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Sudden Cardiac Death Risk Assessment

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Hypertrophic Cardiomyopathy

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Abstract

HCM is a major cause of sudden cardiac death in young adults. The risk can be estimated using different scoring systems based on a number of selected clinical parameters. Other clinical features, such as myocardial fibrosis, LV apical aneurysms and multiple sarcomere protein gene mutations, are also used to guide ICD therapy. Prevention of sudden death relies on lifestyle modification and ICD therapy.

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Bibliography

  1. Maron BJ, McKenna WJ, Danielson GK, Kappenberger LJ, Kuhn HJ, Seidman CE, Shah PM, Spencer WH III, Spirito P, Ten Cate FJ, Wigle ED, ACCF Task Force on Clinical Expert Consensus Documents Members, Vogel RA, Abrams J, Bates ER, Brodie BR, Danias PG, Gregora. American College of Cardiology/European Society of Cardiology Clinical Expert Consensus Document on Hypertrophic Cardiomyopathy: a report of the American College of Cardiology Foundation Task Force on Clinical Expert Consensus Documents and the European Society of Cardiology Committee for Practice Guidelines. Eur Heart J. 2003;24:1965–91.

    Article  PubMed  Google Scholar 

  2. Gersh BJ, Maron BJ, Bonow RO, Dearani JA, Fifer MA, Link MS, Naidu SS, Nishimura RA, Ommen SR, Rakowski H, Seidman CE, Towbin JA, Udelson JE, Yancy CW. 2011 ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy: a report of the American College of Cardiology Foundation/American Heart Association task force on practice guidelines. Developed in collaboration with the American Association for Thoracic Surgery, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. J Am Coll Cardiol. 2011;58:e212–60.

    Article  CAS  PubMed  Google Scholar 

  3. Maron BJ. Contemporary insights and strategies for risk stratification and prevention of sudden death in hypertrophic cardiomyopathy. Circulation. 2010;121:445–56.

    Article  PubMed  Google Scholar 

  4. Maron BJ. Hypertrophic cardiomyopathy; a systemic review. JAMA. 2002;287:1308–20.

    PubMed  Google Scholar 

  5. 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, Hypertrophic Cardiomyopathy Outcomes Investigators. A novel clinical risk prediction model for sudden cardiac death in hypertrophic cardiomyopathy (HCM risk-SCD). Eur Heart J. 2014;35:2010–20.

    Article  PubMed  Google Scholar 

  6. McKenna WJ, Deanfield J, Faruqui A, England D, Oakley C, Goodwin J. Prognosis in hypertrophic cardiomyopathy: role of age and clinical, electrocardiographic and hemodynamic features. Am J Cardiol. 1981;47(3):532–8.

    Article  CAS  PubMed  Google Scholar 

  7. Frenneaux MP. Assessing the risk of sudden cardiac death in a patient with hypertrophic cardiomyopathy. Heart. 2004;90(5):570–5.

    Article  PubMed  PubMed Central  Google Scholar 

  8. Maron BJ, Olivotto I, Spirito P, Casey SA, Bellone P, Gohman TE, Graham KJ, Burton DA, Cecchi F. Epidemiology of hypertrophic cardiomyopathy-related death: revisited in a large non-referral-based patient population. Circulation. 2000;102:858–64.

    Article  CAS  PubMed  Google Scholar 

  9. Elliott PM, Gimeno JR, Thaman R, Shah J, Ward D, Dickie S, Tome Esteban MT, McKenna WJ. Historical trends in reported survival rates in patients with hypertrophic cardiomyopathy. Heart. 2006;92:785–91.

    Article  CAS  PubMed  Google Scholar 

  10. Maron BJ, Casey SA, Poliac LC, Gohman TE, Almquist AK, Aeppli DM. Clinical course of hypertrophic cardiomyopathy in a regional United States cohort. JAMA. 1999;281:65–655.

    Article  Google Scholar 

  11. Briasoulis A, Mallikethi-Reddy S, Palla M, Alesh I, Afonso L. Myocardial fibrosis on cardiac magnetic resonance and cardiac outcomes in hypertrophic cardiomyopathy: a meta-analysis. Heart. 2015;101(17):1406–11.

    Article  CAS  PubMed  Google Scholar 

  12. Adabag AS, Maron BJ. Implications of arrhythmias and prevention of sudden death in hypertrophic cardiomyopathy. Ann Noninvasive Electrocardiol. 2007;12:171–80.

    Article  PubMed  PubMed Central  Google Scholar 

  13. Basso C, Thiene G, Corrado D, Buja G, Melacini P, Nava A. Hypertrophic cardiomyopathy and sudden death in the young: pathologic evidence of myocardial ischemia. Hum Pathol. 2000;31:988–98.

    Article  CAS  PubMed  Google Scholar 

  14. Petersen SE, Jerosch-Herold M, Hudsmith LE, Robson MD, Francis JM, Doll HA, Selvanayagam JB, Neubauer S, Watkins H. Evidence for microvascular dysfunction in hypertrophic cardiomyopathy: new insights from multiparametric magnetic resonance imaging. Circulation. 2007;115:2418–25.

    Article  PubMed  Google Scholar 

  15. Cecchi F, Olivotto I, Gistri R, Lorenzoni R, Chiriatti G, Camici PG. Coronary microvascular dysfunction and prognosis in hypertrophic cardiomyopathy. N Engl J Med. 2003;349:1027–35.

    Article  CAS  PubMed  Google Scholar 

  16. Maron MS, Olivotto I, Maron BJ, Prasad SK, Cecchi F, Udelson JE, Camici PG. The case for myocardial ischemia in hypertrophic cardiomyopathy. J Am Coll Cardiol. 2009;54:866–75.

    Article  PubMed  Google Scholar 

  17. Tsoutsman T, Lam L, Semsarian C. Genes, calcium and modifying factors in hypertrophic cardiomyopathy. Clin Exp Pharmacol Physiol. 2006;33:139–45.

    Article  CAS  PubMed  Google Scholar 

  18. Baudenbacher F, Schober T, Pinto JR, Sidorov VY, Hilliard F, Solaro RJ, Potter JD, Knollmann BC. Myofilament ca2+ sensitization causes susceptibility to cardiac arrhythmia in mice. J Clin Invest. 2008;118:3893–903.

    CAS  PubMed  PubMed Central  Google Scholar 

  19. Frenneaux MP, Counihan PJ, Caforio AL, Chikamori T, McKenna WJ. Abnormal blood pressure response during exercise in hypertrophic cardiomyopathy. Circulation. 1990;82:1995–2002.

    Article  CAS  PubMed  Google Scholar 

  20. Williams L, Frenneaux M. Syncope in hypertrophic cardiomyopathy: mechanisms and consequences for treatment. Europace. 2007;9:817–22.

    Article  PubMed  Google Scholar 

  21. Sorajja P, Ommen SR, Nishimura RA, Gersh BJ, Berger PB, Tajik AJ. Adverse prognosis of patients with hypertrophic cardiomyopathy who have epicardial coronary artery disease. Circulation. 2003;108:2342–8.

    Article  PubMed  Google Scholar 

  22. Autore C, Bernabo P, Barilla CS, Bruzzi P, Spirito P. The prognostic importance of left ventricular outflow obstruction in hypertrophic cardiomyopathy varies in relation to the severity of symptoms. J Am Coll Cardiol. 2005;45:1076–80.

    Article  PubMed  Google Scholar 

  23. Elliott PM, Gimeno JR, Tome MT, Shah J, Ward D, Thaman R, Mogensen J, McKenna WJ. Left ventricular outflow tract obstruction and sudden death risk in patients with hypertrophic cardiomyopathy. Eur Heart J. 2006;27:1933–41.

    Article  PubMed  Google Scholar 

  24. Maron MS, Olivotto I, Betocchi S, Casey SA, Lesser JR, Losi MA, Cecchi F, Maron BJ. Effect of left ventricular outflow tract obstruction on clinical outcome in hypertrophic cardiomyopathy. N Engl J Med. 2003;348:295–303.

    Article  PubMed  Google Scholar 

  25. Sadoul N, Prasad K, Elliott PM, Bannerjee S, Frenneaux MP, McKenna WJ. Prospective prognostic assessment of blood pressure response during exercise in patients with hypertrophic cardiomyopathy. Circulation. 1997;96:2987–91.

    Article  CAS  PubMed  Google Scholar 

  26. Olivotto I, Maron BJ, Montereggi A, Mazzuoli F, Dolara A, Cecchi F. Prognostic value of systemic blood pressure response during exercise in a community-based patient population with hypertrophic cardiomyopathy. J Am Coll Cardiol. 1999;33:2044–51.

    Article  CAS  PubMed  Google Scholar 

  27. Elliott PM, Poloniecki J, Dickie S, Sharma S, Monserrat L, Varnava A, Mahon NG, McKenna WJ. Sudden death in hypertrophic cardiomyopathy: identification of high risk patients. J Am Coll Cardiol. 2000;36:2212–8.

    Article  CAS  PubMed  Google Scholar 

  28. O’Mahony C, Tome-Esteban M, Lambiase PD, Pantazis A, Dickie S, McKenna WJ, Elliott PM. A validation study of the 2003 American College of Cardiology/European Society of Cardiology and 2011 American College of Cardiology Foundation / American Heart Association risk stratification and treatment algorithms for sudden cardiac death in patients with hypertrophic cardiomyopathy. Heart. 2013;99(8):534–41.

    Article  PubMed  Google Scholar 

  29. Elliott PM, Gimeno Blanes JR, Mahon NG, Poloniecki JD, McKenna WJ. Relation between severity of left-ventricular hypertrophy and prognosis in patients with hypertrophic cardiomyopathy. Lancet. 2001;357:420–4.

    Article  CAS  PubMed  Google Scholar 

  30. Muir A, McKeown PP. Risk assessment in hypertrophic cardiomyopathy: contemporary guidelines hampered by insufficient evidence. Heart. 2013;99(8):511–3.

    Article  PubMed  Google Scholar 

  31. Cecchi F, Maron BJ, Epstein SE. Long-term outcome of patients with hypertrophic cardiomyopathy successfully resuscitated after cardiac arrest. J Am Coll Cardiol. 1989;13(6):1283–8.

    Article  CAS  PubMed  Google Scholar 

  32. Maron BJ, Shen WK, Link MS, Epstein AE, Almquist AK, Daubert JP, Bardy GH, Favale S, Rea RF, Boriani G, Estes NA 3rd, Spirito P. Efficacy of implantable cardioverter-defibrillators for the prevention of sudden death in patients with hypertrophic cardiomyopathy. N Engl J Med. 2000;342:365–73.

    Article  CAS  PubMed  Google Scholar 

  33. Zipes DP, Camm AJ, Borggrefe M, Buxton AE, Chaitman B, Fromer M, Gregoratos G, Klein G, Moss AJ, Myerburg RJ, Priori SG, Quinones MA, Roden DM, Silka MJ, Tracy C, Smith SC Jr, Jacobs AK, Adams CD, Antman EM, Anderson JL, Hunt SA, Halperin JL, Nishimura. ACC / AHA / ESC 2006 guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: A report of the american college of cardiology/american heart association task force and the european society of cardiology committee for practice guidelines (writing committee to develop guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death): Developed in collaboration with the european heart rhythm association and the heart rhythm society. Circulation. 2006;114:e385–484.

    Article  PubMed  Google Scholar 

  34. Epstein AE, DiMarco JP, Ellenbogen KA, Estes NA 3rd, Freedman RA, Gettes LS, Gillinov AM, Gregoratos G, Hammill SC, Hayes DL, Hlatky MA, Newby LK, Page RL, Schoenfeld MH, Silka MJ, Stevenson LW, Sweeney MO, Smith SC Jr, Jacobs AK, Adams CD, Anderson JL. ACC/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm abnormalities: A report of the American college of cardiology/American Heart Association task force on practice guidelines (writing committee to revise the ACC/AHA/NASPE 2002 guideline update for implantation of cardiac pacemakers and antiarrhythmia devices) developed in collaboration with the american association for thoracic surgery and society of thoracic surgeons. J Am Coll Cardiol. 2008;51:e1–62.

    Article  PubMed  Google Scholar 

  35. Russo AM, Stainback RF, Bailey SR, Epstein AE, Heidenreich PA, Jessup M, Kapa S, Kremers MS, Lindsay BD, Stevenson LW. ACCF/HRS/AHA/ASE/HFSA/SCAI/SCCT/SCMR 2013 appropriate use criteria for implantable cardioverter-defibrillators and cardiac resynchronization therapy: A report of the american college of cardiology foundation appropriate use criteria task force, heart rhythm society, american heart association, american society of echocardiography, heart failure society of america, society for cardiovascular angiography and interventions, society of cardiovascular computed tomography, and society for cardiovascular magnetic resonance. J Am Coll Cardiol. 2013;61:1318–68.

    Article  PubMed  Google Scholar 

  36. Gimeno JR, Tomé-Esteban M, Lofiego C, Hurtado J, Pantazis A, Mist B, Lambiase P, McKenna WJ, Elliott PM. Exercise-induced ventricular arrhythmias and risk of sudden cardiac death in patients with hypertrophic cardiomyopathy. Eur Heart J. 2009;30(21):2599–605.

    Article  PubMed  Google Scholar 

  37. Spirito P, Autore C, Rapezzi C, Bernabo P, Badagliacca R, Maron MS, Bongioanni S, Coccolo F, Estes NA, Barilla CS, Biagini E, Quarta G, Conte MR, Bruzzi P, Maron BJ. Syncope and risk of sudden death in hypertrophic cardiomyopathy. Circulation. 2009;119:1703–10.

    PubMed  Google Scholar 

  38. Spirito P, Bellone P, Harris KM, Bernabo P, Bruzzi P, Maron BJ. Magnitude of left ventricular hypertrophy and risk of sudden death in hypertrophic cardiomyopathy. N Engl J Med. 2000;342:1778–85.

    Article  CAS  PubMed  Google Scholar 

  39. Monserrat L, Elliott PM, Gimeno JR, Sharma S, Penas-Lado M, McKenna WJ. Non-sustained ventricular tachycardia in hypertrophic cardiomyopathy: an independent marker of sudden death risk in young patients. J Am Coll Cardiol. 2003;42:873–9.

    Article  PubMed  Google Scholar 

  40. Maron BJ, Savage DD, Wolfson JK, Epstein SE. Prognostic significance of 24 hour ambulatory electrocardiographic monitoring in patients with hypertrophic cardiomyopathy: a prospective study. Am J Cardiol. 1981;48:252–7.

    Article  CAS  PubMed  Google Scholar 

  41. Maki S, Ikeda H, Muro A, Yoshida N, Shibata A, Koga Y, Imaizumi T. Predictors of sudden cardiac death in hypertrophic cardiomyopathy. Am J Cardiol. 1998;82:774–8.

    Article  CAS  PubMed  Google Scholar 

  42. Kofflard MJ, Ten Cate FJ, van der Lee C, van Domburg RT. Hypertrophic cardiomyopathy in a large community-based population: clinical outcome and identification of risk factors for sudden cardiac death and clinical deterioration. J Am Coll Cardiol. 2003;41:987–93.

    Article  PubMed  Google Scholar 

  43. Olivotto I, Gistri R, Petrone P, Pedemonte E, Vargiu D, Cecchi F. Maximum left ventricular thickness and risk of sudden death in patients with hypertrophic cardiomyopathy. J Am Coll Cardiol. 2003;41:315–21.

    Article  PubMed  Google Scholar 

  44. Maron BJ, Ommen SR, Semsarian C, Spirito P, Olivotto I, Maron MS. Hypertrophic cardiomyopathy: present and future, with translation into contemporary cardiovascular medicine. J Am Coll Cardiol. 2014;64(1):83–99.

    Article  PubMed  Google Scholar 

  45. Adabag AS, Casey SA, Kuskowski MA, Zenovich AG, Maron BJ. Spectrum and prognostic significance of arrhythmias on ambulatory holter electrocardiogram in hypertrophic cardiomyopathy. J Am Coll Cardiol. 2005;45:697–704.

    Article  PubMed  Google Scholar 

  46. Cecchi F, Olivotto I, Montereggi A, Squillatini G, Dolara A, Maron BJ. Prognostic value of non-sustained ventricular tachycardia and the potential role of amiodarone treatment in hypertrophic cardiomyopathy: assessment in an unselected non-referral based patient population. Heart. 1998;79:331–6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  47. Spirito P, Rapezzi C, Autore C, Bruzzi P, Bellone P, Ortolani P, Fragola PV, Chiarella F, Zoni-Berisso M, Branzi A, et al. Prognosis of asymptomatic patients with hypertrophic cardiomyopathy and nonsustained ventricular tachycardia. Circulation. 1994;90:2743–7.

    Article  CAS  PubMed  Google Scholar 

  48. Elliott PM, Anastasakis A, Borger MA, Borggrefe M, Cecchi F, Charron P, Hagege AA, Lafont A, Limongelli G, Marholdt H, McKenna WJ, Mogensen J, Nihoyannopoulos P, Nistri S, Pieper PG, Pieske B, Rapezzi C, Rutten FH, Tillmanns C, Watkins H. ESC guidelines on diagnosis and management of hypertrophic cardiomyopathy. The task force for the diagnosis and management of hypertrophic cardiomyopathy of the European Society of Cardiology (ESC). Eur Heart J. 2014;35:2733–79.

    Google Scholar 

  49. Woo A, Monakier D, Harris L, Hill A, Shah P, Wigle ED, Rakowski H, Rozenblyum E, Cameron DA. Determinants of implantable defibrillator discharges in high-risk patients with hypertrophic cardiomyopathy. Heart. 2007;93:1044–5.

    Article  PubMed  PubMed Central  Google Scholar 

  50. Lin G, Nishimura RA, Gersh BJ, Phil D, Ommen SR, Ackerman MJ, Brady PA. Device complications and inappropriate implantable cardioverter defibrillator shocks in patients with hypertrophic cardiomyopathy. Heart. 2009;95:709–14.

    Article  CAS  PubMed  Google Scholar 

  51. Liebregts M, Faber L, Jensen MK, Vriesendorp PA, Hansen PR, Seggewiss H, Horstkotte D, Adlova R, Michels M, ten Berg HBJ, Veselka J. Validation of the HCM Risk-SCD model in patients with hypertrophic cardiomyopathy following alcohol septal ablation. Europace. 2017;eux251. https://doi.org/10.1093/europace/eux251

    Article  Google Scholar 

  52. Moolman JC, Corfield VA, Posen B, Ngumbela K, Seidman C, Brink PA, Watkins H. Sudden death due to troponin T mutations. J Am Coll Cardiol. 1997;29:549–55.

    Article  CAS  PubMed  Google Scholar 

  53. Watkins H, McKenna WJ, Thierfelder L, Suk J, Anan R, O’Donoghue A, Spirito P, Matsumori A, Moravec CS, Deidman JG, Seidman CE. Mutations in the genes for cardiac troponin T and alpha-tropomyosin in hypertrophic cardiomyopathy. N Engl J Med. 1995;332:1058–64.

    Article  CAS  Google Scholar 

  54. Varnava AM, Elliott PM, Baboonian C, Davison F, Davies MJ, McKenna WJ. Hypertrophic cardiomyopathy: histopathological features of sudden death in cardiac troponin T disease. Circulation. 2001;104(12):1380–4.

    Article  CAS  PubMed  Google Scholar 

  55. Fiset C, Giles WR. Cardiac troponin T mutations promote life-threatening arrhythmias. J Clin Invest. 2008;118:3845–7.

    CAS  PubMed  PubMed Central  Google Scholar 

  56. Coppini R, Ho CY, Ashley E, Day S, Ferrantini C, Girolami F, Tomberli B, Bardi S, Torricelli F, Cecchi F, Mugelli A, Poggesi C, Tardiff J, Olivotto I. Clinical phenotype and outcome of hypertrophic cardiomyopathy associated with thin-filament gene mutations. 2014;64(24):2589–600.

    Google Scholar 

  57. Pasquale F, Syrris P, Kaski JP, Mogensen J, McKenna WJ, Elliott P. Long-term outcomes in hypertrophic cardiomyopathy caused by mutations in the cardiac troponin T gene. Circ Cardiovasc Genet. 2012;5(1):10–7.

    Article  CAS  PubMed  Google Scholar 

  58. Van Driest SL, Haeger MA, Ommen SR, Will ML, Gersh BJ, Tajik AJ, Ackerman MJ. Comprehensive analysis of the beta-myosin heavy chain gene in 389 unrelated patients with hypertrophic cardiomyopathy. J Am Coll Cardiol. 2004;44(3):602–10.

    Article  PubMed  CAS  Google Scholar 

  59. Van Driest SL, Ackerman MJ, Ommen SR, Shakur R, Will ML, Nishimura RA, Tajik AJ, Gersh BJ. Prevalence and severity of “benign” mutations in the beta-myosin heavy chain, cardiac troponin T, and alpha-tropomyosin genes in hypertrophic cardiomyopathy. Circulation. 2002;106(24):3085–90.

    Article  PubMed  CAS  Google Scholar 

  60. Maron BJ, Maron MS, Semsarian C. Double or compound sarcomere mutations in hypertrophic cardiomyopathy: a potential link to sudden death in the absence of conventional risk factors. Heart Rhythm. 2012;9:57–63.

    Article  PubMed  Google Scholar 

  61. Alcalai R, Seidman JG, Seidman CE. Genetic basis of hypertrophic cardiomyopathy: from bench to the clinics. J Cardiovasc Electrophysiol. 2008;19(1):104–10.

    PubMed  Google Scholar 

  62. Ingles J, Doolan A, Chiu C, Seidman J, Seidman C, Semsarian C. Compound and double mutations in patients with hypertrophic cardiomyopathy: implications for genetic testing and counselling. J Med Genet. 2005;42:e59.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  63. Rubinshtein R, Glockner JF, Ommen SR, Araoz PA, Ackerman MJ, Sorajja P, Bos JM, Tajik AJ, Valeti US, Nishimura RA, Gersh BJ. Characteristics and clinical significance of late gadolinium enhancement by contrast-enhanced magnetic resonance imaging in patients with hypertrophic cardiomyopathy. Circ Heart Fail. 2010;3:51–8.

    Article  PubMed  Google Scholar 

  64. Moon JC, McKenna WJ, McCrohon JA, Elliott PM, Smith GC, Pennell DJ. Toward clinical risk assessment in hypertrophic cardiomyopathy with gadolinium cardiovascular magnetic resonance. J Am Coll Cardiol. 2003;41:1561–7.

    Article  PubMed  Google Scholar 

  65. Moon JC, Reed E, Sheppard MN, Elkington AG, Ho SY, Burke M, Petrou M, Pennell DJ. The histologic basis of late gadolinium enhancement cardiovascular magnetic resonance in hypertrophic cardiomyopathy. J Am Coll Cardiol. 2004;43:2260–4.

    Article  PubMed  Google Scholar 

  66. Adabag AS, Maron BJ, Appelbaum E, Harrigan CJ, Buros JL, Gibson CM, Lesser JR, Hanna CA, Udelson JE, Manning WJ, Maron MS. Occurrence and frequency of arrhythmias in hypertrophic cardiomyopathy in relation to delayed enhancement on cardiovascular magnetic resonance. J Am Coll Cardiol. 2008;51:1369–74.

    Article  PubMed  Google Scholar 

  67. Choudhury L, Mahrholdt H, Wagner A, Choi KM, Elliott MD, Klocke FJ, Bonow RO, Judd RM, Kim RJ. Myocardial scarring in asymptomatic or mildly symptomatic patients with hypertrophic cardiomyopathy. J Am Coll Cardiol. 2002;40:2156–64.

    Article  PubMed  Google Scholar 

  68. Weng Z, Yao J, Chan RH, He J, Yang X, Zhou Y, He Y. Prognostic value of LGE-CMR in HCM: a meta-analysis. JACC Cardiovasc Imaging. 2016;9(12):1392–402.

    Article  PubMed  Google Scholar 

  69. Maron MS, Appelbaum E, Harrigan CJ, Buros J, Gibson CM, Hanna C, Lesser JR, Udelson JE, Manning WJ, Maron BJ. Clinical profile and significance of delayed enhancement in hypertrophic cardiomyopathy. Circ Heart Fail. 2008;1:184–91.

    Article  PubMed  Google Scholar 

  70. Maron MS, Finley JJ, Bos JM, Hauser TH, Manning WJ, Haas TS, Lesser JR, Udelson JE, Ackerman MJ, Maron BJ. Prevalence, clinical significance, and natural history of left ventricular apical aneurysms in hypertrophic cardiomyopathy. Circulation. 2008;118:1541–9.

    Article  PubMed  Google Scholar 

  71. Paul M, Schafers M, Grude M, Reinke F, Juergens KU, Fischbach R, Schober O, Breithardt G, Wichter T. Idiopathic left ventricular aneurysm and sudden cardiac death in young adults. Europace. 2006;8:607–12.

    Article  PubMed  Google Scholar 

  72. Rowin EJ, Maron MS. The role of cardiac MRI in the diagnosis and risk stratification of hypertrophic cardiomyopathy. Arrhythm Electrophysiol Rev. 2016;5(3):197–202.

    Article  PubMed  PubMed Central  Google Scholar 

  73. Harris KM, Spirito P, Maron MS, Zenovich AG, Formisano F, Lesser JR, Mackey-Bojack S, Manning WJ, Udelson JE, Maron BJ. Prevalence, clinical profile, and significance of left ventricular remodeling in the end-stage phase of hypertrophic cardiomyopathy. Circulation. 2006;114:216–25.

    Article  PubMed  Google Scholar 

  74. Maron BJ, Spirito P, Shen WK, Haas TS, Formisano F, Link MS, Epstein AE, Almquist AK, Daubert JP, Lawrenz T, Boriani G, Estes NA 3rd, Favale S, Piccininno M, Winters SL, Santini M, Betocchi S, Arribas F, Sherrid MV, Buja G, Semsarian C, Bruzzi P. Implantable cardioverter-defibrillators and prevention of sudden cardiac death in hypertrophic cardiomyopathy. JAMA. 2007;298:405–12.

    CAS  PubMed  Google Scholar 

  75. Maron BJ, Spirito P, Ackerman MJ, Casey SA, Semsarian C, Estes NA 3rd, Shannon KM, Ashley EA, Day SM, Pacileo G, Formisano F, Devoto E, Anastasakis A, Bos JM, Woo A, Autore C, Pass RH, Boriani G, Garberich RF, Almquist AK, Russell MW, Boni L, Berger S, Maron MS, Link MS. Prevention of sudden cardiac death with implantable cardioverter-defibrillators in children and adolescents with hypertrophic cardiomyopathy. J Am Coll Cardiol. 2013;61:1527–35.

    Article  PubMed  Google Scholar 

  76. Maron BJ, Estes NA 3rd, Maron MS, Almquist AK, Link MS, Udelson JE. Primary prevention of sudden death as a novel treatment strategy in hypertrophic cardiomyopathy. Circulation. 2003;107:2872–5.

    Article  PubMed  Google Scholar 

  77. Hauser RG, Maron BJ. Lessons from the failure and recall of an implantable cardioverter-defibrillator. Circulation. 2005;112:2040–2.

    Article  PubMed  Google Scholar 

  78. Maron BJ. Risk stratification and role of implantable defibrillators for prevention of sudden death in patients with hypertrophic cardiomyopathy. Circ J. 2010;74:2271–82.

    Article  PubMed  Google Scholar 

  79. Trivedi A, Knight BP. ICD therapy for primary prevention in hypertrophic cardiomyopathy. Arrhythm Electrophysiol Rev. 2016;5(3):188–96.

    Article  PubMed  PubMed Central  Google Scholar 

  80. Weinstock J, Bader YH, Maron MS, Rowin EJ, Link MS. Subcutaneous implantable cardioverter defibrillator in patients with hypertrophic cardiomyopathy: an initial experience. J Am Heart Assoc. 2016;5(2). https://doi.org/10.1161/JAHA.115.002488.

  81. Lambiase PD, Gold MR, Hood M, Boersma L, Theuns DA, Burke MC, Weiss R, Russo AM, Kaab S, Knight BP. Evaluation of subcutaneous ICD early performance in hypertrophic cardiomyopathy from the pooled EFFORTLESS and IDE cohorts. Heart Rhythm. 2016;13(5):1066–74.

    Article  PubMed  Google Scholar 

  82. McKenna WJ, Oakley CM, Krikler DM, Goodwin JF. Improved survival with amiodarone in patients with hypertrophic cardiomyopathy and ventricular tachycardia. Br Heart J. 1985;53:412–6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  83. McKenna WJ, Harris L, Rowland E, Kleinebenne A, Krikler DM, Oakley CM, Goodwin JF. Amiodarone for long-term management of patients with hypertrophic cardiomyopathy. Am J Cardiol. 1984;54:802–10.

    Article  CAS  PubMed  Google Scholar 

  84. Melacini P, Maron BJ, Bobbo F, Basso C, Tokajuk B, Zucchetto M, Thiene G, Iliceto S. Evidence that pharmacological strategies lack efficacy for the prevention of sudden death in hypertrophic cardiomyopathy. Heart. 2007;93:708–10.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  85. Lipshultz SE, Orav EJ, Wilkinson JD, Towbin JA, Messere JE, Lowe AE, Sleeper LA, Cox GF, Hsu DT, Canter CE, Hunter JA, Colan SD, for the Pediatric Cardiomyopathy Registry Study Group. Risk stratification at diagnosis for children with hypertrophic cardiomyopathy: an analysis of data from the pediatric cardiomyopathy registry. Lancet. 2013;382(9908):1889–97.

    Google Scholar 

  86. Colan SD. Hypertrophic cardiomyopathy in childhood. Heart Fail Clin. 2010;6(4):433–44.

    Article  PubMed  PubMed Central  Google Scholar 

  87. Decker JA, Rossano JW, Smith EO, Cannon B, Clunie SK, Gates C, Jefferies JL, Kim JJ, Price JF, Dreyer WJ, Towbin JA, Denfield SW. Risk factors and mode of death in isolated hypertrophic cardiomyopathy in children. J Am Coll Cardiol. 2009;54(3):250–4.

    Article  PubMed  Google Scholar 

  88. Maskatia SA, Decker JA, Spinner JA, Kim JJ, Price JF, Jefferies JL, Dreyer WJ, Smith EO, Rossano JW, Denfield SW. Restrictive physiology is associated with poor outcomes in children with hypertrophic cardiomyopathy. Pediatr Cardiol. 2012;33(1):141–9.

    Article  PubMed  Google Scholar 

  89. Moak JP, Leifer ES, Tripodi D, Mohiddin SA, Fananapazir L. Long-term follow-up of children and adolescents diagnosed with hypertrophic cardiomyopathy: risk factors for adverse arrhythmic events. Pediatr Cardiol. 2011;32(8):1096–105.

    Article  PubMed  Google Scholar 

  90. Norrish G, Cantarutti N, Pissaridou E, Ridout DA, Limongelli G, Elliott PM, Kaski JP. Risk factors for sudden cardiac death in childhood hypertrophic cardiomyopathy: a systematic review and meta-analysis. Eur J Prev Cardiol. 2017;24(11):1220–30.

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Institute of Cardiovascular Science, University College London, St Bartholomew’s Hospital, London, UK

    Perry Elliott

  2. Tufts Medical Center, Cardiac Arrhythmia Center, Boston, MA, USA

    Katy E. Bockstall

  3. UT Southwestern Medical Center, Dallas, TX, USA

    Matthew A. Cain & Mark S. Link

  4. Institute of Cardiovascular Science, University College London, ICVD Unit, Barts Heart Centre, St Bartholomew’s Hospital, London, UK

    Mohammed Majid Akhtar

Authors
  1. Perry Elliott
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  2. Katy E. Bockstall
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  3. Matthew A. Cain
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  4. Mohammed Majid Akhtar
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  5. Mark S. Link
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Correspondence to Mark S. Link .

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Editors and Affiliations

  1. Westchester Medical Center, Valhalla, NY, USA

    Srihari S. Naidu

Questions

Questions

  1. 1.

    A 37-year-old female with HCM presents with several episodes of syncope of the last year. They only occur in the shower and only with premenstrual. She has premonitory symptoms of warmth. She has never had exertional syncope but does have palpitations. A Holter did not show NSVT. Her echo has a 16 mm septum, left atrial size of 4.6 cm, and an intracavitary gradient of 144 mmHg. No FMH of SCD or hypotensive response to exercise. Should she get an ICD?

    1. A.

      Yes

    2. B.

      No

    3. C.

      Maybe

    Answer: B. By US criteria she has no high-risk features. Her syncope is most consistent with neurally mediated syncope, and thus she should generally not be treated with an ICD. By European (http://www.doc2do.com/hcm/webHCM.html) criteria, she has a 3.81% chance of SCD in 5 years, an intermediate risk. Thus, by ESC criteria, she is also not a candidate for ICD.

  2. 2.

    A 32-year-old male, diagnosed with HCM 4 years ago. Holter with NSVT of 6 beats at 150 bpm. No FMH of SCD or personal syncope or hypotensive response to exercise. Echo with LA dimension of 4.4 cm, maximal septal thickness of 23 mm, and outflow tract gradient of 77. Should he get an ICD?

    1. A.

      Yes

    2. B.

      No

    3. C.

      Maybe

    Answer: A. US-risk factor of NSVT which is a minor risk factor and thus other risk modifiers such as MRI scar burden, apical aneurysm, double-hit genetic abnormalities, or LVOT gradient need to be weighed. He does have a significant LVOT gradient which increases his risk of SCD. If MRI scar burden is >15%, then both of these would argue for an ICD. European risk calculator places him at 8.92% 5-year SCD risk, and thus he should get ICD.

  3. 3.

    A 17-year-old standout basketball player with syncope during basketball. Echo with maximal thickness of 21 mm, LA size of 4.2 cm, and LVOT gradient of 5 mmHg. No FMH of SCD or hypotensive response to exercise. Should she get an ICD?

    1. A.

      Yes

    2. B.

      No

    3. C.

      Maybe

    Answer: A. By US guidelines he has a single but strong risk factor and thus should get ICD. By ESC calculator a 5-year SCD risk of 15%, and therefore very high risk and should have ICD.

  4. 4.

    A 15-year-old female whose father died suddenly of HCM when he was 32. Echo showed maximal thickness of 17, LA dimension of 35 mm, and no LVOT gradient. No NSVT or syncope or hypotensive response to exercise. Should she get an ICD?

    1. A.

      Yes

    2. B.

      No

    3. C.

      Maybe

    Answer: C. By US criteria she does have a major risk factor and so ICD should be considered. By ESC calculator her 5-year risk is 3.79% and therefore ICD generally not indicated. In these situations, individualized decision-making taking into consideration potential complications and patient wishes is appropriate after a thorough discussion.

  5. 5.

    A 28-year-old male with HCM diagnosed because of LVH on ECG. His echo shows a maximal thickness of 3.2 cm, LA size of 3.8 cm, and no LVOT gradient. Holter with 7 beats NSVT of 140 bpm. No FMH of SCD. No personal history of syncope or hypotensive response to exercise. Should he get an ICD?

    1. A.

      Yes

    2. B.

      No

    3. C.

      Maybe

    Answer: A. By US criteria he has a major and a minor risk factor so ICD is indicated. By ESC, 5.92% 5-year risk of SCD, thus, ICD is also indicated.

  6. 6.

    The patient in question 5 is a laborer digging graves for a funeral home. A 3-lead screening ECG is shown below. Should he get a SC ICD?

    1. A.

      Yes

    2. B.

      No

    3. C.

      Maybe

    figure a
    figure b

    Answer: C. He does meet criteria for S-ICD based on the figures, which show that T wave oversensing should not occur. While there is not as much data about S-ICDs compared to transvenous ICDs, the S-ICD is an attractive alternative for him because of his job. Nevertheless, several drawbacks to S-ICD in the HCM population exist including inability to ATP, no backup pacing, and inability to identify atrial arrhythmias, and thus a careful discussion about the risks and benefits and the lack of long-term data should be performed prior to implantation.

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Elliott, P., Bockstall, K.E., Cain, M.A., Akhtar, M.M., Link, M.S. (2019). Sudden Cardiac Death Risk Assessment. In: Naidu, S. (eds) Hypertrophic Cardiomyopathy. Springer, Cham. https://doi.org/10.1007/978-3-319-92423-6_10

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  • DOI: https://doi.org/10.1007/978-3-319-92423-6_10

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  • Online ISBN: 978-3-319-92423-6

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