Introduction The Fontan procedure provides effective palliation to support the systemic circulation in patients with a functionally single ventricle. It is associated with the formation of a pro-arrhythmogenic environment, due to surgical scar and subsequent haemodynamic abnormalities. These patients now survive into late adulthood, but a significant number are developing arrhythmias, a potentially life threatening event. Changes in surgical technique have been postulated to reduce the incidence of late arrhythmias, however the long term outcome is unknown.
Methods We carried out a retrospective analysis of all adult Fontan patients cared for by the University Hospitals Birmingham Adult Congenital Heart Disease Unit between 2001 and 2013. Patients were identified using electronic databases and data collected from medical records. Patients were stratified according to the surgical approach.
Results 166 adult patients, mean age 29yrs (16–69yrs) with a Fontan circulation were followed for a mean of 19 years post surgery (63% Modified, 13% Lateral Tunnel, 21% Total Cavopulmonary Connection (TCPC), 3% Kawashima). 42% had suffered an arrhythmia (4 SVT, 64 Fib or Flutter). The incidence of arrhythmia was higher in patients with a Modified Fontan (52%). However the Modified Fontan patients were older. There was a high incidence of arrhythmia even in patients with TCPC despite being younger and less time having elapsed since surgery.
Conclusion Arrhythmias are common in patients with a Fontan circulation. Currently the majority of patients with an arrhythmia have a Modified Fontan. However there is already a high arrhythmia burden even in patients with a TCPC despite their younger age and the shorter time that has elapsed since their Fontan surgery. There is likely to be a continued rise in the incidence of arrhythmias in patients with a Fontan circulation despite changes in surgical technique, which in turn may hinder attempts at ablation. This will represent a significant challenge to Electrophysiologists and their Congenital colleagues.
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