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Increased Endothelin-1 Is Associated With Morbidity in Single Ventricle Heart Disease in Children Undergoing Fontan Palliation

Benjamin S. Frank, Sierra Niemiec, Ludmila Khailova, Christopher A. Mancuso, Max B. Mitchell, Gareth J. Morgan, Mark Twite, Michael V. DiMaria, Carmen C. Sucharov, Jesse A. DavidsonUniversity of Colorado. University of Michigan.United States Journal of the American College of Cardiology AdvancesJACC Adv 2025; 4: DOI: 10.1016/j.jacadv.2025.101672 AbstractBackground: Endothelin-1 (ET1) is a potent vasoconstrictor and stimulator of pulmonary […]

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Images: Atypical resolution of sleep-related hypoventilation in congenital central hypoventilation syndrome

Michelle Yoo, Amit Shah, Haitham Shahrour, Hong Li, Ajay S. KasiEmory University and Children’s Healthcare of Atlanta.United States Journal of Clinical Sleep MedicineJ Clin Sleep Med 2025;DOI: 10.5664/jcsm.11644 AbstractPaired-like homeobox 2B (PHOX2B) gene variants cause congenital central hypoventilation syndrome (CCHS) characterized by abnormal ventilatory control necessitating lifelong assisted ventilation (AV). We report a 3-year-old girl who

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Familial Alveolar Capillary Dysplasia With Misalignment of Pulmonary Veins Caused by Paternal FOXF1 Upstream Enhancer Deletion: A Case Report

Hidenori Kawasaki, Kazuhiko Nakabayashi, Masahiko Ikeda, Tetsuo Onda, Seiichi Tomotaki, Masako Torishima, Akiko Saito, Hirofumi Ohashi, Sachiko Minamiguchi, Kenichiro Hata, Masahiro Hayakawa, Masahiko Kawai, Kazutoshi Cho, Shinji Kosugi, akahiro YamadaKyoto University School of Public Health, Kyoto University Hospital and Kyoto University. National Center for Child Health and Development. Hokkaido University Hospital. Nagoya University Hospital. Saitama

Familial Alveolar Capillary Dysplasia With Misalignment of Pulmonary Veins Caused by Paternal FOXF1 Upstream Enhancer Deletion: A Case Report Read More »

Trametinib as a targeted treatment in cardiac and lymphatic presentations of Noonan syndrome

Isabel De Brouchoven, Juan Lorand, Léon Bofferding, Arthur Sorlin, An Van Damme, Olivier DanhaiveSaint-Luc University Hospital,. Luxembourg Hospital Center. Laboratoire National de Santé. University of California San Francisco.Belgium, Luxembourg and United States Frontiers in PediatricsFront Pediatr 2025; 13: DOI: 10.3389/fped.2025.1475143 AbstractIntroduction: Rare pathogenic variants in the PTPN11, KRAS, SOS1 and RAF1 genes are the main molecular causes of Noonan syndrome (NS).

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Prevalence and Predictors of Pulmonary Hypertension in Children with Portal Hypertension: A Single Center Study

Nehal El Koofy, Sawsan Hassan Okasha, Hala Mounir Agha, Noha Ali, Ahmed Said Behairy, Hanan Mina Fouad, Rehab Hamdy ZawamCairo University, Helwan University.Egypt Pediatric Gastroenterology Hepatology and NutritionPediatr Gastroenterol Hepatol Nutr 2025; 28: 101-111DOI: 10.5223/pghn.2025.28.2.101 AbstractPurpose: This study aimed to estimate the prevalence and predictors of portopulmonary hypertension (POPH) in children with portal hypertension.Methods: We recruited children of

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Hemodynamic precision to guide surgical timing for neonates with congenital diaphragmatic hernia: a narrative review

John T. Wren, Neil Patel, Matthew T. Harting, Patrick J. McNamaraUniversity of Iowa Hospitals and Clinics. Royal Hospital for Children. McGovern Medical School at the University of Texas Health Science Center and Children’s Memorial Hermann Hospital. United States and United Kingdom Journal of PerinatologyJ Perinatol 2025; DOI: 10.1038/s41372-025-02265-6 AbstractDespite a near universal approach focused on physiologic

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Airway Anomalies Predict Risk of Pediatric Pulmonary Hypertension

Elana R. Kriegel, Aditya Srinivasen, Luke Mammen, Harini Venkataganesh, Jess T. Randall, Lara ReichertAlbany Medical College and Albany Medical Center.United States Pediatric PulmonologyPediatr Pulmonol 2025; 60: DOI: 10.1002/ppul.71028 AbstractIntroduction: Pediatric pulmonary hypertension (PH) significantly contributes to morbidity and mortality due to the progressive nature of the disease in some subtypes, which leads to severe right heart failure,

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Clinical characteristics and impact of exchange transfusion in infant pertussis with extreme leukocytosis

Yuanyuan Wu, Chuan GanSecond Affiliated Hospital of Chongqing Medical University, Children’s Hospital of Chongqing Medical University and Chongqing Medical University. Chongqing Key Laboratory of Child Rare Diseases in Infection and Immunity.China Italian Journal of PediatricsItal J Pediatr 2025; 51: DOI: 10.1186/s13052-025-01933-9 AbstractBackground: Extreme leukocytosis in pertussis is a rare condition, and without effective interventions to reduce white

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Right Atrial Dysfunction Is Prevalent in Pediatric Acute Respiratory Distress Syndrome and Reflects Pulmonary Hypertension and Right Ventricular Dysfunction

Nathan D. Markiewitz, Yan Wang, Robert A. Berg, Nadir Yehya, Celeste Dixon, Laura Mercer-Rosa, Adam S. HimebauchChildren’s Hospital of Philadelphia and Perelman School of Medicine at the University of Pennsylvania. United States Critical Care ExplorationsCrit Care Exp 2025; 7: DOI: 10.1097/CCE.0000000000001230 AbstractImportance: Right atrial (RA) dysfunction is associated with worse outcomes in some populations with pulmonary hypertension

Right Atrial Dysfunction Is Prevalent in Pediatric Acute Respiratory Distress Syndrome and Reflects Pulmonary Hypertension and Right Ventricular Dysfunction Read More »

Management of Obstructed Complete Left Partial Anomalous Pulmonary Venous Return in a Preterm Infant

Timothy R. Harris, Joiliana Lecointe, Thomas P. Doyle, Nhue L. Do, Dana R. Janssen, Garrett N. CoyanVanderbilt University Medical Center. University of Arizona. Meharry Medical College.United States Annals of Thoracic Surgery Short ReportsAnn Thorac Surg Short Rep 2025; 3: 106-108DOI: 10.1016/j.atssr.2024.09.006 AbstractA 25-week preterm neonate was born with complete left-sided partial anomalous pulmonary venous drainage with

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