Arrhythmia Management in Complex Congenital Heart Disease

Atrioventricular block is a common complication in L-looped single ventricles, and it may be exacerbated by palliative surgical procedures.

We describe the case of a 43-year-old woman with history of double inlet left ventricle after classic Fontan procedure with subsequent Fontan revision, atrial flutter, and complete heart block managed with an epicardial pacemaker with progressive ventricular lead dysfunction. The patient was managed with leadless pacemaker implantation and atrial flutter ablation.

Epicardial pacing is the preferred modality for patients with single-ventricle physiology and atrioventricular block. However, epicardial leads have high rates of lead failure often requiring multiple lead revisions by means of thoracotomies, which carry significant morbidity. Epicardial lead revision is not always successful in patients with multiple surgeries owing to adhesions, thus alternative pacing methods are required.

This case illustrates a true heart team approach in the use of leadless pacemaker and atrial flutter ablation in a patient with a complex single-functioning ventricle.