Circulation, Vol 87, 800-807, Copyright © 1993 by American Heart Association
MJ Silka, J Kron, A Dunnigan and M Dick 2d
BACKGROUND. During the past decade, the implantable cardioverter-
defibrillator (ICD) has emerged as the primary therapeutic option for
survivors of sudden cardiac death (SCD). Investigation of the clinical
efficacy of these devices has primarily assessed outcome in adults with
coronary artery disease. The purpose of this cooperative, international
study was to evaluate the impact of ICDs on the pediatric population of SCD
survivors, based on an analysis of the clinical characteristics and
outcomes of young patients who underwent ICD implantation following an
episode of life-threatening ventricular tachycardia or resuscitation from
SCD. METHODS AND RESULTS. An initial data base, established by contacting
the manufacturers of the various commercially and investigationally
available devices, identified 177 patients who were less than 20 years of
age at the time of initial implantation of an ICD. With this data base as a
reference, detailed responses were subsequently obtained from physicians
involved in the care of 125 (71%) of these patients. The patients ranged in
age from 1.9 to 19.9 years (mean, 14.5 +/- 4 years) and weighted 9.7-117 kg
(mean, 44.6 +/- 14 kg). Of the 125 patients, 76% were survivors of SCD, 10%
had drug refractory ventricular tachycardia, and 10% had syncope with heart
disease and inducible sustained ventricular tachyarrhythmias. The most
common types of associated cardiovascular disease were hypertrophic and
dilated cardiomyopathies (54%), primary electrical diseases (26%), and
congenital heart defects (18%). Ventricular function was abnormal in 46% of
the patients. During a mean follow-up of 31 +/- 23 months, at least one ICD
discharge occurred in 85 of the 125 (68%) patients. Seventy-three patients
(59%) received at least one appropriate ICD discharge, and 25 patients
(20%) had one or more spurious or indeterminate discharges. Duration of
follow-up > 24 months (p = 0.001) and inducibility of a sustained
ventricular arrhythmia (p = 0.05) were correlated with appropriate ICD
discharges. There were nine deaths during the study period: five sudden,
two due to recurrent ventricular arrhythmias, and two related to congestive
heart failure. Abnormal ventricular function (p = 0.002) and prior ICD
discharge (p = 0.01) were univariate correlates of patient mortality; by
multivariate logistic regression, abnormal ventricular function was the
only significant correlate of death (p = 0.005). By actuarial analysis, the
estimated overall post-ICD implant survival rates at 1, 2, and 5 years were
95%, 93%, and 85%, respectively. The corresponding sudden death- free
survival rates were 97%, 95%, and 90%. CONCLUSIONS. Pediatric patients
resuscitated from SCD appear to remain at risk for recurrence of
life-threatening tachyarrhythmias. During a mean follow-up of 31 months,
the ICD provided an effective therapy for such arrhythmias in the majority
of patients in this study. Following ICD implant, impaired ventricular
function was the primary factor correlated with mortality. The patterns of
ICD discharge observed in young patients and, thus, inferred risk of
recurrent life threatening arrhythmias are similar to those of adult
survivors of SCD. Thus, the use of ICDs in pediatric patients, with implant
selection criteria similar to adults, appears valid.
ARTICLES
Sudden cardiac death and the use of implantable cardioverter- defibrillators in pediatric patients. The Pediatric Electrophysiology Society
Division of Cardiology, Oregon Health Sciences University, Portland 97201.
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