Assessment of sinus node function in patients with congenital long QT syndrome

Sinus node function was evaluated in 4 patients with congenital long QT syndrome suffering from recurrent episodes of syncope and ventricular arrhythymias. Three of the 4 patients had bradycardia at rest on a 24-hour ECG. Sinus node function was examined by the atrial overdrive suppression test and the atrial premature stimulation test. Corrected sinus node recovery time (CSNRT) was prolonged in all patients. Total sinoatrial conduction time was also prolonged in 2 of the 4 patients. In all patients with abnormally high values, these values returned to normal after atropine administration, except in one patient. His prolonged CSNRT remained high after atropine administration, indicating abnormal automaticity such as that seen in sick sinus syndrome. These results show that sinus node function in congenital long QT syndrome is often associated with autonomic dysfunction, and sometimes with intrinsic disturbances of sinoatrial conduction.