Background—: Sudden cardiac death and life-threatening ventricular arrhythmia remain a concern in adult patients with repaired tetralogy of Fallot. Longitudinal left ventricular (LV) function is sensitive in detecting early myocardial damage and may have prognostic implications in this setting. Methods and Results—: We included 413 tetralogy of Fallot patients (age, 36±13 years; QRS duration, 148±27 milliseconds; LV ejection fraction, 55±10%). A composite end point of sudden cardiac death/life-threatening ventricular arrhythmia (sustained ventricular tachycardia, resuscitated sudden cardiac death, or appropriate implantable cardioverter-defibrillator discharge) was used. During a median follow-up of 2.9 years, 5 patients died suddenly, 9 had documented sustained ventricular tachycardia, and another 5 had appropriate implantable cardioverter-defibrillator shocks. On univariate Cox analysis, QRS duration (hazard ratio [HR], 1.02 per 1 ms; P =0.046), right atrial area (HR, 1.05 per 1 cm ² ; P =0.02), right ventricular fractional area change (HR, 0.94 per 1%; P =0.02), right ventricular outflow tract diameter (HR, 1.08 per 1 mm; P =0.01), mitral annular plane systolic excursion (HR, 0.84 per 1 mm; P =0.03), and LV global longitudinal 2-dimensional strain (HR, 0.87 per 1%; P =0.03) were related to the combined end point. On bivariable analysis, mitral annular plane systolic excursion and LV global longitudinal 2-dimensional strain were related to outcome independently of QRS duration ( P =0.002 and P =0.01, respectively). In addition, a combination of echocardiographic variables, including right atrial area, right ventricular fractional area change, and LV global longitudinal 2-dimensional strain or mitral annular plane systolic excursion, was also found to be significantly related to outcome ( P <0.001; c statistic, 0.70). Conclusions—: LV longitudinal dysfunction was associated with greater risk of sudden cardiac death/life-threatening ventricular arrhythmias. In combination with echocardiographic right heart variables, also available from routine echocardiography, these measures provide important outcome information and should be considered a useful adjunct to established markers such as QRS duration in the estimation of prognosis in this challenging population.

The aim of this study was to analyze the pacemaker implantation rate (PMIR) with the new balloon-expandable Edwards SAPIEN 3 valve (S3) and the factors associated with it. The introduction of the S3 for transcatheter aortic valve replacement (TAVR) has led to a reduction in paravalvular regurgitation. There are, however, concerns that the new design may increase the PMIR. The first 206 patients treated with the S3 were compared with 371 preceding patients treated with SAPIEN XT valves. Patients who previously underwent pacemaker or implantable cardioverter defibrillator implantation or transapical and valve-in-valve procedures were excluded from the analysis. All patients were monitored for at least 7 days. Previous and new conduction abnormalities were documented, and prosthesis implantation height was assessed for the S3. There were no significant differences in baseline characteristics between groups. The PMIR was, however, significantly higher for the S3 (19.1% vs. 12.2%; p = 0.046). The mean implantation height was significantly lower in patients requiring PMI (67%/33% vs. 72%/28% aortic/ventricular stent extension, p = 0.032). On multivariate regression analysis, implantation height was the only independent predictor of PMI (odds ratio: 0.94 [95% confidence interval: 0.90 to 0.99]; p = 0.009). It increased from 68%/32% to 75%/25% when comparing the first with the second half of S3 implantations (p < 0.0001). This change was associated with a significant decrease in PMIR from 25.9% to 12.3% (p = 0.028), no longer different from the XT valve (12.2%). The PMIR after TAVR is higher with the S3 than with the XT and is independently associated with the implantation height. This increase in the PMIR may be avoided by intending an aortic stent extension >70%.

Parameters of myocardial deformation have been suggested to be superior to conventional measures of ventricular function in patients with tetralogy of Fallot (ToF), but have required non-routine, tagged cardiovascular magnetic resonance (CMR) techniques. We assessed biventricular myocardial function using CMR cine-based feature tracking (FT) and compared it to speckle tracking echocardiography (STE) and to simple endocardial border delineation (EBD). In addition, the relation between parameters of myocardial deformation and clinical parameters was assessed.

Background Parameters of myocardial deformation have been suggested to be superior to conventional measures of ventricular function and to predict outcome in repaired tetralogy of Fallot (ToF). We aimed to test the hypothesis that parameters of myocardial deformation on cardiac MRI (CMR) relate to symptoms and provide prognostic information in patients with repaired ToF. Methods and results We included 372 patients with ToF (median age 16 years; 55% male), recruited within a nationwide, prospective study. Longitudinal (LS), circumferential (CS) and radial global strain (RS) were analysed by CMR-based feature tracking (FT). A combined endpoint of death, successful resuscitation or documented ventricular tachycardia was employed. Parameters of global strain were associated with New York Heart Association (NYHA) class and symptomatic deterioration. During a median follow-up of 7.4 years, 20 events occurred. Left ventricular (LV) CS and right ventricular (RV) LS emerged as predictors of outcome, independent of QRS duration, LV/RV ejection fraction and volumes, NYHA class and peak oxygen uptake. In combination, these parameters also identified a subgroup of patients at significantly increased risk of adverse of outcomes (HR 3.3, p=0.002). Furthermore, LV LS, RS, CS and RV LS were related to the risk of death and nearly missed death (p<0.05 for all). Conclusions FT-CMR provides myocardial deformation parameters, easily derived from standard CMR studies. They relate to symptoms and clinical deterioration in patients with ToF. More importantly, they predict adverse outcome independent of established risk markers, and should be considered as a useful adjunct to established outcome predictors, especially in younger patients with ToF. Clinical trial registration number http://www.clinicaltrials.gov: NCT00266188; Results.