Circulation, Vol 86, 730-740, Copyright © 1992 by American Heart Association
L Fananapazir, AC Chang, SE Epstein and D McAreavey
BACKGROUND. Patients with hypertrophic cardiomyopathy (HCM) frequently have
arrhythmias and hemodynamic abnormalities and are prone to sudden death and
syncope. An important need exists for improved risk stratification and
definition of appropriate investigation and therapy. METHODS AND RESULTS.
The relation of 31 clinical, Holter, cardiac catheterization, and
electrophysiological (EP) variables to subsequent cardiac events in 230 HCM
patients was examined by multivariate analysis. Studies were for cardiac
arrest (n = 32), syncope (n = 80), presyncope (n = 52), ventricular
tachycardia (VT) on Holter (n = 36), a strong family history of sudden
death (n = 9), and palpitations (n = 21). Nonsustained VT on Holter was
present in 115 patients (50%). Sustained ventricular arrhythmia was induced
in 82 patients (36%). Seventeen cardiac events (eight sudden deaths, one
cardiac arrest, and eight syncope with defibrillator discharges) occurred
during a follow- up of 28 +/- 19 months. The 1-year and 5-year event-free
rates were 99% and 79%, respectively. Two variables were significant
independent predictors of subsequent events: sustained ventricular
arrhythmia induced at EP study (beta, 3.5; p = 0.002) and a history of
cardiac arrest or syncope (beta, 2.9; p less than 0.05). Only two of 66
patients without symptoms of impaired consciousness had a cardiac event
(3-year event-free rate, 97%). In contrast, nonsustained VT on Holter was
associated with a worse prognosis only in patients with symptoms of
impaired consciousness: 11 of 79 symptomatic patients with VT on Holter
(14%) had events versus only four of 85 symptomatic patients without VT on
Holter (5%) (p = 0.057). Notably, none of 51 patients without symptoms of
impaired consciousness in whom VT was not induced at EP study had a cardiac
event. CONCLUSIONS. In HCM, VT on Holter is of benign prognostic
significance in the absence of symptoms of impaired consciousness and
inducible VT, and sustained VT induced at EP study, especially when
associated with cardiac arrest or syncope, identifies a subgroup at high
risk for subsequent cardiac events.
ARTICLES
Prognostic determinants in hypertrophic cardiomyopathy. Prospective evaluation of a therapeutic strategy based on clinical, Holter, hemodynamic, and electrophysiological findings
Electrophysiology Section, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892.
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