Medical Therapy. Efficacy or Lack of Efficacy

Misdiagnosed as Idiopathic PAH: Methylmalonic Acidemia as a Reversible Cause of Pediatric Pulmonary Hypertension

Yan Wu, Yuan-Rui Deng, Xiao-Pei Cui, Sheng-Song Zhu, Jiang Shan Tan, Song Hu, Lu HuaFuwai Hospital, Fuwai Shenzhen Hospital and Peking Union Medical College. Qilu Hospital of Shandong University. China Journal of Heart and Lung TransplantationJ Heart Lung Transplant 2026; DOI: 10.1016/j.healun.2026.07.021 AbstractBackground: Methylmalonic acidemia (MMA)-induced pulmonary hypertension (PH) is a rare but treatable cause of pediatric […]

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Meta-analysis of prostacyclin therapy for persistent pulmonary hypertension with congenital diaphragmatic hernia

Shouliang Jiang, Xue Han, Yang He, Qian Gao, Juan Liang, Tingting Zhu, Li Zhang, Jun TangWest China Second University Hospital and Sichuan University. China Journal of Pediatric SurgeryJ Pediatr Surg 2026; DOI: 10.1016/j.jpedsurg.2026.163301 AbstractObjective: To evaluate the efficacy and safety of prostacyclin in the treatment of persistent pulmonary hypertension in congenital diaphragmatic hernia.Methods: A systematic literature search was

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Right Ventricular Function Improves With Pulmonary Vasodilator Treatment in Pediatric Patients With Pulmonary Hypertension and Multivessel Pulmonary Vein Stenosis in a Single Center Cohort

Catherine M. Avitabile, Kimberly Butler, Antara Mondal, David B. Frank, Yan Wang, Devon Ash, Laura Mercer‐Rosa, Michael L. O’Byrne, Ryan CallahanUniversity of Pennsylvania, Perelman School of Medicine and Children’s Hospital of Philadelphia. United States Pulmonary CirculationPulm Circ 2026; 16: DOI: 10.1002/pul2.70346 AbstractPulmonary hypertension (PH) and right ventricular (RV) dysfunction increase mortality in multivessel pediatric pulmonary vein

Right Ventricular Function Improves With Pulmonary Vasodilator Treatment in Pediatric Patients With Pulmonary Hypertension and Multivessel Pulmonary Vein Stenosis in a Single Center Cohort Read More »

Perinatal survival and long-term neurodevelopmental outcomes of congenital chylothorax

Hideyuki Hawaka, Tomoyuki Shimokaze, Jun Shibasaki, Hiromi Nagase, Tomoko Saito, Hiroshi Ishikawa, Katsuaki ToyoshimaKanagawa Children’s Medical Center. Hiroshima City Hiroshima Citizens Hospital. Japan Pediatric ResearchPediatr Res 2026; DOI: 10.1038/s41390-026-05271-2 AbstractBackground: Congenital chylothorax carries a poor prognosis, particularly with delivery at <32 weeks. Survival outcomes under consistent management prioritizing pregnancy prolongation despite worsening hydrops, as well as long-term

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Repair of Sinus Venosus Atrial Septal Defect in 2 Patients With Severe Pulmonary Arterial Hypertension

Ryo Sakuma, Gareth Morgan, Elizabeth Yeung, Benjamin S. Frank, Charles Simpkin, Dunbar IvyUniversity of Colorado School of Medicine.United States Pulmonary CirculationPulm Circ 2026; 16: DOI: 10.1002/pul2.70285 AbstractPretreatment with targeted pulmonary arterial hypertension (PAH) therapy in adults with unrepaired atrial septal defects (ASDs) has been increasingly reported as a means to improve pulmonary hemodynamics and expand candidacy

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Neonatal Survival After Serial Amnioinfusions for Anhydramnios Due to Fetal Kidney Failure: The RAFT Clinical Trial

Jena L. Miller, Ahmet A. Baschat, Anthony Johnson, Yair J. Blumenfeld, Mauro H. Schenone, Juliana S. Gebb, Julie S. Moldenhauer, Michael V. Zaretsky, Ramen H. Chmait, Juan M. Gonzalez, Russel S. Miller, Eyal Krispin, Alireza Shamshirsaz, Katherine H. Bligard, Amanda M. Kalan, Magdalena Sanz Cortes, Anita J. Moon-Grady, Ellen Bendel-Stenzel, Shina Menon, Cynthia Wong, Paul

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Off-Label Sotatercept Use in Pediatric Pulmonary Arterial Hypertension: A Single-Center Retrospective Hemodynamic Study

Dhruv Rao, Shylah Haldeman, Jeanne Carroll, Rohit RaoUniversity of Arizona, Tucson. Rady Children’s Hospital-San Diego and University of California San DiegoUnited States Pediatric CardiologyPediatr Cardiol 2026; DOI: 10.1007/s00246-026-04349-2 AbstractSotatercept, a first-in-class activin signaling inhibitor, has demonstrated disease-modifying efficacy in adults with pulmonary arterial hypertension (PAH). Pediatric data remain limited to ongoing clinical trials. We conducted an

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L-Citrulline: Novel Pharmacotherapy to Improve Outcomes in Infants and Children at Risk of Systemic or Pulmonary Vascular Disease

Candice D. Fike, Frederick E. Barr, Judy L. AschnerUniversity of Utah Health. Diversified Search Group. Hackensack Meridian School of Medicine.United States PharmaceuticalsPharmaceuticals 2026; 19: DOI: 10.3390/ph19060896 AbstractInfants and children suffering from a variety of heart, lung, and blood disorders are known to be at risk of developing systemic or pulmonary vascular disease. Despite progress made in

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L-Citrulline in Maternal-Fetal and Neonatal Health: Metabolic Mechanisms and Emerging Therapeutic Applications

Ana Collins-Smith, Sangeeta Jain, Sunil JainUniversity of Texas Medical Branch at Galveston.United States NutrientsNutrients 2026; 18: DOI: 10.3390/nu18121923 AbstractL-citrulline is a non-protein amino acid with critical roles in perinatal and neonatal physiology through the intestinal-renal arginine-citrulline axis and nitric oxide (NO) production. During pregnancy, L-citrulline supports placental angiogenesis, vascular adaptation, and fetal growth through augmentation of

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Inhaled Nitric Oxide via High-Flow Nasal Cannula in Postrepair Congenital Heart Disease Patients With Pulmonary Arterial Hypertension Following Extubation: A Cohort Study With Propensity Score Matching

Xiaofeng Wang, Qinnan Chen, Shilin Wang, Zhiyuan Zhu, Hong Gu, Xu WangNational Center for Cardiovascular Disease and Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College. Beijing Anzhen Hospital and Capital Medical University.China World Journal of Pediatric and Congenital Heart SurgeryWorld J Pediatr Congenit Heart Surg 2026; DOI: 10.1177/21501351261450515 AbstractObjective: For pediatric patients with

Inhaled Nitric Oxide via High-Flow Nasal Cannula in Postrepair Congenital Heart Disease Patients With Pulmonary Arterial Hypertension Following Extubation: A Cohort Study With Propensity Score Matching Read More »

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