Perioperative management of long QT syndrome in a child with congenital heart disease

During cardiac catheterization, a 2(1/2)-year-old boy developed sudden cardiac arrest. The presence of a long QT interval in the electrocardiogram (ECG) along with ventricular arrhythmia and syncope at that moment enabled us to diagnose long QT syndrome (LQTS). Immediate defibrillation and beta-blocker (metoprolol) therapy saved the life of the child. Cardiac catheterization was completed and the child was planned for Fontan operation. Beta-blocker coverage, prevention of sympathetic stimulation and avoidance of agents which prolong the QT interval made anesthesia uneventful. There were episodes of ventricular fibrillation (VF) in the postoperative period. The child was managed with electrical defibrillation, metoprolol and magnesium.