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CLINICAL STATEMENTS AND GUIDELINES

January 2, 2024 Circulation. 2024;149:e1–e156. DOI: 10.1161/CIR.0000000000001193 e100

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

Recommendation-Specific Supportive Text 1.	 Preexcited AF can conduct rapidly over the path-

way and lead to VF. During an acute episode of preexcited AF with rapid ventricular response and hemodynamic instability, electrical cardioversion must be used to restore sinus rhythm.1,2

2 Pathway ablation has well-established efficacy and

safety for those with preexcited AF for prevention of VF.1 Multiple large-patient series point to a high success rate (93%-95%) balanced with low risk of major short- and long-term complications.3–7 Electrophysiological study and AP ablation can be offered as first-line therapy. 3.	 Pharmacological treatments that increase the

refractory period of atrial tissue (ie, ibutilide or procainamide) can slow AP conduction and terminate AF. Hemodynamically stable patients presenting with preexcited AF with or without rapid ventricular rates can be pharmacologically managed with such agents.8,9 In 1 study involving 22 patients, ibutilide terminated AF in 21 patients and prolonged refractoriness of the atrioventricular node, His-Purkinje system, and AP.9

4 AP conduction can accelerate with atrioventricu-

lar nodal block. Administration of pharmacological agents that block atrioventricular nodal conduction can increase the risk of VF. The use of atrioventricular nodal blocking agents for patients with preexcited AF are potentially harmful due to the risk of accelerated pathway conduction and associated VF.1,10–12 One small series that reviewed intravenous amiodarone for this indication raised significant concerns in view of several published reports on instances of VF precipitated by amiodarone.13 In 1 study of patients with WPW who received verapamil during electrophysiological study, verapamil decreased the shortest RR interval between preexcited ventricular complexes during AF, and 2 patients deteriorated and required cardioversion.14 Data are very limited and observational, but because other safer options are readily available, atrioventricular nodal agents are best avoided in this setting.

10.7 Hypertrophic Cardiomyopathy

The “AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy” was published in December 2020, and this guideline included recommendations on AF.1 Data on AF in patients with HCM published since the 2020 hypertrophic cardiomyopathy guideline were reviewed but did not result in any substantive changes in the recommendations from 2020.2–6 AF is highly prevalent in HCM and increases the risk of stroke. The CHA2DS2-VASc score should not apply to patients with HCM, because they have roughly an equivalent risk to a CHADs-VASc of 3. Data on the use of DOACs in patients with HCM suggest these to be acceptable alternatives to warfarin. When a rhythm-control strategy is needed, several antiarrhythmic drugs have been shown to be safe and effective, allowing for individualization according to underlying substrate and patient preference. Catheter ablation is also an important option, although more confirmatory studies have shown AF ablation is of lower success compared with patients without HCM.7 For specifics, please see the “2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy.”1

10.8 Adult Congenital Heart Disease (ACHD)

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 congeni-

tal 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.3–5

1 C-LD 3 In adults with congenital heart disease and symp-

tomatic 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, blindending cardiac chambers, and cyanosis, it may be reasonable to treat with anticoagulation independent of conventional risk score to reduce risk of thromboembolic events.10

Synopsis Atrial arrhythmias are a leading cause of morbidity in adults with congenital heart disease, and AF surpasses intra-atrial reentrant tachycardia in patients with CHD >50 years of age.11 Risk factors for development of AF in patients with ACHD are similar to those for the general population; however, the presence of an atrial

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