gms | German Medical Science

Cathecholaminergic polymorphic ventricular tachycardia (CPVT) is an uncommon arrhythmia in young patients. The aim of this study was to define of clinical and ECG patterns in pts with CPVT. Methods: CPVT was diagnosed in 20 pts 3 - 15 years (8,5 ± 3,2), 11 boys, 9 girls. 10 (50%) pts had been previosly treated as epilepsy. Evidence heart diseases, long QT syndrome, syndrome Brugada, digoxin toxity were excluded for all pts. Circadian index (CI) was calculated from as ratio mean HR (bpm) during awake to mean HR during sleep by results of HM (normal limit of CI 1,24-1,44, L. Makarov 1997). Results: The CPVT was polymorphic in 5 cases (25%), polymorphic and bidirectional (BCVT) in 15 (75%). 7 pts (age 8,9 ± 3,7, boy/girls – 4/3) had a normal PR interval (NPRI) on rest ECG, 13 - a short PR (SPRI) - 0,11 sec or less (age 7,4 ± 2,4, boy/girls – 7/6), without other signs of WPW syndrome. All CPVT pts had syncope (4,9 ± 3,7 per year in SPRI vs 2,6 ± 2,0 per year in pts with NPRI), 6 - aborted SD (all with SPRI). HR and CI were 56,3 ± 8,7 vs 78,1 ± 2,8 bpm and 1,69 ± 0,09 vs 1,4 2 ± 0,18 (p= 0,009) respectively SPRI vs NPRI. QTc < 350 ms and supraventricular tachycardia were detected only in pts with SPRI - 4 (30,8%) and 7 (53,8%) pts respectively. This ECG pattren no been desribed before as typical for CPVT but we noticed it in some description of CPVT cases (D.Reid et al.1975, S. Eisenberg et al. 1995, J.Fisher et al. and some other) that allow for us decide that SPRI and CPVT is a more freguent association that it was concidered before. Combination of the CPVT and SPRI is the new specific patterns or original clinico-electrocardiographic syndrome with high risk of syncope and sudden death in children. Children and young patients with epilepsy or uncertain syncope with bradycardia and SPRI need in exclusion of CPVT by HM. Role of the SPRI in nature and prognosis of CPVT need in investigation by follow up observation, EPI and genetic studies.