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Arrhythmias Emerging Decades after Tetralogy of Fallot Repair Resolved with Re-Ablation

August 2015

Tetralogy-of-Fallot-diagram web-CDC
— Tetralogy of Fallot is characterized by a ventricular septal defect, pulmonary stenosis, ventricular hypertrophy, and an enlarged aortic valve, which seems to open from both ventricles rather than from the left only. Image appears courtesy of the Centers for Disease Control and Prevention, National Center on Birth Defects and Developmental Disabilities.

Contributed by Cindy M. Martin, MD

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease (CHD).1 Advances in surgical technique and postoperative care now allow well over 85% of patients with TOF to survive into adulthood.2 Yet, many adult patients who underwent TOF repair procedures in childhood must undergo further interventional catheter or surgical procedures later in life.3 Here we describe a middle-aged female patient with a history of 2 TOF surgical procedures who was referred for symptomatic arrhythmias and suspected pulmonary valve regurgitation.


A 57-year-old female patient experiencing symptomatic atrial arrhythmias that were increasing in frequency was referred in 2014 to the Adult Congenital and Cardiovascular Genetics Center. She had a history of TOF, with surgical intervention at age 10 years and 12 years. The patient had felt well after TOF repair and had maintained a high activity level in early adulthood, including walking and hiking 5 to 7 miles per day. At the age of 40 years, symptomatic atrial arrhythmias developed, and at the age of 45 years, the patient underwent cardiac ablation and implantation of a pacemaker. Several months later, she developed ventricular tachycardia, prompting her cardiologist to upgrade her pacemaker to an implantable cardioverter defibrillator. Although she had done well for several years postprocedure, she recently developed exercise intolerance due to recurrent atrial arrhythmias. The referring physician was concerned that she might also be experiencing ventricular arrhythmias and significant pulmonary valve regurgitation.


A cardiac CT scan revealed that although she had substantial pulmonary regurgitation, there was no significant right ventricular dilation or dysfunction and therefore pulmonary valve replacement was not indicated. An electrocardiogram and a stress test revealed that most of the arrhythmias were atrial in origin resulting in significant right ventricular pacing (Figure 1). At University of Minnesota Medical Center, the patient underwent a second cardiac ablation procedure to mitigate the arrhythmias, with an excellent outcome. Her arrhythmias resolved, allowing atrial pacing (Figure 2), and her heart function improved. She has resumed her prior active lifestyle.

— Figure 1. Atrial flutter resulting in ventricular pacing
— Figure 2: Atrial pacing with native AV conduction


Cardiac arrhythmias caused by abnormal anatomy, surgical scars, and suboptimal hemodynamics remain a major source of morbidity and mortality for patients with congenital heart diseases. For example, about 30% of patients with a history of corrective surgery for TOF will develop atrial re-entrant tachycardia during extended follow-up, and about 10% will develop high-grade ventricular arrhythmias.3 In one study of adult patients with CHD, atrial arrhythmias were associated with a 50% increase in mortality, a twofold increased risk of heart failure or stroke, and a threefold higher risk of requiring some major cardiac intervention.4

In the above case, re-ablation was utilized to correct symptomatic arrhythmias in a middle-aged female with a complex CHD history, resulting in a very positive outcome and a return to the patient’s active lifestyle.


1. Reller MD, Strickland MJ, Riehle-Colarusso T, et al. Prevalence of congenital heart defects in metropolitan Atlanta, 1998-2005. J Pediatr. 2008;153:807-813.

2. Murphy JG, Gersh BJ, Mair DD, et al. Long-term outcome in patients undergoing surgical repair of tetralogy of Fallot. N Engl J Med. 1993;329:593-599.

3. Villafañe J, Feinstein JA, Jenkins KJ, et al. Hot topics in tetralogy of Fallot. J Am Coll Cardiol. 2013;62:2155-66.

4. Bouchardy J, Therrien J, Pilote L, et al. Atrial arrhythmias in adults with congenital heart disease. Circulation. 2009;120:1679-1686.

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