Gene analysis for the inherited bradyarrhythmia and characterization of cardiosphere-derived cells to create biological pacemakers

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We evaluated clinical characteristics and performed gene analysis for 58 patients with inherited bradyarrhythmia. Twenty six patients (45%) had family history of bradyarrhythmia and 24 patients (41%) underwent pacemaker implantation. Gene analysis showed mutations were found in 7 patients (12%) who were complicated by QT prolongation, atrial fibrillation, or muscular dystrophy. Cellular electrophysiological study showed functional abnormality in 3 of 4 ion channel gene mutations. We isolated cardiosphere-derived cells from the neonatal mouse heart and confirmed that some cells contracted spontaneously. Patch clamp and RT-PCR showed that at least two types of ionic currents were present in mouse cardiosphere-derived cells including a delayed rectifier K+current and an inward rectifier K+current.