Acquired Patient Factors Associated with Pulmonary Vascular Disease

Novel variants of seryl-tRNA synthetase resulting in HUPRA syndrome featured in pulmonary hypertension

Fan Yang, Dan Wang, Xuehua Zhang, Haoqin Fan, Yu Zheng, Zhenghui Xiao, Zhi Chen, Yunbin Xiao, Qiming LiuSecond Xiangya Hospital and Central South University. Hunan Children’s Hospital. Fujian Children’s Hospital and Fujian Medical University.China Frontiers in Cardiovascular MedicineFront Cardiovasc Med 2023; DOI: 10.3389/fcvm.2022.1058569 AbstractHyperuricemia, pulmonary hypertension, and renal failure in infancy and alkalosis syndrome (HUPRA syndrome) […]

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Hidden cardiovascular morbidity in children and young adults born with congenital diaphragmatic hernia: A population-based study

Katarina Övermo Tydén, Felicia Nordenstam, Björn Frenckner, Carmen Mesas BurgosKarolinska University Hospital. Sweden Journal of Pediatric SurgeryJ Pediatr Surg 2022; 57: 510-515DOI: 10.1016/j.jpedsurg.2022.03.028 AbstractIntroduction: Congenital diaphragmatic (CDH) hernia is a rare congenital malformation with considerable mortality and morbidity in the neonatal period. The majority of the children today survive but little is known about long term cardiovascular

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Acquired von Willebrand syndrome (AVWS) type 2, characterized by decreased high molecular weight multimers, is common in children with severe pulmonary hypertension (PH)

Ivonne Wieland, Franziska Diekmann, Julia Carlens, Laura Hinze, Katharina Lambeck, Thomas Jack, Georg HansmannHannover Medical School.Germany Frontiers in PediatricsFront Pediat 2022; 10: DOI: 10.3389/fped.2022.1012738 AbstractBackground and objectives: Emerging evidence suggests that increased degradation of von Willebrand factor and decrease in high molecular weight multimers occurs in patients with pulmonary hypertension (PH). However, the link between acquired von

Acquired von Willebrand syndrome (AVWS) type 2, characterized by decreased high molecular weight multimers, is common in children with severe pulmonary hypertension (PH) Read More »

A case of acquired von Willebrand disease in severe pediatric pulmonary hypertension contributing to bleeding following reverse Potts shunt

Rachel T. Sullivan, Clara Lo, Elisabeth Martin, Rebecca J. Kameny, Rachel K. HopperStanford University School of Medicine and Lucile Packard Children’s Hospital.United States Pulmonary CirculationPulm Circ 2022; 12: DOI: 10.1002/pul2.12042 AbstractThe reverse Potts shunt is increasingly used as a palliative measure for end-stage pulmonary arterial hypertension (PAH) as a means to offload the right ventricle and

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Kynurenine metabolites predict survival in pulmonary arterial hypertension: A role for IL-6/IL-6Rα

Zongye Cai, Siyu Tian, Theo Klein, Ly Tu, Laurie W. Geenen, Thomas Koudstaal, Annemien E. van den Bosch, Yolanda B. de Rijke, Irwin K. M. Reiss, Eric Boersma, Claude van der Ley, Martijn Van Faassen, Ido Kema, Dirk J. Duncker, Karin A. Boomars, Karin Tran‑Lundmark, Christophe Guignabert, Daphne MerkusErasmus MC University Medical Center. Zhejiang University

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Vascular Disorders of Pregnancy Increase Susceptibility to Neonatal Pulmonary Hypertension in High-Altitude Populations

Alexandra Heath-Freudenthal, Lilian Toledo-Jaldin, Inge von Alvensleben, Litzi Lazo Vega, Rodrigo Mizutani, Margaret Stalker, Hussna Yasini, Fanny Mendizabal, Jesus Dorado Madera, William Mundo, Melany Castro-Monrroy, Julie A. Houck, Any Moreno-Aramayo, Valquiria Miranda-Garrido, Emily J. Su, Dino A. Giussani, Steven H. Abman, Lorna G. Moore, Colleen G. JulianKardiozentrum. Hospital Materno-Infantil. University of Colorado. Universidad Mayor de San Andrés. University of Cambridge.Bolivia, United States and United Kingdom HypertensionHypertension 2022; 79: 1286-1296DOI: 10.1161/HYPERTENSIONAHA.122.19078 AbstractBackground: Preeclampsia and fetal growth restriction increase cardiopulmonary disease risk for affected offspring and occur

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High haemoglobin levels at the time of occlusion predict worse outcome for patients with patent ductus arteriosus and pulmonary hypertension

Kanta Kishi, Jirayut Jarutach, Yinn Khurn Wong, Marhisham Che Mood, Geetha Kandhavello, Mazeni Alwi, Ming Chern Leong Osaka Medical and Pharmaceutical University. Prince of Songkla University. Serdang Hospital. Sarawak General Hospital. Institut Jantung Negara.Japan, Thailand and Malaysia Cardiology in the YoungCardiol Young 2022; DOI: 10.1017/S104795112200244X AbstractIntroduction: Current guidelines discourage shunt closure in patients with pulmonary vascular resistance

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Transpulmonary generation of cell-free hemoglobin contributes to vascular dysfunction in pulmonary arterial hypertension via dysregulated clearance mechanisms

Jamie E. Meegan, Vern Eric Kerchberger, Niki L. Fortune, Joel Brennan McNeil, Julie A. Bastarache, Eric D. Austin, Lorraine B. Ware, Anna R. Hemnes, Evan L. BrittainVanderbilt University Medical CenterUnited States Pulmonary CirculationPulm Circ 2023; 13: DOI: 10.1002/pul2.12185 AbstractCirculating cell-free hemoglobin (CFH) is elevated in pulmonary arterial hypertension (PAH) and associated with poor outcomes but the

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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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