Atrial Arrhythmias in long-QT syndrome under daily life conditions: a nested case control study.

Zellerhoff S, Pistulli R, Mönnig G, Hinterseer M, Beckmann BM, Köbe J, Steinbeck G, Kääb S, Haverkamp W, Fabritz L, Gradaus R, Breithardt G, Schulze-Bahr E, Böcker D, Kirchhof P

Abstract

BACKGROUND: The long-QT syndromes (LQTS) are inherited electrical cardiomyopathies characterized by prolonged ventricular repolarization and ventricular arrhythmias. Several genetic reports have associated defects in LQTS-causing genes with atrial fibrillation (AF). We therefore studied whether atrial arrhythmias occur in patients with LQTS under daily-life conditions. METHODS: We systematically assessed atrial arrhythmias in LQTS patients and matched controls using implanted defibrillators or pacemakers as monitors of atrial rhythm in a nested case-control study. Twenty-one LQTS patients (3 male; 39 +/- 18 years old; 18 on beta blocker, ICD therapy duration 6.3 +/- 2.7 years; 4 LQT1, 6 LQT2, 2 LQT3) were matched to 21 control subjects (13 male; 50 +/- 19 years old; 3 on beta blocker; pacemaker therapy duration 8.5 +/- 5.5 years; 19 higher-degree AV block, 2 others). LQTS patients were identified by a systematic search of the LQTS patient databases in Münster and Munich. RESULTS: One-third (7 of 21) of the LQTS patients developed self-terminating atrial arrhythmias (atrial cycle lengths <250 ms). Only one control patient developed a single episode of postoperative AF (P < 0.05 vs LQTS). CONCLUSIONS: LQTS patients at high risk for ventricular arrhythmias may develop short-lasting atrial arrhythmias under daily-life conditions, suggesting that prolonged atrial repolarization may contribute to the initiation of AF.