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CLINICAL STATEMENTS AND GUIDELINES

Circulation. 2024;149:e1–e156. DOI: 10.1161/CIR.0000000000001193 January 2, 2024 e101

Joglar et al 2023 Guideline for the Diagnosis and Management of Atrial Fibrillation

septal defect is an independent risk factor. Adults may be classified as having simple, moderate, or complex forms of CHD, based on a classification proposed in the “2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease.”12 Choosing pharmacological therapy for treatment of patients with ACHD who have AF should consider factors such as coexisting sinus node dysfunction or atrioventricular block, as well as the ventricular function and other comorbidities. Ablation strategies established in normal hearts can be applied to patients with simple forms of CHD; however, caution must be taken in those patients with moderate or complex forms of ACHD.13 Knowledge of the underlying congenital anatomy is key, because access to the atrial chambers may require specialized approaches. In those patients with moderate or complex forms of CHD, electrophysiological procedures should be performed by operators with expertise in CHD and in collaboration with an ACHD cardiologist.3

Recommendation-Specific Supportive Text 1.	 Adults with CHD often have hemodynamic sequelae

of childhood repair, and any new onset arrhythmia, including AF, should prompt an evaluation for treatable and reversible causes. Studies have shown that AF is correlated with RA dilation, thus necessitating addressing the underlying substrate in addition to treating the arrhythmia.1,2

2 Patients with ACHD who are undergoing inva-

sive electrophysiological procedures in specialized ACHD centers generally have better outcomes, including survival, than those managed in other care settings.3–5 Special attention is required to ensure appropriate periprocedural care, including identification of procedure-related risk factors and availability of ancillary imaging.12

3 Adults with simple forms of CHD and AF are gen-

erally managed according to standard published guidelines. In those patients with moderate or complex forms of CHD, choice of antiarrhythmic therapy should be individualized.6 Most antiarrhythmic drugs are associated with proarrhythmic effects and increased mortality in ACHD patients.14 Rhythm control is generally preferred over rate control in patients with ACHD with moderate or complex disease who may tolerate AF poorly. Class I antiarrhythmic drugs must be avoided in patients with ACHD with CAD or significant subpulmonary or systemic ventricular dysfunction.14–16 Amiodarone is an effective antiarrhythmic agent for maintaining sinus rhythm in patients with ACHD; however, long-term therapy is limited by adverse effects, particularly amiodarone-induced thyrotoxicosis, which is common in women with CHD, cyanotic CHD, and those with Fontan physiology.17 Amiodarone should also be avoided in patients with ACHD who have a BMI <21 kg/m2 due to the increased incidence of thyrotoxicosis in these patients.18 Dofetilide may be a viable alternative to amiodarone in this complex population.19 Extrapolating data from noncongenital populations, dronedarone is best avoided in patients with ACHD who have moderate or complex disease or significant ventricular dysfunction due to the increased risk of HF, stroke, and death.20,21

4 Data on ablative strategies for AF in patients with

ACHD are limited; however, when performed by experienced centers, AF ablation in patients with simple forms of CHD is safe.4,13 Knowledge of the underlying congenital anatomy is key, as access to the atrial chambers may require different approaches, including transseptal puncture. Patients with ACHD who have persistent AF have lower success rates for ablation, and multiple procedures may be required.22 There are insufficient data on the role, safety, and benefit of AF ablation in complex forms of CHD. 5.	 PVI has a lower success rate in patients with

ACHD than in patients with structurally normal hearts. The operator should understand the variations in systemic and pulmonary venous anatomy, such as a left superior vena cava, which may be a trigger for AF.9 Due to related pathology in the right atrium of patients with repaired complex ACHD, additional ablative therapies directed at atriotomy scars and the CTI may be necessary. Cryoballoon ablation is a safe option for patients with simple or moderate forms of CHD and AF.23,24

6 All patients with CHD should be considered

for some form of thromboembolic prophylaxis. Select groups of patients with CHD are at higher risks for thromboembolic events, such as cyanotic lesions, those with low-flow states (Fontan physiology), and those with blind-ending cardiac chambers. The prevalence of cerebrovascular accidents in patients with ACHD is estimated to be 10- to 100-fold higher than age-matched controls, and thromboembolic events account for at least 4% of all-cause mortality in patients with CHD.25 Increased complexity of CHD is associated with thromboembolic events, but CHA2DS2 and CHA2DS2-VASc scores are not predictive of thromboembolic risk in patients with ACHD; therefore, therapies must be individualized.10 Limited data are available for the use of DOACs in patients with ACHD, but in a survey, they appeared to be safe and effective.26

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