Objective. Newborns of mothers positive for antiRo/SSA autoantibodies may develop a series of electrocardiographic (EKG) disturbances. Prolongation of the corrected QT (QTc) interval was recently reported in a significant proportion of children with maternally acquired anti-Ro/SSA antibodies, with a concomitant disappearance of EKG abnormalities and acquired maternal autoantibodies during the first year, suggesting a direct, reversible electrophysiologic effect of anti-Ro/ SSA antibodies on the ventricular repolarization. On this basis, we investigated whether these antibodies may also affect cardiac repolarization in anti-Ro/SSApositive adult patients with connective tissue diseases.Methods. Fifty-seven patients with connective tissue diseases were selected: 31 had anti-Ro/SSA antibodies and 26 did not (controls). In all subjects, we analyzed the QTc interval, heart rate variability, and signalaveraged high-resolution EKG recording.Results. Anti-Ro/SSA-positive patients showed a significant prolongation of the mean QTc interval compared with the controls (mean ؎ SD 445 ؎ 21 versus 419 ؎ 17 msec; P ؍ 0.000005). Eighteen of the 31 anti-Ro/SSA-positive patients (58%) and none of the 26 anti-Ro/SSA-negative patients had QTc values above the upper limit of normal (440 msec). Both groups had a reduction in heart rate variability, with a prevalence for the sympathetic nervous system and a high incidence of ventricular late potentials; these values were not significantly different between the 2 groups.Conclusion. Adult patients with anti-Ro/SSApositive connective tissue diseases showed a high prevalence of QTc interval prolongation. This feature, with the concomitant abnormalities in the autonomic tone and ventricular late excitability observed in all patients studied, suggests that anti-Ro/SSA-positive patients may have a particularly high risk of developing lifethreatening arrhythmias.Neonatal lupus is a rare syndrome related to the transplacental passage of autoantibodies from mothers positive for anti-Ro/SSA (and anti-La/SSB) to their newborns. These mothers frequently have autoimmune disorders, especially connective tissue diseases; however, they are sometimes entirely asymptomatic. The main feature of neonatal lupus is congenital heart block (CHB), an irreversible disturbance in cardiac conduction. Many recent studies have investigated the possible electrophysiologic and molecular mechanisms of CHB, and the findings suggest a direct arrhythmogenic activity of anti-Ro/SSA antibodies. In particular, it has been demonstrated that affinity-purified anti-Ro/SSA antibodies from mothers of children with CHB are able to induce a complete atrioventricular block in the human fetal heart as well as in a rat model of the heart, which inhibits L-type calcium currents (1).CHB is the main, but not the only, electrocardiographic abnormality associated with the transplacental passage of anti-Ro/SSA antibodies. Mazel and colleagues (2) reported sinus bradycardia in a murine model injected with IgG obtained from mothers of childr...