Investigation on Sudden Unexpected Death in the Young (SUDY) in Europe: results of the European Heart Rhythm Association Survey
Por:
Behr ER, Scrocco C, Wilde AAM, Marijon E, Crotti L, Iliodromitis KE, Remme CA, Kosiuk J, Rudaka I, Sarquella-Brugada G, Frampton K, Schulze-Bahr E, Jubele K, de Asmundis C, Hofman N, Tfelt-Hansen J, Boveda S and Conte G
Publicada:
2 feb 2022
Resumen:
The aims of this centre-based survey, promoted and disseminated by the European Heart Rhythm Association (EHRA) was to investigate the current practice for the investigation of Sudden Unexplained Death in the Young (SUDY) amongst European countries. An online questionnaire composed of 21 questions was submitted to the EHRA Research Network, European Cardiac Arrhythmia Genetics (ECGen) Focus Group members, and European Reference Network GUARD-Heart healthcare partners. There were 81 respondents from 24 European countries. The majority (78%) worked in a dedicated clinic focusing on families with inherited cardiac conditions and/or SUDY or had easy access to a nearby one. On average, an autopsy was performed in 43% of SUDY cases. Macroscopic examination of the body and all organs were completed in 71% of cases undergoing autopsy, and expert cardiac examination in 32%. Post-mortem genetic testing was requested on average in 37% of Sudden Arrhythmic Death Syndrome (SADS) cases, but not at all by 20% of survey respondents. Psychological support and bereavement counselling for SADS/SUDY families were available for <= 50% of participants. Whilst electrocardiogram (ECG) and echocardiography were largely employed to investigate SADS relatives, there was an inconsistent approach to the use of provocative testing with exercise ECG, sodium channel blocking drugs, and/or epinephrine and genetic testing. The survey highlighted a significant heterogeneity of service provision and variable adherence to current recommendations for the investigation of SUDY, partly attributable to the availability of dedicated units and specialist tests, genetic evaluation, and post-mortem examination.
Filiaciones:
Behr ER:
Cardiology Research Centre and Cardiovascular Clinical Academic Group, Molecular and Clinical Sciences Institute, St. George's, University of London, Cranmer Terrace, London SW17 0RE, UK
St. George's University Hospitals NHS Foundation Trust, London, UK
Scrocco C:
Cardiology Research Centre and Cardiovascular Clinical Academic Group, Molecular and Clinical Sciences Institute, St. George's, University of London, Cranmer Terrace, London SW17 0RE, UK
St. George's University Hospitals NHS Foundation Trust, London, UK
Wilde AAM:
Department of Clinical and Experimental Cardiology, Heart Center, Amsterdam UMC, Location Academic Medical Center, Amsterdam, The Netherlands
Marijon E:
Paris Cardiovascular Research Center, INSERM Unit 970, Paris, France
Université Paris Descartes, Sorbonne Paris Cité, Paris, France
Cardiology Department, Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Paris, France
Crotti L:
Department of Cardiovascular, Neural and Metabolic Sciences, Istituto Auxologico Italiano, IRCCS, San Luca Hospital, Milan, Italy
Istituto Auxologico Italiano, IRCCS, Center for Cardiac Arrhythmias of Genetic Origin, Laboratory of Cardiovascular Genetics, Milan, Italy
Department of Medicine and Surgery, University of Milano-Bicocca, Milan, Italy
Iliodromitis KE:
Evangelisches Krankenhaus Hagen-Haspe, Clinic for Cardiology and Electrophysiology, Hagen, Germany
Remme CA:
Department of Clinical and Experimental Cardiology, Heart Center, Amsterdam UMC, Location Academic Medical Center, Amsterdam, The Netherlands
Kosiuk J:
1st Chair and Department of Cardiology, Medical University of Warsaw, Warsaw, Poland
Rudaka I:
Arrhythmology Department, Paul Stradins Clinical University Hospital, Riga Stradins University, Riga, Latvia
Sarquella-Brugada G:
Arrhythmia, Inherited Cardiac Diseases and Sudden Death, Hospital Sant Joan de Déu, Barcelona, Spain
Medical Sciences Department, School of Medicine, University of Girona, Girona, Spain
Frampton K:
Cardiology Research Centre and Cardiovascular Clinical Academic Group, Molecular and Clinical Sciences Institute, St. George's, University of London, Cranmer Terrace, London SW17 0RE, UK
St. George's University Hospitals NHS Foundation Trust, London, UK
Schulze-Bahr E:
Institute for Genetics of Heart Diseases (IfGH), University Hospital Münster, Münster, Germany
Jubele K:
Arrhythmology Department, Paul Stradins Clinical University Hospital, Riga Stradins University, Riga, Latvia
de Asmundis C:
Heart Rhythm Management Center, Postgraduate Program in Cardiac Electrophysiology and Pacing, Universitair Ziekenhuis Brussel-Vrije Universiteit Brussel, Brussels, Belgium
Hofman N:
Department of Clinical and Experimental Cardiology, Heart Center, Amsterdam UMC, Location Academic Medical Center, Amsterdam, The Netherlands
Tfelt-Hansen J:
The Heart Centre, Department of Cardiology, Copenhagen University Hospital/Rigshospitalet, Copenhagen, Denmark
Section of Forensic Genetics, Department of Forensic Medicine, Faculty of Health Sciences, University of Copenhagen, Copenhagen, Denmark
Boveda S:
Paris Cardiovascular Research Center, INSERM Unit 970, Paris, France
Université Paris Descartes, Sorbonne Paris Cité, Paris, France
Heart Rhythm Management Center, Postgraduate Program in Cardiac Electrophysiology and Pacing, Universitair Ziekenhuis Brussel-Vrije Universiteit Brussel, Brussels, Belgium
Heart Rhythm Management Department, Clinique Pasteur, 45 Avenue de Lombez, 31076 Toulouse, France
Conte G:
Division of Cardiology, Cardiocentro Ticino Institute, Lugano, Switzerland
Università della Svizzera Italiana Lugano, Lugano, Switzerland
Green Submitted, Bronze
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