[Shortening of PR interval, bradycardia and polymorphic ventricular tachycardia--clinico-electrocardiografical syndrome with high risk of sudden death in children].
OBJECTIVE
To elucidate clinical and electrocardiographical characteristics of children and adolescents with malignant idiopathic polymorphic ventricular tachycardia.


METHODS
Patients aged 6-14 years (n=16) with polymorphic bidirectional tachycardia registered either on standard 12-lead ECG (n=6), or during treadmill exercise test (n=2) and Holter ECG monitoring (n=8). None of the patients had overt heart or coronary artery disease, chronic extracardiac pathology or electrolyte disturbances.


RESULTS
There was no QT, QT(s) prolongations, or ST segment elevations in right precordial leads. Eleven patients (68.8%) had shortening of PR interval -110 ms without signs of Wolf-Parkinson-White syndrome. All patients with shortened PR had history of syncopal states (9.1+/-9.2 per year), 4 patients (36.4%) had family history of sudden death in young age. Among patients without PR shortening 2 (40%) had history of syncopal attacks (1-2 or 0.6+/-0.89 per year). All patients with short PR had bradycardia while in sinus rhythm (55.5+/-9.1 bpm compared with 78.1+/-2.8 bpm in patients without PR shortening).


CONCLUSIONS
Shortened PR and bradycardia were found to be associated with frequent attacks of syncope in this series of children with polymorphic tachycardia. Therefore combination of short PR, bradycardia and polymorphic ventricular tachycardia was suggested to comprise clinico-electrocardiographic syndrome with high risk of malignant ventricular tachyarrhythmia and sudden death.
Makarov
L M
LM
Detskiĭ nauchno-prakticheskiĭ tsentr narusheniĭ ritma serdtsa Minzdrava RF na baze Moskovskogo NII pediatrii i detskoĭ khirurgii Minzdrava RF, 127412 Moskva, ul. Taldomskaia, 2.
Kuryleva
T A
TA
Chuprova
S N
SN
D000293:Adolescent; D001919:Bradycardia / Q000150:complications / Q000175:diagnosis*; D002648:Child; D016757:Death, Sudden, Cardiac / Q000209:etiology; D004562:Electrocardiography; D005260:Female; D006801:Humans; D008297:Male; D017180:Tachycardia, Ventricular / Q000150:complications / Q000175:diagnosis*; D014927:Wolff-Parkinson-White Syndrome / Q000150:complications / Q000175:diagnosis*