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Although patients with systemic right ventricle (SRV) represent a significant proportion of patients with congenital heart disease (CHD) implanted with cardiac resynchronization therapy (CRT), there are limited and conflicting data in this specific patient group.We aimed to analyze outcomes of patients with SRV implanted with a CRT device.Data were analyzed from an observational, retrospective, multicenter cohort study including all patients with CHD implanted with a CRT device from 6 French centers from 2004 to 2020. Response to CRT was defined as an increase in systemic ventricular ejection fraction of ≥10% and/or an improvement in New York Heart Association functional class by at least 1 grade.A total of 85 patients with CHD were enrolled (mean age 39.8 ± 20.0 years; 55 [64.7%] males; 25 defibrillators [29.4%]), including 31 patients with SRV (36.5%) (mean age 43.9 ± 19.8 years; 16 [51.6%] males). The mean change in QRS duration after implantation was similar as compared with patients with systemic left ventricle (-46 ± 26 ms vs -35 ± 32 ms; P = .16). During a mean follow-up of 5.1 ± 3.5 years, late complications included 2 lead dysfunctions (6.5%), 3 CRT-related infections (9.7%), and 1 inappropriate implantable cardioverter-defibrillator shock (3.2%). The proportion of CRT responders at 6, 12, and 24 months were 82.6%, 80.0%, and 77.8% in patients with SRV vs 66.7%, 64.3%, and 69.6% in patients with systemic left ventricle (P = NS).In this multicenter cohort, one-third of patients with CHD implanted with a CRT device had SRV. CRT in patients with SRV was associated with a high rate of responders, comparable to that of patients with systemic left ventricle.

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.

INTRODUCTION Catheter ablation (CA) of atrial tachyarrhythmias (ATs) in patients with complex congenital heart disease (CHD) often requires technically challenging transbaffle or transconduit puncture. The aim was to assess the feasibility and safety of transbaffle/transconduit puncture based on computed tomography (CT) three-dimensional (3D) reconstruction merged with electro-anatomical mapping (EAM) without per-procedure echocardiographic guidance. METHODS AND RESULTS We included 18 consecutive CHD patients in two centers who had atrial-switch or Fontan surgery and underwent CA of AT by an antegrade approach requiring intracardiac puncture. Twelve patients with atrial-switch surgery and six patients with extracardiac Fontan surgery were referred for CA of AT. Cardiac CT with 3D reconstruction was performed before the procedure. The 3D volume of the systemic venous atrium or extracardiac conduit acquired by EAM was merged with the corresponding CT 3D reconstruction. The ablation catheter was positioned at the optimal puncture site. Under fluoroscopic guidance, the needle was positioned next to the ablation and the puncture was performed. Balloon expansion of the puncture site was performed in every case of transconduit puncture and in two (17%) cases of transbaffle puncture. Overall, 17 intra-atrial reentrant tachycarrythmias and 9 focal ATs were successfully ablated, with no acute complications. The median time to access the pulmonary atrium was 78.5 minutes (range, 55-185) and total median fluoroscopy time was 23 minutes (range, 7-53). CONCLUSIONS Transbaffle and transconduit punctures can be performed safely in CHD patients by using a simple technique relying on CT 3D reconstruction and EAM.

Aims In arrhythmogenic cardiomyopathy (ACM), sustained ventricular tachycardia (VT) typically displays a left bundle branch block (LBBB) morphology while a right bundle branch block (RBBB) morphology is rare. The present study assesses the VT morphology in ACM patients with sustained VT and their clinical and genetic characteristics. Methods and results Twenty-six centres from 11 European countries provided information on 954 ACM patients who had ≥1 episode of sustained VT spontaneously documented during patients’ clinical course. Arrhythmogenic cardiomyopathy was defined according to the 2010 Task Force Criteria, and VT morphology according to the QRS pattern in V1. Overall, 882 (92.5%) patients displayed LBBB-VT alone and 72 (7.5%) RBBB-VT [alone in 42 (4.4%) or in combination with LBBB-VT in 30 (3.1%)]. Male sex prevalence was 79.3%, 88.1%, and 56.7% in the LBBB-VT, RBBB-VT, and LBBB + RBBB-VT groups, respectively (P = 0.007). First RBBB-VT occurred 5 years after the first LBBB-VT (46.5 ± 14.4 vs 41.1 ± 15.8 years, P = 0.011). An implanted cardioverter-defibrillator was more frequently implanted in the RBBB-VT (92.9%) and the LBBB + RBBB-VT groups (90%) than in the LBBB-VT group (68.1%) (P Conclusion RBBB-VT accounts for a significant proportion of sustained VTs in ACM. Sex and type of pathogenic mutations were associated with VT type, female sex with LBBB + RBBB-VT, and DSP mutation with RBBB-VT.

BACKGROUND: Cardiac implantable electronic device (CIED) implantation rates as well as the clinical and procedural characteristics and outcomes in patients with known active coronavirus disease 2019 (COVID-19) are unknown. OBJECTIVE: The purpose of this study was to gather information regarding CIED procedures during active COVID-19, performed with personal protective equipment, based on an international survey. METHODS: Fifty-three centers from 13 countries across 4 continents provided information on 166 patients with known active COVID-19 who underwent a CIED procedure. RESULTS: The CIED procedure rate in 133,655 hospitalized COVID-19 patients ranged from 0 to 16.2 per 1000 patients (P

Background Atrial arrhythmias are common complications in adults with congenital heart disease (ACHD) and are associated with high morbi-mortality. Atrial tachycardia (AT) including intra-atrial reentrant tachycardia and focal atrial tachycardia are the most prevalent arrhythmias in ACHD. We aimed to compare mid-term outcomes according to arrhythmia management. Methods All hospitalizations for AT in ACHD were retrospectively identified from medico-administrative database from September 2006 to September 2016 in two centers. Outcomes of patients with AT managed with direct-current cardioversion (DCCV) were compared with patients referred for catheter ablation (CA). Atrial arrhythmia recurrence, stroke, admission for heart failure and death were analyzed during follow-up. Results One hundred and twenty-one ACHD with AT (median age 41 [IQR 32–52] years, male 62%, 41% moderate CHD and 38% complex CHD) were included. Median follow-up was 3.3 years [IQR 1.4–6.2]. Thirty-seven (30.5%) patients underwent DCCV and 84 (69.5%) CA. No significant difference in age, CHD complexity, history of atrial arrhythmia, heart failure, and CHA2DS2-VASC score were observe. Patients undergoing DCCV had higher NYHA class. Atrial arrhythmia recurrence was lower in CA group (Log-rank test, p ​= ​0.045). Most recurrences were AT, but atrial fibrillation was recorded in 35.1% after DCCV and 9.5% after CA (p ​= ​0.001). At the end of follow-up, patients who were initially treated by CA had a significantly lower incidence of severe cardiovascular events (48.6% in DCCV group vs. 16.7% in CA group, HR: 0.46, CI 95%: 0.22–0.93, p ​= ​0.031). Conclusions A proactive management of AT by CA is associated with better long-term outcomes in ACHD.

Aims The roles of implantable cardioverter-defibrillators (ICDs) and radiofrequency catheter ablation (RCA) in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) and well-tolerated monomorphic ventricular tachycardia (MVT) are debated. In this multicentre retrospective study, we aimed at reporting the outcome of selected patients with ARVC after RCA without a back-up ICD. Methods and results Patients with ARVC who underwent RCA of well-tolerated MVT at 10 tertiary centres across 5 countries, without an ICD before and 3 months after RCA, without syncope or electrical storm, and with left ventricular ejection fraction ≥50% were included. In total, 65 ARVC patients [mean age 44.5 ± 13.2 years, 78% males] underwent RCA of MVT between 2003 and 2016. Clinical presentation was palpitations in 51 (80%) patients. One (2%) patient had >1 clinical MVT. At the ablative procedure, clinical MVTs (mean rate 185 ± 32 b.p.m.) were inducible in 50 (81%) patients. Epicardial ablation was performed in 19 (29%) patients. Complete acute success was achieved in 47 (72%) patients. After a median follow-up of 52.4 months (range 12.3–171.4), there was no death or aborted cardiac arrest, and VT recurred in 19 (29%) patients. Survival without VT recurrence was estimated at 88%, 80%, and 68%, 12, 36, and 60 months after RCA, respectively, and was significantly associated with the approach and the procedural outcome. Conclusion In patients with ARVC, well-tolerated MVT without a back-up ICD did not lead to fatal arrhythmic event after RCA despite VT recurrences in some. Our data suggest that RCA may be an alternative to ICD in selected ARVC patients.

Objectives This study sought to assess the feasibility, safety, and outcomes of a stepwise combined percutaneous approach that includes transvenous lead extraction (TLE) followed by baffle stenting and device reimplantation in patients with D-transposition of the great arteries (D-TGA) and atrial baffle dysfunction. Background Management of baffle leak or stenosis in patients with D-TGA and atrial switch surgery is challenging in the presence of transvenous cardiac implantable electronic devices. Baffle complications hinder device-related interventions and addressing baffle dysfunction often requires TLE. Methods All consecutive patients with D-TGA and TLE followed by a percutaneous baffle intervention at the Montreal Heart Institute between 2009 and 2018 were enrolled. Results Ten patients, median 38.6 years of age (range 15.2 to 50.6 years), 5 males (50.0%) were included. Procedures were performed for a device-related indication in 5 patients (50.0%) and for baffle dysfunction in 5 patients (50.0%). A total of 19 leads (17 pacing, 2 defibrillation) were targeted, with a median time from implantation of 8.7 (range 4.3 to 22.1) years. A laser sheath was most frequently required for successful TLE, which was achieved in all patients. Immediate baffle stenting was performed in 9 patients (90.0%) and immediate device reimplantation in 6 (60.0%). During a median follow-up of 3.0 (range 0.1 to 8.2) years, the only complication was subpulmonary atrioventricular valve damage requiring surgery in 1 patient, 8 months after the procedure. Conclusions A combined approach with TLE followed by baffle stenting and reimplantation appears to be safe and feasible in D-TGA patients with atrial switch, baffle dysfunction, and transvenous leads.

BACKGROUND The prevalence of atrial fibrillation (AF) is on the rise in the aging population with congenital heart disease (CHD). A few case series have described the feasibility and early outcomes associated with radiofrequency catheter ablation of AF centered on electrically isolating pulmonary veins (PV) in patients with CHD. In contrast, cryoballoon ablation has not previously been studied in this patient population despite its theoretical advantages, which include a favorable safety profile and shorter procedural time. AIM To assess the safety and feasibility of cryoballoon ablation for AF in an initial cohort of patients with CHD. METHODS The study population consisted of consecutive patients with CHD and cryoballoon ablation for AF at the Montreal Heart Institute between December 2012 and June 2017. Procedural complications, acute success, and 1-year freedom from recurrent AF after a single procedure with or without antiarrhythmic drugs were assessed. Procedures were performed under conscious sedation. Left atrial access was obtained via a single transseptal puncture or through an existing atrial septal defect (ASD). Cryoballoon occlusion was assessed by distal injection of 50% diluted contrast into the pulmonary vein. At least one 240-second cryothermal application was performed upon obtaining complete pulmonary vein occlusion. Following ablation, patients were routinely followed at outpatient visits at 1, 3, 6, and 12 mo, and then annually. RESULTS Ten patients, median age 57.9 (interquartile range 48.2-61.7) years, 60% female, met inclusion criteria and were followed for 2.8 (interquartile range 1.4-4.5) years. Two had moderately complex CHD (sinus venosus ASD with partial anomalous pulmonary venous return; aortic coarctation with a persistent left superior vena cava), with the remainder having simple defects. AF was paroxysmal in 8 (80.0%) and persistent in 2 (20.0%) patients. The pulmonary vein anatomy was normal in 6 (60.0%) patients. Four had left common PV (n = 3) and/or 3 right PV (n = 2). Electrical pulmonary vein isolation (PVI) was acutely successful in all. One patient had transient phrenic nerve palsy that recovered during the intervention. No major complication occurred. One year after a single ablation procedure, 6 (60%) patients remained free from AF. One patient with recurrent AF had recovered pulmonary vein conduction and underwent a second PVI procedure. A second patient had ablation of an extra-pulmonary vein trigger for AF. CONCLUSION Cryoballoon ablation for AF is feasible and safe in patients with simple and moderate forms of CHD, with an excellent acute success rate and modest 1-year freedom from recurrent AF.

Background To date, only a few comparisons between subcutaneous implantable cardioverter-defibrillator (S-ICD) patients undergoing and those not undergoing defibrillation testing (DT) at implantation (DT+ vs DT–) have been reported. Objective The purpose of this study was to compare long-term clinical outcomes of 2 propensity-matched cohorts of DT+ and DT– patients. Methods Among consecutive S-ICD patients implanted across 17 centers from January 2015 to October 2020, DT– patients were 1:1 propensity-matched for baseline characteristics with DT+ patients. The primary outcome was a composite of ineffective shocks and cardiovascular mortality. Appropriate and inappropriate shock rates were deemed secondary outcomes. Results Among 1290 patients, a total of 566 propensity-matched patients (283 DT+; 283 DT–) served as study population. Over median follow-up of 25.3 months, no significant differences in primary outcome event rates were found (10 DT+ vs 14 DT–; P = .404) as well as for ineffective shocks (5 DT– vs 3 DT+; P = .725). At multivariable Cox regression analysis, DT performance was associated with a reduction of neither the primary combined outcome nor ineffective shocks at follow-up. A high PRAETORIAN score was positively associated with both the primary outcome (hazard ratio 3.976; confidence interval 1.339–11.802; P = .013) and ineffective shocks alone at follow-up (hazard ratio 19.030; confidence interval 4.752–76.203; P = .003). Conclusion In 2 cohorts of strictly propensity-matched patients, DT performance was not associated with significant differences in cardiovascular mortality and ineffective shocks. The PRAETORIAN score is capable of correctly identifying a large percentage of patients at risk for ineffective shock conversion in both cohorts.

Recently, the Food and Drug Administration issued a recall for the subcutaneous implantable cardioverter-defibrillator (S-ICD) because of the possibility of lead ruptures and accelerated battery depletion.The aim of this study was to evaluate device-related complications over time in a large real-world multicenter S-ICD cohort.Patients implanted with an S-ICD from January 2015 to June 2020 were enrolled from a 19-institution European registry (Experience from the Long-term Italian S-ICD registry [ELISIR]; ClinicalTrials.gov identifier NCT0473876). Device-related complication rates over follow-up were collected. Last follow-up of patients was performed after the Boston Scientific recall issue.A total of 1254 patients (median age 52.0 [interquartile range 41.0-62.2] years; 973 (77.6%) men; 387 (30.9%) ischemic) was enrolled. Over a follow-up of 23.2 (12.8-37.8) months, complications were observed in 117 patients (9.3%) for a total of 127 device-related complications (23.6% managed conservatively and 76.4% required reintervention). Twenty-seven patients (2.2%) had unanticipated generator replacement after 3.6 (3.3-3.9) years, while 4 (0.3%) had lead rupture. Body mass index (hazard ratio [HR] 1.063 [95% confidence interval 1.028-1.100]; P.001), chronic kidney disease (HR 1.960 [1.191-3.225]; P = .008), and oral anticoagulation (HR 1.437 [1.010-2.045]; P = .043) were associated with an increase in overall complications, whereas older age (HR 0.980 [0.967-0.994]; P = .007) and procedure performed in high-volume centers (HR 0.463 [0.300-0.715]; P = .001) were protective factors.The overall complication rate over 23.2 months of follow-up in a multicenter S-ICD cohort was 9.3%. Early unanticipated device battery depletions occurred in 2.2% of patients, while lead fracture was observed in 0.3%, which is in line with the expected rates reported by Boston Scientific.

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).

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Background Ethanol infusion in the vein of Marshall (EIVM) has shown to be effective for treating atrial fibrillation (AF) and perimitral left atrial (LA) flutter (PMLAF). Aims To assess the persistence of LA lesions created by EIVM by electro-anatomical mapping (EAM) at repeated procedure for recurrent atrial tachycardia (AT) or AF. Methods We included consecutive patients who underwent EIVM then repeated CA for recurrent AT or AF with high-definition EAM in a single center. Acute and long term EIVM effect was assessed at the index and redo procedures by comparing the area of bipolar voltage

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.

A 60-years-old male with remote anterior myocardial infarction (MI) was referred for catheter ablation of electrical storm related to monomorphic ventricular tachycardia (MVT). Radiofrequency applications targeting pre-systolic potentials abolished all clinical MVTs. Scar-associated Purkinje-related MVT mimicking fascicular VT is a rare mechanism of post-MI MVT. The surviving Purkinje cells within scar border zones, responsible for VF during acute MI, may also generate MVT after scar organization occurring with time or after VF ablation. Identification of this mechanism is useful as ablation of a limited area can rapidly eliminate several MVTs.

Funding Acknowledgements Type of funding sources: Public Institution(s). Main funding source(s): INSERM Liliane Bettencourt doctoral grant Background Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is ass­­ociated with complex spatial and temporal right ventricular (RV) wall motion abnormalities. While cardiac magnetic resonance (CMR) is the gold-standard imaging technique, its diagnosic performance remains suboptimal and additional CMR biomarkers reflecting ARVC pathophysiology are needed. Purpose To evaluate the performance of a CMR feature-tracking (FT)-derived parameter combining both longitudinal and radial RV deformation and motion for the characterization of RV wall motion abnormalities in ARVC. Methods Thirty-nine patients with definite or borderline ARVC (median age 45 years, interquartile range 31–51, 56% males) were compared to 20 healthy controls with comparable age, sex and weight distributions. All subjects had 1.5T CMR including short axis and 4-chamber views steady-state free precession acquisitions. A custom FT software adapted to RV wall segmentation and tracking was used to assess RV wall deformation and motion in the 3 space directions resulting in: 1) global longitudinal strain (GLS) estimated on the 4 chamber view from the RV free wall, 2) basal circumferential strain (BCS) and radial motion fraction (BRMF) estimated as an average of short-axis slices comprised in the RV third basal portion. To capture the complex RV motion in ARCV, a longitudinal to radial strain loop (LRSL) was displayed and its area was calculated. Results The ARVC group comprised 28 (72%) patients with definite and 11 (28%) with borderline diagnosis . As compared to controls, LVEF and RVEF were significantly lower in ARVC patients (61(interquartile range (IQR) 52-71) vs. 71%(IQR 55-88) , p = 0.03 and 47%(IQR 16-63) vs. 57%(IQR 49-63) , p = 0.02, respectively), LVEF remaining within normal range limits. While there was no significant difference in RV GLS between ARVC patients and controls (median -17.7%(IQR -24–15) vs. -17.5%(IQR -20.1–15.2), p = 0.67) , BCS and BRMF were significantly lower in ARVC patients vs. controls [-7.5%(IQR -12.3–8.4.) vs. -9.8%(IQR -13.8–8.6.), p = 0.004 and -12.2(IQR -14.4–8.7.) vs. -14.9%(IQR -16.6–13.2) p = 0.0007, respectively] . The LRSL area was significantly and markedly lower in ARVC patients vs. controls [70.6 (IQR 16.3-63.1) vs. 144.1 (IQR 110.4-251.3), p = 0.0002] . LRSL area outperformed RVEF, BCS and BRS in separating ARVC from controls (area under receiving operator characteristics curve 0.82 vs. 0.78, 0.73 and 0.78, respectively). Conclusion In ARVC, a FT-derived parameter combining longitudinal and radial RV wall deformation and motion provided better discrimination of ARVC patients from controls than conventional FT measurements. Its implementation in clinical practice may bolster CMR performance to characterize ARVC wall motion abnormalities. Abstract Figure

The new 5-year ventricular arrhythmia (VA) occurrence risk model is a major breakthrough for arrhythmia risk stratification in the challenging population of patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). In the original study, the model resulted in a 20.6% reduction in implantable cardioverter-defibrillator (ICD) placement compared with the 2015 consensus, for the same protection level. However, only internal validation was performed, limiting generalisation. We externally validated the model in a European tertiary care cohort of 128 patients with ARVC with restrictive indications for primary prevention ICD placement. Overall, 74% were men, none had VA history, and a single patient had an ICD at baseline. Median age at diagnosis was 38 years (interquartile range [IQR] 28-50). During a median follow-up of 7.8 years (IQR 6.1-9.7), 15 patients (12%) experienced VA. The model provided good discrimination, with a C-index for 5-year VA risk prediction of 0.84 (95% confidence interval 0.74-0.93). However, the model led to an overestimation of the 5-year VA risk when applying thresholds50%. With a10% predicted risk, no patient showed VA. With a 7.5% predicted risk, the ICD:VA ratio was 6.3 vs 3.4 in the original study. The model still outperformed the 2015 International Task Force Consensus. Overall, in a relatively large European ARVC cohort with restrictive indications for ICD placement, the ARVC model for VA prediction successfully identified ARVC patients with VA during follow-up. Yet, our study underscores the need for careful threshold selection, considering the model's associated risk overestimation in low- to intermediate-risk patients.

Aims In arrhythmogenic cardiomyopathy (ACM) sustained monomorphic ventricular tachycardia (VT) typically displays left bundle branch block (LBBB) morphology. Sustained VT with right bundle branch block (RBBB) morphology is very rare despite the frequent left ventricular involvement. The present study sought to assess the prevalence of spontaneous sustained LBBB-VT, RBBB-VT or both as well as clinical and genetic differences associated with these VT types. Methods and results Twenty-six centers from 11 European countries provided information on 952 patients with ACM and >1 episode of sustained VT observed during the patients' clinical course. VT was classified as: LBBB-VT; RBBB-VT or LBBB+RBBB-VT. Among 952 patients, 881 (92.5%) had LBBB-VT alone, 71 (7.5%) had RBBB-VT [alone in 42 (4.4%) patients or with LBBB-VT in 29 (3.0%) patients]. Male prevalence was 90.5%, 79.2% and 55.9% in the RBBB-VT, LBBB-VT and LBBB+RBBB-VT groups, respectively (P=0.001). Patients' age at first VT did not differ amongst the 3 VT groups. ICD implantation was more frequent for the RBBB-VT and the LBBB+RBBB groups (≈90% each) vs. 67.9% for the LBBB-VT group (P=0.001). Death incidence (9.5%–17.2%) was not significantly different between the 3 groups (P=0.425). Plakophylin-2 mutations predominated in the LBBB-VT and LBBB-VT+RBBB-VT groups (47.2% and 27.3%, respectively) and Desmoplakin mutations in the RBBB-VT group (36.7%). Conclusion This large European survey demonstrates: 1) Sustained RBBB-VT is documented in 7.5% patients with ACM; 2) Males markedly predominate in the RBBB-VT and LBBB-VT groups but not in the LBBB+RBBB VT group; 3) Distribution of desmosomal mutations appears to be different in the 3 VT groups. Funding Acknowledgement Type of funding source: None

International audience; Background: Clopidogrel associated with aspirin is the recommended treatment for patients undergoing elective percutaneous coronary intervention (PCI). Although severe PCI-related events are rare, evidence suggests that PCI-related myocardial infarction and myocardial injury are frequent complications that can impact the clinical prognosis of the patients. Antiplatelet therapy with a potent P2Y12 receptor inhibitor such as ticagrelor may reduce periprocedural ischemic complications while maintaining a similar safety profile as compared with conventional dual antiplatelet therapy by aspirin and clopidogrel in this setting. Methods: Assessment of Loading with the P2Y12 inhibitor ticagrelor or clopidogrel to Halt ischemic Events in patients Undergoing elective coronary Stenting (ALPHEUS) (NCT02617290) is an international, multicenter, randomized, parallel-group, open-label study in patients with stable coronary artery disease who are planned for an elective PCI. In total, 1,900 patients will be randomized before a planned PCI to a loading dose of ticagrelor 180 mg or a loading dose of clopidogrel (300 or 600 mg) in addition to aspirin. Patients will then receive a dual antiplatelet therapy with aspirin and ticagrelor 90 mg twice daily or clopidogrel 75 mg once daily for 30 days. The primary ischemic end point is PCI-related myocardial infarction (myocardial infarction type 4a or 4b) or major myocardial injury within 48 hours (or at hospital discharge if earlier) after elective PCI/stent. Safety will be evaluated by major bleeding events (Bleeding Academic Research Consortium type 3 or 5) at 48 hours (or discharge if it occurs earlier). Conclusion: ALPHEUS is the first properly sized trial comparing ticagrelor to clopidogrel in the setting of elective PCI and is especially designed to show a reduction in periprocedural events, a surrogate end point for mortality.

Monomorphic ventricular tachycardia (MVT) is common in adults with repaired tetralogy of Fallot (TOF), and is associated with sudden cardiac death. Management of MVT is not defined, and results of catheter ablation (CA) are limited.To evaluate long-term outcomes of MVT CA in repaired TOF.Thirty-four patients (mean age 32±10.3 years; 59% male) with repaired TOF underwent CA for symptomatic MVT between 1990 and 2012 in our centre; direct-current ablation (DCA) was used in 6%, radiofrequency followed by DCA in 29% and radiofrequency alone in 65%.Right ventricular (RV) dysfunction was present in 35% and left ventricular (LV) dysfunction in 21%. Mean numbers of clinical and induced MVTs were 1 and 2, respectively. Mean VT rate was 225±95bpm. Ablation targeted a single site (range 1-2), which was RV outflow tract in 85%. Primary success, defined as ventricular tachycardia (VT) termination during CA and final non-inducibility, was obtained in 82%. Seven patients (21%) required redo ablation in the first 3 months (before 2004; DCA). No death related to CA occurred. Mean follow-up time was 9.5±5.2 years. Antiarrhythmic therapy was discontinued in 71%. There were two cases of sudden cardiac death and four VT recurrences. Freedom from death and arrhythmia recurrence was 94% at 5 years, 81% at 10 years and 70% at 20 years. Global survival was 91% at 20 years. Baseline LV ejection fraction60% was significantly associated with ventricular arrhythmia recurrence (hazard ratio 16.4, 95% confidence interval 1.8-147; P=0.01).CA can safely address macroreentrant MVT in repaired TOF patients with an acceptable long-term rate of recurrence in this high-risk population. Anatomical classification of isthmuses with electroanatomical mapping provides reproducible endpoints for CA. Attention should be given to LV systolic function in risk assessment and selection of candidates for implantable cardioverter-defibrillator.

Up to 30% of patients with aortic stenosis (AS) present with discordant grading (aortic valve area [AVA]

International audience; Background: Percutaneous coronary intervention (PCI)-related myonecrosis is frequent and can affect the long-term prognosis of patients. To our knowledge, ticagrelor has not been evaluated in elective PCI and could reduce periprocedural ischaemic complications compared with clopidogrel, the currently recommended treatment. The aim of the ALPHEUS study was to examine if ticagrelor was superior to clopidogrel in reducing periprocedural myocardial necrosis in stable coronary patients undergoing high-risk elective PCI.Methods: The ALPHEUS study, a phase 3b, randomised, open-label trial, was done at 49 hospitals in France and Czech Republic. Patients with stable coronary artery disease were eligible for the study if they had an indication for PCI and at least one high-risk characteristic. Eligible patients were randomly assigned (1:1) to either ticagrelor (180 mg loading dose, 90 mg twice daily thereafter for 30 days) or clopidogrel (300-600 mg loading dose, 75 mg daily thereafter for 30 days) by use of an interactive web response system, and stratified by centre. The primary outcome was a composite of PCI-related type 4 (a or b) myocardial infarction or major myocardial injury and the primary safety outcome was major bleeding, both of which were evaluated within 48 h of PCI (or at hospital discharge if earlier). The primary analysis was based on all events that occurred in the intention-to-treat population. The trial was registered with ClinicalTrials.gov, NCT02617290.Findings: Between Jan 9, 2017, and May 28, 2020, 1910 patients were randomly assigned at 49 sites, 956 to the ticagrelor group and 954 to the clopidogrel group. 15 patients were excluded from the ticagrelor group and 12 from the clopidogrel group. At 48 h, the primary outcome was observed in 334 (35%) of 941 patients in the ticagrelor group and 341 (36%) of 942 patients in the clopidogrel group (odds ratio [OR] 0·97, 95% CI 0·80-1·17; p=0·75). The primary safety outcome did not differ between the two groups, but minor bleeding events were more frequently observed with ticagrelor than clopidogrel at 30 days (105 [11%] of 941 patients in the ticagrelor group vs 71 [8%] of 942 patients in the clopidogrel group; OR 1·54, 95% CI 1·12-2·11; p=0·0070).Interpretation: Ticagrelor was not superior to clopidogrel in reducing periprocedural myocardial necrosis after elective PCI and did not cause an increase in major bleeding, but did increase the rate of minor bleeding at 30 days. These results support the use of clopidogrel as the standard of care for elective PCI.Funding: ACTION Study Group and AstraZeneca.

Ticagrelor, a direct-acting and reversible P2Y12-adenosine diphosphate receptor antagonist, is recommended as a first-line antithrombotic agent in patients with acute coronary syndromes.1 The superiority of ticagrelor over other P2Y12 antagonists is thought to be mediated in part by pleiotropic properties associated with an increased concentration of adenosine, including cardioprotection, anticoagulant effects, and anti-inflammatory properties.2 However, these pleiotropic properties can also be responsible for major adverse effects, including electrophysiological consequences. Herein, we describe the case of a patient with severe cyclical sinus bradycardia and atrioventricular (AV) block related to ticagrelor.

Background In arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D), implantable cardioverter-defibrillators (ICD) after an episode of sustained monomorphic ventricular tachycardia (MVT) are currently recommended in most situations. However, radiofrequency catheter ablation (RCA) is effective in reducing recurrent VT and whether MVT is a surrogate of sudden cardiac death is debated when other risk factors are lacking. Purpose To report the outcomes of patients with ARVC/D who underwent RCA of well-tolerated MVT without a back-up ICD. Methods Patients with a definite ARVC/D diagnosis according to the 2010 Task Force revised criteria who underwent RCA of well-tolerated MVT at 9 tertiary centers across 5 countries, without an ICD prior to RCA and in the 3 following months were retrospectively included. Patients presenting with syncope or electrical storm, and patients with left ventricular ejection fraction Results Sixty-five patients [median age 46.1 years, range (19.5–73.8), 75% males] underwent RCA of MVT between 2003 and 2016. Familial history of ARVC/D was found in 11% of patients. Epsilon-waves were present in 19% and T-waves inversion beyond V2 in 43%. A right ventricular (RV) ejection fraction ≤40% or fractional area change ≤33% was found in 14 (25%) patients. Median left ventricular ejection fraction was 61% (50–70). Clinical presentation was palpitations in 81% of patients and near-syncope in 14%. Prior to RCA, patients were on beta-blockers alone in 18%, class I drugs in 37% and amiodarone in 9%, while 15% of patients were free any antiarrhythmic medication. Only 1 patient (2%) had >1 clinical VT morphology. Median VT rate was 180 (110–270). An epicardial approach was used in 31% patients. The clinical VT was inducible in 84% of patients. The median number of targeted RV site was 1 (1–3) (RV outflow tract in 72%). Full acute success defined inability to induce any VT was achieved in 72% of patients. During a median follow-up time of 49 month (1.4–162), there was no death or aborted cardiac arrest. Survival without VT recurrence was estimated at 82%, 71% and 60%, 12-, 36- and 60-months after RCA. No VT recurrence was observed among patient who had undergone an epicardial ablation. Among patients with VT recurrence, 6 (35%) did not receive an ICD, and 14 (70%) underwent redo RCA. An ICD was implanted in 10 patients, including 5 for VT recurrence. Fifty-eight patients constituted the control group, and 64% had appropriate ICD interventions during follow-up. Conclusions Despite a significant rate of VT recurrence, selected patients with ARVC/D who underwent RCA for stable MVT without an ICD did not experience any arrhythmic death. Further prospective studies are mandatory to precise the respective places of ICD and RCA in the management of ARVC/D patients with well-tolerated MVT. Acknowledgement/Funding None

International audience; BACKGROUND: Therapeutic strategies for electrical storm (ES) in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) are not well defined.OBJECTIVE: The purpose of this study was to report the acute and long-term results of ventricular tachycardia (VT) radiofrequency catheter ablation (RFCA) as a treatment of ES in patients with ARVC.METHODS: This multicenter study retrospectively enrolled 23 consecutive patients with ARVC (mean age 43.6 ± 16.7 years; all men) who underwent 24 RFCA procedures for ES between 2003 and 2015.RESULTS: Thirteen patients (57%) had a previous VT RFCA procedure; 14 (61%) had right ventricular dysfunction and 7 (30%) left ventricular ejection fraction ≤ 50%. The clinical VT was inducible in 19 procedures (79%). Epicardial ablation was performed in 4 procedures (17%). The median number of targeted VTs was 1 (range 1-6). Complete acute success (no VT inducible) was achieved in 11 procedures (46%) and partial acute success (clinical VT nor inducible) in 11 (46%). After a median follow-up of 3.9 years (range 1 month-10 years), ES recurred in 2 patients and end-stage heart failure developed in 4 (17%), leading to 1 death and 3 heart transplantations. At 1-year follow-up, the probability of freedom from VT recurrence was 75% and did not significantly predict long-term survival. At the last evaluation, 8 patients (35%) were free of non-β-blocker antiarrhythmic drugs as compared with 1 (4%) at baseline (P = .02).CONCLUSION: Catheter ablation was efficient to prevent ES recurrence in patients with ARVC. However, these patients were at high risk of evolution toward ARVC-related heart failure that was not associated with VT recurrence.

Aims Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy associated with a high risk of ventricular arrhythmia (VA). Current guidelines recommend beta-blockers as first-line medical therapy and if ineffective, sotalol or amiodarone. We describe our experience, as a tertiary centre for ARVC, with the effectiveness and tolerance of flecainide in addition to beta-blockers to prevent VA in ARVC. Methods and results We retrospectively included 100 consecutive ARVC patients who received flecainide with beta-blockers between May 1999 and November 2017. Treatment persistence and related side effects were assessed, as was VA-free survival on treatment, 24-h Holter monitoring and programmed ventricular stimulation (PVS) off- and on-treatment. Tolerance was good, with 10% flecainide discontinuations (lack of efficacy in six, atrial fibrillation in one, and side effects in three). No Brugada-induced electrocardiography pattern on flecainide or haemodynamic impairment was reported. Premature ventricular contraction burden at 24-h Holter monitoring was significantly decreased under treatment [median 415 (interquartile range, IQR 97–730) vs. 2370 (1572–3400) at baseline, P < 0.0001, n = 46]. Among the 33 patients with PVS under treatment, PVS was positive in 40% on-treatment vs. 94% off-treatment (P < 0.001). During a median follow-up of 47 months (IQR 23–73), 22 patients presented sustained VA on treatment, corresponding to an event rate of 5% [95% confidence interval (CI) (0.6–9)] at 1 year and 25% [95% CI (14–35)] at 5 years under treatment. No patient died. Conclusion This study suggests that flecainide and beta-blockers association is complementary to implantable cardioverter-defibrillator and catheter ablation and is safe for treating persistent symptomatic VA in patients with ARVC.

Introduction As opposed to ventricular arrhythmias (VA), clinical implications of atrial tachyarrhythmias (AT) in arrhythmogenic right ventricular cardiomyopathy (ARVC) remain scarcely explored. This study sought to evaluate the prevalence and prognostic significance of AT in ARVC. Methods In total, 171 patients diagnosed with ARVC between 1985 and 2018 in a single tertiary center were retrospectively included. Were defined as follows: AT: sustained atrial fibrillation, atrial flutter and focal atrial tachycardia; major adverse cardiovascular events (MAE) as a composite criterion including heart failure, cardiac assistance, transplantation, and death. Results After a median follow-up of 6 years (IQR 3 to 11), prevalence of AT, MAE and VA were respectively of 16%, 8% and 60%. AT occurred later in the disease course: mean age 54 ± 14.4, while 50% of patients had VA at diagnosis. Age at diagnosis (hazard-ratio [HR]: 1.05, 95% CI [1.02–1.08]; P Conclusion AT are common in ARVC and AT is associated with intensive sport activity and MAE. Our results mandate careful monitoring of ARVC patients with new-onset AT.

Background Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy associated with a high risk of ventricular arrhythmia (VA). Current guidelines recommend beta-blockers as first line medical therapy and if ineffective, treatment by sotalol or amiodarone. Purpose To describe our experience as a tertiary centre for ARVC on flecainide effectiveness and tolerance in addition to beta-blockers to prevent ventricular arrhythmia in ARVC. Methods We included one hundred ARVC patients treated with flecainide and beta-blocker diagnosed between May 1999 and November 2017. Ventricular events including sustained ventricular tachycardia (VT), ventricular fibrillation (VF) and ICD therapy were collected retrospectively in patient's medical records. Besides, a 24 hours Holter monitoring and a programmed ventricular stimulation (PVS) before and after introduction of flecainide were performed. Results Tolerance of flecainide was good with 9% discontinuation (6 for VA occurrence, 2 for side effect, 1 for atrial fibrillation). No Brugada-induced ECG pattern on flecainide was reported. PVC burden on 24 hours Holter monitoring (n = 46 patients) was significantly decreased under treatment (2370; IQR1572–3400 versus 415; IQR97–730, P Proportion of positive PVS was significantly decreased (n = 32 patients) with 94% having positive PVS before treatment compared to 37.5% on flecainide (P Fig. 1 ). During a median follow-up of 47 (IQR24–74) months, 23 patients had sustained VA (all monomorphic VT) on treatment (5% per patient–year). No sudden death occurred. Conclusion This study suggests that flecainide and beta-blockers association is complementary to ICD and catheter ablation, and is safe to treat ARVC patients with persistent symptomatic VA.

Background Atrial arrhythmias are common complications in adults with congenital heart disease (ACHD) and are associated with a high morbidity and mortality. Intra-atrial reentrant tachycardia (IART) is the most prevalent arrhythmia in this population. This study aimed to compare outcomes according to arrhythmia management. Methods All hospitalisations for IART in ACHD patients were retrospectively identified from medico-administrative database from September 2006 to September 2016. Outcomes of patients with IART managed with direct-current cardioversion (DCCV) were compared with patients referred for catheter ablation (CA). Atrial arrhythmia recurrence, death, stroke and first admission for heart failure (HF) were analyzed during follow-up. Results Ninety-one ACHD patients with IART (baseline median age 38 years [IQR 29-50], male 64%, 45% moderate CHD and 41% complex CHD) were included. Median follow-up was 3 years [IQR 1.6-6.7]. Twenty-six (28.6%) patients underwent DCCV and 65 (71.4%) CA. No significant difference in CHD complexity, past medical history of atrial arrhythmia and HF were observed between the 2 groups. Patients who underwent DCCV were significantly older (43 years [IQR 34–51] vs. 36 years [IQR 26–44], P = 0.03), with a higher CHADS-VASC score (CHADS-VASC ≥ 1 in 61% in DCCV group vs. 44% in CA group, P = 0.001). Atrial arrhythmia recurrence was observed in 52% after CA and 65% after DCCV ( Fig. 1 A, P = 0.28). Most of recurrence were IART, but atrial fibrillation was recorded in 37.5% after DCCV and 25.8% after CA (P = 0.4). At the end of follow-up, patients who were initially treated by CA were more frequently in sinus rhythm (84% vs. 27%, P = 0.02), with a significant lower incidence of severe cardiovascular events ( Fig. 1 B, P Conclusions A proactive management of IART by catheter CA is associated with better mid-term outcomes in adult patients with CHD.

A convergence of epidemiological and clinical features has prompted a precipitous rise in the prevalence of atrial fibrillation (AF) in the expanding population of adults with congenital heart disease. Herein, we synthesize the current state of knowledge on epidemiological features, associated morbidities, and pathophysiological insights regarding AF in adults with congenital heart disease. Management issues are examined including surgical, pharmacological, and catheter-based therapies. Throughout, knowledge gaps and avenues for future research are identified. Although AF has been coined the next arrhythmic epidemic to strike adults with congenital heart disease, it has already usurped atrial macroreentrant tachycardia as the most common presenting arrhythmia over the age of 50 years. Much remains to be discovered about the mitigating role of types of congenital defects, residual hemodynamic lesions, surgical sequelae, and ramifications of shunts and cyanosis on mechanisms and determinants of AF. Thromboprophylaxis is the cornerstone of pharmacological management, with anticoagulation recommended in patients with moderate or complex congenital heart disease and those with significant valve disease or risk factors for stroke. Considering the limited success with antiarrhythmic drugs, catheter ablation is increasingly performed. Non-pulmonary vein sources, focal and reentrant arrhythmias, appear to be important triggers for AF in this population. As such, they should be identified and addressed during catheter ablation interventions. The nascent literature on electrical isolation of pulmonary veins suggests that it is feasible and safe, although initial success rates appear to be modest. A more thorough understanding of underlying mechanisms and substrates carries the potential to further improve outcomes.

The incidence of atrial fibrillation (AF), the most common sustained arrhythmia and a major public health burden, increases exponentially with age. However, mechanisms underlying this long-recognized association remain incompletely understood. Experimental and human studies have demonstrated the involvement of aging in several arrhythmogenic processes, including atrial electrical and structural remodelling, disturbed calcium homeostasis, and enhanced atrial ectopic activity/increased vulnerability to re-entry induction. Given this wide range of putative mechanisms, the task of delineating the specific effects of aging responsible for AF promotion is not simple, as aging is itself associated with increasing prevalence of a host of AF-predisposing conditions, including heart failure, coronary artery disease, and hypertension. Although we usually think of old age promoting AF, there is also evidence that young age may actually have a protective effect against AF occurrence. For example, the low AF incidence among populations of young patients with significant structural congenital heart disease and substantial atrial enlargement/remodelling suggests that younger age might protect against fibrillation in the diseased atrium; efforts at understating how younger age may prevent AF might be helpful in elucidating missing mechanistic links between AF and age. The goal of this paper is to review the epidemiologic and pathophysiologic evidence regarding mechanisms underlying age-related AF. Although the therapeutic options for AF have recently improved, major gaps still remain and a better understanding of the special relationship between age and AF may be important for the identification of new targets for therapeutic innovation.

Objective Transaortic septal myectomy is the gold standard surgery in obstructive hypertrophic cardiomyopathy, but it is not optimal for children aged less than 5 years and with right ventricular outflow tract obstruction. We evaluated outcomes with the modified Konno procedure in children with severe forms of obstructive hypertrophic cardiomyopathy. Methods A total of 79 consecutive children who underwent the modified Konno procedure in our center between 1991 and 2016 were included. Results Clinical features included age less than 5 years (38%), maximal septal thickness 25 mm or more (32%), extension to the left ventricular apex (29%), and right ventricular outflow tract obstruction (28%). In total, 25% of patients had Noonan syndrome. Five children (6%) aged less than 15 months with Noonan syndrome and biventricular obstruction died in the hospital. Mean follow-up was 6 ± 5.7 years. Survival without death and heart transplantation was 82% at 20 years. Atrioventricular block occurred in 9 patients (11%) and was associated with right ventricular outflow tract obstruction and surgery before 2010. Death, resuscitated sudden cardiac death, and appropriate implantable defibrillator shock were associated with maximal septal thickness before surgery (adjusted odds ratio, 1.20; 95% confidence interval, 1.07-1.35; P = .002) and need for an associated procedure (adjusted odds ratio, 8.84; 95% confidence interval, 2.01-38.93; P = .004). There was no case of recurrent obstruction. Reoperation was required in 4 patients (5%) for other reasons. Conclusions The modified Konno procedure provided durable obstruction relief and good long-term survival in children with severe forms of obstructive hypertrophic cardiomyopathy. Children with Noonan syndrome undergoing surgery early in life were at higher risk of early mortality.

Background Organized atrial arrhythmias (OAAs) are common after orthotopic heart transplantation (OHT). Some controversies remain about their clinical presentation, relationship with atrial anastomosis and electrophysiologic features. The objectives of this retrospective study were to determine the mechanisms of OAAs after OHT and describe the outcomes of radiofrequency catheter ablation (RFCA). Methods Thirty consecutive transplanted patients (mean age 48 ± 17 years, 86.6% male) underwent 3-dimensional electroanatomic mapping and RFCA of their OAA from 2004 to 2012 at our center. Results Twenty-two patients had biatrial anastomosis and 8 had bicaval anastomosis. Macro-reentry was the arrhythmia mechanism for 96% of patients. The electrophysiologic diagnoses were: cavotricuspid isthmus (CTI)-dependent atrial flutter (AFL) in 93% of patients ( n = 28); perimitral AFL in 3% (n = 1); and focal atrial tachycardia (FAT) in 3% ( n = 1). In 5 patients with biatrial anastomosis, a right FAT was inducible. Primary RFCA success was obtained in 93% of patients. Mean follow-up time was 39 ± 26.8 months. Electrical repermeation between recipient and donor atria , present in 20% of patients ( n = 6), did not account for any of the OAAs observed. Survival without OAA relapse at 12, 24 and 60 months was 93%, 89% and 79%, respectively. Conclusions CTI-dependent AFL accounted for most instances of OAA after OHT, regardless of anastomosis type. Time from transplantation to OAA was shorter with bicaval than with biatrial anastomosis. RFCA was safe and provided good long-term results.

Aims The aim of this study was to evaluate the feasibility, safety and outcomes of TAVI performed via the suprasternal brachiocephalic approach in selected patients at high or prohibitive surgical risk who are not eligible for transfemoral or subclavian TAVI. Methods and results From March 2014 to March 2016, 26 high-risk patients without transfemoral or subclavian access options were considered for TAVI via a suprasternal brachiocephalic approach. The feasibility of the suprasternal brachiocephalic approach was determined according to computed tomography findings. In 23 (88.4%) patients the procedure was performed as intended, whereas in three (11.5%) patients the approach was converted to a right carotid access. Both self-expanding (n=20, 76.9%) and balloon-expandable prostheses (n=6, 23.1%) were used. At 30 days, no patient had died; there was one major stroke (3.8%) and there were three major vascular access site-related complications (11.5%). After a median follow-up of 317 days (57-705), two patients had died, both from cardiovascular causes, and 19 out of 24 survivors (79.2%) were in New York Heart Association functional Class I or II. Conclusions This single-centre case series suggests that TAVI using the suprasternal brachiocephalic approach is feasible in selected patients and may represent an additional alternative route in patients who are not eligible for other approaches.