Class 1. Idiopathic Pulmonary Hypertension

Use of sotatercept in an adolescent patient with idiopathic pulmonary arterial hypertension

Kohei Kurosaki, Yohei Akazawa, Tomoyasu Yuki, Kohta Takei, Kiyohiro TakigikuNagano Children’s Hospital. Japan Journal of Cardiology CasesJ Cardiol Cases 2026; 34: 173-176DOI: 10.1016/j.jccase.2026.06.010 AbstractAn adolescent boy with idiopathic pulmonary arterial hypertension (iPAH) presented with persistent pulmonary hypertension with right ventricular dysfunction despite upfront oral triple combination therapy. Additional sotatercept treatment led to a striking improvement

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[Etiological and clinical classification of pulmonary hypertension in children]

Ruxuan He, Haiming Yang, Jinrong Liu, Xiaolei Tang, Hui Xu, Hui Liu, Yuelin Shen, Xiaoyan Zhang, Huimin Li, Shunying ZhaoBeijing Children’s Hospital, Capital Medical University and National Center for Children’s Health.China Chinese Journal of PediatricsZhonghua Er Ke Za Zhi 2026; 64: 1168-1172DOI: 10.3760/cma.j.cn112140-20260402-00264 AbstractObjective: To investigate the etiological and across clinical classification features in children

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Histology-guided 3D virtual staining of microCT-imaged lung tissue via deep learning

Cristina Almagro-Pérez, Niccolò Peruzzi, Csaba Galambos, Andrew H.Song, Hans Brunnström, KingaI. Gawlik, MarcoStampanoni, Karin Tran-Lundmark, Goran LovricPaul Scherrer Institut. Institute for Biomedical Engineering. Lund University and Skåne University Hospital Lund. University of Colorado Anschutz Medical Campus School of Medicine. University of Texas MD Anderson Cancer Center. Switzerland, Sweden and United States Journal of the Royal

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Non-invasive surrogate of main pulmonary artery stiffness is associated with right ventricular function in experimental pulmonary arterial hypertension

John D. Dauz, Bahram Mirani, J. Paul Santerre, Michelle P. Bendeck, Craig A. Simmons, Mark K. FriedbergHospital for Sick Children and University of Toronto. Ted Rogers Centre for Heart Research. Canada American Journal of Physiology Heart and Circulatory PhysiologyAm J Physiol Heart Circ Physiol 2026; DOI: 10.1152/ajpheart.00459.2026 AbstractMain pulmonary artery (MPA) stiffening increases right ventricular (RV)

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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 »

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