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. Author manuscript; available in PMC: 2024 May 15.
Published in final edited form as: Circulation. 2023 Nov 30;149(1):e1–e156. doi: 10.1161/CIR.0000000000001193

Recommendations for ACHD

Referenced studies that support the recommendations are summarized in the Online Data Supplement.

COR LOE Recommendations
1 B-NR 1. In adults with congenital heart disease and AF, it is recommended to evaluate for and treat precipitating factors and reversible causes of AF, recognizing that residual hemodynamic sequelae may contribute to the occurrence of the arrhythmia.1,2
1 C-LD 2. In adults with AF and moderate or complex congenital heart disease, electrophysiological procedures should be performed by operators with expertise in ACHD procedures and in collaboration with an ACHD cardiologist, ideally in specialized centers, when available.35
1 C-LD 3. In adults with congenital heart disease and symptomatic or hemodynamically significant paroxysmal or persistent AF, an initial strategy of rhythm control is recommended regardless of lesion severity as AF in this population is often poorly tolerated.6
2a B-NR 4. In symptomatic patients with simple congenital heart disease with antiarrhythmic drug–refractory AF, it is reasonable to choose ablation over long-term antiarrhythmic therapies.4,7
2b C-LD 5. In adults with congenital heart disease with AF undergoing PVI, it may be reasonable to include an ablative strategy in the right atrium directed at reentrant arrhythmia secondary to atriotomy scars and the CTI.8,9
2b C-LD 6. In adults with AF and moderate or severe forms of congenital heart disease, particularly those with low-flow states such as Fontan circulation, blind-ending cardiac chambers, and cyanosis, it may be reasonable to treat with anticoagulation independent of conventional risk score to reduce risk of thromboembolic events.10