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In repaired tetralogy of Fallot (TOF), little is known about characteristics of patients with rapid ventricular tachycardia (VT). Also, whether patients with a first episode of nonrapid VT may subsequently develop rapid VT or ventricular fibrillation (VF) has not been addressed.The objectives of this study were to compare patients with rapid VT/VF with those with nonrapid VT and to assess the evolution of VT cycle lengths (VTCLs) overtime.Data were analyzed from a nationwide registry including all patients with TOF and implantable cardioverter-defibrillator (ICD) since 2000. Patients with ≥1 VT episode with VTCL ≤250 ms (240 beats/min) formed the rapid VT/VF group.Of 144 patients (mean age 42.0 ± 12.7 years; 104 [72%] men), 61 (42%) had at least 1 VT/VF episode, including 28 patients with rapid VT/VF (46%), during a median follow-up of 6.3 years (interquartile range 2.2-10.3 years). Compared with patients in the nonrapid VT group, those in the rapid VT/VF group were significantly younger at ICD implantation (35.2 ± 12.6 years vs 41.5 ± 11.2 years; P = .04), had more frequently a history of cardiac arrest (8 [29%] vs 2 [6%]; P = .02), less frequently a history of atrial arrhythmia (11 [42%] vs 22 [69%]; P = .004), and higher right ventricular ejection fraction (43.3% ± 10.3% vs 36.6% ± 11.2%; P = .04). The median VTCL of VT/VF episodes was 325 ms (interquartile range 235-429 ms). None of the patients with a first documented nonrapid VT episode had rapid VT/VF during follow-up.Patients with TOF and rapid VT/VF had distinct clinical characteristics. The relatively low variation of VTCL over time suggests a room for catheter ablation without a backup ICD in selected patients with well-tolerated VT.Clinicaltrials.gov identifier: NCT03837574.

There is no consensus on the most effective radiofrequency ablation strategy for the initial or repeated treatment of persistent atrial tachyarrhythmia (ATa).To describe success and complication rates after the creation of left atrial (LA) linear lesions for recurrent persistent ATa following an index cryoballoon pulmonary vein isolation (PVI), and to identify predictors of ATa recurrence.During March 2013 to March 2020, we prospectively included all consecutive patients undergoing repeat procedures for the treatment of persistent ATa. Radiofrequency ablation consisting of the creation of linear lesions: a roofline, a mitral isthmus and/or septal line, and a cavotricuspid isthmus line. All patients were prospectively followed up for ATa recurrence.Overall, 133 patients underwent 170 procedures after initial cryoballoon PVI (n=715). At least one pulmonary vein reconnection was observed in 60 patients (45.1%), all of whom underwent successful re-disconnection. After all the procedures,90% of patients had a lesion pattern consisting of a roofline, a mitral isthmus and/or septal line, and a cavotricuspid isthmus line. ATa was terminated in 41 patients (35%). There were three cases of tamponade (3/170 procedures, 1.8%). Ninety-two patients (69.2%) were in sinus rhythm after a median (interquartile range) of 36 (21-53) months since the index cryoballoon PVI. Diagnosis-to-ablation time and LA area were predictors of recurrence in multivariable analysis.The creation of linear lesions is a safe and effective treatment strategy for the recurrence of persistent ATa after cryoballoon PVI. Longer diagnosis-to-ablation time and larger LA area were predictive of ATa recurrence.

Background: Recurrences of atrial fibrillation (AF) after pulmonary vein isolation (PVI) are mainly due to pulmonary vein reconnection. However, a growing number of patients have AF recurrences despite durable PVI. The optimal ablative strategy for these patients is unknown. We analyzed the impact of current ablation strategies in a large multicenter study. Methods: Patients undergoing a redo ablation for AF and presenting durable PVI were included. The freedom from atrial arrhythmia after pulmonary vein-based, linear-based, electrogram-based, and trigger-based ablation strategies were compared. Results: Between 2010 and 2020, 367 patients (67% men, 63±10 years, 44% paroxysmal) underwent a redo ablation for AF recurrences despite durable PVI at 39 centers. After durable PVI was confirmed, linear-based ablation was performed in 219 (60%) patients, electrogram-based ablation in 168 (45%) patients, trigger-based ablation in 101 (27%) patients, and pulmonary vein-based ablation in 56 (15%) patients. Seven patients (2%) did not undergo any additional ablation during the redo procedure. After 22±19 months of follow-up, 122 (33%) and 159 (43%) patients had a recurrence of atrial arrhythmia at 12 and 24 months, respectively. No significant difference in arrhythmia-free survival was observed between the different ablation strategies. Left atrial dilatation was the only independent factor associated with arrhythmia-free survival (HR, 1.59 [95% CI, 1.13–2.23]; P =0.006). Conclusions: In patients with recurrent AF despite durable PVI, no ablation strategy used alone or in combination during the redo procedure appears to be superior in improving arrhythmia-free survival. Left atrial size is a significant predictor of ablation outcome in this population.

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)

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.

Cryoballoon pulmonary vein isolation (PVI) for persistent atrial fibrillation (AF) ablation is an increasingly used strategy. We aimed to determine the results and predictors of arrhythmia recurrence after a single procedure of cryoballoon PVI for patients with persistent and long-standing persistent AF. We included all consecutive patients who underwent cryoballoon PVI for the treatment of persistent symptomatic drug-refractory AF since 2012. All patients were prospectively followed to detect the recurrence of atrial tachyarrhythmia (ATa). Predictors of recurrence were assessed. Cryoballoon PVI was performed on 399 patients with persistent AF, among whom 52 (13%) had long-standing persistent AF. Patients with long-standing persistent AF had a significantly larger left atrium than those with persistent AF. A 28-mm cryoballoon was used for 322 patients (93%). In total, 359 patients (90%) completed the 12-month follow-up visit and the median follow-up was 24 months (interquartile range 43 to 13). The 2-year probability of freedom from ATa recurrence was 51% for persistent AF and 27% for long-standing persistent AF. Long-standing persistent AF and left atrial area/volume were independent predictors of ATa recurrence. Ten patients (2.5%) experienced phrenic nerve palsy, 1 tamponade (0.25%), 2 stroke (0.5%), 2 pericardial effusions (0.5%), and 5 vascular complications (1.25%). In conclusion, 2-year ATa-free survival rates were 51 and 27% for persistent and long-standing persistent AF patients, respectively. Complications were rare. Long-standing persistent AF and left-atrial area/volume were predictors of recurrence.

Aims Acute cardiac tamponade (ACT) is the most common life-threatening complication of interventional electrophysiology. Urgent drainage by percutaneous pericardiocentesis and anticoagulation reversal are required. Immediate direct transfusion of the blood volume aspirated from the pericardial space to the patient has been rarely described. This study was designed to assess the efficacy and safety of immediate direct autologous blood transfusion (AutoBT). Methods A retrospective case series of direct AutoBT performed for ACT was collected. Urgent drainage by percutaneous pericardiocentesis and immediate direct AutoBT were performed to achieve hemodynamic stabilization without a cell-saver system. Results Twenty-two electrophysiology centers were contacted to participate in the case series. Fourteen centers reported not to use direct AutoBT. Three centers reported using direct AutoBT with the cell-saver system. Fourteen cases of immediate direct AutoBT without cell-saver system were included from the five remaining centers. Electrophysiological procedures were performed for ventricular tachycardia (n = 5), atrial fibrillation (n = 5), atrial tachycardia (n = 2), left accessory pathway (n = 1), and premature ventricular contraction (n = 1) with transseptal (n = 9), retroaortic (n = 4), and/or epicardial access (n = 4). Pericardial drainage was performed by percutaneous pericardiocentesis for 13 patients and via the transseptal sheath for one patient. Surgical hemostasis was required for seven patients. The mean volume of autologous blood directly transfused was 1207 ± 963 mL. Direct AutoBT permitted to resume the procedure in four patients. No major complication related to the use of AutoBT occurred. Conclusion Direct AutoBT without a cell-saver system is a feasible, safe, and useful technique for salvage therapy in ACT in interventional electrophysiology.

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.

Introduction Atrial fibrillation (AF) is the most documented arrhythmia in COVID-19 pneumonia. Left atrial (LA) strain analysis, assessing LA contractility have been associated with the development of new-onset atrial fibrillation (NOAF) in several clinical situations. We aimed to assess the diagnostic ability of LA strain parameters to predict NOAF in patients with severe hypoxemic COVID-19 pneumonia. Method We conducted a prospective single center study at Amiens Hospital University (NCT04354558). The population study was composed of adult patients with a severe or critical Covid-19 pneumonia in sinus rhythm at inclusion. Transthoracic echocardiography was performed within 48 hours of admission. LA strain analysis was performed by an automated software. The following LA strain parameters were recorded: LA strain during reservoir phase (LASr), LA strain during conduit phase (LAScd) and LA strain during contraction phase (LASct). The primary endpoint was the occurrence of NOAF during the intensive care unit (ICU) stay. Results From March 2020 to February 2021, 79 patients were included. Sixteen patients (18%) developed NOAF in ICU. LAScd and LASr were significantly reduced in the NOAF group compared to the other group (8.1[6.3-10.9] vs. 17.2[5.0-10.2]%; P 11% (log rank test P value

We report a detailed case of type 2 TS due to a p.(Gly402Ser) mutation in exon 8 of the CACNA1C gene. The patient shows a marked prolongation of repolarization with a mean QTc of 540 ms. He shows no structural heart disease, syndactyly, or cranio-facial abnormalities. However, he shows developmental delays, without autism, and dental abnormalities. The cardiac phenotype is very severe, with a resuscitated cardiac arrest at 2.5 years of age, followed by 26 appropriate shocks during nine years of follow-up. Adding mexiletine to nadolol resulted in a reduction of the QTc and a slight decrease in the number of appropriate shocks.

Background Several liver function tests have been identified as predictors of hospitalization for heart failure (HF) and death in patients with chronic HF. The relationship between serum γ-glutamyltranferase (GGT) and albumin (SA) levels with the response to cardiac resynchronization therapy (CRT) has not been reliably determined. The aim of the study was to evaluate the impact of liver function tests on the results of CRT in the elderly. Methods Baseline GGT and SA were assessed before CRT device implantation in the elderly (> 70-year-old) patients. The endpoints were: (1) CRT response defined as > 5% left ventricular ejection fraction improvement and no hospitalization for HF or cardiovascular death; (2) hospitalizations; and (3) mortality. Results Eighty of 138 (58%) included patients were responders at nine months. Compared to responders, the SA levels were not significantly different (35.1 ± 5.4 vs. 33.6 ± 5.5 g/L, P = 0.103); but the GGT levels, higher (81.6 ± 69.3 vs. 54.7 ± 49.6 U/L, P = 0.013) in non-responders to CRT. GGT level was independently associated with non-response to CRT (P < 0.001, OR = 0.17; 95% CI: 0.08–0.38, P < 0.001). GGT cut-off value ≥ 55 U/L was highly predictive of non-response [AUC = 0.65, 64% Sensitivity, 69% Specificity (95% CI: 0.56–0.74)]. GGT ≥ 55 U/L was also associated with higher risk of hospitalization for atrial fibrillation (AF) (95% vs. 83%, P = 0.024). Both SA and GGT had no impact on overall (P = 0.220, P = 0.723) mortality. Conclusions Higher level of GGT is an independent predictor of non-response to CRT in patients over age 70 years and is associated with higher risk of hospitalization for AF. Baseline serum levels of albumin and GGT and have no impact on mortality in elderly patients undergoing CRT.

Background Long-term outcomes in terms of thromboembolic events (TEs) are poorly described after cryoballoon pulmonary vein isolation (cryo-PVI) for atrial fibrillation (AF). We evaluated the long-term incidence and predictive factors of TE after cryo-PVI. Methods All consecutive patients who underwent cryo-PVI for paroxysmal or persistent AF between November 2012 and October 2017 were included. They were prospectively followed for at least 12 months in the ablation center and then by their cardiologist. Data on all ischemic events (stroke, transient ischemic attack (TIA), systemic embolism) were collected. Results In total, 450 patients (78% men, median age 61 years) were included. The mean CHA2DS2-VASc score was 1.6 ± 1.3 and 26 patients (6%) had a history of stroke/TIA before the procedure. OAT was discontinued for 75 patients (17%) after the procedure, among whom 50 (67%) had no indication for long-term anticoagulation. Six patients experienced an ischemic event, all considered as a TE: three strokes, two TIAs, and one acute lower-limb ischemia. The mean follow up was 30 months. Thus, the incidence of TE was 0.53%/year. Three of the six patients who experienced a TE had no recurrence of atrial arrhythmia documented before, at the time, or after the event. All patients who experienced a TE had a class I or class IIa indication for long-term anticoagulation. After multivariate analysis, the CHA2DS2-VASc Score (p = .0005) was a predictor of TEs. Conclusion The long-term incidence of TEs after cryo-PVI was 0.53%/year. The CHA2DS2-VASc Score was the only independent predictor of TEs.

Introduction Assess the prevalence and predictors of Left Atrial Tachycardia (LAT) after cryoballoon ablation of pulmonary veins. Methods and results Patients who underwent catheter ablation of pulmonary veins with a second-generation cryoballoon for symptomatic paroxysmal (151/270, 56%) or persistent (119/270, 44%) atrial fibrillation were entered in a single-centre prospective registry. Patients who experienced post-cryoballoon LAT (pcryo-LAT) were selected on the basis of 12-lead ECG characteristics. Left atrial origin was confirmed during conventional EP study and electroanatomical activation mapping, and patients were treated by RF catheter ablation. Pcryo-LAT was observed in 15 (5.6%) of 270 patients and was attributed to a reentrant mechanism in 11 patients (73%). The other 4 cases of pcryo-LAT were due to focal atrial tachycardia associated with reconnection of one pulmonary vein. In comparison with patients who remained in sinus rhythm, LA area (HR = 1.09; CI 1.01,1.2; p = 0.02), LVEF (HR = 0.94; CI 0.90,0.97; p

Background The electrocardiogram (ECG) is widely available and may contribute to a better risk stratification for sudden cardiac death in tetralogy of Fallot. Purpose To identify ECG predictors of appropriate therapies in patients with tetralogy of Fallot and implantable cardioverter defibrillator (ICD). 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 A total of 134 patients (median age 41.7 years, 70.7% males) were enrolled. During a median (IQR) follow-up of 6.1 (2.7–10.2) years, 59 (44.0%) patients received at least one appropriate ICD therapy, giving annual incidence of 5.5% and 7.1% in primary and secondary prevention, respectively (P = 0.058). Overall, QRSd ≥ 180ms (P = 0.073), QRS fragmentation (P = 0.052), and QRS vector magnitude (vm, P = 0.327) were not significantly associated with appropriate ICD therapies, whereas the association of QRSd ≥ 180ms and QRS fragmentation (HR = 1.9, 95% CI: 1.1–3.4, P = 0.036) were associated with an increased risk of appropriates ICD therapies. In patients with ICD for primary prevention (47 patients, 35.1%), while non-sustained ventricular tachycardia (NSVT) considered isolated was not associated with events during follow-up (P = 0.069), combinations with QRSd ≥ 180 ms (HR = 7.2, 95% CI: 1.6–32.7, P = 0.011), QRS fragmentation (HR = 3.8, 95% CI: 1.2–12.4, P = 0.025), or decreased QRS vm (HR = 3.6, 95% CI: 1.1–12.1, P = 0.042) were all associated with a higher incidence of appropriate ICD therapies. Conclusions Our findings highlight that cumulative risk score derived from ECG may contribute to improve risk stratification in patients with tetralogy of Fallot, in particular QRS fragmentation and QRS vm in association with QRS duration and other traditional risk factors.

Background The electrocardiogram (ECG) is widely available and may contribute to a better risk stratification for sudden cardiac death in patients with tetralogy of Fallot. QRS duration has been consistently associated with outcomes, with a lack of specificity for sudden mortality and a relatively low predictive value. New markers such as QRS fragmentation and vectocardiographic parameters have been recently suggested. Purpose To identify ECG predictors of appropriate therapies in patients with tetralogy of Fallot and implantable cardioverter defibrillator (ICD). 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 A total of 134 patients (median age 41.7 years, 70.7% males) were enrolled. During a median (IQR) follow-up of 6.1 (2.7–10.2) years, 59 (44.0%) patients received at least one appropriate ICD therapy, giving annual incidence of 5.5% and 7.1% in primary and secondary prevention, respectively (p=0.058). Overall, QRSd ≥180ms (p=0.073), QRS fragmentation (p=0.052), and QRS vector magnitude (vm, p=0.327) were not significantly associated with appropriate ICD therapies, whereas QRS fragmentation in right leads (HR=1.7, 95% CI: 1.1–2.9, p=0.039) and the association of QRSd ≥180ms and overall QRS fragmentation (HR=1.9, 95% CI: 1.1–3.4, p=0.036) were associated with an increased risk of appropriates ICD therapies. In patients with ICD for primary prevention (47 patients, 35.1%), 53.8% had QRS fragmentation, 48.6% had decreased QRS vm, and 41.0% had QRSd ≥180ms. In this group, while non-sustained ventricular tachycardia (NSVT) considered isolated was not associated with ventricular events during follow-up (p=0.069), respective combinations with QRSd ≥180 ms (HR=7.2, 95% CI: 1.6–32.7, p=0.011), QRS fragmentation (HR=3.8, 95% CI: 1.2–12.4, p=0.025), or decreased QRS vm (HR=3.6, 95% CI: 1.1–12.1, p=0.042) were all associated with a higher incidence of appropriate ICD therapies. Positive predictive value and negative predictive value were 0.33 and 0.85, 0.58 and 0.74, and 0.36 and 0.84 in patients with NSVT and QRS ≥180ms, NSVT and QRS fragmentation, and NSVT and decreased QRS vm, respectively. Conclusions Our findings highlight that cumulative risk score derived from ECG may contribute to improve risk stratification in patients with tetralogy of Fallot, in particular QRS fragmentation and QRS vm in association with QRS duration and other traditional risk factors.

Aims Acute cardiac tamponade (ACT) is the most common life-threatening complication of interventional electrophysiology. Urgent drainage by percutaneous pericardiocentesis and anticoagulation reversal are required. Immediate direct transfusion of the blood volume aspirated from the pericardial space to the patient has been rarely described. This study was designed to assess the efficacy and safety of immediate direct autologous blood transfusion (AutoBT). Methods A retrospective case series of direct AutoBT performed for ACT was collected. Urgent drainage by percutaneous pericardiocentesis and immediate direct AutoBT into the femoral sheath of the blood volume aspirated from the pericardial space, were performed to achieve hemodynamic stabilization. Results Twenty-two electrophysiology centers were contacted to participate to the case series. Fourteen centers reported not to use direct AutoBT. Fourteen cases of immediate direct AutoBT were included from the five remaining centers. Electrophysiological procedures were performed for ventricular tachycardia (n = 5), atrial fibrillation (n = 5), atrial tachycardia (n = 2), left accessory pathway (n = 1) and premature ventricular contraction (n = 1) with transseptal (n = 9), retroaortic (n = 4) and/or epicardial access (n = 4). Pericardial drainage was performed by percutaneous pericardiocentesis for 13 patients and via the transseptal sheath for one patient. Surgical hemostasis was required for 7 patients. The mean volume of autologous blood directly transfused was 1207 ± 963 ml Direct AutoBT permitted to resume the procedure in 4 patients. 13 patients (93%) were discharged with a hospital stay of 12.3 ± 17.1 days. No major complication related to the use of AutoBT occurred ( table 1 ). Conclusion Direct AutoBT without cell-saver system is a feasible and useful technique for salvage therapy in acute cardiac tamponade in interventional electrophysiology.

Introduction Atrial fibrillation (AF) catheter ablation is more and more performed without interruption of direct oral anticoagulants (DOAC), with a risk of puncture site complications. Purpose To evaluate safety and feasibility of figure-of eight-suture to achieve hemostasis of puncture site in AF catheter ablation without interruption of DOAC. Methods All patients receiving DOAC who underwent AF catheter ablation from November 2017 to December 2018 were prospectively enrolled. Unfractionated heparin (UH) was administrated during the procedure to obtain a target ACT > 300s. At the end of the procedure, a last ACT was assayed and a figure-of-eight suture was performed to remove the sheaths, without further manual compression. Puncture site complications were recorded during the hospitalization and at the 3 months control consultation. Results One hundred and thirty-eight patients underwent AF catheter ablation without DOAC interruption with a median follow-up of 119 days (99–149). Median last ACT before sheath removal was 300s (259–337). Figure-of-eight suture was able to be performed in all patients. No protamine reversal was necessary to obtain puncture site hemostasis. Puncture site complications occured in 8 patients (hematoma: 4; 2.9% − pseudoaneurysm: 1;0.7% − arteriovenous fistula (AVF): 2;1.4%). All complications were detected during hospitalization except for one patient for which pseudoaneurysm was diagnosed and treated ambulatory. Only 2 (1.4%) patients needed to be treated surgically for AVF. No death was reported. Conclusion Figure-of-eight suture is safe and feasible to achieve puncture site hemostasis, without manual compression or protamine reversal, in AF catheter ablation without DOAC interruption.

Background Long-term outcomes in term of cardioembolic events (CE) is poorly described after cryoballoon pulmonary vein isolation (cryo-PVI) for atrial fibrillation (AF). Purpose To evaluate the long-term incidence of CE after cryo-PVI. Methods All consecutive patients who underwent cryo-PVI for paroxysmal or persistent AF between November 2012 and October 2017 were included. They were prospectively followed at least 12 months in the ablation center, and then by their cardiologist. Decision of oral anticoagulation (OAC) discontinuation was let at the appreciation of the referent cardiologist. All ischemic events (stroke, transient ischemic attack (TIA), systemic embolism) were collected. Results Four hundred and fifty patients (78% men, median age 61 years) were included, among which 208 (46%) were initially in persistent AF. Median CHA2DS2-VASc score was 1 (IQR 1), and 26 patients (6%) had a history of stroke/TIA before the procedure. Two hundred and seventy patients (60%) were in sinus rhythm after one procedure and a median follow-up of 26 months (IQR 23). OAC was discontinued for 75 patients (17%) after the procedure, among which 50 (67%) had no indication for long-term anticoagulation. Six patients presented an ischemic event, all considered as cardioembolic origin: 3 stroke, 2 TIA, 1 acute lower limb ischemia. Mean follow-up was 30 months, therefore incidence of CE was 0.53%/year. Three out of six of the patient who presented CE had no recurrence documented before or at the time of the event. All patients who presented a CE had class I or class IIa indication for long-term anticoagulation. Conclusion In our cohort, long-term incidence of CE after cryo-PVI was 0.53%/year. Absence of documented recurrence could not be an argument for stopping OAC, decision who need to be guided only by CHA2DS2-VASc score.

International audience; BACKGROUND:Previous studies suggested that genetic status affects the clinical course of arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) patients. The aim of this study was to compare the outcome of desmoglein-2 (DSG2) mutation carriers to those who carry the plakophilin-2 (PKP2) mutation, the most common ARVC/D-associated gene.METHODS AND RESULTS:Consecutive ARVC/D patients carrying a pathogenic mutation in PKP2 or DSG2 were selected from a national ARVC/D registry. The cumulative freedom from sustained ventricular arrhythmia and cardiac transplantation/death from heart failure (HF) during follow-up was assessed, compared between PKP2 and DSG2, and predictors for ventricular arrhythmia and HF events determined. Overall, 118 patients from 78 families were included: 27 (23%) carried a DSG2 mutation and 91 (77%) a PKP2 mutation. There were no significant differences between DSG2 and PKP2 mutation carriers concerning gender, proband status, age at diagnosis, T-wave inversion, or right ventricular dysfunction at baseline. DSG2 patients displayed more frequent epsilon wave (37% vs. 17%, P = 0.048) and left ventricular dysfunction at diagnosis (54% vs. 10%, P < 0.001). During a median follow-up of 5.6 years (2.5-16), DSG2 and PKP2 mutation carriers displayed a similar risk of sustained ventricular arrhythmia (log-rank P = 0.20), but DSG2 mutation carriers were at higher risk of transplantation/HF-related death (log-rank P < 0.001). The presence of a DSG2 mutation vs. PKP2 mutation was a predictor of transplantation/HF-related death in univariate Cox analysis (P = 0.0005).CONCLUSIONS:In this multicentre cohort, DSG2 mutation carriers were found to be at high risk of end-stage HF compared to PKP2 mutation carriers, supporting careful haemodynamic monitoring of these patients. The benefit of early HF treatment needs to be assessed in DSG2 carriers.

Summary Background Brugada syndrome is a genetic heart disease with autosomal dominant inheritance. Family screening commonly detects one parent responsible for transmission of the disease. Aims To describe atypical transmission of Brugada syndrome. Methods Between 2001 and 2007, systematic screening, including an electrocardiogram, ajmaline challenge and DNA sequencing of the SCN5A gene, of the first-degree relatives of 62 probands with Brugada syndrome was performed (Programme Hospitalier de Recherche Clinique). Results In two families, both parents transmitted Brugada syndrome to their offspring. In the first family, the proband presented Brugada electrocardiogram features with ajmaline challenge and carried a new SCN5A mutation (p.V1281F). The mutation was also identified in the mother, who had a type 1 aspect on inferior leads with ajmaline. The proband's father presented a typical Brugada electrocardiogram pattern on lead V2 with ajmaline and no SCN5A gene mutation. In the second family, the proband was a boy aged 2.5 years who had been resuscitated from sudden cardiac death. Ajmaline challenge revealed a typical Brugada electrocardiogram pattern in both parents but with no mutation in the genes studied. Conclusion Family studies should always be exhaustive and discovery of one parent with Brugada syndrome does not eliminate the need for screening of the other parent.

The familial prevalence of Brugada syndrome (BrS) in a consecutive series of patients was prospectively determined. BrS is genetically determined with autosomal dominant transmission. The familial prevalence of the BrS is unknown. A detailed pedigree of each family of patients with BrS was assembled and permission was obtained to invite relatives for electrocardiography and an ajmaline challenge. Sixty-two of 98 patients participated in the study and were included over a 6-year period. SCN5A genotyping was performed in 56 of these 62 patients (90%). Electrocardiograms (ECGs) of 488 relatives (mean age 38 ± 20 years, 45% men) were recorded and 270 of these relatives agreed to undergo an ajmaline challenge. Spontaneous type 1 BrS ECG was found in 4 of 488 relatives (0.8%). In the group of relatives in whom ajmaline challenge was performed (n = 270), the finding was positive in 79 subjects (29%). SCN5A genotyping identified 5 other affected relatives. As a result, the total number of affected relatives was 88. Standard 12-lead ECG was normal in 64 of the 88 affected relatives (73%). Mean percentage of affected relatives per family was 27 ± 32% (95% confidence interval 19 to 35). Familial forms of BrS were observed in 41 of the 62 families (66%) and no SCN5A mutations were found in sporadic forms. In conclusion, after active family screening affected relatives were found in almost 1/3 of subjects. BrS appeared to be a familial disease in 2/3 of subjects.

Contexte : le cannabis est la substance illicite la plus consommée en France. L’impact du cannabis chez les fumeurs victimes d’infarctus du myocarde reste incertain. Les effets cardiovasculaires du cannabis sont peu connus comparativement à ses effets psychotropes. De nombreux cas cliniques ont été rapportés ainsi que des revues de littérature mais peu d’études comparatives ont été réalisées et aucune comportant un suivi de cette population spécifique. Objectif : l’objectif principal était de déterminer l’évolution des patients pendant le suivi, en étudiant la survenue d’une complication cardiovasculaire. L’objectif secondaire était de déterminer les facteurs de risque de survenue de ces complications pendant le suivi. Méthode : cent-huit patients d’âge inférieur ou égal à 45 ans ont été rétrospectivement inclus entre le premier janvier 2010 et le 31 décembre 2019, aux soins intensifs cardiologiques du CHU d’Amiens. Quarante-sept dans le groupe fumeurs de cannabis et 61 dans le groupe non-fumeurs. L’ensemble des caractéristiques cliniques, paracliniques ainsi que les traitements médicamenteux et interventionnels ont été recueillis. Les données du suivi ont été intégrées par l’intermédiaire des consultations avec le médecin cardiologue, le médecin traitant et par un entretien téléphonique avec chaque patient. Résultats : le pic de troponinémie était plus élevé chez les fumeurs de cannabis (51 ± 54 ng/mL, p 25ng/mL étaient des facteurs pronostics indépendant à la survenue d’une complication cardiaque. La prévention primaire et secondaire, le strict contrôle des facteurs de risques cardiovasculaires sont essentiels pour améliorer le pronostic de ces jeunes sujets. Ces résultats sont à confirmer et à confronter à des études prospectives intégrant un suivi à long terme de ces patients.

Aim : the ablation strategy in the treatment of persistent atrial fibrillation (AF) is still an ongoing debate. A pulmonary vein isolation must be performed. Ablation targeting others substrate in the left atria (LA) doesn’t show superiority and is indicated in IIb class. We reported results of cryoablation as a first procedure, followed by radiofrequency linear ablation in case of an arythmia recurrence. Methods : forty-four patients with recurrence of persistent AF following a second-generation cryoballoon pulmonary vein isolation underwent at least one repeat ablation in radiofrequency, with creation of a mitral isthmus line and a LA roof line, between march 2013 and april 2018. Our prospective follow-up evaluated the freedom from AF and the predictors of arythmia recurrence. Results : thirty-one patients (70.5%) were in sinus rythm after a mean of 1.3 ± 0.6 radiofrequency procedures, with a follow-up of 20.6 ± 13.4 months since the first repeat ablation and 17 ± 13 months since the last procedure. On univariate analysis, LA size was a predictor of relapse, for an area at or beyond 30 cm² (HR 4.712, IC 1.357-16.362, p=0.015) and a volume at or beyond 200 mL (HR 3.279, IC 1.057-10.174, p=0.04). We had only one serious complication, a cardiac tamponade. Conclusion : our strategy of repeated ablation(s) with creation of LA lines in radiofrequency after persistent AF recurrence following an index procedure of cryoballoon pulmonary vein isolation, apparead safe and effective, with 70.5% of freedom from any atrial arythmia at 20 months follow-up.; Objectif : la stratégie d’ablation de la fibrillation atriale (FA) persistante fait débat. L’isolation des veines pulmonaires est impérative. L’ajout d’autres lésions dans l’oreillette gauche (OG) n’a pas prouvé sa supériorité et est recommandée en classe IIb. Notre étude a évalué les résultats d’une approche commençant par la cryoisolation des veines pulmonaires seule, complétée par la création de lignes en radiofréquence en cas de récidive. Méthodes : quarante-quatre patients porteurs de FA persistante, ayant présenté une récidive après une procédure de cryoablation au ballon de deuxième génération ont bénéficié d’au moins une reprise en radiofréquence avec création d’une ligne de l’isthme mitral et une ligne du toit de l’OG, entre mars 2013 et avril 2018. Notre suivi prospectif a évalué le taux de maintien en rythme sinusal et les facteurs prédictifs de récidive Résultats : trente et un patients (70.5%) étaient en rythme sinusal après 1.3 ± 0.6 reprises en moyenne, avec un suivi de 20.6 ± 13.4 mois depuis la première reprise et de 17 ± 13 mois depuis la dernière procédure. En analyse univariée, on retrouvait une différence significative en termes de dimensions de l’OG entre les patients ayant récidivé et ceux indemnes d’arythmie, pour une surface supérieure ou égale à 30 cm² (HR 4.712, IC 1.357-16.362, p=0.015) et un volume supérieur ou égal à 200 mL (HR 3.279, IC 1.057-10.174, p=0.04). Il y eu une seule complication grave, une tamponnade. Conclusion : la stratégie de reprise(s) en radiofréquence avec création de lignes dans l’OG en cas de récidive de FA persistante après une procédure initiale de cryoisolation des veines pulmonaires s’est avérée sûre et efficace, avec 70.5% de maintien en rythme sinusal à 20 mois.