Most office-based physicians have limited experience in caring for adults with congenital heart disease (ACHD). Because of the poor prognosis associated with most of these conditions, almost all the care for these patients has been delivered at high-volume academic centers. As treatments continue to improve, however, prognosis is better and life expectancy longer; thus more mainstream and community practices are seeing ACHD.
Nevertheless, the paucity of data to guide care for this population continues to pose a challenge. Antiarrhythmic treatment in patients with atrial fibrillation (AF) and congenital heart disease is an example—standard therapy is less effective in ACHD and may have a paradoxical effect, promoting arrhythmias. Based on results of the CABANA trial (catheter ablation for AF was superior to drug therapy in an on-treatment analysis) and as techniques for AF ablation become more refined, the treatment is emerging as an exciting option for ACHD patients.
First study of safety, efficacy
A recent analysis in JACC EP assessed 57 consecutive ACHD patients with drug-refractory AF who underwent ablation for AF between 2004 and 2017. It was a heterogeneous patient population including 2 patients with left persistent superior caval vein (1 with situs solitus, 1 with situs inversus plus azygous continuity), 1 patient with situs inversus totalis alone, and one with double aortic arch; 30 patients underwent corrective surgery and 6 palliative cardiac surgery; 5 patients underwent catheter-mediated interventional treatment of their CHD. Patients were followed for a median of 41 ± 36 months and arrhythmia-free survival was tracked.
For the initial ablation procedure (essentially wide pulmonary vein isolation ablation), the arrhythmia-free survival was 63% for 1 year and 22% for 5 years. For subsequent ablation procedures (which included more extensive ablation lines), the arrhythmia-free survival was better--99% at 1 year and 83% at 5 years. Of the 57 patients, 32 (56.1%) underwent a second ablation and 11 of those (34.4%) underwent a third ablation.
This study comes with a few important caveats as well as promising take-aways:
- AF in the ACHD patient population is heterogeneous and difficult to manage.
- AF ablation provides an appealing, safe, and effective alternative for management of ACHD, with results that are comparable to the general population. This is the first study to report the safety and mid- to long-term success rate of AF ablation in ACHD.
- The rate of need for re-ablation procedures in this population is much higher than the general population. Most patients have undergone prior surgical procedures. Therefore, AF ablation for ACHD should be referred to specialized academic centers.
- ACHD studies are small as patient populations are relatively limited, so this study of 57 patients is actually a reasonably-sized study for this patient group.
Source: Sohns C, Nürnberg J-H, Hebe J, et al. Catheter ablation for atrial fibrillation in adults with congenital heart disease. Lessons learned from more than 10 years following a sequential ablation approach. JACC: Clin Electrophys. 2018;4:733-743; DOI:10.1016/j.jacep.2018.01.015