Successful implantation of a dual-chamber pacemaker in an ELBW infant for long QT syndrome.

Long QT syndromes encompass the most prevalent group of ion channelopathies. Long QT syndromes are predominantly familial and predispose the affected individual to ventricular arrhythmias and sudden death. Permanent pacemaker insertion for long QT syndrome is discouraged apart from younger patients exhibiting 2:1 atrioventricular block. However, permanent pacemaker insertion is a relatively common procedure in neonates with atrioventricular block, and dual-chamber permanent pacemaker insertion in low birth weight infants is challenging. We describe the management of long QT syndrome - type 2 - presenting in an extremely preterm neonate including epicardial, dual-chamber permanent pacemaker insertion.

Ten-year experience in atenolol use and exercise evaluation in children with genetically proven long QT syndrome.

BACKGROUND: Due to its availability, atenolol is the primary beta-blocker used in Australia for children with long QT syndrome. There is limited data on long-term follow-up of its use. METHODS: A single-tertiary-center, retrospective, observational study investigating all children and adolescents who had genetically proven long QT syndrome type 1 (LQT1) and type 2 (LQT2) was conducted. Their pretreatment exercise tests were evaluated for QTc intervals into the recovery phase of exercise. RESULTS: Eighty six patients were identified (LQT1, 67, and LQT2, 19) from 2004 to 2014. The majority (86%) of patients were initially referred for family screening. Atenolol was administered at a mean dose of 1.58 ± 0.51 mg/kg/day. During the median follow-up period of 4.29 years, only one proband developed ventricular arrhythmia whilst taking atenolol, No patient had cardiac arrest or aborted cardiac arrest. With respect to side effects of atenolol, only two patients had intolerable side effects necessitating changes of medication. Evaluation of exercise tests (pretreatment) demonstrated that corrected QT (QTc) intervals at 2-3 min into the recovery phase of exercise were significantly prolonged for LQT1 patients. LQT1 patients with transmembrane mutation had longer QTc intervals than their C-terminus mutation counterparts, reaching statistical significance at 3 min into the recovery phase of exercise. CONCLUSIONS: Atenolol is an effective treatment for genetically proven LQT1 and LQT2 children and adolescents, with good tolerability. In LQT1 patients, QTc intervals at 2-3 min into the recovery phase of exercise were significantly prolonged, particularly in patients with transmembrane mutations.

The QT and corrected QT interval in recovery after exercise in children.

BACKGROUND: Prolongation of the QT interval after exercise can be used to help diagnose long-QT syndrome, especially when the resting QT interval is borderline. The aim of this study was to determine the normal ranges for QT and corrected QT in the recovery phase after exercise in children. METHODS AND RESULTS: Ninety-four volunteer boys and girls aged 8 to < 17 years without any history of heart disease underwent exercise testing and had a 12-lead ECG performed in the supine position for 10 minutes of recovery. The QT was measured using a standardized tangent method, with the baseline defined as the Q-Q line. The recovery QT was maximally short at 1 minute of recovery in 93 of 94 children then lengthened and stabilized at 4 to 5 minutes recovery. The recovery QT lengthens as heart rate decreases in an approximately linear fashion with a mean increase of 15 ms per 10-beat decrease in heart rate. The 98 th percentiles for the corrected QT using the Bazett formula during minutes 4 to 6 in recovery were from 482 to 491 ms. There was excellent intraobserver and interobserver reliability, with intraclass correlation coefficients of 0.95 and 0.88, respectively. CONCLUSIONS: There is substantial individual variability of the normal repolarization process in the postexercise recovery period in children. The study provides a reference for normal responses for similar populations using a specific measurement protocol that can be easily applied.