[Clinico-physiological characteristics of syncopal conditions in the long QT syndrome (Romano-Ward syndrome)]. / Kliniko-fiziologicheskaia kharakteristika sinkopal'nykh sostoianii pri sindrome udlinennogo intervala Q-T (sindrom Romano-Uorda).

A complex of methods including clinical analysis, ECG, and exercise EEG was used to examine 52 patients aged 2 to 20 years with Romano-Ward syndrome. There were two groups: group I with syncopal attacks, group II without them. The patients with Romano-Ward syndrome had dysfunction of the suprasegmental systems regulated autonomously in the form of functional insufficiency of the mesencephalic activating system. In the patients without syncopal attacks, there was hyperproduction of the theta-rhythm, which may reflect the mechanisms of compensation. According to the EEG readings, criteria for the syndrome gravity and sudden death risk factors were distinguished.

Holter monitoring in the long QT syndrome of children and adolescents.

Results of Holter monitoring (HM) of ECG in 45 patients with long QT syndrome (LOTS) and 26 healthy control children are presented. 15 patients had a history of syncopal attacks (group I), other patients were symptomless (group II). Heart rate-corrected QT interval (QTc) on standard resting ECG exceeded 460 ms in 73.3% of patients in group I and in 44.8% of group II patients. On Holter monitoring, it was present in 100% of group I and 83.9% of group II patients. T wave alternation was registered in 66.6% of group I and 56.7% of group II patients. Ventricular arrhythmia was not found on standard resting ECG in any of the examined children, whereas Holter monitoring revealed ventricular extrasystoles in 53.3% of group I and 6.7% of group II patients (p less than 0.05). In one case early ventricular extrasystoles accompanied an attack of ventricular tachycardia. Mathematical analysis of 24-h extracardial heart rhythm regulation revealed its disturbance in LQTS patients compared to healthy children, as well as asthenic signs in the sympathetic nervous system. These changes were more pronounced in patients with a more severe course of the disease (group I).