Résumé
Abstract
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is defined by bidirectional or polymorphic ventricular tachycardia during adrenergic situations and is associated to a poor long-term prognosis. Clinical cases suggest an association between epilepsy and/or neuro-psycho-behavioral troubles (NPBT) to cardiac channelopathies.
Methods
This is a retrospective observational study based on case analysis from the INTEGRALIS database of the referral center for inherited cardiac arrythmias in Nantes. Epidemiological and clinical-biological features of the population have been studied. Patients with Presence or Absence of NPBT were compared. Results:From 8306 pts in the whole database, 533 presented with VT and 71 pts were diagnosed with CPVT and genotyped. Symptom onset occurred at a medium age of 17.1±13.5 years. Median LVEF was 65% (IQR 9,8%) and median corrected QT interval (QTc) was 399 ms (IQR 27 ms). 77.5% of pts had fainting and/or syncopes, and there were 28.2% patients with a history of cardiac arrest. Time to diagnosis was below1 year for 44.2% of symptomatic pts. Symptoms occurred during exertion for 42.3% of pts including swimming. The prevalence of NPBT was 23,9%. 74% of NPBT were convulsive seizures, 21% psycho-behavioral troubles and 5% epilepsy proved by EEG. Median age of symptom onset was younger in the group “NPBT” (12.2±4 yo vs 19.2±15.5 yo). The rate of patients with symptoms during exertion was higher in the group “NPBT” (29.4 vs 7.4% P=0.031). A mutation in the gene of Ryanodine receptor-2 was found in 64.8% of pts.
Comparisons patients w/wo NPBT
NPBT (N=17)
Without NPBT (N=54)
Familial history of Sudden death
7/17 (41.2%)
24/54 (44.4%)
NS
Familial history of CPVT
5/17 (29.4%)
29/54 (53.7%)
NS
Medium age of symptom onset (yo)
12.1±4
19.2±15.5
P=0.021
Time to diagnosis <1 year
4/17 (23.5%)
16/54 (27.8%)
NS
Malaises and/or syncopes
17/17 (100%)
38/54 (70.4%)
P=0.035
Cardiac arrest
9/17 (52.9%)
11/54 (20.4%)
P=0.025
ICD Implantation
6/17 (35.3%)
12/54 (22.2%)
NS
Supraventricular arrhythmias
3/17 (17.7%)
6/54 (11.1%)
NS
Antiepileptic treatment
5/17 (29.4%)
2/54 (3.7%)
P=0.009
Conclusion
NPBT appears to be associated to a younger age of symptom onset and a higher rate of serious cardiac events particularly during swimming. This study will serve as preliminary data for further clinical and experimental protocols.