Investigation on Sudden Unexpected Death in the Young (SUDY) in Europe: results of the European Heart Rhythm Association Survey

Elijah R Behr; Chiara Scrocco; Arthur A M Wilde; Eloi Marijon; Lia Crotti; Konstantinos E Iliodromitis; Carol A Remme; Jedrzej Kosiuk; Irina Rudaka; Georgia Sarquella Brugada; Katie Frampton; Eric Schulze-Bahr; Kristine Jubele; Carlo de Asmundis; Nynke Hofman; Jacob Tfelt-Hansen; Serge Boveda; Giulio Conte

doi:10.1093/europace/euab176

Investigation on Sudden Unexpected Death in the Young (SUDY) in Europe: results of the European Heart Rhythm Association Survey

Europace. 2022 Feb 2;24(2):331-339. doi: 10.1093/europace/euab176.

Authors

Elijah R Behr^{1

2}, Chiara Scrocco^{1

2}, Arthur A M Wilde³, Eloi Marijon^{4

5

6}, Lia Crotti^{7

8

9}, Konstantinos E Iliodromitis¹⁰, Carol A Remme³, Jedrzej Kosiuk¹¹, Irina Rudaka¹², Georgia Sarquella Brugada^{13

14}, Katie Frampton^{1

2}, Eric Schulze-Bahr¹⁵, Kristine Jubele¹², Carlo de Asmundis¹⁶, Nynke Hofman³, Jacob Tfelt-Hansen^{17

18}, Serge Boveda^{4

5

16

19}, Giulio Conte^{20

21}

Affiliations

¹ 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.
² St. George's University Hospitals NHS Foundation Trust, London, UK.
³ Department of Clinical and Experimental Cardiology, Heart Center, Amsterdam UMC, Location Academic Medical Center, Amsterdam, The Netherlands.
⁴ Paris Cardiovascular Research Center, INSERM Unit 970, Paris, France.
⁵ Université Paris Descartes, Sorbonne Paris Cité, Paris, France.
⁶ Cardiology Department, Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Paris, France.
⁷ Department of Cardiovascular, Neural and Metabolic Sciences, Istituto Auxologico Italiano, IRCCS, San Luca Hospital, Milan, Italy.
⁸ Istituto Auxologico Italiano, IRCCS, Center for Cardiac Arrhythmias of Genetic Origin, Laboratory of Cardiovascular Genetics, Milan, Italy.
⁹ Department of Medicine and Surgery, University of Milano-Bicocca, Milan, Italy.
¹⁰ Evangelisches Krankenhaus Hagen-Haspe, Clinic for Cardiology and Electrophysiology, Hagen, Germany.
¹¹ 1st Chair and Department of Cardiology, Medical University of Warsaw, Warsaw, Poland.
¹² Arrhythmology Department, Paul Stradins Clinical University Hospital, Riga Stradins University, Riga, Latvia.
¹³ Arrhythmia, Inherited Cardiac Diseases and Sudden Death, Hospital Sant Joan de Déu, Barcelona, Spain.
¹⁴ Medical Sciences Department, School of Medicine, University of Girona, Girona, Spain.
¹⁵ Institute for Genetics of Heart Diseases (IfGH), University Hospital Münster, Münster, Germany.
¹⁶ Heart Rhythm Management Center, Postgraduate Program in Cardiac Electrophysiology and Pacing, Universitair Ziekenhuis Brussel-Vrije Universiteit Brussel, Brussels, Belgium.
¹⁷ The Heart Centre, Department of Cardiology, Copenhagen University Hospital/Rigshospitalet, Copenhagen, Denmark.
¹⁸ Section of Forensic Genetics, Department of Forensic Medicine, Faculty of Health Sciences, University of Copenhagen, Copenhagen, Denmark.
¹⁹ Heart Rhythm Management Department, Clinique Pasteur, 45 Avenue de Lombez, 31076 Toulouse, France.
²⁰ Division of Cardiology, Cardiocentro Ticino Institute, Lugano, Switzerland.
²¹ Università della Svizzera Italiana Lugano, Lugano, Switzerland.

PMID: 34351417
DOI: 10.1093/europace/euab176

Abstract

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.

Keywords: Autopsy; European Heart Rhythm Association survey; Genetic testing; Inherited cardiac conditions; Provocation testing; Sudden Arrhythmic Death Syndrome; Sudden Unexplained Death in the Young; Sudden death.

MeSH terms

Arrhythmias, Cardiac / diagnosis
Arrhythmias, Cardiac / epidemiology
Arrhythmias, Cardiac / genetics
Death, Sudden, Cardiac* / epidemiology
Europe / epidemiology
Genetic Predisposition to Disease*
Humans
Surveys and Questionnaires