Single-ventricle physiology occurs in patients with hypoplastic ventricular heart defects, either on the right or left, who have undergone stepwise palliation surgeries ending with the Fontan procedure. After Fontan completion, these patients are dependent on passive venous return to the pulmonary circulation. The implications of passive flow are potentially devastating to the patient. We discuss some of the basic changes to the patient’s experience after a Fontan procedure, as well as the common complications. We also touch on some of the emerging management strategies for the common complications.
Purpose of reviewThe adult congenital heart patient population is rapidly growing due to increasing survival rates, and they often face chronic physiologic challenges as sequalae of both their defects and repairs. Among the most common sequalae are arrhythmias. Here we describe intra-atrial reentrant tachycardia (IART), one of the most commonly seen arrhythmias in the adult congenital heart population, and the approaches to successful ablation in adult congenital heart patients.
Recent findingsIART has increasing ablation success rates due to the increasing exposure of electrophysiologists to congenital cases, advances in technology, and the increasing application of both our pediatric congenital knowledge and adult acquired knowledge to the adult congenital population.
SummaryIART is a frequently seen arrhythmia in the adult congenital population, and it can have life-threatening consequences in the setting of congenital disease. Ablation techniques and treatment rates have improved over time, despite the challenges these cases present to clinicians; success of the ablation depends on careful, often creative, preplanning, and understanding of the complex individualized anatomy and circuits of the patient.
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