Class 1. Idiopathic Pulmonary Hypertension

Hemodynamic Effects of a High-Frequency Oscillatory Ventilation Open-Lung Strategy in Critically Ill Children With Acquired or Congenital Cardiac Disease

Pauline de Jager, Martha A. Q. Curley, Ira M. Cheifetz, Martin C. J. KneyberBeatrix Children’s Hospital and University Medical Center Groningen. Children’s Hospital of Philadelphia and University of Pennsylvania. Rainbow Babies and Children’s Hospital and Case Western Reserve University School of Medicine. Netherlands and United States Pediatric Critical Care MedicinePediatr Crit Care Med 2023; DOI: 10.1097/PCC.0000000000003211 […]

Hemodynamic Effects of a High-Frequency Oscillatory Ventilation Open-Lung Strategy in Critically Ill Children With Acquired or Congenital Cardiac Disease Read More »

Idiopathic Pulmonary Arterial Hypertension in Paediatrics Represents Still a Serious Challenge: A Case Series Study

Pier Paolo Bassareo, Paola Argiento, Colin Joseph McMahon, Esme Dunne, Kevin Patrick Walsh, Maria Giovanna Russo, Michele D’AltoMater Misercordiae University Hospital. Children’s Health Ireland at Crumlin. University College Dublin. Università della Campania “Luigi Vanvitelli” and Ospedale Monaldi. Ireland and Italy ChildrenChildren 2023; 10: DOI: 10.3390/children10030518 AbstractIntroduction: Paediatric pulmonary hypertension (PH) represents a heterogeneous illness that is responsible

Idiopathic Pulmonary Arterial Hypertension in Paediatrics Represents Still a Serious Challenge: A Case Series Study Read More »

Metabolomic Profiles Differentiate Scleroderma-PAH From Idiopathic PAH and Correspond With Worsened Functional Capacity

Mona Alotaibi, Junzhe Shao, Michael W. Pauciulo, William C. Nichols, Anna R. Hemnes, Atul Malhotra, Nick H. Kim, Jason X-J Yuan, Timothy Fernandes, Kim M. Kerr, Laith Alshawabkeh, Ankit A. Desai, Andreea M. Bujor, Robert Lafyatis, Jeramie D. Waltrous, Tao Long, Susan Cheng, Stephen Y. Chan, Mihit Jain University of California San Diego. Peking University.

Metabolomic Profiles Differentiate Scleroderma-PAH From Idiopathic PAH and Correspond With Worsened Functional Capacity Read More »

Differential biventricular adaption to pulmonary vascular disease in patients with idiopathic/heritable and congenital heart disease: a prospective cardiac magnetic resonance and invasive study

Zhuoyuan Xu, Ruiyu Dou, Zhen Zhou, Hongsheng Zhang, Chen Zhang, Qiangqiang Li, Lei Xu, Hong GuBeijing Anzhen Hospital and Capital Medical University. China European Heart Journal Cardiovascular ImagingEur Heart J Cardiovasc Imag 2023; DOI: 10.1093/ehjci/jead106 AbstractAims: Despite shared pathophysiological mechanisms, patients with idiopathic/heritable pulmonary arterial hypertension (IPAH/HPAH) have a poorer prognosis than those with PAH after congenital

Differential biventricular adaption to pulmonary vascular disease in patients with idiopathic/heritable and congenital heart disease: a prospective cardiac magnetic resonance and invasive study Read More »

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 »

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