Detecting and managing atrial arrhythmias in adults with congenital heart disease

Atrial arrhythmias are an important source of morbidity in adults with congenital heart disease (CHD) and have been linked to sudden death in certain subgroups. Whereas intra-atrial reentrant tachycardia (IART) is the most common arrhythmia, the prevalence of atrial fibrillation (AF) is increasing. Research efforts have therefore been directed toward early detection and the assessment of management options. Risk stratification for sudden death is challenging in patients with atrial switch surgery for D-transposition of the great arteries in whom some fatal events are provoked by atrial arrhythmias. Kakarla et al1Kakarla J. Crossland D.S. Murray S. et al.An unmet need: arrhythmia detection by implantable loop recorder in the systemic right ventricle.Europace. 2023; 25euad304Crossref Scopus (1) Google Scholar assessed the value of implantable loop recorders in 24 adults with D-transposition of the great arteries/atrial switch (median age 35 years) who had no pacemaker or implantable cardioverter-defibrillator. Moderate or severe systemic right ventricular dysfunction was present in 42%. Over a median follow-up of 40 months, arrhythmias were detected in 75%, prompting changes to clinical management in 64% including anticoagulation in 21%. In particular, IART was detected in 46%. One patient with recurrent IART who declined an implantable cardioverter-defibrillator died suddenly of sustained IART resulting in cardiac arrest. Although the study was underpowered to assess the cost-effectiveness of this monitoring strategy or the value of the implantable loop recorder in sudden death prediction, the high detected burden of arrhythmias suggests a potential role for continuous rhythm monitoring in this population. Wu et al2Wu M.H. Chiu S.N. Tseng W.C. Lu C.W. Kao F.Y. Huang S.K. Atrial fibrillation in adult congenital heart disease and the general population.Heart Rhythm. 2023; 20: 1248-1254Abstract Full Text Full Text PDF PubMed Scopus (5) Google Scholar conducted a nationwide cohort study in Taiwan that included 32,325 adults with CHD (simple in 84%), 7.3% of whom had a diagnostic code for AF between 2003 and 2014. Among the general population without CHD, 347,979 experienced AF within the same time frame. The corresponding annual incidence rates for AF were 7.62 per 1000 vs 1.41 per 1000 among adults with CHD vs the general population. The incidence ratio of AF in adults with CHD/general population was >20 in those younger than 50 years and decreased gradually in higher age categories (Figure 1). On average, AF appears 30 years earlier in adults with CHD than in the general population. Patients with atrial septal defects are among the CHD subgroups with the highest prevalence of AF. Concerns have been raised about the potential for septal occluders to increase the risk of developing AF. A Danish nationwide study3Skibsted C.V. Korsholm K. Pedersen L. Bonnesen K. Nielsen-Kudsk J.E. Schmidt M. Long-term risk of atrial fibrillation or flutter after transcatheter patent foramen ovale closure: a nationwide Danish study.Eur Heart J. 2023; 44: 3469-3477Crossref PubMed Scopus (13) Google Scholar examined the onset of AF or atrial flutter in 817 patients after transcatheter closure of patent foramen ovale (PFO) between 2008 and 2020, along with 1224 patients with an identified PFO but no closure device and 8170 matched controls with no PFO. Among these 3 groups, the 5-year incidence of AF or atrial flutter was 7.8%, 3.1%, and 1.2%, respectively. The corresponding adjusted hazard ratios were 1.6 (95% confidence interval [CI] 1.1–2.6) for PFO closure vs no closure and 8.6 (95% CI 5.5–14) for PFO closure vs matched controls. In comparison to the nonclosed PFO group, the heightened risk period was limited to 3 months after closure (hazard ratio 2.3; 95% CI 1.3–4.0 for the initial 3 months and 0.7; 95% CI 0.3–1.7 thereafter). Catheter ablation is increasingly performed for AF in adults with CHD. Hu et al4Hu T.Y. Janga C. Amin M. et al.Catheter ablation of atrial fibrillation in adult congenital heart disease: procedural characteristics and outcomes.Circ Arrhythm Electrophysiol. 2023; 16: 437-446Crossref PubMed Scopus (3) Google Scholar reported the Mayo Clinic experience with 145 adults with CHD (mean age 57 ± 12 years; 28% female) who had ablation for AF [paroxysmal in 92 (63%) patients] between 2000 and 2020. The type of CHD was simple in 110 (76%), moderate in 26 (18%), and complex in 9 (6%) patients. Pulmonary vein isolation was attempted in all and successful in 134 (92%) patients. Ablation sites apart from the pulmonary veins and cavotricuspid isthmus were pursued in 79 (54%) patients, with a proportion that increased with CHD complexity. The recurrence rates 3–12 months after ablation were 34%, 42%, and 56% in patients with simple, moderate, and complex CHD. In univariable analyses, recurrences were associated with a larger left atrium and inducible IART. Further research is required to refine the selection of appropriate candidates and optimize procedural techniques, including targeting non–pulmonary vein triggers that are common in this population. Anticoagulation is generally indicated in adults with CHD with IART or AF who have moderate or complex CHD or standard risk factors for stroke. Kartas et al5Kartas A, Papazoglou AS, Moysidis DV, et al. Use of apixaban in adults with congenital heart disease and atrial arrhythmias: the PROTECT-AR study [published online ahead of print March 31, 2024]. Int J Cardiol. https://doi.org/10.1016/j.ijcard.2024.131993.Google Scholar conducted a prospective cohort study in 218 adults with CHD (median age 51 years) with AF or IART treated with apixaban and followed for a median of 2.8 years. The type of CHD was simple in 34%, moderate in 39%, and complex in 26%. A historical comparator group consisted of 73 (33%) patients who received an oral non–vitamin K antagonist before enrollment. The annualized rate of stroke or thromboembolism was 0.6% vs 1.8% with apixaban vs vitamin K antagonist, which met the noninferiority criterion. The corresponding rates of major bleeding were 1.5% and 2.4% (P = .64). While the study provides reassuring data in support of direct oral anticoagulants, caution is warranted in extending this therapy to underrepresented high-risk subgroups, such as those with Fontan palliation or severe cyanosis, in whom concerns have been raised. The author has no conflicts of interest to disclose. Dr Khairy was supported by the endowed André Chagnon Research Chair in Electrophysiology and Congenital Heart Disease.

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