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Electrophysiologic findings after Fontan repair of functional single ventricle

C C Kürer1, C S Tanner, V L Vetter

  • 1Department of Pediatrics, Children's Hospital, Philadelphia University of Pennsylvania School of Medicine, Pennsylvania 19104.

Insights

The Fontan procedure for single ventricle anomalies can cause significant cardiac arrhythmias. Electrophysiologic studies reveal abnormal sinus node function, prolonged atrial refractoriness, and inducible atrial arrhythmias in many patients.

Area of Science:

  • Cardiology
  • Electrophysiology
  • Pediatric Cardiac Surgery

Background:

  • The Fontan operation is a palliative procedure for complex congenital heart defects, creating a single ventricle physiology.
  • Cardiac arrhythmias are known complications following the Fontan procedure.

Purpose of the Study:

  • To evaluate the electrophysiologic effects of the Fontan procedure in patients with functional single ventricle.
  • To identify specific electrophysiologic abnormalities and their prevalence post-Fontan repair.

Main Methods:

  • Cardiac catheterization with electrophysiologic study in 30 patients post-modified Fontan repair.
  • Assessment of sinus node and atrial pacemaker function, sinoatrial conduction, and atrial refractory periods.
  • Atrial endocardial mapping and programmed atrial/ventricular stimulation to induce arrhythmias.

Main Results:

  • 50% of patients showed abnormal sinus node or ectopic pacemaker automaticity.
  • Prolonged sinoatrial conduction time and atrial refractory periods were observed in 50% and 43% of patients, respectively.
  • Intraatrial conduction delays were found in 76%, and inducible supraventricular arrhythmias, primarily intraatrial reentry, occurred in 37% of patients.

Conclusions:

  • The Fontan procedure is associated with significant electrophysiologic abnormalities, including impaired sinus node function and delayed intraatrial conduction.
  • Intraatrial reentry is a common mechanism for inducible atrial arrhythmias after Fontan repair.
  • These findings highlight the proarrhythmic substrate created by the Fontan circulation, necessitating careful long-term monitoring.

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