Long-term noninvasive arrhythmia assessment after total anomalous pulmonary venous connection repair

Pediatric patients with a history of atrial surgery are at risk for the development of sinus node dysfunction and atrial arrhythmias. However, there has been no comprehensive, long-term, electrophysiologic study of patients who have undergone repair of total anomalous pulmonary venous connection.

We evaluated school-aged and adolescent survivors of isolated total anomalous pulmonary venous connection repair from January 1983 to December 1996 to assess for sinus node dysfunction, atrioventricular block, and atrial and ventricular arrhythmias. Assessment was limited to an electrocardiogram, 24-hour Holter monitor, and exercise stress test.

Twenty-nine children were evaluated 11.2 +/- 3.6 years after their initial operative repair. The mean age at repair was 36.0 +/- 43.0 days. Electrophysiologic studies revealed evidence of sinus node dysfunction, including sinus bradycardia, sinus pauses, and chronotropic impairment, in most of the patients. Twenty-nine percent of patients showed chronotropic impairment on exercise testing. Atrioventricular conduction abnormalities occurred in 2 patients. Single atrial and ventricular premature complexes were frequent, but complex tachyarrhythmias were less common. There was 1 patient who had nonsustained supraventricular tachycardia and 2 patients who had nonsustained ventricular tachycardia. No statistically significant relationships were found between hypothesized variables and arrhythmia outcomes.

Survivors of total anomalous pulmonary venous connection repair appear to have a high incidence of signs of sinus node dysfunction and a low incidence of atrioventricular block in follow-up. Significant atrial and ventricular arrhythmias appear to be uncommon. Despite a favorable overall long-term outcome, these patients warrant ongoing clinical follow-up for arrhythmia surveillance.