Class 1. Idiopathic Pulmonary Hypertension

Lifesaving Compassionate-Use of Sotatercept in a 12-Year-old With Idiopathic Pulmonary Arterial Hypertension

Weronika Pelczar‐Płachta, Rafał Surmacz, Waldemar BobkowskiPoznan University of Medical Sciences.Poland Pulmonary CirculationPulm Circ 2026; 16DOI: 10.1002/pul2.70293 AbstractPediatric idiopathic pulmonary arterial hypertension (IPAH) refractory to maximal medical therapy is associated with high morbidity and mortality, and therapeutic options remain limited. We describe a 12-year-old girl with end-stage IPAH who developed acute decompensated right heart failure despite triple […]

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Clinical Utility of Stroke Volume Index in Children with Idiopathic and Heritable Pulmonary Arterial Hypertension

Shinichi Takatsuki, Kinjal Parikh, Mio Sato, Yuta Kawamura, Yurika Shimizu, Reiko Kawai, Dunbar IvyToho University Omori Medical Center. Inova Children’s Hospital. University of Colorado School of Medicine.Japan and United States Pediatric CardiologyPediatr Cardiol 2026; DOI: 10.1007/s00246-026-04241-z AbstractTo determine the prognostic value of stroke volume index (SVi) in children with idiopathic and heritable pulmonary arterial hypertension (PAH).

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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

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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

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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

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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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