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Background: Adults with repaired Tetralogy of Fallot (rTOF) comprise one of the largest cohorts among adults with congenital heart disease (ACHD). These patients have a higher burden of atrial arrhythmias (AA), leading to increased adverse events, including stroke and transient ischemic attack (TIA). However, the data on factors associated with stroke/TIA in rTOF are limited, and classic risk factors may not apply. We studied event rates and associated factors for thromboembolism in a rTOF cohort. Methods: Retrospective cohort study of all adult patients age >18 years with rTOF followed at a single ACHD tertiary care center. AA of interest were atrial fibrillation (AF) and atrial flutter (AFL). Results: Data from 260 patients were identified, mean age 37.6 SD 13.3 years, followed over 5108 patient‐years (mean 16.6 SD 8.2 years). 43 patients had AF and/or AFL, and 30 patients had thromboembolic events, of which 19 patients had stroke/TIA. The event rate for any thromboembolism was 3.39 per 100 patient‐years follow‐up in patients with AA, compared to 1.80 in patients without (P =.07). In univariate analysis, older age and diabetes were associated with thromboembolic events. In multivariate analysis, only older age was associated with thromboembolic events. Conclusions: In our relatively young cohort of adults with rTOF, there was a high prevalence of AA, associated with nearly double the rate of thromboembolic events compared to patients without AA. Older age alone is independently associated with thromboembolic events. Further studies into assessment of silent AA are required, and routine assessments should be considered at an earlier age. [ABSTRACT FROM AUTHOR]

Special considerations for the management of adult congenital heart disease include pregnancy-related cardiovascular disease, pulmonary arterial hypertension, and arrhythmias. Improvements in the treatment of congenital heart disease mean that more women with congenital heart disease are reaching childbearing age. In BC the Cardiac Obstetrics clinic at St. Paul's Hospital provides coordinated care for pregnant women with cardiac conditions. The clinic also offers preconception counseling so that couples can make informed choices about pregnancy. Another service based at St. Paul's Hospital, the Pacific Adult Congenital Heart clinic, helps manage patients with pulmonary arterial hypertension, a progressive condition affecting around 10% of adult congenital heart disease patients. Agents used to treat pulmonary arterial hypertension include endothelin receptor antagonists and phosphodiesterase type 5 inhibitors. The Pacific Adult Congenital Heart clinic also manages patients with a significant arrhythmia, often the first manifestation of deterioration in complex congenital heart disease. Complications that can result from arrhythmias include heart failure, thromboembolism, and sudden cardiac death. A range of complementary imaging modalities aid in the management of all these conditions by enabling the assessment of ventricular and valvular function (echocardiography and magnetic resonance imaging), quantification of right ventricular volume (multidetector computed tomography), and exclusion of coronary stenosis (coronary CT angiography). [ABSTRACT FROM AUTHOR]

Adults with congenital heart disease (ACHD) experience a high prevalence of atrial arrhythmia (AA) and thromboembolic cerebrovascular complications. However, data on AA and associated long-term outcomes are limited in ACHD patients with coarctation of the aorta (CoA). This study aimed to characterize the prevalence and risk factors for AA and thromboembolic complications in adults with CoA. We conducted a retrospective cohort study in a tertiary ACHD care center and included consecutive CoA patients older than 18 years old with more than one year of follow-up. Two hundred seventy patients with CoA were followed for 7.2 ± 3.95 years. The mean age was 35.3 ± 11.1 and 55.2% were male. Patients had a mean of 2.1 ± 1.8 cardiovascular surgical or transcatheter procedures. Thirty-five patients (13%) had AA. Ten subjects (3.8%) had a thromboembolic cerebrovascular event, of which four (1.4%) had AA. In univariate analysis, age (p = 0.005) and total intracardiac interventions (p = 0.007) were associated with the presence of AA. Age (p = 0.021), history of heart failure (p = 0.022), and dyslipidemia (p = 0.019) were associated with thromboembolism. In multivariate analysis, age (p < 0.001) and intracardiac interventions (p = 0.007) were associated with AA. The rate of AA is higher in adults with CoA than in the general population but lower than in other ACHD. Increasing age and intracardiac interventions were associated with AA. The rate of thromboembolic events was low. Some traditional risk factors for stroke may apply. Larger studies are needed to validate predictors for stroke in this population. • Atrial arrhythmia occurs at a greater rate in adults with aortic coarctation. • Increasing age and intracardiac interventions are associated with atrial arrhythmia. • Strokes develop at younger ages in adults with aortic coarctation. • Larger studies are required to better characterize risk factors for stroke. [ABSTRACT FROM AUTHOR]