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Background: Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease, and sudden cardiac death represents an important mode of death in these patients. Data evaluating the implantable cardioverter defibrillator (ICD) in this patient population remain scarce. Methods: A Nationwide French Registry including all patients with tetralogy of Fallot with an ICD was initiated in 2010 by the French Institute of Health and Medical Research. The primary time to event end point was the time from ICD implantation to first appropriate ICD therapy. Secondary outcomes included ICD-related complications, heart transplantation, and death. Clinical events were centrally adjudicated by a blinded committee. Results: A total of 165 patients (mean age, 42.2±13.3 years, 70.1% males) were included from 40 centers, including 104 (63.0%) in secondary prevention. During a median (interquartile range) follow-up of 6.8 (2.5–11.4) years, 78 (47.3%) patients received at least 1 appropriate ICD therapy. The annual incidence of the primary outcome was 10.5% (7.1% and 12.5% in primary and secondary prevention, respectively; P =0.03). Overall, 71 (43.0%) patients presented with at least 1 ICD complication, including inappropriate shocks in 42 (25.5%) patients and lead dysfunction in 36 (21.8%) patients. Among 61 (37.0%) patients in primary prevention, the annual rate of appropriate ICD therapies was 4.1%, 5.3%, 9.5%, and 13.3% in patients with, respectively, 0, 1, 2, or ≥3 guidelines-recommended risk factors. QRS fragmentation was the only independent predictor of appropriate ICD therapies (hazard ratio, 3.47 [95% CI, 1.19–10.11]), and its integration in a model with current criteria increased the 5-year time-dependent area under the curve from 0.68 to 0.81 ( P =0.006). Patients with congestive heart failure or reduced left ventricular ejection fraction had a higher risk of nonarrhythmic death or heart transplantation (hazard ratio, 11.01 [95% CI, 2.96–40.95]). Conclusions: Patients with tetralogy of Fallot and an ICD experience high rates of appropriate therapies, including those implanted in primary prevention. The considerable long-term burden of ICD-related complications, however, underlines the need for careful candidate selection. A combination of easy-to-use criteria including QRS fragmentation might improve risk stratification. Registration: URL: https://www.clinicaltrials.gov ; Unique identifier: NCT03837574.

International audience; Background: Women with congenital heart disease at high risk for sudden cardiac death have been poorly studied thus far.Objectives: The aim of this study was to assess sex-related differences in patients with tetralogy of Fallot (TOF) and implantable cardioverter-defibrillators (ICDs).Methods: Data were analyzed from the DAI-T4F (French National Registry of Patients With Tetralogy of Fallot and Implantable Cardioverter Defibrillator) cohort study, which has prospectively enrolled all patients with TOF with ICDs in France since 2010. Clinical events were centrally adjudicated by a blinded committee.Results: A total of 165 patients (mean age 42.2 ± 13.3 years) were enrolled from 40 centers, including 49 women (29.7%). Among the 9,692 patients with TOF recorded in the national database, the proportion of women with ICDs was estimated to be 1.1% (95% CI: 0.8%-1.5%) vs 2.2% (95% CI: 1.8%-2.6%) in men (P < 0.001). The clinical profiles of patients at implantation, including the number of risk factors for ventricular arrhythmias, were similar between women and men. During a median follow-up period of 6.8 years (IQR: 2.5-11.4 years), 78 patients (47.3%) received at least 1 appropriate ICD therapy, without significant difference in annual incidences between women (12.1%) and men (9.9%) (HR: 1.22; 95% CI: 0.76-1.97; P = 0.40). The risk for overall ICD-related complications was similar in women and men (HR: 1.33; 95% CI: 0.81-2.19; P = 0.30), with 24 women (49.0%) experiencing at least 1 complication.Conclusions: Our findings suggest that women with TOF at high risk for sudden cardiac death have similar benefit/risk balance from ICD therapy compared with men. Whether ICD therapy is equally offered to at-risk women vs men warrants further evaluation in TOF as well as in other congenital heart disease populations. (French National Registry of Patients With Tetralogy of Fallot and Implantable Cardioverter Defibrillator [DAI-T4F]; NCT03837574)

International audience; Objectives: This study aimed to assess the impact of pulmonary valve replacement (PVR) on ventricular arrhythmias burden in a population of tetralogy of Fallot (TOF) patients with continuous cardiac monitoring by implantable cardioverter-defibrillators (ICDs).Background: Sudden cardiac death is a major cause of death in TOF, and right ventricular overload is commonly considered to be a potential trigger for ventricular arrhythmias.Methods: Data were analyzed from a nationwide French ongoing study (DAI-T4F) including all TOF patients with an ICD since 2000. Survival data with recurrent events were used to compare the burden of appropriate ICD therapies before and after PVR in patients who underwent PVR over the study period.Results: A total of 165 patients (mean age 42.2 ± 13.3 years, 70.1% male) were included from 40 centers. Over a median follow-up period of 6.8 (interquartile range: 2.5 to 11.4) years, 26 patients (15.8%) underwent PVR. Among those patients, 18 (69.2%) experienced at least 1 appropriate ICD therapy. When considering all ICD therapies delivered before (n = 62) and after (n = 16) PVR, the burden of appropriate ICD therapies was significantly lower after PVR (HR: 0.21; 95% confidence interval [CI]: 0.08 to 0.56; p = 0.002). Respective appropriate ICD therapies rates per 100 person-years were 44.0 (95% CI: 35.7 to 52.5) before and 13.2 (95% CI: 7.7 to 20.5) after PVR (p < 0.001). In the overall cohort, PVR before ICD implantation was also independently associated with a lower risk of appropriate ICD therapy in primary prevention patients (HR: 0.29 [95% CI: 0.10 to 0.89]; p = 0.031).Conclusions: In this cohort of high-risk TOF patients implanted with an ICD, the burden of appropriate ICD therapies was significantly reduced after PVR. While optimal indications and timing for PVR are debated, these findings suggest the importance of considering ventricular arrhythmias in the overall decision-making process. (French National Registry of Patients With Tetralogy of Fallot and Implantable Cardioverter Defibrillator [DAI-T4F]; NCT03837574).

Introduction Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease, and sudden cardiac death represents an important mode of death in these patients. Data evaluating the implantable cardioverter defibrillator (ICD) in this patient population remain scarce. Objective We aimed to describe long-term follow-up of TOF patients implanted with ICD through a nationwide French registry. Methods Nationwide French Registry including all TOF patients with an ICD initiated in 2010 by the French Institute of Health and Medical Research. The primary time to event endpoint was the time from ICD implantation to first appropriate ICD therapy. Clinical events were centrally adjudicated by a blinded committee. Results A total of 165 patients (mean age 42.2 ± 13.3 years, 70.1% males) were included from 40 centers, including 104 (63.0%) in secondary prevention. During a median (IQR) follow-up of 6.8 (2.5–11.4) years, 78 (47.3%) patients received at least one appropriate ICD therapy. The annual incidence of the primary outcome was 10.5% (7.1% and 12.5% in primary and secondary prevention, respectively, P = 0.03). Overall, 71 (43.0%) patients presented with at least one ICD complication, including inappropriate shocks in 42 (25.5%) patients and lead dysfunction in 36 (21.8%) patients. Among 61 (37.0%) primary prevention patients, the annual rate of appropriate ICD therapies was 4.1%, 5.3%, 9.5%, and 13.3% in patients with respectively no, one, two, or ≥ three guideline-recommended risk factors. QRS fragmentation was the only independent predictor of appropriate ICD therapies (HR 3.47, 95% CI 1.19–10.11), and its integration in a model with current criteria increased the 5-year time-dependent area under the curve from 0.68 to 0.81 (P = 0.006) ( Fig. 1 ). Conclusions Patients with TOF and an ICD experience high rates of appropriate therapies, including those implanted in primary prevention. The considerable long-term burden of ICD-related complications, however, underlines the need for careful candidate selection. A combination of easy-to-use criteria including QRS fragmentation might improve risk stratification.

Background Ventricular arrhythmias and sudden death are feared late complications in patients with tetralogy of Fallot. Selection of candidates for primary prevention implantable cardioverter defibrillator (ICD) remains challenging in this population. Non-sustained ventricular tachycardia (NSVT), altered left ventricular ejection fraction (LVEF), positive programmed ventricular stimulation, and enlarged QRS are currently used for risk stratification. Purpose To identify high-risk patients with tetralogy of Fallot in the setting of primary prevention of sudden cardiac death. Methods The DAI-T4F study is a large ongoing national French registry including all patients with tetralogy of Fallot and ICD (NCT03837574). Information have been collected prospectively since 2010 with annual update. Baseline patient characteristics and clinical events during the follow-up were analyzed with central adjudication. Cox proportional hazard models were used to identify factors associated with appropriate ICD therapies. Results Among 134 patients enrolled, 47 (35.1%) underwent ICD implantation for primary prevention (median age 49.1 years, 76.6% males). At baseline, 20 (42.6%) patients had NSVT, 17 (36.2%) had severe altered LVEF ≤35%, 16 (34.0%) had positive programmed ventricular stimulation, and 16 (34.0%) had QRS duration ≥180ms. Overall, 20 (42.6%), 15 (31.9%), and 6 (12.8%) patients had respectively one, two, or ≥ three of these risk factors. Six (12.8%) patients were implanted for other indications. During a median (IQR) follow-up duration of 5.3 (2.1–8.0) years, 14 (29.8%) patients had at least one appropriate ICD therapy. The annual incidence of appropriate ICD therapies were 2.8%, 4.6%, 6.3%, and 8.6% in patients with none, one, two, or ≥ three of these factors (p for trend = 0.145). None of predictors, considered isolated, was significantly associated with ICD appropriate therapies. Patients with non-sustained ventricular tachycardia (NSVT) and positive programmed ventricular stimulation had a significant increased risk of ICD appropriate therapies (HR=3.8, 95% CI: 1.1–14.3, p=0.035), as well as patients with NSVT and QRSd ≥180 ms (HR=7.2, 95% CI: 1.6–32.7, p=0.003). No patient with severe altered LVEF without other risk factor had appropriate ICD therapy. Patients with congestive heart failure and/or altered LVEF had a higher risk of non-sudden death or cardiac transplantation (HR=14.4, 95% CI: 1.8–112.7, p Conclusions Our data illustrate that specific risk stratification and primary prevention for sudden cardiac death in patients with tetralogy of Fallot may be improved. The value of a severely altered LVEF appears low in the absence of other risk factors, and combination of different predictors is essential. The high rate of complications as well as consideration of competing risk situation have to be integrated in the benefit-risk equation.

Background Ventricular arrhythmias and sudden death are feared late complications in patients with tetralogy of Fallot. Selection of candidates for primary prevention implantable cardioverter defibrillator (ICD) remains challenging. Purpose To identify patients with tetralogy of Fallot at high-risk of sudden cardiac death in primary prevention. Methods The DAI-T4F study is an ongoing national French registry including all patients with tetralogy of Fallot and ICD ( NCT03837574 ). Information have been collected prospectively since 2010. Cox proportional hazard models were used to identify factors associated with appropriate ICD therapies. Results Among 134 patients enrolled, 47 (35.1%) underwent ICD implantation for primary prevention (median age 49.1 years, 76.6% males). At baseline, 20 (42.6%) patients had NSVT, 17 (36.2%) had altered LVEF ≤ 35%, 16 (34.0%) had positive programmed ventricular stimulation, and 16 (34.0%) had QRS duration ≥ 180ms. During a median (IQR) follow-up duration of 5.3 (2.1–8.0) years, 14 (29.8%) patients had at least one appropriate ICD therapy. The annual incidence of appropriate ICD therapies were 2.8%, 4.6%, 6.3%, and 8.6% in patients with none, one, two, or ≥ three of these factors (P for trend = 0.145). None of predictors, considered isolated, was significantly associated with ICD appropriate therapies. Patients with non-sustained ventricular tachycardia (NSVT) and positive programmed ventricular stimulation had a significant increased risk of ICD appropriate therapies (HR = 3.8, 95% CI: 1.1–14.3, P = 0.035), as well as patients with NSVT and QRSd ≥ 180 ms (HR = 7.2, 95% CI: 1.6–32.7, P = 0.003). No patient with severe altered LVEF without other risk factor had appropriate ICD therapy. Conclusions Our data illustrate that specific risk stratification and primary prevention for sudden cardiac death in patients with tetralogy of Fallot may be improved. The value of a severely altered LVEF appears low in the absence of other risk factors.

Summary Background Meta-analyses have confirmed the high performance of multislice computed tomography (MSCT) in coronary stenosis detection. Recent reports have described the study of left ventricular anatomy and function and coronary venous anatomy with MSCT. Aims We sought to compare, in patients with cardiomyopathy of unknown origin, the performance of MSCT versus angiography for significant coronary artery disease detection and versus transthoracic echocardiography (TTE) for left ventricular anatomy and function evaluation, and to assess its ability to characterize coronary venous anatomy. Methods Fifty-nine patients with cardiomyopathy (left ventricular ejection fraction [LVEF] less than or equal to 40%) of unknown origin, in sinus rhythm, underwent MSCT, TTE and coronary angiography. Results Twenty-four (3%) of 724 analysable coronary segments (97%) and 12 (20%) patients had significant coronary artery disease. MSCT sensitivity, specificity, and positive and negative predictive values for coronary artery disease detection were 87.5%, 98.5%, 67.7% and 99.6% in the per-segment assessment and 100%, 91%, 75% and 100% in the per-patient evaluation, respectively. Statistical analyses showed good agreement between MSCT and TTE in LVEF measurement (33 ± 10% vs 32 ± 11%, p = 0.4, mean difference = 0.7%, limits of agreement ± 13.6%) and a small LVED diameter overestimation (65.0 ± 9.3 mm vs 63.6 ± 9.4 mm, p = 0.03). MSCT allowed detection of the posterolateral vein in 86% of cases. Conclusions In selected patients presenting with idiopathic cardiomyopathy, MSCT is accurate for coronary artery disease detection and is a useful coronary venous imaging tool. MSCT studies of left ventricular function and morphology were mostly concordant with TTE measurements.