Medical Therapy. Efficacy or Lack of Efficacy

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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Sildenafil for bronchopulmonary dysplasia-associated pulmonary hypertension: A systematic search and narrative synthesis

Yoshinori Katayama, Katsuya Hirata, Yutaka Nishimura, Atsuko, Nakahari, Mami Takeoka, Masahiko Watanabe, Tetsuya Isayama, Japan Evidence Based NeonatologyTakatsuki General Hospital. Osaka Women’s and Children’s Hospital. Hiroshima City Hiroshima Citizens Hospital. Jichi Medical University Saitama Medical Center. Mie University Graduate School of Medicine. National Center for Child Health and Development.Japan Pediatrics InternationalPediatr Int 2026; 68: DOI: 10.1111/ped.70385

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Postnatal Steroids in Preterm Infants: A Narrative Review Series-Part 2: Cardiovascular Impacts

Phoenix Plessas-Azurduy, Anie Lapointe, Punnanee Wutthigate, Sarah Spénard, Andréanne Villeneuve, Audrey Hébert, Eilon Shany, Justin Richardson, Neta Geva, Wadi Mawad, Tiscar Cavallé-Garrido, Marc Beltempo, Wissam Shalish, Guilherme Sant’Anna, Gabriel AltMcGill University. Université de Montréal, CHU Sainte-Justine. Mahidol University. Montreal Children’s Hospital. CHU de Québec and Laval University. Saban Children’s Hospital and Soroka Medical Center. Ben

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Parenteral treprostinil in paediatric pulmonary arterial hypertension: a systematic review and meta-analysis

Julie Wacker, Raphael Joye, Maurice BeghettiGeneva University Hospitals and Faculty of Medicine and University of Geneva.Switzerland European Respiratory ReviewEur Respir Rev 2026; DOI: 10.1183/16000617.0033-2025 AbstractBackground: Paediatric pulmonary arterial hypertension (PAH) shares commonalities with adult disease but is essentially different regarding complexity and is usually more challenging to treat. Current treatment recommendations are based on expert opinion, small-scale

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Updates in the Treatment of Pediatric Pulmonary Arterial Hypertension

Danya Z. Jafri, James M. Beck, Samantha A. Kaplan, Allegra Keeler, Bryan Sanchez, Daniela Brady, William H. Frishman, Wilbert S. Aronow, Avi Levine, Erika Rosenzweig, Gregg M. LanierNew York Medical College School of Medicine. Westchester Medical Center.United States Cardiology in ReviewCardiol Rev 2026; DOI: 10.1097/CRD.0000000000001239 AbstractPediatric pulmonary arterial hypertension (PAH) is a relentless and potentially fatal

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Neonatal pulmonary vascular remodeling induced by increased blood flow is associated with an antiviral-like immune signature

Sixie Zheng, Hao Li, Siqi She, Yiting Xue, Debao Li, Jiapei Wang, Jing Wang, Yuqing Hu, Lincai YeShanghai Children’s Medical Center and Shanghai Jiao Tong University School of Medicine. Children’s Hospital of Fudan University and National Children’s Medical Center. Affiliated Women and Children’s Hospital of Ningbo University. China Frontiers in ImmunologyFront Immunol 2026; 17: DOI: 10.3389/fimmu.2026.1780303

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Pulmonary Hypertension Associated With Vitamin C Deficiency Is Rapidly Reversible

Tomás Woodgate, Jay Patel, Thomas Day, Brodie Knight, Adriani Spanaki, Alistair Calder, Shahin Moledina, Sadia QuyamGreat Ormond Street Hospital. Royal Hospital for Children. Evelina London Children’s Hospital,United Kingdom PediatricsPediatrics 2026; DOI: 10.1542/peds.2025-073645 AbstractWe report 4 children (aged 3-9 years) with severe pulmonary hypertension (PH) associated with vitamin C deficiency. All presented with either musculoskeletal symptoms or

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Protocol for an open-label, randomised, controlled trial to evaluate the efficacy and safety of sotatercept add-on therapy compared with pulmonary vasodilator-based standard of care for pulmonary vasodilator-resistant pulmonary arterial hypertension associated with unrepaired congenital shunts (atrial septal defect, ventricular septal defect or patent ductus arteriosus), including Eisenmenger syndrome: the SuMILE trial

Keimei Yoshida, Kazuya Hosokawa, Takahiro Hiraide, Satoshi Akagi, Kentaro Ejiri, Yu Taniguchi, Shiro Adachi, Takumi Inami, Naohiko Nakanishi, Masaharu Kataoka, Taijyu Satoh, Shunsuke Tatebe, Toshiro Shinke, Hideshi Tomita, Yusuke Akazawa, Takashi Higaki, Koshiro Tagawa, Ayako Ishikita, Soshun Asakawa, Kohtaro AbeKyushu University and Kyushu University Hospital. Keio University School of Medicine. Okayama University. Kobe University Hospital.

Protocol for an open-label, randomised, controlled trial to evaluate the efficacy and safety of sotatercept add-on therapy compared with pulmonary vasodilator-based standard of care for pulmonary vasodilator-resistant pulmonary arterial hypertension associated with unrepaired congenital shunts (atrial septal defect, ventricular septal defect or patent ductus arteriosus), including Eisenmenger syndrome: the SuMILE trial Read More »

Sotatercept in Patients With Eisenmenger Syndrome

Oktay Tutarel, Oliver Miera, Felix BergerDeutsches Herzzentrum der Charité, Freie Universität Berlin and Humboldt-Universität zu Berlin.Germany Pulmonary CirculationPulm Circ 2026; 16: DOI: 10.1002/pul2.70283 AbstractEisenmenger syndrome represents a complex and severe form of pulmonary arterial hypertension (PAH) associated with congenital heart disease. Sotatercept, a first-in-class activin-signaling inhibitor, offers a novel therapeutic approach in the treatment of PAH,

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Reversing the irreversible? A case of successful surgical repair in a late-presenting aortopulmonary window with severe pulmonary hypertension

Rido Mulawarman, Ericko Ongko Joyo, Muhamad Adrin Aefiansyah Putra, Aditya Agita Sembiring, Sisca Natalia Siagian, Oktavia LilyasarUniversitas Indonesia. Indonesia Egyptian Heart JournalEgypt Heart J 2026; 78: DOI: 10.1186/s43044-026-00726-7 AbstractBackground: Aortopulmonary window (APW) is a rare congenital heart defect, accounting for only 0.2-0.6% of all congenital cardiac anomalies, and is usually identified and surgically corrected within the first

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