Elliott P, Andersson B, Arbustini E et al (2008) Classification of the cardiomyopathies: a position statement from the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases. Eur Heart J [Internet] 29:270–276. Available from: http://www.ncbi.nlm.nih.gov/pubmed/17916581

Finocchiaro G, Papadakis M, Robertus J-L, Dhutia H, Steriotis AK, Tome M, Mellor G, Merghani A, Malhotra A, Behr E, Sharma S, Sheppard MN (2016) Etiology of sudden death in sports insights from a United Kingdom Regional Registry. J Am Coll Cardiol 67

Stecker EC, Reinier K, Marijon E, Narayanan K, Teodorescu C, Uy-Evanado A, Gunson K, Jui J, Chugh SS (2014) Public health burden of sudden cardiac death in the United States. Circ Arrhythm Electrophysiol [Internet] 7:212–217. Available from: http://www.ncbi.nlm.nih.gov/pubmed/24610738

Smith T, Jordaens L, Theuns DAMJ, van Dessel PF, Wilde AA, Hunink MGM (2013) The cost-effectiveness of primary prophylactic implantable defibrillator therapy in patients with ischaemic or non-ischaemic heart disease: a European analysis. Eur Heart J 34:211–219

PubMed  Google Scholar 

O’Mahony C, Jichi F, Pavlou M, Monserrat L, Anastasakis A, Rapezzi C, Biagini E, Gimeno JR, Limongelli G, McKenna WJ, Omar RZ, Elliott PM (2014) A novel clinical risk prediction model for sudden cardiac death in hypertrophic cardiomyopathy (HCM Risk-SCD). Eur Heart J [Internet] 35:2010–2020. Available from: http://www.ncbi.nlm.nih.gov/pubmed/24126876

Maron MS, Rowin EJ, Wessler BS, Mooney PJ, Fatima A, Patel P, Koethe BC, Romashko M, Link MS, Maron BJ (2019) Enhanced American College of Cardiology/American Heart Association strategy for prevention of sudden cardiac death in high-risk patients with hypertrophic cardiomyopathy. JAMA Cardiol [Internet] 4:644–657. Available from: http://www.ncbi.nlm.nih.gov/pubmed/31116360

Elliott PM, Anastasakis A, Borger MA et al (2014) 2014 ESC guidelines on diagnosis and management of hypertrophic cardiomyopathy. Eur Heart J 35:2733–2779

Article  Google Scholar 

Elliott PM, Gimeno JR, Thaman R, Shah J, Ward D, Dickie S, Tome Esteban MT, McKenna WJ (2006) Historical trends in reported survival rates in patients with hypertrophic cardiomyopathy. Heart 92:785–791

CAS  PubMed  Google Scholar 

Norrish G, Ding T, Field E et al (2019) Development of a novel risk prediction model for sudden cardiac death in childhood hypertrophic cardiomyopathy (HCM Risk-Kids). JAMA Cardiol [Internet] 4:918–927. Available from: http://www.ncbi.nlm.nih.gov/pubmed/31411652

Bonow RO, McNally EM (2019) Risk prediction model in children with hypertrophic cardiomyopathy: a work in progress. JAMA Cardiol [Internet] 4:927. Available from: http://www.ncbi.nlm.nih.gov/pubmed/31411634

Ponikowski P, Voors AA, Anker SD et al (2016) ESC guidelines for the diagnosis and treatment of acute and chronic heart failure. Eur Heart J 2016:2129–2200m

Google Scholar 

Køber L, Thune JJ, Nielsen JC et al (2016) Defibrillator implantation in patients with nonischemic systolic heart failure. N Engl J Med [Internet] 375:1221–1230. Available from: http://www.ncbi.nlm.nih.gov/pubmed/27571011

Melacini P, Basso C, Angelini A, Calore C, Bobbo F, Tokajuk B, Bellini N, Smaniotto G, Zucchetto M, Iliceto S, Thiene G, Maron BJ (2010) Clinicopathological profiles of progressive heart failure in hypertrophic cardiomyopathy. Eur Heart J 31:2111–2123

PubMed  PubMed Central  Google Scholar 

Niknian M, McKinlay SM, Rakowski W, Carleton RA (1989) A comparison of perceived and objective CVD risk in a general population. Am J Public Health [Internet] 79:1653–1654. Available from: http://www.ncbi.nlm.nih.gov/pubmed/2817194

van der Weijden T, van Steenkiste B, Stoffers HEJH, Timmermans DRM, Grol R Primary prevention of cardiovascular diseases in general practice: mismatch between cardiovascular risk and patients’ risk perceptions. Med Decis Making [Internet] 27:754–761. Available from: http://www.ncbi.nlm.nih.gov/pubmed/17873263

Health Literacy (1999) Report of the Council on Scientific Affairs. Ad Hoc Committee on Health Literacy for the Council on Scientific Affairs, American Medical Association. JAMA [Internet] 281:552–557. Available from: http://www.ncbi.nlm.nih.gov/pubmed/10022112

Lipkus IM Numeric, verbal, and visual formats of conveying health risks: suggested best practices and future recommendations. Med Decis Making [Internet] 27:696–713. Available from: http://www.ncbi.nlm.nih.gov/pubmed/17873259

Paling J (2003) Strategies to help patients understand risks. BMJ [Internet] 327:745–748. Available from: http://www.ncbi.nlm.nih.gov/pubmed/14512489

Maurizi N, Tanini I, Olivotto I et al (2017) Effectiveness of subcutaneous implantable cardioverter-defibrillator testing in patients with hypertrophic cardiomyopathy. Int J Cardiol [Internet] 231:115–119. Available from: http://www.ncbi.nlm.nih.gov/pubmed/28073660

Fumagalli C, Maurizi N, Day SM, Ashley EA, Michels M, Colan SD, Jacoby D, Marchionni N, Vincent-Tompkins J, Ho CY, Olivotto I (2020) Share Investigators: Association of obesity with adverse long-term outcomes in hypertrophic cardiomyopathy. JAMA Cardiol [Internet] 5:65–72. Available from: http://www.ncbi.nlm.nih.gov/pubmed/31693057

Olivotto I, Maron BJ, Tomberli B et al (2013) Obesity and its association to phenotype and clinical course in hypertrophic cardiomyopathy. J Am Coll Cardiol [Internet] 62:449–457. Available from: http://www.ncbi.nlm.nih.gov/pubmed/23643593

Magavern EF, Finocchiaro G, Sharma S, Papadakis M, Borry P (2018) Time out: ethical reflections on medical disqualification of athletes in the context of mandated pre-participation cardiac screening. Br J Sports Med [Internet] 52:1207–1210. Available from: http://www.ncbi.nlm.nih.gov/pubmed/29021245

Maresso A, Mladovsky P, Thomson S, Sagan A, Karanikolos M, Richardson E, Cylus J, Evetovits T, Jowett M, Figueras J, Kluge H (2015) Economic crisis, health systems and health in Europe-country experience. Econ Cris Heal Syst Heal Eur

Xu K, Soucat A, Kutzin J, Brindley C, Vande Maele N, Toure H, Garcia MA, Li D, Xu H (2018) Public spending on health: a closer look at global trends

Camerini F, Fabris E, Sinagra G (2019) Appropriateness, inappropriateness and waste of resources: unfulfilled expectations? Eur J Intern Med [Internet] 63:15–18. Available from: http://www.ncbi.nlm.nih.gov/pubmed/31006508

Gialama F, Prezerakos P, Maniadakis N (2014) The cost effectiveness of implantable cardioverter defibrillators: a systematic review of economic evaluations. Appl Health Econ Health Policy 12:41–49

PubMed  Google Scholar 

Mark DB, Hlatky MA (2002) Medical economics and the assessment of value in cardiovascular medicine: part I. Circulation 

Sanders GD, Hlatky MA, Owens DK (2005) Cost-effectiveness of implantable cardioverter-defibrillators. N Engl J Med 

Buchanan DR (2008) Autonomy, paternalism, and justice: ethical priorities in public health. Am J Public Health 98:15–21

PubMed  PubMed Central  Google Scholar 

Aggarwal A, Davies J, Sullivan R (2014) “Nudge” in the clinical consultation—an acceptable form of medical paternalism? BMC Med Ethics 15:31

PubMed  PubMed Central  Google Scholar 

Feinberg J (1986) Harm to self. Oxford University Press, New York, NY

Google Scholar 

Williams B (1981) Moral luck. Cambridge University Press, Cambridge

Google Scholar 

Monserrat L, Elliott PM, Gimeno JR, Sharma S, Penas-Lado M, McKenna WJ (2003) Non-sustained ventricular tachycardia in hypertrophic cardiomyopathy: an independent marker of sudden death risk in young patients. J Am Coll Cardiol

Merlo M, Pyxaras SA, Pinamonti B, Barbati G, Di Lenarda A, Sinagra G (2011) Prevalence and prognostic significance of left ventricular reverse remodeling in dilated cardiomyopathy receiving tailored medical treatment. J Am Coll Cardiol 57:1468–1476

PubMed  Google Scholar 

Mazzanti A, Ng K, Faragli A et al (2016) Arrhythmogenic right ventricular cardiomyopathy: clinical course and predictors of arrhythmic risk. J Am Coll Cardiol 68:2540–2550

PubMed  Google Scholar 

Maron BJ, Olivotto I, Spirito P, Casey SA, Bellone P, Gohman TE, Graham KJ, Burton DA, Cecchi F (2000) Epidemiology of hypertrophic cardiomyopathy–related death. Circulation 102:858–864

CAS  PubMed  Google Scholar 

Kadish A, Dyer A, Daubert JP et al (2004) Prophylactic defibrillator implantation in patients with nonischemic dilated cardiomyopathy. N Engl J Med 350:2151–2158

CAS  PubMed  Google Scholar 

Brun F, Groeneweg JA, Gear K, Sinagra G, van der Heijden J, Mestroni L, Hauer RN, Borgstrom M, Hughes T, Marcus FI (2016) Risk stratification in arrhythmic right ventricular cardiomyopathy without implantable cardioverter-defibrillators. JACC Clin Electrophysiol 2:558–564

PubMed  PubMed Central  Google Scholar 

Maron BJ, Casey SA, Poliac LC, Gohman TE, Almquist AK, Aeppli DM (1999) Clinical course of hypertrophic cardiomyopathy in a regional United States cohort. JAMA 281:650–655

CAS  PubMed  Google Scholar 

Strickberger SA, Hummel JD, Bartlett TG, Frumin HI, Schuger CD, Beau SL, Bitar C, Morady F (2003) AMIOVIRT Investigators: Amiodarone versus implantable cardioverter-defibrillator: randomized trial in patients with nonischemic dilated cardiomyopathy and asymptomatic nonsustained ventricular tachycardia—AMIOVIRT. J Am Coll Cardiol 41:1707–1712

PubMed  Google Scholar 

Nava A, Bauce B, Basso C, Muriago M, Rampazzo A, Villanova C, Daliento L, Buja G, Corrado D, Danieli GA, Thiene G (2000) Clinical profile and long-term follow-up of 37 families with arrhythmogenic right ventricular cardiomyopathy. J Am Coll Cardiol 36:2226–2233

CAS  PubMed  Google Scholar 

O’Mahony C, Jichi F, Ommen SR et al (2018) International external validation study of the 2014 European Society of Cardiology Guidelines on sudden cardiac death prevention in hypertrophic cardiomyopathy (EVIDENCE-HCM). Circulation 137:1015–1023

PubMed  Google Scholar 

Pinamonti B, Dragos AM, Pyxaras SA, Merlo M, Pivetta A, Barbati G, Di Lenarda A, Morgera T, Mestroni L, Sinagra G (2011) Prognostic predictors in arrhythmogenic right ventricular cardiomyopathy: results from a 10-year registry. Eur Heart J 32:1105–1113

PubMed  Google Scholar 

Lorenzini M, Anastasiou Z, O’Mahony C et al (2020) Mortality among referral patients with hypertrophic cardiomyopathy vs the general European population. JAMA Cardiol 5:73–80

PubMed  Google Scholar 

Sanna T, Dello Russo A, Toniolo D, Vytopil M, Pelargonio G, De Martino G, Ricci E, Silvestri G, Giglio V, Messano L, Zachara E, Bellocci F (2003) Cardiac features of Emery-Dreifuss muscular dystrophy caused by lamin A/C gene mutations. Eur Heart J

Aquaro GD, De Luca A, Cappelletto C et al (2020) Prognostic value of magnetic resonance phenotype in patients with arrhythmogenic right ventricular cardiomyopathy. J Am Coll Cardiol [Internet] 75:2753–2765. Available from: http://www.ncbi.nlm.nih.gov/pubmed/32498802

Aquaro GD, Grigoratos C, Bracco A, Proclemer A, Todiere G, Martini N, Habtemicael YG, Carerj S, Sinagra G, Di Bella G (2020) Late gadolinium enhancement-dispersion mapping: a new magnetic resonance imaging technique to assess prognosis in patients with hypertrophic cardiomyopathy and low-intermediate 5-year risk of sudden death. Circ Cardiovasc Imaging 13:e010489.

Gulati A, Jabbour A, Ismail TF et al (2013) Association of fibrosis with mortality and sudden cardiac death in patients with nonischemic dilated cardiomyopathy. JAMA [Internet] 309:896–908. Available from: http://www.ncbi.nlm.nih.gov/pubmed/23462786

Halliday BP, Gulati A, Ali A et al (2017) Association between midwall late gadolinium enhancement and sudden cardiac death in patients with dilated cardiomyopathy and mild and moderate left ventricular systolic dysfunction. Circulation [Internet] 135:2106–2115. Available from: http://www.ncbi.nlm.nih.gov/pubmed/28351901

Chan RH, Maron BJ, Olivotto I et al (2014) Prognostic value of quantitative contrast-enhanced cardiovascular magnetic resonance for the evaluation of sudden death risk in patients with hypertrophic cardiomyopathy. Circulation [Internet] 130:484–495. Available from: http://www.ncbi.nlm.nih.gov/pubmed/25092278

Merlo M, Cannatà A, Pio Loco C et al (2020) Contemporary survival trends and aetiological characterization in non-ischaemic dilated cardiomyopathy. Eur J Heart Fail 22:1111–1121

PubMed  Google Scholar 

Maron BJ, Spirito P, Shen WK et al (2007) Implantable cardioverter-defibrillators and prevention of sudden cardiac death in hypertrophic cardiomyopathy. JAMA [Internet] 298:405–412. Available from: http://www.ncbi.nlm.nih.gov/pubmed/17652294

Bänsch D, Antz M, Boczor S, Volkmer M, Tebbenjohanns J, Seidl K, Block M, Gietzen F, Berger J, Kuck KH (2002) Primary prevention of sudden cardiac death in idiopathic dilated cardiomyopathy: the Cardiomyopathy Trial (CAT). Circulation 105:1453–1458

PubMed  Google Scholar 

Corrado D, Calkins H, Link MS et al (2010) Prophylactic implantable defibrillator in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia and no prior ventricular fibrillation or sustained ventricular tachycardia. Circulation 122:1144–1152

PubMed  Google Scholar 

Alsheikh-Ali AA, Link MS, Semsarian C, Shen W-K, Estes NAM, Maron MS, Haas TS, Formisano F, Boriani G, Spirito P, Maron BJ (2013) Ventricular tachycardia/fibrillation early after defibrillator implantation in patients with hypertrophic cardiomyopathy is explained by a high-risk subgroup of patients. Hear Rhythm 10:214–218

Google Scholar 

James CA, Tichnell C, Murray B, Daly A, Sears SF, Calkins H (2012) General and disease-specific psychosocial adjustment in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy with implantable cardioverter defibrillators: a large cohort study. Circ Cardiovasc Genet 

Hodgkinson KA, Parfrey PS, Bassett AS, Kupprion C, Drenckhahn J, Norman MW, Thierfelder L, Stuckless SN, Dicks EL, McKenna WJ, Connors SP (2005) The impact of implantable cardioverter-defibrillator therapy on survival in autosomal-dominant arrhythmogenic right ventricular cardiomyopathy (ARVD5). J Am Coll Cardiol 45:400–408

PubMed  PubMed Central  Google Scholar 

Magnusson P, Gadler F, Liv P, Mörner S (2016) Risk markers and appropriate implantable defibrillator therapy in hypertrophic cardiomyopathy. Pacing Clin Electrophysiol 39:291–301

PubMed  Google Scholar 

Nazer B, Dale Z, Carrassa G et al (2020) Appropriate and inappropriate shocks in hypertrophic cardiomyopathy patients with subcutaneous implantable cardioverter-defibrillators: an international multicenter study. Hear Rhythm

Gigli M, Merlo M, Graw SL et al (2019) Genetic risk of arrhythmic phenotypes in patients with dilated cardiomyopathy. J Am Coll Cardiol [Internet] 74:1480–1490. Available from: http://www.ncbi.nlm.nih.gov/pubmed/31514951

Maron BJ, Spirito P, Ackerman MJ et al (2013) Prevention of sudden cardiac death with implantable cardioverter-defibrillators in children and adolescents with hypertrophic cardiomyopathy. J Am Coll Cardiol 61:1527–1535

PubMed  Google Scholar 

O’Mahony C, Lambiase PD, Quarta G, Cardona M, Calcagnino M, Tsovolas K, Al-Shaikh S, Rahman SM, Arnous S, Jones S, McKenna W, Elliott P (2012) The long-term survival and the risks and benefits of implantable cardioverter defibrillators in patients with hypertrophic cardiomyopathy. Heart 98:116–125

PubMed  Google Scholar 

Bardy GH, Lee KL, Mark DB et al (2005) Amiodarone or an implantable cardioverter-defibrillator for congestive heart failure. N Engl J Med 352:225–237

CAS  PubMed  Google Scholar 

Hauser RG, Maron BJ, Marine JE, Lampert R, Kadish AH, Winters SL, Scher DL, Biria M, Kalia A (2008) Safety and efficacy of transvenous high-voltage implantable cardioverter-defibrillator leads in high-risk hypertrophic cardiomyopathy patients. Hear Rhythm 5:1517–1522

Google Scholar 

Corrado D, Leoni L, Link MS et al (2003) Implantable cardioverter-defibrillator therapy for prevention of sudden death in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia. Circulation 108:3084–3091

PubMed  Google Scholar 

Elliott PM, Gimeno Blanes JR, Mahon NG, Poloniecki JD, McKenna WJ (2001) Relation between severity of left-ventricular hypertrophy and prognosis in patients with hypertrophic cardiomyopathy. Lancet (London, England) 357:420–424

CAS  Google Scholar 

O’Mahony C, Elliott P, McKenna W (2012) Sudden cardiac death in hypertrophic cardiomyopathy. Circ Arrhythm Electrophysiol [Internet] 1–29. Available from: http://www.ncbi.nlm.nih.gov/pubmed/23022709

Halliday BP, Cleland JGF, Goldberger JJ, Prasad SK (2017) Personalizing risk stratification for sudden death in dilated cardiomyopathy: the past, present, and future. Circulation

Link MS, Laidlaw D, Polonsky B, Zareba W, McNitt S, Gear K, Marcus F, Estes NAM (2014) Ventricular arrhythmias in the North American multidisciplinary study of ARVC: predictors, characteristics, and treatment. J Am Coll Cardiol 64:119–125

PubMed  PubMed Central  Google Scholar