Family History and Warning Symptoms Precede Sudden Cardiac Death in Arrhythmogenic Right Ventricular Cardiomyopathy (from a Nationwide Study in Sweden)

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiac disease explaining about 4% of sudden cardiac death (SCD) cases in the young in Sweden. This study aimed to describe the circumstances preceding SCD in all victims <35 years of age who received an autopsy-confirmed diagnosis of ARVC from January 1, 2000, to December 31, 2010, in Sweden (n = 22). Data on demographics, medical and family history, circumstances of death, and anatomopathological findings were collected from several compulsory national health registries, clinical records, family interviews, and autopsy reports. Registry-based data were compared with age-matched, gender-matched, and geographically-matched population controls. During the 6 months preceding SCD, 15 cases (68%) had experienced symptoms of cardiac origin, mainly syncope or presyncope (54%) and chest discomfort (27%). A total of 8 cases (36%) had sought medical care because of cardiac symptoms. The occurrence of hospital visits was significantly increased in cases compared with controls (odds ratio 4.62 1.35 to 15.8). A total of 10 cases (45%) had a family history of SCD. The most common activity at the time of death was exercise (41%). A complete cardiac investigation was seldom performed; only 1 case was diagnosed with ARVC before death. In conclusion, in this nationwide study, we observed a high prevalence of symptoms of cardiac origin, healthcare use, and family history of SCD preceding SCD in the young caused by ARVC. Increased awareness of these warning signals in younger patients is critical to improving risk stratification and early disease detection. Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiac disease explaining about 4% of sudden cardiac death (SCD) cases in the young in Sweden. This study aimed to describe the circumstances preceding SCD in all victims <35 years of age who received an autopsy-confirmed diagnosis of ARVC from January 1, 2000, to December 31, 2010, in Sweden (n = 22). Data on demographics, medical and family history, circumstances of death, and anatomopathological findings were collected from several compulsory national health registries, clinical records, family interviews, and autopsy reports. Registry-based data were compared with age-matched, gender-matched, and geographically-matched population controls. During the 6 months preceding SCD, 15 cases (68%) had experienced symptoms of cardiac origin, mainly syncope or presyncope (54%) and chest discomfort (27%). A total of 8 cases (36%) had sought medical care because of cardiac symptoms. The occurrence of hospital visits was significantly increased in cases compared with controls (odds ratio 4.62 1.35 to 15.8). A total of 10 cases (45%) had a family history of SCD. The most common activity at the time of death was exercise (41%). A complete cardiac investigation was seldom performed; only 1 case was diagnosed with ARVC before death. In conclusion, in this nationwide study, we observed a high prevalence of symptoms of cardiac origin, healthcare use, and family history of SCD preceding SCD in the young caused by ARVC. Increased awareness of these warning signals in younger patients is critical to improving risk stratification and early disease detection. IntroductionArrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiac disease affecting 1:1000 to 1:5000 patients.1Basso C Corrado D Marcus FI Nava A Thiene G. Arrhythmogenic right ventricular cardiomyopathy.Lancet. 2009; 373: 1289-1300Abstract Full Text Full Text PDF PubMed Scopus (630) Google Scholar,2James CA Syrris P van Tintelen JP Calkins H. The role of genetics in cardiovascular disease: arrhythmogenic cardiomyopathy.Eur Heart J. 2020; 41: 1393-1400Crossref PubMed Scopus (36) Google Scholar ARVC is characterized by integrity loss of the intercalated disk, followed by apoptosis and fibrofatty replacement of the myocardium, leading to arrhythmia and impaired systolic function.1Basso C Corrado D Marcus FI Nava A Thiene G. Arrhythmogenic right ventricular cardiomyopathy.Lancet. 2009; 373: 1289-1300Abstract Full Text Full Text PDF PubMed Scopus (630) Google Scholar,3Saffitz JE. The pathobiology of arrhythmogenic cardiomyopathy.Annu Rev Pathol. 2011; 6: 299-321Crossref PubMed Scopus (34) Google Scholar Early disease recognition is a pervasive challenge; patients can present with a spectrum of ventricular arrhythmias, from benign premature extrasystoles (ventricular extrasystoles) to sustained ventricular tachycardias (VTs) and sudden cardiac death (SCD) before detectable structural changes occur.1Basso C Corrado D Marcus FI Nava A Thiene G. Arrhythmogenic right ventricular cardiomyopathy.Lancet. 2009; 373: 1289-1300Abstract Full Text Full Text PDF PubMed Scopus (630) Google Scholar,3Saffitz JE. The pathobiology of arrhythmogenic cardiomyopathy.Annu Rev Pathol. 2011; 6: 299-321Crossref PubMed Scopus (34) Google Scholar ARVC is indeed one of the leading causes of SCD in the younger population, explaining 5% to 15% of SCD cases in Europe and up to 32% of SCD in competitive athletes.4Corrado D Thiene G Nava A Rossi L Pennelli N. Sudden death in young competitive athletes: clinicopathologic correlations in 22 cases.Am J Med. 1990; 89: 588-596Abstract Full Text PDF PubMed Scopus (474) Google Scholar, 5Corrado D Basso C Thiene G. Sudden cardiac death in young people with apparently normal heart.Cardiovasc Res. 2001; 50: 399-408Crossref PubMed Scopus (299) Google Scholar, 6Tabib A Loire R Chalabreysse L Meyronnet D Miras A Malicier D Thivolet F Chevalier P Bouvagnet P. Circumstances of death and gross and microscopic observations in a series of 200 cases of sudden death associated with arrhythmogenic right ventricular cardiomyopathy and/or dysplasia.Circulation. 2003; 108: 3000-3005Crossref PubMed Scopus (266) Google Scholar, 7Finocchiaro G Papadakis M Robertus JL Dhutia H Steriotis AK Tome M Mellor G Merghani A Malhotra A Behr E Sharma S Sheppard MN. Etiology of sudden death in sports: insights from a United Kingdom regional registry.J Am Coll Cardiol. 2016; 67: 2108-2115Crossref PubMed Scopus (286) Google Scholar, 8Sadjadieh G Jabbari R Risgaard B Olesen MS Haunso S Tfelt-Hansen J Winkel BG. Nationwide (Denmark) study of symptoms preceding sudden death due to arrhythmogenic right ventricular cardiomyopathy.Am J Cardiol. 2014; 113: 1250-1254Abstract Full Text Full Text PDF PubMed Scopus (19) Google Scholar In Sweden, the incidence of SCD in patients aged 1 to 35 years between 2000 and 2010 was 1.3 × 100.000 person-year, and ARVC was the cause of death in 22 cases (4%).9Wisten A Krantz P Stattin EL. Sudden cardiac death among the young in Sweden from 2000 to 2010: an autopsy-based study.Europace. 2017; 19: 1327-1334PubMed Google Scholar,10Stattin EL Hagström E Dahl N Strömsöe A Delgado-Vega AM Klar J Bodil S Börjesson M Wisten A. The Swedish study of sudden cardiac death in the young (SUDDY) 2000–2010 – a complete nationwide cohort of SCDs.BMJ Open. 2022; 12e055557Crossref Scopus (3) Google Scholar In the present study, we describe the family history, symptoms, hospital care use, and circumstances of the death of these 22 patients, based on data from national health registries, medical records (MRs), family interviews, and autopsy reports.MethodsAll subjects aged 1 to 35 years who died of SCD with autopsy-confirmed ARVC as the probable cause of death from January 1, 2000, to December 31, 2010, in Sweden were included in the study (n = 22). Cases were identified through nationwide registry data from the Swedish National Board of Forensic Medicine and the Swedish Cause of Death Registry. Autopsy reports and death certificates were manually reviewed by a single forensic pathologist to confirm the postmortem ARVC diagnosis. The nationally standardized autopsy protocols followed during the study period, and the criteria by which SCD subjects were selected have been previously described.9Wisten A Krantz P Stattin EL. Sudden cardiac death among the young in Sweden from 2000 to 2010: an autopsy-based study.Europace. 2017; 19: 1327-1334PubMed Google Scholar For every case, 5 controls matched by gender, birth and were identified from the Swedish of the and (n = of the cases were identified from the Swedish from patients, and controls were from the Death of the study which data from several Swedish National EL Hagström E Dahl N Strömsöe A Delgado-Vega AM Klar J Bodil S Börjesson M Wisten A. 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Nationwide (Denmark) study of symptoms preceding sudden death due to arrhythmogenic right ventricular cardiomyopathy.Am J Cardiol. 2014; 113: 1250-1254Abstract Full Text Full Text PDF PubMed Scopus (19) Google A preceding sudden cardiac death in the young common J. PubMed Scopus Google C G Papadakis M B J B J G E C Malhotra A Robertus JL A A Tome M Sharma S Behr Sheppard Sudden death and ventricular in arrhythmogenic PubMed Scopus Google R C B S A H Thiene G Basso C Calkins H CA of of cardiac in patients with arrhythmogenic right ventricular J Cardiol. 2017; Full Text Full Text PDF PubMed Scopus Google Scholar In Sweden, the a was observed in young victims of SCD because of ARVC in a nationwide study from to A preceding sudden cardiac death in the young common J. PubMed Scopus Google Scholar was in the risk of young patients who sought medical care because of symptoms during the 10 with the prevalence of SCD observed in ARVC subjects was in all SCD victims from the cohort A Loire R Chalabreysse L Meyronnet D Miras A Malicier D Thivolet F Chevalier P Bouvagnet P. Circumstances of death and gross and microscopic observations in a series of 200 cases of sudden death associated with arrhythmogenic right ventricular cardiomyopathy and/or dysplasia.Circulation. 2003; 108: 3000-3005Crossref PubMed Scopus (266) Google G Jabbari R Risgaard B Olesen MS Haunso S Tfelt-Hansen J Winkel BG. Nationwide (Denmark) study of symptoms preceding sudden death due to arrhythmogenic right ventricular cardiomyopathy.Am J Cardiol. 2014; 113: 1250-1254Abstract Full Text Full Text PDF PubMed Scopus (19) Google D C H J A C C Calkins H. Arrhythmogenic right ventricular a United PubMed Scopus Google C G Papadakis M B J B J G E C Malhotra A Robertus JL A A Tome M Sharma S Behr Sheppard Sudden death and ventricular in arrhythmogenic PubMed Scopus Google A Börjesson M Krantz P Stattin EL. sudden cardiac death (SCD) in the young of a Swedish nationwide a in SCD among Full Text Full Text PDF PubMed Scopus Google Scholar only of in subjects competitive most subjects as of the of most victims (68%) were in high to a study in the United Kingdom on SCD victims because of arrhythmogenic cardiomyopathy a of symptoms and a of C G Papadakis M B J B J G E C Malhotra A Robertus JL A A Tome M Sharma S Behr Sheppard Sudden death and ventricular in arrhythmogenic PubMed Scopus Google Scholar this cohort of an population with a spectrum of arrhythmogenic total of 8 cases in cohort were during and at the time of SCD were to in ARVC and C B van van H Calkins H van Tintelen JP CA and in with arrhythmogenic right ventricular 2016; PubMed Scopus (34) Google Scholar, E E G P C C C F J. and in arrhythmogenic right ventricular J Cardiol. Full Text Full Text PDF PubMed Scopus Google Scholar, A H J ventricular arrhythmias, and in with arrhythmogenic right ventricular cardiomyopathy in the ARVC 2020; PubMed Scopus (3) Google Scholar the role of in ARVC of the present study is to ARVC early and young patients at risk of SCD. most cases who sought medical care before death an to symptoms in younger symptoms of impaired cardiac in and and complete cardiac in the of a family history of SCD or inherited cardiovascular as the time to is R C B S A H Thiene G Basso C Calkins H CA of of cardiac in patients with arrhythmogenic right ventricular J Cardiol. 2017; Full Text Full Text PDF PubMed Scopus Google of the study is and based on at standardized protocols at the time of all autopsy reports in the cohort were manually to confirm the we ARVC cases Cases were sudden and victims of SCD this study a series of cases with the data through and to family to or of symptoms, and history of activity of the data medical was with the by the postmortem was during the study the ARVC diagnosis was in of the in to as of the postmortem investigation of F van P AM F S H A C D E Sheppard AM A Basso C on of of of of on and prevalence and of the of sudden cardiac J PubMed Scopus Google C B J S G C A J L P S S Sheppard S Thiene G van A A autopsy investigation of sudden cardiac from the 2017; PubMed Scopus Google conclusion, in this study of SCD in the we observed most victims of SCD caused by ARVC had warning symptoms of cardiac origin, with the most before death. of the patients with cardiac symptoms had sought medical only 1 was as a family history of SCD and arrhythmogenic disease was common in most and inherited cardiac disease was IntroductionArrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiac disease affecting 1:1000 to 1:5000 patients.1Basso C Corrado D Marcus FI Nava A Thiene G. Arrhythmogenic right ventricular cardiomyopathy.Lancet. 2009; 373: 1289-1300Abstract Full Text Full Text PDF PubMed Scopus (630) Google Scholar,2James CA Syrris P van Tintelen JP Calkins H. The role of genetics in cardiovascular disease: arrhythmogenic cardiomyopathy.Eur Heart J. 2020; 41: 1393-1400Crossref PubMed Scopus (36) Google Scholar ARVC is characterized by integrity loss of the intercalated disk, followed by apoptosis and fibrofatty replacement of the myocardium, leading to arrhythmia and impaired systolic function.1Basso C Corrado D Marcus FI Nava A Thiene G. Arrhythmogenic right ventricular cardiomyopathy.Lancet. 2009; 373: 1289-1300Abstract Full Text Full Text PDF PubMed Scopus (630) Google Scholar,3Saffitz JE. The pathobiology of arrhythmogenic cardiomyopathy.Annu Rev Pathol. 2011; 6: 299-321Crossref PubMed Scopus (34) Google Scholar Early disease recognition is a pervasive challenge; patients can present with a spectrum of ventricular arrhythmias, from benign premature extrasystoles (ventricular extrasystoles) to sustained ventricular tachycardias (VTs) and sudden cardiac death (SCD) before detectable structural changes occur.1Basso C Corrado D Marcus FI Nava A Thiene G. Arrhythmogenic right ventricular cardiomyopathy.Lancet. 2009; 373: 1289-1300Abstract Full Text Full Text PDF PubMed Scopus (630) Google Scholar,3Saffitz JE. The pathobiology of arrhythmogenic cardiomyopathy.Annu Rev Pathol. 2011; 6: 299-321Crossref PubMed Scopus (34) Google Scholar ARVC is indeed one of the leading causes of SCD in the younger population, explaining 5% to 15% of SCD cases in Europe and up to 32% of SCD in competitive athletes.4Corrado D Thiene G Nava A Rossi L Pennelli N. Sudden death in young competitive athletes: clinicopathologic correlations in 22 cases.Am J Med. 1990; 89: 588-596Abstract Full Text PDF PubMed Scopus (474) Google Scholar, 5Corrado D Basso C Thiene G. Sudden cardiac death in young people with apparently normal heart.Cardiovasc Res. 2001; 50: 399-408Crossref PubMed Scopus (299) Google Scholar, 6Tabib A Loire R Chalabreysse L Meyronnet D Miras A Malicier D Thivolet F Chevalier P Bouvagnet P. Circumstances of death and gross and microscopic observations in a series of 200 cases of sudden death associated with arrhythmogenic right ventricular cardiomyopathy and/or dysplasia.Circulation. 2003; 108: 3000-3005Crossref PubMed Scopus (266) Google Scholar, 7Finocchiaro G Papadakis M Robertus JL Dhutia H Steriotis AK Tome M Mellor G Merghani A Malhotra A Behr E Sharma S Sheppard MN. Etiology of sudden death in sports: insights from a United Kingdom regional registry.J Am Coll Cardiol. 2016; 67: 2108-2115Crossref PubMed Scopus (286) Google Scholar, 8Sadjadieh G Jabbari R Risgaard B Olesen MS Haunso S Tfelt-Hansen J Winkel BG. Nationwide (Denmark) study of symptoms preceding sudden death due to arrhythmogenic right ventricular cardiomyopathy.Am J Cardiol. 2014; 113: 1250-1254Abstract Full Text Full Text PDF PubMed Scopus (19) Google Scholar In Sweden, the incidence of SCD in patients aged 1 to 35 years between 2000 and 2010 was 1.3 × 100.000 person-year, and ARVC was the cause of death in 22 cases (4%).9Wisten A Krantz P Stattin EL. Sudden cardiac death among the young in Sweden from 2000 to 2010: an autopsy-based study.Europace. 2017; 19: 1327-1334PubMed Google Scholar,10Stattin EL Hagström E Dahl N Strömsöe A Delgado-Vega AM Klar J Bodil S Börjesson M Wisten A. The Swedish study of sudden cardiac death in the young (SUDDY) 2000–2010 – a complete nationwide cohort of SCDs.BMJ Open. 2022; 12e055557Crossref Scopus (3) Google Scholar In the present study, we describe the family history, symptoms, hospital care use, and circumstances of the death of these 22 patients, based on data from national health registries, medical records (MRs), family interviews, and autopsy reports.