Class 1. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease

Distinctive hemodynamic phenotype in Fontan-type circulation patients with distal esophageal varices

Luis Téllez, Luis, María Toledano, María Álvarez, Elvira Garrido-Lestache, Elena Garrido, Antonio Guerrero, Jesús Donate, Lorenzo Canova, María Torres, Cristian Perna, María Jesús del Cerro, Agustín AlbillosHospital Universitario Ramón y Cajal, IRYCIS, CIBERehd, Universidad de Alcalá. Spain HepatologyHepatology 2025;  DOI: 10.1097/HEP.0000000000001472 AbstractBackground aims: Esophageal varices (EV) are common in adults with Fontan-type circulation and may arise via […]

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c-Myc promotes metabolic reprogramming in pulmonary hypertension via the stimulation of glutaminolysis and the reductive tricarboxylic acid cycle

Manivannan Yegambaram, Xutong Sun, Qing Lu, Alejandro Garcia Flores, Marina Zemskova, Jamie Soto, Adam Rauckhorst, Emin Maltepe, Ting Wang, Jeffrey R. Fineman, Stephen M. BlackHerbert Wertheim College of Medicine and Florida International University. University of California San Francisco. United States Redox BiologyRedox Biol 2025; DOI: 10.1016/j.redox.2025.103765 AbstractEndothelial cell (EC) dysfunction is key in initiating and progressing

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Prostacyclin Therapy as Adjunctive Treatment for Pulmonary Hypertension in Pediatric Heart Transplantation: A Case Report

Anusha Konduri, Aly J. Sonnen, Heang M. Lim, Kurt R. Schumacher, David M. Peng, Vikram Sood, Mary K. Olive, Amanda D. McCormickUniversity of Michigan.United States Pediatric TransplantationPediatr Transplant 2025; 29: DOI: 10.1111/petr.70129 AbstractBackground: Elevated pulmonary vascular resistance is a significant risk factor for right-ventricle failure and poor outcomes following pediatric heart transplantation. Pulmonary vasodilator therapy can help

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Macitentan in Children with Pulmonary Arterial Hypertension: A Multicenter Experience

A. Rodriguez Ogando, A. Sabaté-Rotés, A. Moya Bonora, L. Albert de la Torre, J. M. Espín López, C. Labrandero de Lera, Maria Jesus Del Cerro MarinGregorio Marañon General University Hospital. Hospital Materno Infantil Gregorio Marañon. Vall d’Hebron University Hospital. La Fe University Hospital. 12 de Octubre University Hospital. Virgen dee la Arrixaca University Hospital. La

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The Use of Vasodilator Therapy in Fontan Patients: A Single-Centre Experience

Alessia Faccini, Martina Avesani, Roberta Biffanti, Elettra Pomiato, Domenico Sirico, Alice Pozza, Alessia Cerutti, Elena Reffo, Biagio Castaldi, Giovanni Di SalvoUniversity Hospital Padua.Italy ChildrenChildren 2025; 12:DOI: 10.3390/children12060751 AbstractBackground: The aim of this study was to describe our centre experience in the use of pulmonary vasodilator therapy in Fontan patients.Methods: We retrospectively enrolled patients that underwent Fontan operation between

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Usefulness of exercise stress echocardiography in a patient with unilateral pulmonary branch stenosis

Ryohei Yokoyama, Yoshihiko Kodama, Kazunari Takamura, Masako Takahashi, Miyo Tanaka, Nozomi Watanabe, Hiroshi MoritakeUniversity of Miyazaki.Japan Journal of Cardiology CasesJ Cardiol Cases 2025; 31: 155-157DOI: 10.1016/j.jccase.2025.02.003 AbstractExercise stress echocardiography (ESE) is a feasible and valuable tool for evaluating subclinical pulmonary hypertension (PH). However, its utility in patients with unilateral pulmonary branch stenosis remains unclear. We present

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Whole exome sequencing unravels genetic architecture and its clinical implications in pediatric pulmonary arterial hypertension

Dai-Ji Jiang, Yi-Jia Yang, Yu-Zhen Wang, Xu Zhang, Wen-Xiu, Chan, Ting-Ting Yu, Hao Chen, Hao Zhang, Yi Yan, Li-Jun FuShanghai Children’s Medical Center, National Children’s Medical Center and Shanghai Jiao Tong University School of Medicine.China International Journal of CardiologyInt J Cardiol 2025; DOI: 10.1016/j.ijcard.2025.133515 AbstractBackground: Pulmonary arterial hypertension (PAH) is a severe disease with significant genetic predisposition.

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Down syndrome and congenital heart disease: perioperative planning and management

Dennis R. Delany, Stephanie S. Gaydos, Deborah A. Romeo, Heather T. Henderson, Kristi L. Fogg, Angela S. McKeta, Minoo N. Kavarana, John M. CostelloMedical University of South Carolina. United States Journal of Congenital CardiologyJ Congenit Cardiol 2021; 5: DOI: 10.1186/s40949-021-00061-3 AbstractApproximately 50% of newborns with Down syndrome have congenital heart disease. Non-cardiac comorbidities may also be

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Common shunt lesions with pulmonary hypertension-who will benefit from surgery?

Shine Kumar, Raman Krishna KumarAmrita Hospital and Amrita Vishwa Vidyapeetham University.India Indian Journal of Thoracic and Cardiovascular SurgeryInd J Thorac Cardiovasc Surg 2025; 41: 718-729DOI: 10.1007/s12055-024-01786-7 AbstractLeft to right shunts comprise a specific group of congenital heart disease, when identified and treated on time result in excellent outcomes. However, a proportion of these defects do not

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Focal Dermal Hypoplasia with Unusual Cardiac Anomalies Presentation: A Report of Two Cases and Literature Review

Nagehan Bilgeça, Mahmut Gökdemirb, Özgür Balasar, Fayize Maden Bedela, Hüseyin ÇaksenNecmettin Erbakan University. Başkent University. Konya City Hospital.Turkey Molecular SyndromologyMol Syndromol 2025; DOI: 10.1159/000545533 AbstractIntroduction: Focal dermal hypoplasia (FDH), also known as Goltz syndrome, is an exceedingly rare multisystemic disease with X-linked dominant inheritance involving meso-ectodermal tissues. FDH is characterized by specific cutaneous lesions, ectodermal findings, craniofacial

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