Class 1. Idiopathic Pulmonary Hypertension

High-altitude pulmonary edema in children with underlying cardiopulmonary disorders and pulmonary hypertension living at altitude

Bibhuti B. Das, Robert R. Wolfe, Kak-Chen Chan, Gary L. Larsen, John T. Reeves, Dunbar IvyChildren’s Hospital, Denver.United States Archives of Pediatric and Adolescent MedicineArch Pediatr Adolesc Med 2004; 158: 1170-1176DOI: 10.1001/archpedi.158.12.1170 AbstractBackground: Pulmonary hypertension has not been described as a predisposing risk factor for high-altitude pulmonary edema (HAPE) in children. Previous studies have shown an association […]

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A prospective, multicenter, open-label, single-arm Phase 2 study to investigate the pharmacokinetics, safety, tolerability, and exploratory efficacy of selexipag in children with pulmonary arterial hypertension

Maurice Beghetti, Lene Nygaard Axelsen, Julian I. Borissoff, Mahdi Farhan, Simon Grill, Sining Leng, Alberto Russu, Catherine Lesage, Tatiana Remeňová, Shu-Fang Hsu Schmitz, Shahin MoledinaUniversity Hospitals of Geneva. Johnson & Johnson. Great Ormond Street Hospital. Switzerland and United Kingdom ChestChest 2025; DOI: 10.1016/j.chest.2025.12.013 AbstractBackground: Selexipag is an oral selective prostacyclin receptor agonist approved for treating pulmonary arterial

A prospective, multicenter, open-label, single-arm Phase 2 study to investigate the pharmacokinetics, safety, tolerability, and exploratory efficacy of selexipag in children with pulmonary arterial hypertension Read More »

Meta-Analysis of Differential Gene Expression in Idiopathic Pulmonary Arterial Hypertension

Andy P. Huang, Sarah Voskamp, Ameneh A. Ebadi, Jennifer L. Liedel, Jennifer S. Nelson, Joseph KuruvillaUniversity of Central Florida. Nemours Children’s Hospital. United States Cardiovascular PathologyCardiovasc Pathol 2025;  DOI: 10.1016/j.carpath.2025.107805 AbstractBackground: Idiopathic pulmonary arterial hypertension (IPAH) is a subtype of pulmonary arterial hypertension and impacts both children and adults. IPAH has overall poor survival, highlighting the importance

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Cause of death in children with heart disease: a cohort study

Neil Derridj, Daphné Madec, Olivier Raisky, Sophie Malekzadeh-Milani, Diala Khraiche, Antoine Legendre, Lucile Houyel, Zahra Belhadjer, Franck Iserin, Mathilde Méot, Marilyne Levy, Bertrand Stos, Daniela Laux, Victor Waldmann, Vanessa Lopez, Ayman Haydar, Segolene Bernheim, Régis Gaudin, Fanny Bajolle, Damien Bonnet, Members of M3C Necker Collaborative GroupHôpital Universitaire Necker-Enfants Malades. Paris Cité University. France Archives of

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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

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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 »

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