Class 1. Heritable Pulmonary Hypertension

Characteristics, Long-term Survival, and Risk Assessment of Pediatric Pulmonary Arterial Hypertension in China: Insights From a National Multicenter Prospective Registry

Yuling Qian, Ruilin Quan, Xiaoxi Chen, Qing Gu, Changming Xiong, Huijun Han, Gangcheng Zhang, Yucheng Chen, Zaixin Yu, Hongyan Tian, Yuhao Liu, Xianyang Zhu, Shengqing Li, Caojin Zhang, Jianguo HeChinese Academy of Medical Sciences and Peking Union Medical College and Fuwai Hospital. School of Basic Medicine Peking Union Medical College. Wuhan Asia Heart Hospital. West […]

Characteristics, Long-term Survival, and Risk Assessment of Pediatric Pulmonary Arterial Hypertension in China: Insights From a National Multicenter Prospective Registry Read More »

TGF-β receptor mutations and clinical prognosis in Chinese pediatric patients with idiopathic/hereditary pulmonary arterial hypertension

Xinyu Zhang, Chen Zhang, Qiangqiang, Li, Hong GuBeijing Anzhen Hospital and Capital Medical University.China Pulmonary CirculationPulm Circ 2022; 12: DOI: 10.1002/pul2.12076 AbstractThe relationship between clinical prognosis and transforming growth factor-β (TGF-β) receptor mutations in Chinese pediatric patients with idiopathic/hereditary pulmonary arterial hypertension (IPAH/HPAH) remains unclear. We retrospectively studied the clinical characteristics and outcomes of pediatric patients

TGF-β receptor mutations and clinical prognosis in Chinese pediatric patients with idiopathic/hereditary pulmonary arterial hypertension Read More »

Association between Genotype, Presentation, and Outcome in Childhood Idiopathic and Hereditary Pulmonary Arterial Hypertension

Zhuoyuan Xu, Hongsheng Zhang, Chen Zhang, Qiangqiang Li, Hong GuBeijing Anzhen Hospital and Capital Medical University, No. 2.China Journal of Clinical MedicineJ Clin Med 2022; 11: DOI: 10.3390/jcm11247331 AbstractBackground: Paediatric-onset idiopathic/hereditary pulmonary arterial hypertension (IPAH/HPAH) is partially linked to genetic factors that may also affect treatment response and outcome. The relation between clinical characteristics and pathogenicity of

Association between Genotype, Presentation, and Outcome in Childhood Idiopathic and Hereditary Pulmonary Arterial Hypertension Read More »

Chest computed tomography findings of ground-glass nodules with enhancing central vessel/nodule in pediatric patients with BMPR2 mutations and plexogenic arteriopathy

Jason P. Weinman, David A. Mong, LaDonna J. Malone, D. Dunbar Ivy, Robin R. Deterding, Csaba GalambosChildren’s Hospital Colorado.United States Pediatric RadiologyPediatr Radiol 2022; 52: 2549-2556DOI: 10.1007/s00247-022-05413-8 AbstractBackground: Germline mutation in bone morphogenetic protein type II (BMPR2) is the most common cause of idiopathic/heritable pulmonary hypertension in pediatric patients. Despite the discovery of this gene there are

Chest computed tomography findings of ground-glass nodules with enhancing central vessel/nodule in pediatric patients with BMPR2 mutations and plexogenic arteriopathy Read More »

Clinical efficacy and safety of selexipag in children and young adults with idiopathic and heritable pulmonary arterial hypertension

Shinichi Takatsuki, Tomotaka Nakayama, Yurika Shimizu, Reiko Kawai, Hiroyuki MatsuuraToho University Omori Medical Center. Kochi Medical School Hospital.Japan Cardiology in the YoungCardiol Young 2023; 33: 196-200DOI: 10.1017/S1047951122000415 AbstractObjective: This study aimed to investigate the safety, tolerability, and efficacy of selexipag in children and young adults with idiopathic and heritable pulmonary arterial hypertension.Methods: This retrospective cohort study included clinical

Clinical efficacy and safety of selexipag in children and young adults with idiopathic and heritable pulmonary arterial hypertension Read More »

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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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,

BMPR2 Mutation and Metabolic Reprogramming in Pulmonary Arterial Hypertension Read More »

An emerging phenotype of pulmonary arterial hypertension patients carrying SOX17 variants

David Montani, Benoit Lechartier, Barbara Girerd, Mélanie Eyries, Maria-Rosa Ghigna, Laurent Savale, Xavier Jaïs, Andrei Seferian, Mitja Jevnikar, Athénais Boucly, Marianne Riou, Julie Traclet, Ari Chaouat, Maryline Levy, Jerome LePavec, Elie Fadel, Frédéric Perros, Florent Soubrier, Martine Remy-Jardin, Olivier Sitbon, Damien Bonnet, Marc HumbertHôpitaux de Paris, Université Paris-Saclay, Hôpital Marie Lannelongue, Hôpital Pitié-Salpêtrière, Nouvel Hôpital

An emerging phenotype of pulmonary arterial hypertension patients carrying SOX17 variants Read More »

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