Class 1. Idiopathic Pulmonary Hypertension

Gene and metabolite changes triggered by downregulation of JUNB and ZNF281 in idiopathic pulmonary arterial hypertension: potential mechanisms revealed by multi-omics study

Yanfang Zong, Wei Liu, Jiahe Tian, Cuilan Hou, Tingting Xiao, Sirui Song, Xunwei JiangShanghai Children’s Hospital School of Medicine and Shanghai Jiao Tong University.China Translational PediatricsTransl Pediatr 2025; 14: 2572-2585DOI: 10.21037/tp-2025-370 AbstractBackground: Pulmonary arterial hypertension (PAH) is a severe pulmonary vascular disease causing right heart failure. Idiopathic PAH (IPAH), a type of PAH with unknown causes, has […]

Gene and metabolite changes triggered by downregulation of JUNB and ZNF281 in idiopathic pulmonary arterial hypertension: potential mechanisms revealed by multi-omics study Read More »

Pre-existing pulmonary arterial hypertension decompensation associated with e-cigarettes in an adolescent

James Andrew Bishara, Patrick EversOregon Health and Science University and Doernbecher Children’s Hospital.United States Cardiology in the YoungCardiol Young 2025; DOI: 10.1017/S1047951125100577 AbstractAn adolescent girl with a long-standing history of pulmonary arterial hypertension experienced severe clinical decompensation after starting to use e-cigarettes. A combination of e-cigarette cessation, atrial septostomy, increasing treprostinil, and initiation of sotatercept led

Pre-existing pulmonary arterial hypertension decompensation associated with e-cigarettes in an adolescent Read More »

Pulmonary Artery Pulsatility Index: A Novel Marker for Risk Assessment and Prognosis in Pediatric Idiopathic Pulmonary Arterial Hypertension

Emine Gülsah Torun, Nevin Özdemiroglu, Denizhan Bagrul, Ibrahim EceTurkish Ministry of Health, Ankara Bilkent City Hospital and Gaziantep City Hospital.Turkey Pediatric CardiologyPediatr Cardiol 2025; DOI: 10.1007/s00246-025-03970-x AbstractThe pulmonary artery pulsatility index (PAPi), calculated as (systolic pulmonary artery pressure – diastolic pulmonary artery pressure)/mean right atrial pressure). This study aimed to assess the clinical utility of PAPi

Pulmonary Artery Pulsatility Index: A Novel Marker for Risk Assessment and Prognosis in Pediatric Idiopathic Pulmonary Arterial Hypertension Read More »

Macitentan in Children with Pulmonary Arterial Hypertension: A Multicenter Experience

A. Rodriguez Ogando, A. Sabaté-Rotés, A. Moya Bonora, L. Albert de la Torre, J. M. Espín López, C. Labrandero de Lera, Maria Jesus Del Cerro MarinGregorio Marañon General University Hospital. Hospital Materno Infantil Gregorio Marañon. Vall d’Hebron University Hospital. La Fe University Hospital. 12 de Octubre University Hospital. Virgen dee la Arrixaca University Hospital. La

Macitentan in Children with Pulmonary Arterial Hypertension: A Multicenter Experience Read More »

The genetic epidemiology and genotype-phenotype correlations among Chinese children with idiopathic and heritable pulmonary arterial hypertension

Yuan He, Qiangqiang Li, Chen Zhang, Bradley B. Keller, Yiping Shen, Hong GuBeijing Anzhen Hospital and Capital Medical University. Cincinnati Children’s Hospital Medical Center. Boston Children’s Hospital and Harvard Medical School.China and United States Respiratory ResearchRespir Res 2025; 26: DOI: 10.1186/s12931-025-03249-y AbstractObjective: This study aims to analyze the genetic characteristics, genotype-phenotype correlation and long-term prognosis of children

The genetic epidemiology and genotype-phenotype correlations among Chinese children with idiopathic and heritable pulmonary arterial hypertension Read More »

Whole exome sequencing unravels genetic architecture and its clinical implications in pediatric pulmonary arterial hypertension

Dai-Ji Jiang, Yi-Jia Yang, Yu-Zhen Wang, Xu Zhang, Wen-Xiu, Chan, Ting-Ting Yu, Hao Chen, Hao Zhang, Yi Yan, Li-Jun FuShanghai Children’s Medical Center, National Children’s Medical Center and Shanghai Jiao Tong University School of Medicine.China International Journal of CardiologyInt J Cardiol 2025; DOI: 10.1016/j.ijcard.2025.133515 AbstractBackground: Pulmonary arterial hypertension (PAH) is a severe disease with significant genetic predisposition.

Whole exome sequencing unravels genetic architecture and its clinical implications in pediatric pulmonary arterial hypertension Read More »

Reduced pulmonary arterial compliance predicts poor short-term outcome in children with pulmonary arterial hypertension independent of pulmonary vascular resistance

Eva Gouwy, Mark-Jan Ploegstra, Meindina G. Haarman, Marcus T. R. Roofthooft, Rolf M. F. Berger, Johannes M. DouwesBeatrix Children’s Hospital, University Medical Center Groningen and University of Groningen.Netherlands Frontiers in Cardiovascular MedicineFront Cardiovasc Med 2025; 12: DOI: 10.3389/fcvm.2025.1526435 AbstractBackground and study aim: Pulmonary arterial hypertension (PAH) is a progressive pulmonary vascular disease with pulmonary vascular remodelling leading

Reduced pulmonary arterial compliance predicts poor short-term outcome in children with pulmonary arterial hypertension independent of pulmonary vascular resistance Read More »

Pediatric lung transplantation in China, 2019-2023

Xiao‑Shan Li, Zi‑Tao Wang, Bo Wu, Shu‑Gao Ye, Feng Liu, Chun‑Xiao Hu, Yi Lu, Wen‑Jie Hua, Wei‑Wei Xu, Man Huang, Jing‑Yu ChenAffiliated Wuxi People’s Hospital of Nanjing Medical University, Wuxi People’s Hospital, Wuxi Medical Center and Nanjing Medical University. Chinese Lung Transplantation Quality Management and Control Center. Wuxi School of Medicine and Jiangnan University. Second

Pediatric lung transplantation in China, 2019-2023 Read More »

Single-Cell and Spatial Transcriptomics Identified Fatty Acid-Binding Proteins Controlling Endothelial Glycolytic and Arterial Programming in Pulmonary Hypertension

Bin Liu, Dan Yi, Shuai Li, Karina Ramirez, Xiaomei Xia, Yanhong Cao, Hanqiu Zhao, Ankit Tripathi, Shenfeng Qiu, Mrinalini Kala, Ruslan Rafikov, Haiwei Gu, Vinicio de jesus Perez, Sarah-Eve Lemay, Christopher C. Glembotski, Kenneth S. Knox, Sebastien Bonnet, Vladimir V. Kalinichenko, You-Yang Zhao, Michael B. Fallon, Olivier Boucherat, Zhiyu DaiCollege of Medicine-Phoenix and University of Arizona. Washington University School of Medicine in St. Louis. Guangzhou Medical University. Indiana University College of Medicine. Arizona State University. Stanford University. Laval University. Phoenix Children’s Hospital. Ann & Robert H. Lurie Children’s Hospital of Chicago and Northwestern University Feinberg School of Medicine.United

Single-Cell and Spatial Transcriptomics Identified Fatty Acid-Binding Proteins Controlling Endothelial Glycolytic and Arterial Programming in Pulmonary Hypertension Read More »

USP11 Promotes Endothelial Apoptosis-Resistance in Pulmonary Arterial Hypertension by Deubiquitinating HINT3

Bum-Yong Kang, Jiwoong Choi, Victor Tseng, Yutong Zhao, Jing Zhao, Robert S. Stearman, Wilbur A. Lam, Viranuj Sueblinvong, Benjamin T. Kopp, Michael J. Passineau, Changwon Park, John Lister, Raymond J. Benza, Andrew J. JangEmory University School of Medicine. Atlanta Veterans Healthcare System. University of Kansas School of Medicine and University of Kansas. Ansible Health. Ohio

USP11 Promotes Endothelial Apoptosis-Resistance in Pulmonary Arterial Hypertension by Deubiquitinating HINT3 Read More »

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