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European central hypoventilation syndrome consortium description of congenital central hypoventilation syndrome neonatal onset.
Eur J Pediatr
January 2025
Service de Physiologie Pédiatrique-Centre du Sommeil-CRMR Hypoventilations Alvéolaires Rares, INSERM NeuroDiderot, Université Paris-Cité, AP-HP, Hôpital Robert Debré, Paris, France.
Unlabelled: It is known that in most cases of congenital central hypoventilation syndrome (CCHS), apnoeas and hypoventilation occur at birth. Nevertheless, a detailed description of initial symptoms, including pregnancy events and diagnostic tests performed, is warranted in infants with neonatal onset of CCHS, that is, in the first month of life. The European Central Hypoventilation Syndrome Consortium created an online patient registry from which 97 infants (44 females) with CCHS of neonatal onset and PHOX2B mutation from 10 countries were selected.
View Article and Find Full Text PDFBerlin Heart EXCOR sVAD implantation technique for neonates and infants with functionally univentricular ductal-dependent systemic circulation.
Multimed Man Cardiothorac Surg
January 2025
Congenital Heart Center, Division of Cardiovascular Surgery, Department of Surgery, University of Florida, Gainesville, FL, USA.
The Berlin Heart EXCOR is a pulsatile paracorporeal ventricular assist device (VAD) for neonates, infants, children and adults with congenital or acquired severe ventricular dysfunction. Berlin Heart EXCOR VADs are routinely used as either a bridge to a cardiac transplantation, or occasionally as a bridge to ventricular recovery. Our programmatic philosophy is to bridge neonates and infants with functionally univentricular ductal-dependent systemic circulation or functionally univentricular ductal-dependent pulmonary circulation who are at high risk for staged palliation because of important cardiac risk factors with a single-ventricle VAD (sVAD) as a bridge to a cardiac transplant.
View Article and Find Full Text PDFSystemic venous collaterals between Glenn and Fontan: prevalence, therapy, and impact on outcomes.
Cardiol Young
January 2025
Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, Technische Universität München, Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universität München, Europäisches Kinderherzzentrum München, Munich, Germany.
Objective: This study aimed to evaluate veno-venous collaterals between bidirectional cavopulmonary shunt and total cavopulmonary connection.
Methods: Patients who underwent staged total cavopulmonary connection between 1995 and 2022 were reviewed. Veno-venous collaterals between bidirectional cavopulmonary shunt and total cavopulmonary connection were depicted using angiograms.
Percutaneous permeabilisation with electrosurgery of intrahepatic inferior vena cava agenesis in a 12-year-old boy.
Cardiol Young
January 2025
Pediatric Interventional Cardiology Unit, Autònoma de Barcelona University, Vall d'Hebron Hospital, Barcelona, Spain.
We describe a 12-year-old boy with agenesis of the intrahepatic segment of the inferior vena cava (a rare congenital anomaly) in whom an electrified coronary guidewire with electrocautery was used percutaneously to perforate and open the atretic segment, followed by stent implantation. This technique may be a safe and feasible option for paediatric patients, offering a therapeutic alternative for complex cardiac anatomies.
View Article and Find Full Text PDFIncreased cardiac macrophages in -deficient hearts: revealing a potential role for macrophage in responding to embryonic myocardial abnormalities.
Front Genet
January 2025
Pediatric Translational Medicine Institute and Pediatric Congenital Heart Disease Institute, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, China.
Macrophages are known to support cardiac development and homeostasis, contributing to tissue remodeling and repair in the adult heart. However, it remains unclear whether embryonic macrophages also respond to abnormalities in the developing heart. Previously, we reported that the structural protein Sorbs2 promotes the development of the second heart field, with its deficiency resulting in atrial septal defects (ASD).
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