Class 1. Idiopathic Pulmonary Hypertension

Sotatercept administration in a young infant with severe pulmonary arterial hypertension: A case report

Arjith V. Rathakrishnan, Jenna Torgeson, Arij Beshish, Monica C. Bogenschutz, Vivek Balasubramaniam, Luke J. LamersUniversity of Wisconsin–Madison and UW Health Kids-Madison.United States Frontiers in PediatricsFront Pediatr 2026; DOI: 10.3389/fped.2026.1806079 AbstractIdiopathic pulmonary arterial hypertension (PAH) in infants is a rare, life-threatening condition characterized by elevated pulmonary artery pressure resulting from endothelial dysfunction, vasoconstriction, and vascular remodeling. The […]

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Pediatric pulmonary hypertension registry: Single-center experience from India

Wadile Santosh, Mishra Arpita, Banpurkar Ashishkumar Moreshwar, Raeen Shahena Parveen, Kulkarni SnehalSri Sathya Sai Sanjeevani Centre for Child Heart Care and Training in Pediatric Cardiac Skills.India Annals of Pediatric CardiologyAnn Pediatr Cardiol 2026; 19: 14-22DOI: 10.4103/apc.apc_181_24 AbstractBackground: Pulmonary hypertension (PH) is a rare and usually irreversible disease, eventually leading to right heart failure (RHF). There are multiple

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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

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

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