CarusoUniversity of Cambridge School of Clinical MedicineUnited Kingdom Circulation ResearchCirc Res 2023; 132(1):109-126DOI: 10.1161/CIRCRESAHA.122.321554 AbstractPulmonary 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 wall cells,

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Prematurity and Pulmonary Vein Stenosis: The Role of Parenchymal Lung Disease and Pulmonary Vascular Disease

Shilpa Vyas-Read, Nidhy P. Varghese, Divya Suthar, Carl Backes, Satyan Lakshminrusimha, Christopher J. Petit, Philip T. LevyEmory University and Children’s Healthcare of Atlanta. Baylor College of Medicine and Texas Children’s Hospital. Ohio State University and Nationwide Children’s Hospital. University of California Davis. Harvard University and Boston Children’s Hospital.United States Children (Basel)Children 2022; 9: DOI: 10.3390/children9050713 AbstractPulmonary

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The vascular phenotype of BPD: new basic science insights-new precision medicine approaches

Wojciech Durlak, Bernard ThébaudUniversity of Ottawa. Jagiellonian University Medical College. Poland and Canada Pediatric ResearchPediatr Res 2022; DOI: 10.1038/s41390-022-02428-7 AbstractBronchopulmonary dysplasia (BPD) is the most common complication of preterm birth. Up to 1/3 of children with BPD develop pulmonary hypertension (PH). PH increases mortality, the risk of adverse neurodevelopmental outcome and lacks effective treatment. Current vasodilator

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Primary pulmonary vein stenosis during infancy: state of the art review

David B. Frank, Philip T. Levy, Corey A. Stiver, Brian A. Boe, Christopher W. Baird, Ryan M. Callahan, Charles V. Smith, Rachel D. Vanderlaan, Carl H. BackesUniversity of Pennsylvania and Children’s Hospital of Philadelphia. Harvard University and Boston Children’s Hospital. Ohio State University and Nationwide Children’s Hospital. University of Washington and Seattle Children’s Research Institute.

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Risk stratification in adult and pediatric pulmonary arterial hypertension: A systematic review

Chantal Lokhorst, Sjoukje van der Werf, Rolf M. F. Berger, Johannes M. DouwesUniversity of Groningen, Beatrix Children’s Hospital and University Medical Center GroningenNetherlands Frontiers in Cardiovascular MedicineFront Cardiovasc Med 2022; 9: 1035453DOI: 10.3389/fcvm.2022.1035453 AbstractIntroduction: Currently, risk stratification is the cornerstone of determining treatment strategy for patients with pulmonary arterial hypertension (PAH). Since the 2015 European Society of

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Paediatric pulmonary arterial hypertension: updates on definition, classification, diagnostics and management

Erika B. Rosenzweig, Steven H. Abman, Ian Adatia, Maurice Beghetti, Damien Bonnet, Sheila Haworth, D. Dunbar Ivy, Rolf M. F. BergerColumbia University and New York Presbyterian Hospital. University of Colorado and Children’s Hospital Colorado. Glenwood Children’s Heart Clinic. University Hospitals of Geneva and Lausanne. Hôpital Necker Enfants Malades, AP-HP, Université Paris Descartes. University College London.

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Diagnosis and management of pulmonary hypertension in congenital diaphragmatic hernia

Shazia Bombal, Neil PatelStanford University and Lucille Packard Children’s Hospital. Royal Hospital for Children, Glasgow. United States and United Kingdom Seminars in Fetal and Neonatal MedicineSemin Fetal Neonatal Med 2022; 27: DOI: 10.1016/j.siny.2022.101383 AbstractCongenital diaphragmatic hernia (CDH) contributes to neonatal morbidity and mortality worldwide. Pulmonary hypertension (PH) is a key component of CDH pathophysiology and critical

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