Sudden cardiac death (SCD) is a rare and devastating event in children and remains a leading cause of death in young athletes. Channelopathies and cardiomyopathies, in particular long QT syndrome (LQTS), catecholaminergic polymorphic ventricular tachycardia (CPVT), hypertrophic cardiomyopathy (HCM), and arrhythmogenic cardiomyopathy (ACM) are associated with exercise-related SCD. Implantable cardioverter-defibrillators (ICDs) are often placed for secondary prevention for athletes with cardiomyopathy or channelopathy. There remains concern regarding the safety of return to participation with an ICD in place. Guidelines have historically recommended that patients with inherited heart rhythm disorders be restricted from competitive sports participation. Increasing evidence suggests a lower risk of exercise-related cardiac events in young athletes with inherited heart rhythm disorders. In this review, we highlight current knowledge, evolving guidelines, and present a multidisciplinary approach involving shared decision-making and appropriate planning for safe sports participation of children with inherited heart rhythm disorders.
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http://dx.doi.org/10.3389/fped.2023.1151286 | DOI Listing |
Eur Heart J Open
January 2025
Institute of Health Informatics Research, University College London, 222 Euston Road, London NW1 2DA, UK.
Aims: Causes of death remain largely unexplored in the atrial fibrillation (AF) population. We aimed to (i) thoroughly assess causes of death in patients with AF, especially those associated with sudden cardiac death (SCD) and (ii) evaluate the potential association between AF and SCD.
Methods And Results: Linked primary and secondary care United Kingdom Clinical Practice Research Datalink dataset comprising 6 529 382 individuals aged ≥18.
Eur J Haematol
January 2025
Division of Hematology and Medical Oncology, Oregon Health & Science University, Portland, Oregon, USA.
Hematologic complications are common in pregnancy and can significantly impact both maternal and fetal health. Recognizing and treating these complications can be challenging due to the limited evidence available to guide clinical consultants. Iron deficiency anemia is the most prevalent hematologic issue in pregnancy and often occurs due to increased maternal blood volume and the nutritional demands of the growing fetus.
View Article and Find Full Text PDFNat Rev Dis Primers
January 2025
European Reference Network for Rare Multisystemic Vascular Disease (VASCERN), HHT Rare Disease Working Group, Paris, France.
Hereditary haemorrhagic telangiectasia (HHT) is a vascular dysplasia inherited as an autosomal dominant trait and caused by loss-of-function pathogenic variants in genes encoding proteins of the BMP signalling pathway. Up to 90% of disease-causal variants are observed in ENG and ACVRL1, with SMAD4 and GDF2 less frequently responsible for HHT. In adults, the most frequent HHT manifestations relate to iron deficiency and anaemia owing to recurrent epistaxis (nosebleeds) or bleeding from gastrointestinal telangiectases.
View Article and Find Full Text PDFHeart Rhythm
January 2025
Department of Molecular Biosciences, University of California, Davis, CA, USA; Department of Basic Sciences, California Northstate University, Elk Grove, CA. Electronic address:
Background: Friedreich's ataxia (FA) is a rare inherited neuromuscular disorder, where most patients die from lethal cardiomyopathy and arrhythmias. Mechanisms leading to arrhythmic events in FA patients are poorly understood.
Objective: This study aims to examine cardiac electrical signal propagation in mouse model of FA with severe cardiomyopathy and evaluate effects of omaveloxolone (OMAV), the first FDA-approved therapy.
Heart Rhythm
January 2025
Department of Coronary Artery Disease and Cardiac Rehabilitation, National Institute of Cardiology, Warsaw, Poland.
Background: Sudden cardiac arrest (SCA) risk stratification in patients with mitral valve prolapse (MVP) may be complicated by other potential causes of arrhythmia.
Objectives: We aimed to characterize SCA survivors with isolated (iMVP) and non-isolated MVP (non-iMVP) and to assess their long-term follow-up.
Methods: This ambispective study included 75 patients with MVP who experienced SCA and were treated in our center between 2009-2024.
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