Hypertrophic cardiomyopathy in children, adolescents, and young adults associated with low cardiovascular mortality with contemporary management strategies

Circulation

Volumn 133, Issue 1, 2016, Pages 62-73

  Maron, Barry J ^a   Rowin, Ethan J ^b   Casey, Susan A ^a   Lesser, John R ^a   Garberich, Ross F ^a   McGriff, Deepa M ^a   Maron, Martin S ^b  

^a MINNEAPOLIS HEART INSTITUTE FOUNDATION   (United States)

^b TUFTS MEDICAL CENTER   (United States)

Author keywords

cardiomyopathy, hypertrophic; death, sudden; defibrillators, implantable; heart failure; heart transplantation

Indexed keywords

ADOLESCENT; ADULT; ARTICLE; CARDIOVASCULAR MORTALITY; CHILD; DISEASE ASSOCIATION; FEMALE; FOLLOW UP; HEART ARRHYTHMIA; HEART FAILURE; HEART TRANSPLANTATION; HEART VENTRICLE TACHYCARDIA; HIGH RISK PATIENT; HUMAN; HYPERTROPHIC CARDIOMYOPATHY; IMPLANTABLE CARDIOVERTER DEFIBRILLATOR; LONG TERM CARE; MAJOR CLINICAL STUDY; MALE; OUT OF HOSPITAL CARDIAC ARREST; OUTCOME ASSESSMENT; POSTOPERATIVE COMPLICATION; PRIORITY JOURNAL; SCHOOL CHILD; SUDDEN CARDIAC DEATH; SURVIVAL; YOUNG ADULT; AGE; CARDIOMYOPATHY, HYPERTROPHIC; CLINICAL TRIAL; COHORT ANALYSIS; DISEASE MANAGEMENT; LONGITUDINAL STUDY; MORTALITY; MULTICENTER STUDY; TRENDS;

ADOLESCENT; ADULT; AGE FACTORS; CARDIOMYOPATHY, HYPERTROPHIC; CHILD; COHORT STUDIES; DISEASE MANAGEMENT; FEMALE; FOLLOW-UP STUDIES; HUMANS; LONGITUDINAL STUDIES; MALE; MORTALITY; YOUNG ADULT;

EID: 84953839863     PISSN: 00097322     EISSN: 15244539     Source Type: Journal    
DOI: 10.1161/CIRCULATIONAHA.115.017633     Document Type: Article

References (51)

1. 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 1535. doi: 10.1016/j.jacc.2013.01.037.
2. Berul CI, Van Hare GF, Kertesz NJ, Dubin AM, Cecchin F, Collins KK, Cannon BC, Alexander ME, Triedman JK, Walsh EP, Friedman RA., Results of a multicenter retrospective implantable cardioverter-Defibrillator registry of pediatric and congenital heart disease patients. J Am Coll Cardiol 2008 51 1685 1691. doi: 10.1016/j.jacc.2008.01.033.
3. Kaski JP, Tomé Esteban MT, Lowe M, Sporton S, Rees P, Deanfield JE, McKenna WJ, Elliott PM., Outcomes after implantable cardioverter-Defibrillator treatment in children with hypertrophic cardiomyopathy. Heart 2007 93 372 374. doi: 10.1136/hrt.2006.094730.
4. Korte T, Köditz H, Niehaus M, Paul T, Tebbenjohanns J., High incidence of appropriate and inappropriate ICD therapies in children and adolescents with implantable cardioverter defibrillator. Pacing Clin Electrophysiol 2004 27 924 932. doi: 10.1111/j.1540-8159.2004.00560.x.
5. McKenna WJ, Deanfield JE., Hypertrophic cardiomyopathy: an important cause of sudden death. Arch Dis Child 1984 59 971 975
6. Maron BJ, Henry WL, Clark CE, Redwood DR, Roberts WC, Epstein SE., Asymetric septal hypertrophy in childhood. Circulation 1976 53 9 19
7. Shah PM, Adelman AG, Wigle ED, Gobel FL, Burchell HB, Hardarson T, Curiel R, De La Calzada C, Oakley CM, Goodwin JF., The natural (and unnatural) history of hypertrophic obstructive cardiomyopathy. Circ Res 1974 35 suppl II):179 suppl II:195
8. Maron BJ, Doerer JJ, Haas TS, Tierney DM, Mueller FO., Sudden deaths in young competitive athletes: analysis of 1866 deaths in the United States, 1980-2006. Circulation 2009 119 1085 1092. doi: 10.1161/CIRCULATIONAHA.108.804617.
9. Maron BJ, McKenna WJ, Danielson GK, Kappenberger LJ, Kuhn HJ, Seidman CE, Shah PM, Spencer WH 3rd, Spirito P, Ten Cate FJ, Wigle ED., American College of Cardiology/European Society of Cardiology clinical expert consensus document on hypertrophic cardiomyopathy. J Am Coll Cardiol 2003 42 1687 713. Eur Heart J. 2003;24:1965-1991
10. 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, American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines; 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; Society of Thoracic Surgeons 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. Circulation 2011 124 2761 2796
11. Maron BJ, Maron MS., Hypertrophic cardiomyopathy. Lancet 2013 381 242 255. doi: 10.1016/S0140-6736(12)60397-3.
12. 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 83 99. doi: 10.1016/j.jacc.2014.05.003.
13. Wheeler MT, Ashley EA., Hypertrophic cardiomyopathy: can the horse be put back in the barn? J Am Coll Cardiol 2015 65 570 572. doi: 10.1016/j.jacc.2014.12.004.
14. Dutchen S., Silencing sudden death. Harvard Medical School News. October 3, 2013. http://hms.harvard.edu/news/genetics/silencing-suddendeath-10-3-13. Accessed March 6, 2013
15. Marian AJ., Hypertrophic cardiomyopathy: from genetics to treatment. Eur J Clin Invest 2010 40 360 369
16. Watkins H., Sudden death in hypertrophic cardiomyopathy. N Engl J Med 2000 342 422 424. doi: 10.1056/NEJM200002103420609.
17. Marian AJ., Experimental therapies in hypertrophic cardiomyopathy. J Cardiovasc Transl Res 2009 2 483 492. doi: 10.1007/s12265-009-9132-7.
18. 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 342 365 373. doi: 10.1056/NEJM200002103420601.
19. 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 298 405 412. doi: 10.1001/jama.298.4.405.
20. Schinkel AF, Vriesendorp PA, Sijbrands EJ, Jordaens LJ, ten Cate FJ, Michels M., Outcome and complications after implantable cardioverter defibrillator therapy in hypertrophic cardiomyopathy: systematic review and meta-Analysis. Circ Heart Fail 2012 5 552 559. doi: 10.1161/CIRCHEARTFAILURE.112.969626.
21. Maron BJ, Rowin EJ, Casey SA, Link MS, Lesser JR, Chan RH, Garberich RF, Udelson JE, Maron MS., Hypertrophic Cardiomyopathy in Adulthood Associated With Low Cardiovascular Mortality With Contemporary Management Strategies. J Am Coll Cardiol 2015 65 1915 1928. doi: 10.1016/j.jacc.2015.02.061.
22. Spirito P., The dawn of a better day for patients with hypertrophic cardiomyopathy. J Am Coll Cardiol 2015 65 1929 1930. doi: 10.1016/j.jacc.2015.03.516.
23. Maron BJ, Spirito P., Implantable defibrillators and prevention of sudden death in hypertrophic cardiomyopathy. J Cardiovasc Electrophysiol 2008 19 1118 1126. doi: 10.1111/j.1540-8167.2008.01147.x.
24. Vriesendorp PA, Schinkel AF, Van Cleemput J, Willems R, Jordaens LJ, Theuns DA, van Slegtenhorst MA, de Ravel TJ, ten Cate FJ, Michels M., Implantable cardioverter-Defibrillators in hypertrophic cardiomyopathy: patient outcomes, rate of appropriate and inappropriate interventions, and complications. Am Heart J 2013 166 496 502. doi: 10.1016/j.ahj.2013.06.009.
25. Von Bergen NH, Atkins DL, Dick M 2nd, Bradley DJ, Etheridge SP, Saarel EV, Fischbach PS, Balaji S, Sreeram N, Evans WN, Law IH., Multicenter study of the effectiveness of implantable cardioverter defibrillators in children and young adults with heart disease. Pediatr Cardiol 2011 32 399 405. doi: 10.1007/s00246-010-9866-7.
26. Kamp AN, Von Bergen NH, Henrikson CA, Makhoul M, Saarel EV, Lapage MJ, Russell MW, Strieper M, Yu S, Dick M, Day SM, Bradley DJ., Implanted defibrillators in young hypertrophic cardiomyopathy patients: a multicenter study. Pediatr Cardiol 2013 34 1620 1627. doi: 10.1007/s00246-013-0676-6.
27. Maron BJ, Braunwald E., Evolution of hypertrophic cardiomyopathy to a contemporary treatable disease. Circulation 2012 126 1640 1644. doi: 10.1161/CIRCULATIONAHA.112.123174.
28. Rowin EJ, Maron BJ, Kiernan MS, Casey SA, Feldman DS, Hryniewicz KM, Chan RH, Harris KM, Udelson JE, DeNofrio D, Roberts WC, Maron MS., Advanced heart failure with preserved systolic function in nonobstructive hypertrophic cardiomyopathy: under-recognized subset of candidates for heart transplant. Circ Heart Fail 2014 7 967 975. doi: 10.1161/CIRCHEARTFAILURE.114.001435.
29. 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 114 216 225. doi: 10.1161/CIRCULATIONAHA.105.583500.
30. Maron MS, Kalsmith BM, Udelson JE, Li W, DeNofrio D., Survival after cardiac transplantation in patients with hypertrophic cardiomyopathy. Circ Heart Fail 2010 3 574 579. doi: 10.1161/CIRCHEARTFAILURE.109.922872.
31. Maron BJ, Seidman CE, Ackerman MJ, Towbin JA, Maron MS, Ommen SR, Nishimura RA, Gersh BJ., How should hypertrophic cardiomyopathy be classified?: What's in a name? Dilemmas in nomenclature characterizing hypertrophic cardiomyopathy and left ventricular hypertrophy. Circ Cardiovasc Genet 2009 2 81 85; discussion 86. doi: 10.1161/CIRCGENETICS.108.788703.
32. Maron BJ, Roberts WC, Arad M, Haas TS, Spirito P, Wright GB, Almquist AK, Baffa JM, Saul JP, Ho CY, Seidman J, Seidman CE., Clinical outcome and phenotypic expression in LAMP2 cardiomyopathy. JAMA 2009 301 1253 1259. doi: 10.1001/jama.2009.371.
33. Maron BJ, Maron MS, Semsarian C., Genetics of hypertrophic cardiomyopathy after 20 years: clinical perspectives. J Am Coll Cardiol 2012 60 705 715. doi: 10.1016/j.jacc.2012.02.068.
34. 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. doi: 10.1056/NEJMoa021332.
35. Chan RH, Maron BJ, Olivotto I, Pencina MJ, Assenza GE, Haas T, Lesser JR, Gruner C, Crean AM, Rakowski H, Udelson JE, Rowin E, Lombardi M, Cecchi F, Tomberli B, Spirito P, Formisano F, Biagini E, Rapezzi C, De Cecco CN, Autore C, Cook EF, Hong SN, Gibson CM, Manning WJ, Appelbaum E, Maron MS., Prognostic value of quantitative contrast-Enhanced cardiovascular magnetic resonance for the evaluation of sudden death risk in patients with hypertrophic cardiomyopathy. Circulation 2014 130 484 495. doi: 10.1161/CIRCULATIONAHA.113.007094.
36. Maron BJ., Contemporary insights and strategies for risk stratification and prevention of sudden death in hypertrophic cardiomyopathy. Circulation 2010 121 445 456. doi: 10.1161/CIRCULATIONAHA.109.878579.
37. 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 1785. doi: 10.1056/NEJM200006153422403.
38. Spirito P, Autore C, Rapezzi C, Bernabò P, Badagliacca R, Maron MS, Bongioanni S, Coccolo F, Estes NA, Barillà CS, Biagini E, Quarta G, Conte MR, Bruzzi P, Maron BJ., Syncope and risk of sudden death in hypertrophic cardiomyopathy. Circulation 2009 119 1703 1710. doi: 10.1161/CIRCULATIONAHA.108.798314.
39. Bos JM, Maron BJ, Ackerman MJ, Haas TS, Sorajja P, Nishimura RA, Gersh BJ, Ommen SR., Role of family history of sudden death in risk stratification and prevention of sudden death with implantable defibrillators in hypertrophic cardiomyopathy. Am J Cardiol 2010 106 1481 1486. doi: 10.1016/j.amjcard.2010.06.077.
40. Maron BJ, Casey SA, Chan RH, Garberich RF, Rowin EJ, Maron MS., Independent Assessment of the European Society of Cardiology Sudden Death Risk Model for Hypertrophic Cardiomyopathy. Am J Cardiol 2015 116 757 764. doi: 10.1016/j.amjcard.2015.05.047.
41. Elliott PM, Anastasakis A, Borger MA, Borggrefe M, Cecchi F, Charron P, Hagege AA, Lafont A, Limongelli G, Mahrholdt H, McKenna WJ, Mogensen J, Nihoyannopulos P, Nistri S, Pieper PG, Pieske B, Rapezzi C, Rutten FH, Tillmanns C, Watkins H., 2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy: the Task Force for the Diagnosis and Management of Mypertrophic Cardiomyopathy of the European Society of Cardiology (ESC). Eur Heart J 2014 35 2733 2779
42. Therneau TM, Grambsch PM., Modeling Survival Data: Extending the Cox Model 2000 New York, NY Springer 16 17
43. Hauser RG, Maron BJ., Lessons from the failure and recall of an implantable cardioverter-Defibrillator. Circulation 2005 112 2040 2042. doi: 10.1161/CIRCULATIONAHA.105.580381.
44. Maron BJ, Spirito P., Impact of patient selection biases on the perception of hypertrophic cardiomyopathy and its natural history. Am J Cardiol 1993 72 970 972
45. Maron BJ, Dearani JA, Ommen SR, Maron MS, Schaff HV, Nishimura RA, Ralph-Edwards A, Rakowski H, Sherrid MV, Swistel DG, Balaram S, Rastegar H, Rowin EJ, Smedira NG, Lytle BW, Desai MY, Lever HM., Low operative mortality achieved with surgical septal myectomy: role of dedicated hypertrophic cardiomyopathy centers in the management of dynamic subaortic obstruction. J Am Coll Cardiol 2015 66 1307 1308. doi: 10.1016/j.jacc.2015.06.1333.
46. Ommen SR, Maron BJ, Olivotto I, Maron MS, Cecchi F, Betocchi S, Gersh BJ, Ackerman MJ, McCully RB, Dearani JA, Schaff HV, Danielson GK, Tajik AJ, Nishimura RA., Long-Term effects of surgical septal myectomy on survival in patients with obstructive hypertrophic cardiomyopathy. J Am Coll Cardiol 2005 46 470 476. doi: 10.1016/j.jacc.2005.02.090.
47. Maron BJ, Rowin EJ, Casey SA, Haas TS, Chan RH, Udelson JE, Garberich RF, Lesser JR, Appelbaum E, Manning WJ, Maron MS., Risk stratification and outcome of patients with hypertrophic cardiomyopathy ≥60 years of age. Circulation 2013 127 585 593. doi: 10.1161/CIRCULATIONAHA.112.136085.
48. Bardy GH, Lee KL, Mark DB, Poole JE, Packer DL, Boineau R, Domanski M, Troutman C, Anderson J, Johnson G, McNulty SE, Clapp-Channing N, Davidson-Ray LD, Fraulo ES, Fishbein DP, Luceri RM, Ip JH, Sudden Cardiac Death in Heart Failure Trial (SCD-HeFT) Investigators Amiodarone or an implantable cardioverter-Defibrillator for congestive heart failure. N Engl J Med 2005 352 225 237. doi: 10.1056/NEJMoa043399.
49. Moss AJ, Hall WJ, Cannom DS, Daubert JP, Higgins SL, Klein H, Levine JH, Saksena S, Waldo AL, Wilber D, Brown MW, Heo M., Improved survival with an implanted defibrillator in patients with coronary disease at high risk for ventricular arrhythmia. Multicenter Automatic Defibrillator Implantation Trial Investigators. N Engl J Med 1996 335 1933 1940. doi: 10.1056/NEJM199612263352601.
50. Kadish A, Dyer A, Daubert JP, Quigg R, Estes NA, Anderson KP, Calkins H, Hoch D, Goldberger J, Shalaby A, Sanders WE, Schaechter A, Levine JH, Defibrillators in Non-Ischemic Cardiomyopathy Treatment Evaluation (DEFINITE) Investigators Prophylactic defibrillator implantation in patients with nonischemic dilated cardiomyopathy. N Engl J Med 2004 350 2151 2158. doi: 10.1056/NEJMoa033088.
51. 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., The long-Term survival and the risks and benefits of implantable cardioverter defibrillators in patients with hypertrophic cardiomyopathy. Heart 2012 98 116 125. doi: 10.1136/hrt.2010.217182.