The long QT syndrome is characterized by intermittent or permanent prolongation of the QT interval, ventricular tachyarrhythmias and frequent sudden death. There is three previous reports in the literature, two unsuccessful. In all these patients a postoperative pacemaker was implanted. In our Institute, in October 1993 to May 1994, three patients with this syndrome, one female and two male aged 10,11 and 13 years were submitted to total denervation of the heart through autotransplantation. Despite full dosis of propranolol these patients were severaly symptomatic, with recurrent ventricular tachycardia. The technique was orientated to avoid lesion of the conduction system. The superior and inferior vena cava, the aortic and the pulmonary trunk were transected. Only the posterior wall of the left atrium between the pulmonary veins was incised. After surgery the patients remained in sinus rhythm without episodes of ventricular tachycardia even during serial ergometric test. Considering these preliminary results we recommend the described technique for patients with the long QT syndrome refractarles to adequate medication.
Long QT syndrome; QT, long; Heart autotransplantation; Heart transplantation; Heart transplantation