[Long term course of catecholaminergic polymorphic ventricular tachycardia in children. Apropos of 20 cases with an 8 year-follow-up].
BACKGROUND
Primary ventricular arrhythmias are rarely seen in children. Some of them have a poor prognosis; they should be diagnosed because adequate treatment can prevent sudden death.


METHODS
Twenty children (11 male, nine female), aged 3 to 16 years (mean: 7.7 +/- 4), with apparently normal hearts and normal QTc intervals were referred for stress or emotion-induced syncope. Primary ventricular arrhythmia, consisting of isolated polymorphic ventricular extrasystoles followed by salvos eventually degenerating into ventricular fibrillation, was reproducibly induced by physical exertion. The syncopal events and "torsades de pointe" disappeared with beta-blocking therapy. A total of four syncopal events and two sudden deaths occurred during a mean follow-up of 8 years, probably due to discontinuation of treatment.


CONCLUSIONS
Fifty-four-cases of stress-induced severe polymorphous ventricular arrhythmia have been reported in the literature. There were four sudden deaths in 37 patients on beta-blocking therapy, and ten sudden deaths in 21 untreated patients.


CONCLUSIONS
Clinically close to the congenital long QT syndrome, this primary ventricular arrhythmia must be looked for in cases of stress or emotion-induced syncope. The diagnosis relies on Holter monitoring and a stress test. Life-long beta blocker therapy is required.
Lucet
V
V
Unité de rythmologie pédiatrique, Château des Côtes, Les Loges-en-Josas, France.
Grau
F
F
Denjoy
I
I
Do Ngoc
D
D
Geiger
K
K
Ghisla
R
R
Mselati
J C
JC
Leenhardt
A
A
Coumel
P
P
D000293:Adolescent; D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use; D002395:Catecholamines / Q000502:physiology; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D005602:France; D006801:Humans; D008297:Male; D013315:Stress, Psychological / Q000150:complications; D017180:Tachycardia, Ventricular / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology; D016171:Torsades de Pointes
D000319:Adrenergic beta-Antagonists; D002395:Catecholamines
Devenir à long terme des tachycardies ventriculaires polymorphes catécholergiques de l'enfant. A propos de 20 cas suivis pendant 8 ans.