Arrhythmia and prognosis in infants, children and adolescents with hypertrophic cardiomyopathy

Division of Cardiovascular Disease, Hammersmith Hospital, London, England.

In adults with hypertrophic cardiomyopathy, the annual mortality rate from sudden death is 2 to 3%, and the finding of nonsustained ventricular tachycardia during electrocardiographic (ECG) monitoring provides a marker of the patient who is at increased risk. In the young, the annual mortality rate from sudden death is even higher, approximately 6%, but the prognostic significance of arrhythmia is unknown. To determine the prevalence of arrhythmia and its relation to prognosis, 2 days of ECG monitoring was performed in 6 infants, 14 children and 33 adolescents with hypertrophic cardiomyopathy receiving no cardioactive medications. An additional 1 to 9 days (median 2) of monitoring was performed in 29 patients. All patients had sinus rhythm; 4 adolescents had episodes of paroxysmal supraventricular tachycardia, a child with the Wolff-Parkinson-White syndrome had symptomatic reentrant atrioventricular tachycardia and 5 adolescents had asymptomatic nonsustained ventricular tachycardia. During follow-up of 1 week to 7 years (median 3 years), five patients died suddenly and two had successful resuscitation from out-of-hospital ventricular fibrillation; none of these seven patients had ventricular arrhythmias during 2 to 7 days (median 3) of ECG monitoring. The two patients with ventricular fibrillation, the five with ventricular tachycardia, the one with Wolff-Parkinson-White syndrome and the seven with recurrent syncope or adverse family history, or both, received low dose amiodarone. None of these "high risk" patients died during 6 months to 6 years (median 3 years) of follow-up.(ABSTRACT TRUNCATED AT 250 WORDS)

This article has been cited by other articles:

				K Prasad, L Williams, R Campbell, P M Elliott, W J McKenna, and M Frenneaux Episodic syncope in hypertrophic cardiomyopathy: evidence for inappropriate vasodilation Heart, October 1, 2008; 94(10): 1312 - 1317. [Abstract] [Full Text] [PDF]

				L. Williams and M. Frenneaux Syncope in hypertrophic cardiomyopathy: mechanisms and consequences for treatment Europace, September 1, 2007; 9(9): 817 - 822. [Abstract] [Full Text] [PDF]

				G Limongelli, T Miele, G Pacileo, G Di Salvo, P Calabro', R Ancona, S Gala, A Rea, M Verrengia, and R Calabro' Heart rate variability is a weak predictor of sudden death in children and young patients with hypertrophic cardiomyopathy Heart, January 1, 2007; 93(1): 117 - 118. [Full Text] [PDF]

				Developed in Collaboration With the European Heart, D. P. Zipes, A. J. Camm, M. Borggrefe, A. E. Buxton, B. Chaitman, M. Fromer, G. Gregoratos, G. Klein, A. J. Moss, et al. 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) J. Am. Coll. Cardiol., September 5, 2006; 48(5): e247 - e346. [Full Text] [PDF]

				Writing Committee Members, D. P. Zipes, A. J. Camm, M. Borggrefe, A. E. Buxton, B. Chaitman, M. Fromer, G. Gregoratos, G. Klein, A. J. Moss, et al. 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 Europace, September 1, 2006; 8(9): 746 - 837. [Full Text] [PDF]

				A. W. Nugent, P. E.F. Daubeney, P. Chondros, J. B. Carlin, S. D. Colan, M. Cheung, A. M. Davis, C.W. Chow, R. G. Weintraub, and for the National Australian Childhood Cardiomyopat Clinical Features and Outcomes of Childhood Hypertrophic Cardiomyopathy: Results From a National Population-Based Study Circulation, August 30, 2005; 112(9): 1332 - 1338. [Abstract] [Full Text] [PDF]

				M. P Frenneaux Assessing the risk of sudden cardiac death in a patient with hypertrophic cardiomyopathy Heart, May 1, 2004; 90(5): 570 - 575. [Full Text] [PDF]

				L. Monserrat, P. M. Elliott, J. R. Gimeno, S. Sharma, M. Penas-Lado, and W. J. McKenna Non-sustained ventricular tachycardia in hypertrophic cardiomyopathy: an independent marker of sudden death risk in young patients J. Am. Coll. Cardiol., September 3, 2003; 42(5): 873 - 879. [Abstract] [Full Text] [PDF]

				G Butera, D Bonnet, J Kachaner, D Sidi, and E Villain Heart rate variability in children with hypertrophic cardiomyopathy Heart, February 1, 2003; 89(2): 205 - 206. [Full Text] [PDF]

				Committee Members, G. Gregoratos, J. Abrams, A. E. Epstein, R. A. Freedman, D. L. Hayes, M. A. Hlatky, R. E. Kerber, G. V. Naccarelli, M. H. Schoenfeld, et al. ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices--Summary Article: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (ACC/AHA/NASPE Committee to Update the 1998 Pacemaker Guidelines) J. Am. Coll. Cardiol., November 6, 2002; 40(9): 1703 - 1719. [Full Text] [PDF]

				G. Gregoratos, J. Abrams, A. E. Epstein, R. A. Freedman, D. L. Hayes, M. A. Hlatky, R. E. Kerber, G. V. Naccarelli, M. H. Schoenfeld, M. J. Silka, et al. ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices: Summary Article: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (ACC/AHA/NASPE Committee to Update the 1998 Pacemaker Guidelines) Circulation, October 15, 2002; 106(16): 2145 - 2161. [Full Text] [PDF]

				W. J McKenna and E. R Behr Hypertrophic cardiomyopathy: management, risk stratification, and prevention of sudden death Heart, February 1, 2002; 87(2): 169 - 176. [Full Text] [PDF]

				S.G. Priori, E. Aliot, C. Blomstrom-Lundqvist, L. Bossaert, G. Breithardt, P. Brugada, A.J. Camm, R. Cappato, S.M. Cobbe, C. Di Mario, et al. Task Force on Sudden Cardiac Death of the European Society of Cardiology Eur. Heart J., August 2, 2001; 22(16): 1374 - 1450. [PDF]

				R.N.W. Hauer, E. Aliot, M. Block, A. Capucci, B. Luderitz, M. Santini, and P.E. Vardas Indications for implantable cardioverter defibrillator (ICD) therapy. Study Group on Guidelines on ICDs of the Working Group on Arrhythmias and the Working Group on Cardiac Pacing of the European Society of Cardiology Eur. Heart J., July 1, 2001; 22(13): 1074 - 1081. [PDF]

				R. N. W. Hauer, E. Aliot, M. Block, A. Capucci, B. Luderitz, M. Santini, and P. E. Vardas Indications for implantable cardioverter defibrillator (ICD) therapy: Study Group on Guidelines on ICD of the Working Group on Arrhythmias and the Working Group on Cardiac Pacing of the European Society of Cardiology Europace, January 1, 2001; 3(3): 169 - 176. [PDF]

				R.C. Saumarez and A.A. Grace Paced ventricular electrogram fractionation and sudden death in hypertrophic cardiomyopathy and other non-coronary heart diseases Cardiovasc Res, July 1, 2000; 47(1): 11 - 22. [Full Text] [PDF]

				M. H. Crawford, S. J. Bernstein, P. C. Deedwania, J. P. DiMarco, K. J. Ferrick, A. Garson Jr, L. A. Green, H. L. Greene, M. J. Silka, P. H. Stone, et al. ACC/AHA guidelines for ambulatory electrocardiography: A report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Committee to Revise the Guidelines for Ambulatory Electrocardiography) developed in collaboration with the North American Society for Pacing and Electrophysiology J. Am. Coll. Cardiol., September 1, 1999; 34(3): 912 - 948. [Full Text] [PDF]

				P. M. Elliott, S. Sharma, A. Varnava, J. Poloniecki, E. Rowland, and W. J. McKenna Survival after cardiac arrest or sustained ventricular tachycardia in patients with hypertrophic cardiomyopathy J. Am. Coll. Cardiol., May 1, 1999; 33(6): 1596 - 1601. [Abstract] [Full Text] [PDF]

				A. T. Yetman, B. W. McCrindle, C. MacDonald, R. M. Freedom, and R. Gow Myocardial Bridging in Children with Hypertrophic Cardiomyopathy -- A Risk Factor for Sudden Death N. Engl. J. Med., October 22, 1998; 339(17): 1201 - 1209. [Abstract] [Full Text] [PDF]

				K PRASAD and M P FRENNEAUX Hypertrophic cardiomyopathy: is there a role for amiodarone? Heart, April 1, 1998; 79(4): 317 - 318. [Full Text]

				N. Sadoul, K. Prasad, P. M. Elliott, S. Bannerjee, M. P. Frenneaux, and W. J. McKenna Prospective Prognostic Assessment of Blood Pressure Response During Exercise in Patients With Hypertrophic Cardiomyopathy Circulation, November 4, 1997; 96(9): 2987 - 2991. [Abstract] [Full Text]

				P. Spirito, C. E. Seidman, W. J. McKenna, and B. J. Maron The Management of Hypertrophic Cardiomyopathy N. Engl. J. Med., March 13, 1997; 336(11): 775 - 785. [Full Text] [PDF]

				D. F. Vitale, R. O. Bonow, R. Calabro, M. De Cristofaro, G. Pacileo, P. Caso, G. Gerundo, C. Bordini, M. A. Losi, C. Rengo, et al. Myocardial Ultrasonic Tissue Characterization in Pediatric and Adult Patients With Hypertrophic Cardiomyopathy Circulation, December 1, 1996; 94(11): 2826 - 2830. [Abstract] [Full Text]

				M. P Ryan, J. French, S. Al-Mahdawi, P. Nihoyannopoulos, J. G F Cleland, and C. M Oakley An Ethical Debate: Genetic testing for familial hypertrophic cardiomyopathy in newborn infants: Clinicians' perspective BMJ, April 1, 1995; 310(6983): 856 - 857. [Full Text]