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Serial changes of systemic ventricular function and atrioventricular valve function in patients with failing Fontan.
Eur J Cardiothorac Surg
January 2025
Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, University Hospital of Technische Universität München, Munich Germany, Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universität München, Munich, Germany, Europäisches Kinderherzzentrum München, Munich, Germany.
Objectives: This study investigated the longitudinal changes in ventricular function and atrioventricular valve function to clarify the timing and the mechanisms of failing Fontan.
Methods: Patients who underwent total cavopulmonary connection between 1994 and 2023 were reviewed, and longitudinal echocardiographic examinations of ventricular function and atrioventricular valve regurgitation were analyzed.
Results: A total of 650 patients were included.
Vitamin D deficiency and secondary hyperparathyroidism in adult Fontan patients.
Int J Cardiol Congenit Heart Dis
September 2024
Medizinische Hochschule Hannover, Department of Cardiology and Angiology, Hannover, Germany.
Background: The prevalence of vitamin D deficiency and secondary hyperparathyroidism (sHPT) in adult Fontan patients remains unstudied, and the role of vitamin D and parathyroid hormone (PTH) levels in assessing heart and circulatory failure in these patients is unclear.
Methods: We compared vitamin D deficiency and sHPT prevalence in adult Fontan patients (n = 35; mean age 33 ± 7.5 years) to adults with mild congenital heart disease (ACHD, n = 14).
An evaluation of pozelimab for the treatment of CHAPLE disease.
Expert Opin Biol Ther
January 2025
Division of Allergy and Immunology, Department of Pediatrics, School of Medicine, Marmara University, Istanbul, Turkey.
Introduction: CHAPLE disease is a severe, ultra-rare disorder caused by CD55 gene mutations, leading to uncontrolled complement hyperactivation, protein-losing enteropathy, and systemic thrombosis. Recent advances in targeted therapies, particularly the C5 inhibitor pozelimab (Veopoz), offer new treatment options by addressing complement dysregulation, marking a shift from symptomatic to precision therapy.
Areas Covered: This review explores the pathophysiology, clinical manifestations, and current treatments for CHAPLE disease.
Fontan-associated liver disease: the importance of multidisciplinary teamwork in its management.
Front Med (Lausanne)
November 2024
Department of Pediatrics, Wakayama Medical University, Wakayama, Japan.
The Fontan operation, which directly connects the superior and inferior vena cava to the pulmonary artery, is a palliative surgery for children with a functional or anatomic single ventricle. This procedure leads to hemodynamic changes (Fontan circulation) in patients, who tend to develop congestive hepatic fibrosis characterized by sinusoidal fibrosis and dilatation beginning approximately 10 years after the procedure. In addition, in the context of severe fibrosis and cirrhosis, hepato-gastrointestinal complications including hepatocellular carcinoma, focal nodular hyperplasia, and portal hypertension can arise.
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