Arrhythmias in patients with congenital heart disease and their impact on prognosis

Patients with congenital heart disease have an increased chance to suffer f rom brady- as well as tachyarrhythmias. The impact of these on quality of l ife, morbidity and mortality is more often imperative as compared to heart- healthy individuals. The substrate for these may be either congenital or ac quired. Improvements of the surgical management of these patients have led, on the one hand, to improved survival rates with prolonged life expectancy within the last 2 decades, which on the other hand provided the basis for a higher rate of acquired cardiac anhythmias. Together, this not only chall enges diagnostics and therapy but also the prognostic relevance of these ar rhythmias. The therapeutic strategies and prognostic markers have until now mostly been based on retrospective studies limited by the low number of pa tients and inhomogeneous patient selection. Despite these limitations, an i ncreased risk of sudden cardiac death has been substantiated for certain pa tient groups, e.g., those operated on by the Mustard- or Senning procedures in patients with transposition of the great arteries and patients operated on with correction of the tetralogy of Fallot. However, until now it has n ot been possible to identify reliable markers for establishing the risk on an individual basis within these patient cohorts. For achieving reliable da ta on the symptomatic and prognostic effects of present-day - as well as ne w-coming - therapeutic strategies, it is mandatory to perform prospectively based, randomized multicenter studies. Furthermore, the well-appreciated s ynergism of hemodynamically and primarily of arrhythmia-based effects on pr ognosis could potentially be divided into their relative weight to better g uide appropriate, substrate-related therapy. In addition, this should help to get better estimates of the risk for sudden cardiac death in different, etiologically homogeneous, groups of patients with congenital heart disease .