Learn more: PMC Disclaimer | PMC Copyright Notice
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
Abstract
Background—Amiodarone has been reported to reduce the likelihood of sudden death in patients with hypertrophic cardiomyopathy (HCM). However, data regarding the clinical course in HCM have traditionally come from selected referral populations biased toward assessment of high risk patients.
Aims—To evaluate antiarrhythmic treatment for sudden death in an HCM population not subject to tertiary referral bias, closely resembling the true disease state present in the community.
Methods—Cardiovascular mortality was assessed in relation to the occurrence of non-sustained ventricular tachycardia (NSVT) on 24 or 48 hour ambulatory Holter recording, a finding previously regarded as a marker for sudden death, particularly when the arrhythmia was frequent, repetitive or prolonged. 167 consecutive patients were analysed by multiple Holter ECG recordings (mean (SD) 157 (129) hours) and followed for a mean of 10 (5) years. Only patients with multiple repetitive NSVT were treated with amiodarone, and in relatively low doses (220 (44) mg/day).
Results—Nine HCM related deaths occurred: 8 were the consequence of congestive heart failure, but only 1 was sudden and unexpected. Three groups of patients were segregated based on their NSVT profile: group 1 (n = 39), multiple (⩾ 2 runs) and repetitive bursts (on ⩾ 2 Holters) of NSVT, or prolonged runs of ventricular tachycardia, included 4 deaths due to heart failure; group 2 (n = 38), isolated infrequent bursts of NSVT, included 1 sudden death; group 3 (n = 90), without NSVT, included 4 heart failure deaths. Kaplan-Meier survival analysis showed no significant differences in survival between the three groups throughout follow up.
Conclusions—In an unselected patient population with HCM, isolated, non-repetitive bursts of NSVT were not associated with adverse prognosis and so this arrhythmia does not appear to justify chronic antiarrhythmic treatment. Amiodarone, administered in relatively low doses, did not carry an independent and additive risk for cardiac mortality. Amiodarone may have contributed to the absence of sudden cardiac death in patients believed to be at higher risk because of multiple repetitive NSVT.
Keywords: hypertrophic cardiomyopathy; ventricular tachycardia; amiodarone
Full Text
The Full Text of this article is available as a PDF (143K).
Selected References
These references are in PubMed. This may not be the complete list of references from this article.
- Maron BJ, Bonow RO, Cannon RO, 3rd, Leon MB, Epstein SE. Hypertrophic cardiomyopathy. Interrelations of clinical manifestations, pathophysiology, and therapy (1). N Engl J Med. 1987 Mar 26;316(13):780–789. [PubMed] [Google Scholar]
- McKenna W, 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 Mar;47(3):532–538. [PubMed] [Google Scholar]
- Frank S, Braunwald E. Idiopathic hypertrophic subaortic stenosis. Clinical analysis of 126 patients with emphasis on the natural history. Circulation. 1968 May;37(5):759–788. [PubMed] [Google Scholar]
- Wigle ED, Sasson Z, Henderson MA, Ruddy TD, Fulop J, Rakowski H, Williams WG. Hypertrophic cardiomyopathy. The importance of the site and the extent of hypertrophy. A review. Prog Cardiovasc Dis. 1985 Jul-Aug;28(1):1–83. [PubMed] [Google Scholar]
- Maron BJ, Roberts WC, Epstein SE. Sudden death in hypertrophic cardiomyopathy: a profile of 78 patients. Circulation. 1982 Jun;65(7):1388–1394. [PubMed] [Google Scholar]
- Swan DA, Bell B, Oakley CM, Goodwin J. Analysis of symptomatic course and prognosis and treatment of hypertrophic obstructive cardiomyopathy. Br Heart J. 1971 Sep;33(5):671–685. [PMC free article] [PubMed] [Google Scholar]
- Hardarson T, De la Calzada CS, Curiel R, Goodwin JF. Prognosis and mortality of hypertrophic obstructive cardiomyopathy. Lancet. 1973 Dec 29;2(7844):1462–1467. [PubMed] [Google Scholar]
- Loogen F, Kuhn H, Gietzen F, Lösse B, Schulte HD, Bircks W. Clinical course and prognosis of patients with typical and atypical hypertrophic obstructive and with hypertrophic non-obstructive cardiomyopathy. Eur Heart J. 1983 Nov;4 (Suppl F):145–153. [PubMed] [Google Scholar]
- Hecht GM, Panza JA, Maron BJ. Clinical course of middle-aged asymptomatic patients with hypertrophic cardiomyopathy. Am J Cardiol. 1992 Apr 1;69(9):935–940. [PubMed] [Google Scholar]
- McKenna WJ, Deanfield JE. Hypertrophic cardiomyopathy: an important cause of sudden death. Arch Dis Child. 1984 Oct;59(10):971–975. [PMC free article] [PubMed] [Google Scholar]
- Fiddler GI, Tajik AJ, Weidman W, McGoon DC, Ritter DG, Giuliani ER. Idiopathic hypertrophic subaortic stenosis in the young. Am J Cardiol. 1978 Nov;42(5):793–799. [PubMed] [Google Scholar]
- Cecchi F, Maron BJ, Epstein SE. Long-term outcome of patients with hypertrophic cardiomyopathy successfully resuscitated after cardiac arrest. J Am Coll Cardiol. 1989 May;13(6):1283–1288. [PubMed] [Google Scholar]
- McKenna WJ, Camm AJ. Sudden death in hypertrophic cardiomyopathy. Assessment of patients at high risk. Circulation. 1989 Nov;80(5):1489–1492. [PubMed] [Google Scholar]
- Watkins H, Rosenzweig A, Hwang DS, Levi T, McKenna W, Seidman CE, Seidman JG. Characteristics and prognostic implications of myosin missense mutations in familial hypertrophic cardiomyopathy. N Engl J Med. 1992 Apr 23;326(17):1108–1114. [PubMed] [Google Scholar]
- Dambrink JH, Tuininga YS, van Gilst WH, Peels KH, Lie KI, Kingma JH. Association between reduced heart rate variability and left ventricular dilatation in patients with a first anterior myocardial infarction. CATS Investigators. Captopril and Thrombolysis Study. Br Heart J. 1994 Dec;72(6):514–520. [PMC free article] [PubMed] [Google Scholar]
- 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 Aug;48(2):252–257. [PubMed] [Google Scholar]
- Savage DD, Seides SF, Maron BJ, Myers DJ, Epstein SE. Prevalence of arrhythmias during 24-hour electrocardiographic monitoring and exercise testing in patients with obstructive and nonobstructive hypertrophic cardiomyopathy. Circulation. 1979 May;59(5):866–875. [PubMed] [Google Scholar]
- McKenna WJ, England D, Doi YL, Deanfield JE, Oakley C, Goodwin JF. Arrhythmia in hypertrophic cardiomyopathy. I: Influence on prognosis. Br Heart J. 1981 Aug;46(2):168–172. [PMC free article] [PubMed] [Google Scholar]
- McKenna WJ, Oakley CM, Krikler DM, Goodwin JF. Improved survival with amiodarone in patients with hypertrophic cardiomyopathy and ventricular tachycardia. Br Heart J. 1985 Apr;53(4):412–416. [PMC free article] [PubMed] [Google Scholar]
- 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 Dec;90(6):2743–2747. [PubMed] [Google Scholar]
- Cecchi F, Olivotto I, Montereggi A, Santoro G, Dolara A, Maron BJ. Hypertrophic cardiomyopathy in Tuscany: clinical course and outcome in an unselected regional population. J Am Coll Cardiol. 1995 Nov 15;26(6):1529–1536. [PubMed] [Google Scholar]
- Maron BJ, Spirito P. Impact of patient selection biases on the perception of hypertrophic cardiomyopathy and its natural history. Am J Cardiol. 1993 Oct 15;72(12):970–972. [PubMed] [Google Scholar]
- Shapiro LM, Zezulka A. Hypertrophic cardiomyopathy: a common disease with a good prognosis. Five year experience of a district general hospital. Br Heart J. 1983 Dec;50(6):530–533. [PMC free article] [PubMed] [Google Scholar]
- Kofflard MJ, Waldstein DJ, Vos J, ten Cate FJ. Prognosis in hypertrophic cardiomyopathy observed in a large clinic population. Am J Cardiol. 1993 Oct 15;72(12):939–943. [PubMed] [Google Scholar]
- Spirito P, Chiarella F, Carratino L, Berisso MZ, Bellotti P, Vecchio C. Clinical course and prognosis of hypertrophic cardiomyopathy in an outpatient population. N Engl J Med. 1989 Mar 23;320(12):749–755. [PubMed] [Google Scholar]
- Fananapazir L, Leon MB, Bonow RO, Tracy CM, Cannon RO, 3rd, Epstein SE. Sudden death during empiric amiodarone therapy in symptomatic hypertrophic cardiomyopathy. Am J Cardiol. 1991 Jan 15;67(2):169–174. [PubMed] [Google Scholar]
- McKenna WJ, Harris L, Perez G, Krikler DM, Oakley C, Goodwin JF. Arrhythmia in hypertrophic cardiomyopathy. II: Comparison of amiodarone and verapamil in treatment. Br Heart J. 1981 Aug;46(2):173–178. [PMC free article] [PubMed] [Google Scholar]
- 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 Oct 1;54(7):802–810. [PubMed] [Google Scholar]
- Maron BJ, Gottdiener JS, Epstein SE. Patterns and significance of distribution of left ventricular hypertrophy in hypertrophic cardiomyopathy. A wide angle, two dimensional echocardiographic study of 125 patients. Am J Cardiol. 1981 Sep;48(3):418–428. [PubMed] [Google Scholar]
- Shapiro LM, McKenna WJ. Distribution of left ventricular hypertrophy in hypertrophic cardiomyopathy: a two-dimensional echocardiographic study. J Am Coll Cardiol. 1983 Sep;2(3):437–444. [PubMed] [Google Scholar]
- Panza JA, Petrone RK, Fananapazir L, Maron BJ. Utility of continuous wave Doppler echocardiography in the noninvasive assessment of left ventricular outflow tract pressure gradient in patients with hypertrophic cardiomyopathy. J Am Coll Cardiol. 1992 Jan;19(1):91–99. [PubMed] [Google Scholar]
- Spirito P, Maron BJ, Bonow RO, Epstein SE. Occurrence and significance of progressive left ventricular wall thinning and relative cavity dilatation in hypertrophic cardiomyopathy. Am J Cardiol. 1987 Jul 1;60(1):123–129. [PubMed] [Google Scholar]
- Frenneaux MP, Counihan PJ, Porter A, Lipkin DP, McKenna WJ. Effects of amiodarone on erect and supine exercise haemodynamics and exercise capacity in patients with hypertrophic cardiomyopathy. Eur Heart J. 1992 May;13(5):687–696. [PubMed] [Google Scholar]
- Sugrue DD, Dickie S, Myers MJ, Lavender JP, McKenna WJ. Effect of amiodarone on left ventricular ejection and filling in hypertrophic cardiomyopathy as assessed by radionuclide angiography. Am J Cardiol. 1984 Nov 1;54(8):1054–1058. [PubMed] [Google Scholar]
- Debbas NM, du Cailar C, Bexton RS, Demaille JG, Camm AJ, Puech P. The QT interval: a predictor of the plasma and myocardial concentrations of amiodarone. Br Heart J. 1984 Mar;51(3):316–320. [PMC free article] [PubMed] [Google Scholar]
- Spirito P, Seidman CE, McKenna WJ, Maron BJ. The management of hypertrophic cardiomyopathy. N Engl J Med. 1997 Mar 13;336(11):775–785. [PubMed] [Google Scholar]
- Friedman PL, Stevenson WG. Unsustained ventricular tachycardia--to treat or not to treat? N Engl J Med. 1996 Dec 26;335(26):1984–1985. [PubMed] [Google Scholar]
- Singh BN. Amiodarone: historical development and pharmacologic profile. Am Heart J. 1983 Oct;106(4 Pt 2):788–797. [PubMed] [Google Scholar]
Figures and Tables
Flow diagram showing the three groups identified among the 202 HCM patients based on ambulatory Holter ECG findings. AF, atrial fibrillation; SVT supraventricular tachycardia; NSVT, non-sustained ventricular tachycardia.
Kaplan-Meier estimates of cumulative survival during the period of follow up from the time of initial diagnosis of HCM in the three patient groups. There was no significant difference in survival between the three groups independent of whether a pooled or pairwise analysis was performed (cumulative p value was 0.4; for all pairwise comparisons, p was > 0.1).
Kaplan-Meier estimates of cumulative survival during the period following the first detection of NSVT in 77 study patients. Survival did not differ significantly (p = 0.2) between patients with isolated infrequent NSVT receiving no systematic antiarrhythmic treatment from those with multiple repetitive NSVT treated with amiodarone.