Transposition with mustard operation patient with risk of sudden death: Case submitted by Jim T. Vehmeijer, MD, Barbara J.M. Mulder, MD, PhD, Joris R. de Groot, MD, PhD

Abstract

1. Patients with atrial switch (Mustard’s and Senning’s operations for transposition) and a combination of arrhythmias and systemic RV failure present extremely difficult management conundrums in adult congenital heart disease. 2. Ablation therapy for atrial arrhythmias is encouraging in this group and should be considered early. 3. The frequent presence of baffle stenosis and or baffle leaks should be considered in such patients prior to any interventional and/or device procedures. 4. In general, transplantation should be considered early in the course of patients with complex CHD and systemic ventricular failure.