This long-term study sought to determine the clinical implication of defective sinus node and AV conduction tissue in patients with left atrial isomerism (LAI). From 1984 to 1998, a total of 22 patients were identified as LAI. Patient age at the last follow-up ranged from 2 to 276 months (90+/-70 months). Associated cardiac anomalies were interruption of the inferior vena cava (n = 18, 82%), common atrium (n = 9, 41%), AV canal (n = 14, 64%), double-outlet right ventricle (n = 8, 36%), and pulmonary stenosis (n = 15, 68%). Palliative interventions were performed in 16 patients (Fontan-type operation in 4 patients, shunt followed by Fontan-type operation in 2, repair of septal defect in 4, and extracardiac intervention in 6). During the follow-up, over half of the patients (n = 14, 64%) developed bradyarrhythmia (onset age: from 1 to 264 months; median 78 months): junctional rhythm (n = 11), sinus bradycardia (n = 8) (5 patients also had junctional rhythm), and AV block (n = 2, both also had junctional rhythm). The probability free from bradyarrhythmia was 80% and 46% at the age of 2 and 6 years, respectively. None of the bradyarrhythmias were directly related to open-heart surgery. Besides, junctional ectopic tachycardia occurred after Fontan-type operation in three of six patients. In two patients, a Mahaim-like pathway was identified during the electrophysiological study. The patients with LAI had a high probability of developing bradyarrhythmias due to abnormal sinus node function. Varied AV conduction abnormalities may include compromised AV conduction, junctional ectopic tachycardia after Fontan-type operation, and an association of Mahaim-like pathway.