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ObjectivePatients with repaired tetralogy of Fallot (TOF) are followed serially by cardiac magnetic resonance (CMR) for surveillance of RV dilation and dysfunction. We sought to define the prevalence of progressive RV disease and the optimal time interval between CMR evaluations.MethodsCandidates were selected from a multicentre TOF registry and were included if ≥2 CMR studies performed ≥6 months apart were available without interval cardiovascular interventions. Patients with ‘disease progression’ (defined as increase in RV end-diastolic volume index (RVEDVi) ≥30 mL/m2, decrease in RVEF ≥10% or decrease in LVEF ≥10%) were compared with those with ‘disease non-progression’ (defined as RVEDVi increase ≤5 mL/m2, RVEF decrease ≤3% and LVEF decrease ≤3%).ResultsA total of 849 CMR studies in 339 patients (median age at first CMR 23.6 years) were analysed. Over a median interval of 2.2 years between CMR pairs, RVEDVi increased 4±18 mL/m2 (p<0.001), RV end-systolic volume index increased 3±13 mL/m2 (p<0.001), RVEF decreased 1%±6% (p=0.02) and LVEF decreased 1%±6% (p=0.001). Disease progression was observed in 15% (n=76) and non-progression in 26% (n=133). There were no significant differences between those with and without progression in baseline demographic, anatomic, ECG, exercise or baseline CMR characteristics. The optimal time interval between CMR studies for detection of progression was a 3-year interval (63% sensitivity, 65% specificity, area under the receiver operating characteristic curve 0.65).ConclusionsAlthough progressive RV dilation and decline in biventricular systolic function occur at a slow pace in the majority of adults with repaired TOF, 15% of patients experience rapid disease progression. The results of this study support the practice of serial CMR examinations to identify progressive disease at a time interval of up to 3 years.

Supraventricular tachycardias (SVTs) are a major cause of morbidity in adults with congenital heart disease (CHD). Few data exist on safety and efficacy of antiarrhythmic drugs in this population. Our aim was to determine the efficacy of antiarrhythmic drugs in adults with CHD and first-onset SVT on maintaining sinus rhythm after conversion. This was a multicenter retrospective study including adults with CHD and first-onset SVT from January 2008 to January 2011. First-onset SVT occurred in 92 of 7,171 patients without previous SVT (mean age 51 ± 16 years, 57% women). SVTs included atrial fibrillation and flutter in >80% of the patients. Most of these patients had septal defects (50%) and left-sided lesions (21%). The acute management of SVTs resulted in sinus rhythm in 83 patients, and 89% of these patients were instituted on oral antiarrhythmics to prevent SVT recurrence. After a mean follow-up of 2.5 ± 1.4 years, only 45% of the patients were free from SVT. Class III antiarrhythmics (85% sotalol and 15% amiodarone) were associated with a significantly lesser risk of SVT recurrence compared with all other antiarrhythmic drugs (hazard ratio 0.5, 95% confidence interval 0.27 to 0.96, p = 0.036). However, adverse effects of medication occurred in 22% of the patients, mainly in patients taking amiodarone. In conclusion, in adults with CHD and first-onset SVTs, class III antiarrhythmics are more efficacious in maintaining sinus rhythm after cardioversion than other antiarrhythmics. Sotalol may be considered as the first-choice therapy as this is associated with fewer adverse effects than amiodarone.

Objective Patients with repaired tetralogy of Fallot (TOF) experience increased rates of mortality and morbidity in adulthood. This study was designed to identify risk factors for death and ventricular tachycardia (VT) in a large contemporary cohort of patients with repaired TOF. Methods Subjects with repaired TOF from four large congenital heart centres in the USA, Canada and Europe were enrolled. Clinical, ECG, exercise, cardiac magnetic resonance (CMR) and outcome data were analysed. Results Of the 873 patients (median age 24.4 years), 32 (3.7%) reached the primary outcome (28 deaths, 4 sustained VT; median age at outcome 38 years; median time from CMR to outcome 1.9 years). Cox proportional-hazards regression identified RV mass-to-volume ratio ≥0.3 g/mL (HR, 5.04; 95% CI 2.3 to 11.0; p<0.001), LV EF z score<−2.0 (HR, 3.34; 95% CI 1.59 to 7.01; p=0.001), and history of atrial tachyarrhythmia (HR, 3.65; 95% CI 1.75 to 7.62; p=0.001) as outcome predictors. RV dysfunction was predictive of the outcome similar to LV dysfunction. In subgroup analysis of 315 subjects with echocardiographic assessment of RV systolic pressure, higher pressure (HR 1.39; 95% CI 1.19 to 1.62; p<0.001) was associated with death and sustained VT independent of RV hypertrophy and LV dysfunction. Conclusions RV hypertrophy, ventricular dysfunction and atrial tachyarrhythmias are predictive of death and sustained VT in adults with repaired TOF. These findings may inform risk stratification and the design of future therapeutic trials.

The process of establishing a core congenital CMR laboratory for uniform data analysis and an electronic database for standardized data entry are the first steps to identifying predictors of adverse outcomes meaningful to this patient population.

Although early survival after tetralogy of Fallot (TOF) repair in the modern era is excellent, studies on late outcomes have shown increasing rates of mortality and morbidity. Despite multiple publications on factors associated with late complications, risk factors for major outcomes (death and sustained ventricular tachycardia [VT]) remain poorly defined. Consequently, the International Multicenter TOF Registry (INDICATOR) was established. This article describes the development, structure, and goals of this registry and characterizes the initial cohort derived from four large congenital heart centers in the United States, Canada, and Europe. A data coordinating center with a core cardiac magnetic resonance (CMR) laboratory and statistical core was established. Subjects with repaired TOF who had CMR imaging performed between 1997 and 2010 and ≥1 year follow-up were included. Clinical end points were death and sustained VT. Demographic, electrophysiologic, exercise, and outcome data were collected. A total of 873 subjects fulfilled inclusion criteria (median age at repair 2.9 years and at CMR imaging 22.8 years). Of these, 9 % had QRS duration >180 ms on electrocardiogram (ECG). On CMR imaging, 38 % had severe right-ventricular (RV) dilatation (≥160 mL/m2), and 6 % had severe RV dysfunction (ejection fraction < 35 %). Of the 551 subjects with exercise testing available, 28 % had severely decreased exercise capacity with <50 % predicted peak oxygen consumption. The INDICATOR cohort allows robust statistical analysis to evaluate major clinical outcomes in patients with repaired TOF. Continued follow-up and further expansion of the registry may provide new insights into innovative therapeutic strategies to improve late outcomes.

Arrhythmias are a major cause of morbidity, mortality and hospital admission in adults with congenital heart disease (CHD). The etiology of arrhythmias in this population is often multifactorial and includes electrical disturbances as part of the underlying defect, surgical intervention or hemodynamic abnormalities. Despite the numerous existing arrhythmia management tools including drug therapy, pacing and ablation, management of arrhythmias in adults with CHD remains difficult and challenging. Owing to improvement in mapping and ablation techniques, ablation and arrhythmia surgery are being performed more frequently in adults with CHD. However, there is little information on the long-term results of these treatment strategies. The purpose of this article is therefore to review the available data on nonpharmacological treatment of cardiac arrhythmias in adult patients with CHD and to give an overview of the available data on the early and late outcomes of these treatment strategies.