Implantation of Cardioverter-Defibrillator in Children With Long-QT Syndrome: Assessment of Indications, Efficacy, and Safety Based on 10-Year Experience.

PURPOSE: to assess specificities of course of the long-QT syndrome in children before and after implantation of cardioverter-defibrillator (ICD), and optimization of indications to ICD-therapy. MATERIALS AND METHODS: We included in this study 48 children with long-QT syndrome from 44 unrelated families (28 boys and 20 girls), who underwent ICD implantation at the mean age 11.8±3.8 years. Mean duration of follow-up after implantation was 5.2±2.8 years. Data from these children were compared with those from 59 children of comparable age and gender with long-QT syndrome from 46 unrelated families receiving antiarrhythmic therapy (ß-adrenoblockers). We assessed clinical and electrocardiographic characteristics of the disease obtained at initial visit and their dynamics thereafter. RESULTS: Children with long-QT syndrome and ICD were mainly probands with interval QT longer than 500 ms, recurrent syncope and often history of sudden cardiac arrest requiring high doses of ß-adrenoblockers for control of ventricular tachyarrhythmias. CONCLUSION: ICD implantation is an effective and safe method both of primary and secondary prevention of sudden cardiac death in children with long-QT syndrome.

[Left ventricular diastolic dysfunction in endovascular treatment of atrial fibrillation in patients with preserved ejection fraction].

AIM: To study prevalence of left ventricular diastolic dysfunction (LVDD) and to evaluate the effect of pulmonary vein catheter isolation on left ventricular (LV) diastolic function in patients with paroxysmal and persistent forms of atrial fibrillation (AF). MATERIALS AND METHODS: 109 patients with paroxysmal (n=90; 82.6 %) and persistent (n=19; 17.4 %) AF were evaluated after 109 pulmonary vein catheter isolations. The patients were divided into two groups based on the operation efficacy. Heart ultrasound including evaluation of the LV diastolic function was performed for all patients on the day of operation and at 6 and 12 months. RESULTS: After the surgery, 61 (56.5 %) patients maintained sinus rhythm (SR) for 12 months and comprised Group 1. Group 2 consisted of patients with a relapse of AF (47 patients; 43.5 %). At baseline, LVDD with SR was observed in 53 patients (48.6 %), at 6 months - in 34 (31.2 %) patients (p=0.001), and at 12 months - in 27 patients (24.8 %) (p.

[The Brugada Syndrome in a Teenager].

The Brugada syndrome (BS) belongs to the group of hereditary channelopathies associated with elevated risk of sudden death (SD) in the absence of structural heart diseases. The disorder phenotypically manifests by specific electrocardiographic pattern, associated with ventricular tachycardia (VT). VT can be accompanied by loss of conscience, and after transformation to ventricular fibrillation result in SD. BS is extremely rare among children and adolescents. We present here a clinical case of teenager (age 17 years) with BS manifested by syncopal state at the background of fever.

[General anesthesia tools in endovascular surgery of the conductive heart system]. / Metody obshchei anestezii pri éndovascularnykh operatsiiakh na provodiashchei sisteme serdtsa.

The anesthetic management course was analyzed in 224 patients who underwent nonvascular surgeries on the conductive heart system. Analgesic and anti-stress techniques, which do not affect the intracardial conductivity and ensure the successful outcome of surgery with spontaneous or auxiliary ALV, were designed on the basis of research. The above schemes were introduced in practice with their efficiency being confirmed. They are based on a balanced use of the new-generation non-steroid anti-inflammatory drugs, like Xephocam, bezodiazepines and fentanyl (when used at subnarcotic doses that do not affect the intracardial conductivity). The main analgesic component of lornoxycam was sufficient when used at a dose of 0.1 mg/kg and at a total dose of equal to or below 16 mg.

[Anesthesiology in the surgical treatment of supraventricular tachyarrhythmias]. / Anesteziologicheskoe obespechenie pri khirurgicheskom lechenii nadzheludochkovykh takhiaritmii.

Different anesthesias were used in 197 patients operated for supraventricular tachiarrhythmias through a transvenous access. Hypnoanalgesia based on preventive injection of a potent nonnarcotic antiinflammatory agent xefocame (lornoxicame), drip infusion of propofol (2-3 mg/kg/h), and bolus injection of dormicum under conditions of spontaneous respiration proved to be the best method.

[Experience with the implantation of sensory multiple programmable pacemakers of the Siemens firm]. / Opyt implantatsii sensornykh mul'tiprogrammiruemykh élektrokardiostimuliatorov firmy Simens.

The paper summarizes the experience on 27 implantations of sensory multiprogrammable pacemakers (two-chamber Synchrony-II and one-chamber Sensolog-III) produced by Siemens-Elema. The implantations were performed in the Moscow Cardiostimulation Center in 1993. Primary pacemaker implantation was performed in 19 patients, 8 patients needed the replacement of one-chamber pacemakers because of the power source exhaustion or circulatory insufficiency. The two-chamber pacemaker was implanted to 14 patients with a complete AV-block and chronotropic sinus node incompetence and to 4 patients with sick sinus syndrome in intact AV-conduction. In 6 patients the one-chamber pacemaker was implanted to stimulate the right ventricle. They had permanent bradysystolic auricular fibrillation and complete AV block with persistent or transitory tachysystolic auricular fibrillation. 3 patients with sick sinus syndrome received one-chamber pacemaker. The authors think their experience with sensory Siemens-Elema pacemakers positive.