Late Arrhythmia After Fontan Repair

The patient in this case was born with the most prevalent cyanotic congenital heart defect and was submitted to surgical correction in a two-stage procedure. After the surgical procedures, the patient remained asymptomatic until 2014 when he sought medical care evolving with sustained tachycardia episodes in a row which required drug reversal in the emergency room several times. This case illustrates a congenital heart defect in which part of or the entire aorta arises from the right ventricle and part of or the entire pulmonary trunk arises from the left ventricle, and Jatene’s arterial repair is traditionally performed, for the surgical procedure would be indicated for this heart defect. In the last decades, sophisticated corrective procedures improved the quality of life and life expectancy of patients with congenital heart defects. Nevertheless, due to surgical procedure healing, the use of patches and suture lines, and consequent fibrosis, the development of arrhythmias is increasingly common in this population, and several therapies are suggested to treat arrhythmias in these cases such as reoperations, antiarrhythmic medications, and ablation of the electric circuit that provokes the arrhythmia. The long-term following of the patients with this congenital heart defect is extremely important due to substantially increased mortality in this group after developing this arrhythmia.