A 38-year-old woman underwent atriopulmonary Fontan surgery at age 18 years and subsequently successfully delivered a girl by cesarean section at age 34. Her condition later deteriorated due to atrial tachyarrhythmia and progressed to New York Heart Association (NYHA) class IV heart failure. Her treatment, at age 36, comprised total cavopulmonary connection conversion, direct right atrial ablation with bipolar radiofrequency devices, the creation of an atrial septal defect, and placement of a dual-chamber permanent pacemaker. Three years after the conversion, her condition has improved to NYHA class I.