Class 1. Idiopathic Pulmonary Hypertension

RASA3 is a candidate gene in sickle cell disease-associated pulmonary hypertension and pulmonary arterial hypertension

Clare C. Prohaska, Xu Zhang, Tae‐Hwi L. Schwantes‐An, Robert S. Stearman, Stanley Hooker, Rick A. Kittles, Micheala A. Aldred, Katie A. Lutz, Michael W. Pauciulo, William C. Nichols, Ankit A. Desai, Victor R. Gordeuk, Roberto F. MachadoIndiana University. University of Illinois at Chicago. City of Hope. Morehouse School of Medicine. Cincinnati Children’s Hospital Medical Center […]

RASA3 is a candidate gene in sickle cell disease-associated pulmonary hypertension and pulmonary arterial hypertension Read More »

Case Report: Selexipag in pediatric pulmonary hypertension: Initiation, transition, and titration

Jenna M. Faircloth, Neelam D. Bhatt, Corey A. Chartan, Ryan D. Coleman, Natalie Villafranco, Fadel E. Ruiz, Raysa Morales-Demori, Elise Whalen, Erin Ely, Rozmeen Fombin, Nidhy P. VargheseTexas Children’s Hospital and Baylor College of Medicine.United States Frontiers in PediatricsFront Pediatr 2023; 11: DOI: 10.3389/fped.2023.1050508 AbstractSelexipag, a selective prostacyclin receptor agonist, is approved for treating pulmonary arterial

Case Report: Selexipag in pediatric pulmonary hypertension: Initiation, transition, and titration Read More »

Safety of chronic high-dose calcium channel blockers exposure in children with pulmonary arterial hypertension

Yan Wu, Fu-Hua Peng, Xin Gao, Xin-Xin Yan, FengWen Zhang, Jiang-Shan Tan, Song Hu, Lu HuaFuwai Hospital and Chinese Academy of Medical Sciences and Peking Union Medical College.China Frontiers in Cardiovascular MedicineFront Cardiovasc Med 2022; DOI: 10.3389/fcvm.2022.918735 AbstractBackground: Chronic calcium channel blockers (CCBs) are indicated in children with idiopathic/heritable pulmonary arterial hypertension (IPAH/HPAH) and positive response to

Safety of chronic high-dose calcium channel blockers exposure in children with pulmonary arterial hypertension Read More »

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 »

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 »

Proteomics discovery of pulmonary hypertension biomarkers: Insulin-like growth factor binding proteins are associated with disease severity

Melanie K. Nies, Jun Yang, Megan Griffiths, Rachel Damico, Jie Zhu, Dhananjay Vaydia, Zongming Fu, Stephanie Brandal, Eric D. Austin, Dunbar D. Ivy, Paul M. Hassoun, Jennifer E. Van Eyk, Allen D. EverettJohns Hopkins University. Columbia University New York. Vanderbilt University. Children’s Hospital Colorado. Cedars-Sinai Medical Center.United States Pulmonary CirculationPulm Circ 2022; 12:DOI: 10.1002/pul2.12039 AbstractPulmonary arterial

Proteomics discovery of pulmonary hypertension biomarkers: Insulin-like growth factor binding proteins are associated with disease severity Read More »

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 »

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