Circulation, Vol 87, 1866-1872, Copyright © 1993 by American Heart Association
A Garson Jr, M Dick 2d, A Fournier, PC Gillette, R Hamilton, JD Kugler, GF van Hare 3d, V Vetter and GW Vick 3d
BACKGROUND. The Pediatric Electrophysiology Society studied children with
the long QT syndrome (LQTS) to describe the features of LQTS in patients
less than 21 years old, define potential "low-risk" and "high- risk"
subpopulations, and determine optimal treatment. METHODS AND RESULTS.
Patients less than 21 years old were included if either QTc was more than
0.44; they had unexplained syncope, seizures, or cardiac arrest preceded by
emotion or exercise; or family history of LQTS. We found 287 patients from
26 centers in seven countries. Mean +/- SD age at presentation was 6.8 +/-
5.6; 9% presented with cardiac arrest, 26% with syncope, and 10% with
seizures. Of those with symptoms, 67% had symptoms related to exercise.
Family history was positive for long QT interval in 39% and for sudden
death in 31%. Hearing loss was present in 4.5%. A normal QTc was present in
6%, and QTc of more than 0.60 was in 13%. Atrioventricular block occurred
in 5%, but 13 of 15 patients had second-degree atrioventricular block
(2:1), and only two of 287 had complete atrioventricular block. Ventricular
arrhythmias were found on 16% of initial routine ECGs: 4% uniform premature
ventricular contractions, 5% multiform premature ventricular contractions,
1% monomorphic ventricular tachycardia, and 6% torsade de pointes. Overall,
treatment was effective for symptoms in 76% and for ventricular arrhythmias
in 60%. There was no difference between propranolol and other beta-blockers
in effective treatment. Left stellectomy was performed in nine patients,
and defibrillators were implanted in four; no sudden death occurred in
these 13 patients. In follow-up (duration, 5.0 +/- 4 years; age, 10.9 +/-
6.3 years), 5% had cardiac arrest, 4% had syncope, and 1% had seizures. The
two multivariate predictors of symptoms at follow-up were symptoms at
presentation and propranolol failure. Sudden death occurred in 8%;
multivariate predictors of sudden death were length of QTc at presentation
of more than 0.60 and medication noncompliance. CONCLUSIONS. The appearance
of 2:1 atrioventricular block, multiform premature ventricular
contractions, and torsade de pointes are relatively more common in children
with LQTS than other children and should raise the index of suspicion for
LQTS. Because 9% of patients presented with cardiac arrest and no preceding
symptoms, perhaps prophylactic treatment in asymptomatic children is
indicated. Asymptomatic patients with normal QTc and positive family
history may be a low-risk group. Patients with QTc of more than 0.60 are at
particularly high risk for sudden death, and if treatment is not effective,
consideration should be given to cardiac sympathetic denervation, pacemaker
implantation, and perhaps implantation of a defibrillator.
ARTICLES
The long QT syndrome in children. An international study of 287 patients
Duke University Medical Center, NC 27710.
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