Electrophysiology Study for Complex Supraventricular Tachycardia in Congenital Heart Disease Patients With Single-Ventricle Physiology.

BACKGROUND: Supraventricular tachycardia (SVT) is common in complex congenital heart disease (CCHD) patients with single-ventricle physiology and may cause hemodynamic deterioration. We reported the outcomes of catheter ablation for such complex SVT in these single-ventricle CCHD patients.

METHODS AND RESULTS: Patients with single-ventricle physiology (defined as CCHD patients) who received electrophysiology studies and catheter ablation between 1995 and 2015 were studied. We enrolled 30 CCHD patients (18 with right atrial isomerism, 5 with left atrial isomerism, and 7 with other CCHDs; 17 male, 13 female). The age of onset of clinical SVT was 6.7 years (±4.7 years). Electrophysiology studies and ablation were performed at age 7.1 years (±3.9 years); body weight was 20.7 kg (±10.0 kg). Twin atrioventricular nodes were present in 60% of patients (right atrial isomerism, 72.2%; left atrial isomerism, 40%; other CCHDs, 42.9%). Manifested preexcitation was noted in 10% of patients. SVT was induced in 21 patients. Twin atrioventricular nodal reentrant tachycardia was the most common (57.1%), followed by atrioventricular reentrant tachycardia (28.6%), junctional tachycardia (14.3%), and atrioventricular nodal reentrant tachycardia (9.5%). Multiple arrhythmias were common (33.3%), particularly in patients with atrioventricular reentrant tachycardia (50%). Ablation successfully eliminated SVT in 12 of 14 patients (85.7%), with a recurrence rate of 16.7% during 6 years of follow-up.

CONCLUSIONS: Transcatheter ablation of complex SVT substrates, including minor atrioventricular node of twin atrioventricular nodal reentrant tachycardia, accessory pathways of atrioventricular reentrant tachycardia, and a slow pathway of atrioventricular nodal reentrant tachycardia, is effective in CCHD patients. The limitations are limited vascular access and the risk of atrioventricular block.