Prolonged left ventricular contraction duration in apical segments as a marker of arrhythmic risk in patients with long QT syndrome.

AIMS: Long QT syndrome (LQTS) is an inherited cardiac ion channelopathy predisposing to life-threatening ventricular arrhythmias and sudden cardiac death. The aim of this study was to investigate left ventricular mechanical abnormalities in LQTS patients and establish a potential role of strain as a marker of arrhythmic risk. METHODS AND RESULTS: We included 47 patients with genetically confirmed LQTS (22 LQT1, 20 LQT2, 3 LQT3, and 2 SCN3B) and 25 healthy controls. A history of cardiac events was present in 30 LQTS subjects. Tissue Doppler and speckle tracking echocardiography were performed and contraction duration was measured by radial and longitudinal strain. The radial strain characteristic was subdivided into two planes - the basal and the apical. Left ventricular ejection fraction and global longitudinal strain were normal in LQTS patients. Mean contraction duration was longer in LQTS patients compared with controls in regard to basal radial strain (491 ± 57 vs. 437 ± 55 ms, P < 0.001), apical radial strain (450 ± 53 vs. 407 ± 53 ms, P = 0.002), and longitudinal strain (445 ± 34 vs. 423 ± 43 ms, P = 0.02). Moreover, contraction duration obtained from apical radial strain analysis was longer in symptomatic compared with asymptomatic LQTS mutation carriers (462 ± 49 vs. 429 ± 55 ms, P = 0.024), as well as in subject with mutations other than LQT1 considered to be at higher risk (468 ± 50 vs. 429 ± 49 ms, P = 0.01). CONCLUSION: Myocardial contraction duration is prolonged for both radial and longitudinal directions in LQTS patients. Regional left ventricular function analysis may contribute to risk stratification. Apical radial deformation seems to select subjects at higher risk of arrhythmic events.

Atrial tachycardia ablation in patients with a functional single ventricle after the Fontan surgery.

BACKGROUND: Atrial tachyarrhythmias are a leading source of morbidity and mortality after Fontan-type procedures and antiarrhythmic drug therapy is often ineffective in these patients. AIM: To evaluate short- and long-term outcomes of radiofrequency current ablation for atrial tachycardia (AT) in patients after the Fontan procedure, and to report clinical, electrophysiological and electroanatomical characteristics of these arrhythmias. METHODS: We retrospectively analysed data obtained in 8 patients (5 males, 3 females) after the Fontan procedure who underwent ablation for AT between 2002 and 2013. In order to compare the clinical impact of arrhythmia before and after ablation, we used the modified arrhythmia score, ranging from 0 (no arrhythmia activity) to 12 (very severe arrhythmia). In all patients, electroanatomical mapping using the CARTO system was performed, allowing semiquantification of low-voltage (< 0.5 mV) areas and scars. RESULTS: Seven patients had an atriopulmonary connection and 1 patient had an extracardiac conduit. The mean patient age was 9.4 ± 3.1 years at the time of the Fontan procedure and 26.2 ± 4.6 years at the time of the first ablation. A total of 18 ablations were performed with no complications, 1 to 4 (median 2.5) procedures per patient. In patients who had more than 1 ablation, the mean time from the first to the last procedure was 34.8 months (range 1-64 months). In individual patients, 1 to 4 (median 2.5) different ATs were observed, with the mean tachycardia cycle length of 334 ± 95 ms. In 6 patients, low-voltage area (< 0.5 mV) comprised 25-50% of the right atrium, and in two others it comprised 10-25% and < 10% of the right atrium, respectively. Seven procedures were fully successful (ablation of all ATs), 7 were partially successful (ablation of only some AT, including clinical arrhythmia, but not of all ATs) and 4 were unsuccessful (failed ablation of clinical AT). The mean procedural, fluoroscopy and ablation times were 176 ± 54.6, 13.7 ± 5.7 and 21.7 ± 11.9 min, respectively. Freedom from arrhythmia during the mean follow-up of 58.6 ± 46 months (range 11-127 months) since the last procedure was obtained in 4 patients. The median arrhythmia score after the last ablation was significantly reduced compared to baseline (4.5 vs. 8; p < 0.05). CONCLUSIONS: Catheter ablation of AT in patients after the Fontan procedure is safe but its acute and long-term efficacy is limited. Due to complex and extensive substrate, along with complex anatomy, recurrences are frequent and patients may require repeat ablation procedures. Suppression of arrhythmia is associated with an improved clinical status of the patients.