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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 Relations between voltage mapping and diagnosis or genetic background in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) have not been investigated so far. Objective We investigate if diagnosis or genetic background were linked to voltage mapping in ARVC. Method 70 patients with proved or suspected ARVC undergoing 3D endocardial mapping and genetic testing have been retrospectively included. Localisation and extension of bipolar low voltage areas were correlated to ARVC diagnosis and presence of a culprit mutation. Results 44/70 fulfilled ARVC Task Force criteria and 25/70 had culprit mutations. Endocardial (38/44 vs 16/26, p=0.02) and especially infero-lateral scars (31/44 vs 9/26, p=0.003) were more often present in patients fulfilling Task force criteria vs suspected ARVC, with larger scars (area 23±27 vs 8±11 cm2, p=0.04, perimeter 17±10 vs 11±7 cm, p=0.03) (sensitivity 86%). Mutated patients had more infero-lateral (19/25 vs 21/45, p=0.01), multiple (12/20 vs 11/34, p=0.04) and larger scars (perimeter 21±10 vs 12±7 cm, p=0.01) vs non mutated patients. In patients with ARVC diagnosed according to the Task Force criteria, there was a trend toward more infero-lateral (p=0.09) and larger scars (p=0.08) in mutated cases. PKP2-mutated cases tended to have less ourflow tract (p=0.08) and less multiple scars (p=0.09) vs other mutations. Conclusion 3D endocardial mapping could have an important role for ARVC diagnosis and may be able to detect minor forms with otherwise insufficiant criteria for diagnosis. More frequent and larger infero-lateral scars are present in mutated patients with bordeline differences according to the mutated genes. Funding Acknowledgement Type of funding source: None

Introduction Relations between scars characteristics and genetic background in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) has not been investigated so far. Objective To investigate if genetic background change the scars characteristics in ARVC. Method 66 patients with ARVC (24 with culprit mutation) having undergone endocardial mapping have been retrospectively included. Every patient had a diagnosis of ARVC according to the task force criteria. Localisations, surfaces and perimeters of low votage areas ( Results Mutated patients more often had altered right ventricular ejection fraction ( Conclusion In our ARVC population, culprit mutations increase size and number of intracardiac scars and degree of biventricular involvment, and localize scars in infero-lateral areas compared to patients without mutation.

Background Ventricular arrhythmias and sudden death are potential late complications in patients with tetralogy of Fallot. Data regarding the value of implantable cardioverter defibrillators (ICD) are scarce in this population. Purpose To assess long-term rates of appropriate ICD therapies and ICD-related complications in a large registry. Methods The DAI-T4F study is an ongoing national French registry including all patients with tetralogy of Fallot and ICD ( NCT03837574 ). Information has been collected prospectively since 2010 with annual update. Cox proportional hazard models were used to identify factors associated with appropriate ICD therapies and complications. Results A total of 134 patients (median age 41.7 years, 70.7% males) were enrolled, including 47 (35.1%) for primary prevention. The median (IQR) follow-up duration was 6.1 (2.7–10.2) years. Overall, 14 (29.8%) and 45 (51.7%) patients received at least one appropriate ICD therapy in primary and secondary prevention, respectively, giving annual incidences of 5.5% and 7.1% (P = 0.06). Patients with altered left ventricle ejection fraction (LVEF) at inclusion ≤ 35% experienced less appropriate ICD therapies (HR = 0.31, 95% CI: 0.11-0.86, P = 0.02), whereas a history of sustained or non-sustained ventricular arrhythmia (HR = 2.7, 95% CI: 1.2–3.9, P = 0.03) was positively associated with appropriate therapies. Fifty-seven (42.5%) patients had ICD-related complications, including 32 (24.2%) inappropriate ICD shocks. During follow-up, 7 (5.2%) patients underwent cardiac transplantation and 12 (9.0%) patients died, mainly from progressive heart failure (n = 5). Only one sudden death due to electrical storm was recorded ( Fig. 1 ). Conclusions Appropriate therapies are frequent in patients with tetralogy of Fallot and ICDs, including in primary prevention. The relatively important proportion of ICD-related complication highlights the need for improving risk stratification in this population.

Background Cardiotoxicity of some medical anti-cancer treatment and radiotherapy (RT) is well known for breast cancer (BC) patients. However, heart rhythm disorders are almost absent of research in this area, while many studies have reported cases of syncope, atrioventricular block or atrial fibrillation occurring several years after cancer treatment. Purpose To evaluate whether BC treatment, in particular RT, is associated with an increased risk of cardiac arrhythmias and conduction disorders (ACD) characterized by implantation of a pacemaker or defibrillator or hospitalization for rhythm disorders possibly associated with interventional procedures (EP Study and/or ablation). Methods Our study is based on the Echantillon Generaliste de Beneficiaires (EGB), the 1/97 random sample of Systeme National des Donnees de Sante, the French nationwide health database. Within the EGB we will identify patients treated for BC between 2008 and 2012 after excluding cases of prevalent cancer, arrhythmia or conduction disorders and identify incident cases of ACD arising post BC and until December 2017. The ACD incidence rates in the population of BC according to the BC treatment (surgery ± chemotherapy ± RT) will be estimated and compared to the national incidence rates estimated from the whole EGB population by age and calendar year standardization. Results With an initial cohort of approximately 260 000 women in the EGB, we plan to identify nearly 3 000 patients treated for BC between 2008 and 2012. Based on the estimation of PM or DEF implantations or AF cases in France, we should observe approximately 5 000 incident cases of ACD in the general population and 60 incident cases in the BC subgroup if the BC treatment has no effect. The observation of at least 85 cases instead of 60 (RR = 1.4) will have a statistical power of 80%. Conclusion The study is ongoing. It will allow quantifying the excess risk of ACD suspected for BC treatment, in particular mediastinal RT.

Background TOF is the most frequent congenital heart disease managed by cardiologists, at particularly high risk of sudden cardiac death (SCD). However, few studies have reported long-term outcomes of TOF with ICD. Purpose We aimed to report characteristics and outcomes from a large population of TOF with ICD. Methods Between 2005 and 2016, all TOF implanted with an ICD in 22 centers were enrolled to determine characteristics at implantation and outcomes. Results Overall, 101 patients (43 ± 13 years, 69% males) were enrolled. A majority was implanted for secondary prevention (71%), whereas the remaining (29%) was for primary prevention. Among the latter, risk factors for SCD were: severe pulmonary regurgitation (25%), prior palliative shunt (43%), syncope (21%), inducible ventricular tachycardia (VT) (39%), non-sustained VT (29%), QRS duration ≥ 180 ms (19%), left ventricular significant dysfunction (29%, with ejection fraction ≤ 45%), and sustained supra-VT (46%). After a mean follow-up of 6.0 ± 4 years, 44 (43%) experienced ≥ one appropriate therapy (25% in the primary vs. 51% in the secondary prevention group), giving annual-incidences of 4.95% and 14.1%, respectively (P = 0.009). The median time between ICD implantation and the first appropriate therapy was 0.70 years (0.26–3.75), without significant differences between primary and secondary prevention (P = 0.27). Overall, ≥ 1 complication occurred in 40 patients (39%), including inappropriate shock (n = 26), major pocket hematoma (n = 1), lead dysfunction (n = 15), infection (n = 6), algodystrophia (n = 2), device failure needing reintervention (n = 3). Eventually, 6 patients received heart transplant (6%), and 9 (9%) died during the course of follow-up. Conclusions TOF and ICDs experience high rates of appropriate therapies, especially in secondary prevention. ICD-related complications remain very high. Selection of candidates for primary prevention remains challenging and can be potentially improved.

RésuméBien que les indications d’implantation de pacemaker présentent de nombreuses similitudes entre enfants et adultes, il existe plusieurs particularités dans la population pédiatrique justifiant une approche spécifique. Le bloc auriculo-ventriculaire congénital, le bloc auriculo-ventriculaire postopératoire, la dysfonction sinusale, le syndrome bradycardietachycardie, le syndrome du QT long congénital et la resynchronisation biventriculaire constituent les principales indications d’implantation en pédiatrie. La caractéristique propre des jeunes patients est qu’ils seront stimulés sur une durée prolongée, impliquant : 1) une réflexion parfois difficile concernant la décision d’implanter un dispositif définitif de stimulation, d’autant qu’en l’absence d’études cliniques randomisées menées en pédiatrie, les recommandations émanant des sociétés savantes sont pour la plupart basées sur des consensus ; 2) une fois la décision prise d’implanter un stimulateur cardiaque, la question de la voie d’abord est également essentielle. L’implantation d’un pacemaker peut être réalisée chez l’enfant par voie endocardique ou épicardique avec un faible taux de complication à court et moyen terme. Cependant, les complications à plus long terme, moins étudiées, pourraient s’avérer plus problématiques. L’effet d’une stimulation prolongée durant toute une vie sur un site ventriculaire suboptimal ou même délétère pourrait avoir été sous-évalué. De même, les enfants sont exposés à un risque plus important de complications tardives en rapport avec leur matériel de stimulation.Dans cette revue, nous proposons la description des principales indications de stimulation cardiaque en pédiatrie, discutons de l’intérêt et des limites des différentes voies d’abord et essayons d’envisager les futurs progrès qui pourraient permettre une véritable révolution dans la discipline.SummaryAlthough the general indications for pacemaker implantation in children and adolescents are similar to those in adults, there are several important considerations in young patients. Congenital atrioventricular block, surgical or acquired atrioventricular block, sinus node dysfunction, bradycardia - tachycardia syndrome, long QT syndrome and cardiac resynchronization therapy are the main indications for pacing in the paediatric population. One of the peculiar characteristics in young patients is the long duration of pacing, which involves: difficult reflection regarding the indications for and timing of pacing therapy (all the more so given that the absence of randomized clinical trials of cardiac pacing conducted in the paediatric population, which means that the level of evidence for most recommendations is based on consensus); and the choice between endocardial and epicardial approaches. Indeed, children can now undergo implantation of epicardial or endocardial pacing systems with low rates of short- and mid-term compl ications. However, despite no clear demonstration in the literature because of the absence of studies with life-long follow-up, long-term complications may impact on the life expectancy of these patients. Lifelong pacing effects on a suboptimal or detrimental ventricular site may have been underestimated. Moreover, children are prone to a higher incidence of longterm adverse events because of the pacing device. In this review, we present the pacing indications and recommendations in the paediatric population, we discuss the benefits and limitations of epicardial and endocardial approaches and we examine whether new cardiac stimulation techniques might enable an absolute revolution in pacing therapy.

IntroductionFrequent premature ventricular beats (PVB) may induce cardiomyopathy (CM). Characteristics and prognosis factor for recovery after RF ablation remain debated.Methods93 patients (74% men, 58±14 yo) with dilated CM associated with frequent isolated PVB were included. A group of 75 pts undergoing ablation for symptomatic PVB without significant cardiac disease serves as the control group.ResultsEF was 38±10% and left ventricular end diastolic diameter (LVEDD) was 63±8mm. One third have various associated cardiomyopathy.PVB burden was 27±12%. PVB arose from the left ventricle in 96 pts (LVOT 61, mitral 16, apex 7, septal 12) and from the right ventricle in 61 pts (RVOT 58) and multiple in 11. Epicardial ablation in the CS was needed in 25.In multivariate analysis, lack of palpitations (OR 9.09 [3.45-33.33]), VPB number > 20000 (OR 5.40 [1.98-14.70]), left ventricular origin (OR 4.12 [1.53-11.11]), epicardial location (OR 11.00 [1.92-62.50]), VPB right inferior axis (OR 2.31 [0.85-6.27]), baseline QRS width > 100ms (OR 3.66 [1.2810.43]), VPB coupling interval > 500ms (OR 3.11 [1.14-8.55]) and polymorphic VPB (OR 10.40 [1.05-103.05]) were independantly associated with CM compared to controls (p 80% reduction). In these, EF increased (36±9 to 51±12%, p 10% increase in EF. Only a VPB > 2mV (OR 19.2 [1.84-200.00], p=0.01) was independanlty associated with reversal of CM in multivariate analysis.ConclusionMechanisms leading to PVB-induced CM may involve lack of palpitations, a high VPB number, a left ventricular origin, an epicardial location, a VPB right inferior axis, a large baseline QRS duration, a long VPB coupling interval and polymorphic VPB. Reversal of CM after RF ablation may associate a high VPB amplitude and a shorter VPB coupling interval. This may help in selecting patients for RF ablation of suspected VPB-induced CM.

IntroductionAtrial fibrillation (AFib) progressively leads to electrical remodeling (ER) and anatomical-mechanical remodeling (AMR) whose relationships remain poorly known.MethodsER and AMR were compared in patients undergoing percutaneous RF ablation for AFib. ER was defined by right and left appendage activation rate (RAAAR and LAAAR) as a surrogate for atrial refractory periods. AMR was approached by left atrium (LA) diameters and area and left atrial appendage (LAA) area and contractile function (mean emptying flow velocity) (LAAFV) as determined during transoesophageal and transthoracic echocardiography performed during AFib the day before or immediately before RF ablation. Mean duration between successive LAA contractions was considered as LAA mechanical rate.Results40 pts with paroxysmal AFib (n=10), persistent AFib (n=25) or long-persistent AFib (n=5) were included (30 men, 64±9 yo, EF 39±14%). 63% were on amiodarone.Parameters exploring AMR were highly correlated to each other: LA area 27±7cm2; LAA area 5.5±2cm2; LA transv 48±14 mm; LA ant-post 58±13 mm; LAA velocity 28±13cm/sec (p

IntroductionFrequent premature ventricular beats (PVB) may induce cardiomyopathy (CM) whose characteristics and underlying mechanisms are poorly knownMethods38 patients (27 men, 57±16yo) with dilated CM associated with frequent isolated PVB suspected to be responsible the CM were ablated between 2005 and 2011. PVB were documented 5 years (n=10) before (unknown in 9). Ejection fraction (EF), left ventricular end-diastolic diameter (LVEDD) and NYHA class were compared before and after radio-frequency ablation. 16 pts with symptomatic PVB without CM serves as the control groupResultsBaseline EF was 39±1%, LVEDD was 61±7mm and mean NYHA class was 1.9±0.8. PVB arose from the RV in 10 pts (RVOT in 7) and from the LV in 28 pts (LVOTt in 10 and coronary cusps in 10)Compared to the control group, daily PVB number was not different in pts with suspected VPB-induced CM (22000±12000 vs 20000±14000, p=ns), as was the presence of PVB left bundle branch pattern (24/38 vs 13/16, p=ns) and gender or age. Pts with suspected PVB-induced CM had more often right axis VPB (31/37 vs 10/16, p=0.08). History of PVB was shorter in controls. Origin of the PVB did not differ between groupsRF ablation completely eliminated PVB in 26 pts (68%), partially in 4 and was inefficient in 8. During a follow up of 19±19 months, EF increased from 39±10 to 52±13% (p=0.003) while LVEDD decrease from 61±7 to 56±7mm (p=0.002) leading to a decrease in NYHA class (1.9±0.8 to 1.4±0.6, p=0.02). Parameters related to the failure of ablation were an older age, a higher NYHA class and a LVOT location of the focus. Parameters related to the lack of reversal of CM in successful ablations, were an older age, a lower baseline EF, but not the length of PVB history.ConclusionRF ablation of frequent PVB may lead to cure or significant improvement in 78% of pts with associated CM. Plausible mechanisms leading to a PVB-induced CM may associate a more longer history of PVB.

Introduction T wave alternans (TWA) is a reliable marker of risk for malignant ventricular arrhythmias whose prognosis value has been established in many populations. Short QT syndrome (SQTS) is a recently described and very rare channelopathy defined by a decrease in repolarisation duration and carrying a risk of ventricular fibrillation. TWA in SQTS has not been evaluated at yet. Methods 6 patients with SQTS (QT 310 ± 16 ms, QTc 329 ± 11 ms) underwent microvolt TWA measurement using spectral analysis. TWA testing was performed using Heartwave II (Cambridge HeartTM) during bicycle exercice and classified as negative, positive or indeterminate according to the published standards for clinical interpretation. Results there were 6 men (mean age 24 ± 2 yo): 4 asymptomatic, 1 with previous sudden cardiac death and 1 with unexplained syncope. 3 patients belonged from the same family. Familial history of SQTS was present in 4 (2 families) and history of unexplained sudden death was found in both families. Ventricular fibrillation was induced in 2 of 5 investigated patients. Two patients were implanted with an ICD without any appropriate therapy during follow-up. No patient was on any medical therapy. Genetic analysis was pending in each but without result at yet. TWA was negative in each patient except in one inducible asymptomatic patient (indeterminate due to too fast heart rate). Maximal negative heart rate was 113 ± 5 bpm. Conclusions Conversely to what is observed in other settings, TWA testing is negative in most SQTS patients even in the symptomatic or inducible ones. This may be explained by the longer diastolic interval according to the restitution curve theory. Measurement of TWA for risk stratification in SQTS is therefore useless.

PurposeThe infections after cardiac device implantation (CDI) are not well known in the real life because of the multiplicity of the circumstances. This is a monocentric prospective study.Method304 consecutive pts had a CDI in 6 months (feb to aug 2009): male 69% age 70±15 yo. The data of the pts were consecutively collected:type of device (VVI PM 10%, DDD PM 42%, CRT P 7%, VVI ICD 13%, DDD ICD 10%, CRT D 17%) (Primo Implant 73%, Device Replacement 21%, Lead Replacement 8%, Burying 2.3%).components of the NNIS score (N1. Nosocomial Infection Surveillance): −1 duration of the procedure (83±40mn); 7minus;2 ASA score (Am. Soc. Anesth)(asal=4%, asa2=22%, asa3=43%, asa4=30%, asa5=0); −3 surgical site Infection (SSI) Class: Clean wound 73%, fever the days before 6%.the prevention with antibiotic therapy was: – conventional: Cefuroxine 1.5 g 30 mn before and 0.75g each 2hr (93%; 69% timing conformity); – Vancomicyne 1gr 1hr before (3%); previous adjusted therapy (4%).the follow-up was of 3 months.Results7 SSI occurred at 43±36 days (2.3%) for DDD PM = 5, VVI ICD = 1, CRT D = 1. They were 2 endocarditis (for 2 implantations on the other side after an extraction, with the same germ) sepsis = 1 (diabetes), loge infection = 4 (2 after burying). The 7 pts had an extraction of the device and the leads. The risk factors of SSI were anticoagulation (7/7; p

BackgroundICD is an effective treatment to prevent SCD but very long term follow-up is poorly known.MethodsWe retrospectively studied the 67 patients implanted with an ICD at the University Hospital of Toulouse between 1989 and 1999.ResultsCharacteristics of the population are (number of patients): males: 56, ischemic heart disease (IHD): 42, mean EF 42%, secondary prevention: 62, abdominal implantation: 30, dual chamber devices: 57. Data were complete for 58 patients (mean follow-up: 100 months +/− 103), nine patients were lost to follow up (mean 52 +/− 40 months). Survival rate (of the 58 patients) is respectively 93%, 71% and 48% at 1, 5 and 10 years. Cause of death was cardiac for 10 patients (7 terminal cardiac failures, 2 sudden cardiac deaths and 1 endocarditis), extra cardiac for 7 patients and unknown for 13.Independent factors of global mortality are: age (p = 0.038), IHD (p = 0.035), NYHA>2 (p = 0.0058), EF

Introduction radio-frequency (RF) ablation of premature ventricular beats (PVB) has been only reported as isolated cases or in short series. Methods 34 successive pts (25 men, 53 ± 18 yo) underwent RF ablation of frequent or symptomatic isolated PVB at our institution from 2005 to 2009. Indication, technique and results were reported. Results 43 RF ablation of PVB was performed for post-ischemic electrical storm in 7 pts (6 men), for symptomatic PVB in 7 (3 men) and for suspected VPB-induced tachycardiomyopathy in 20 (16 men) with 1,2 procedure/pt. RF was performed in each case using pace mapping and activation mapping with conventional technique. RF ablation of PVB during intractable post-ischemic electrical storm was initially successful in each pt but fatal electrical storm recurred in 4 during the following days. PVB were targeted in the left septal Purkinje network. 10 procedures were performed in 7 pts for palpitations or syncope, leading to complete or partial clinical success in 6 (PVB reduction 9400 ± 12200 to 420 ± 610 /24h, NS). 25 procedures were performed for suspected VPB-induced tachycardiomyopathy in 20 pts leading to complete success in 9 pts and to significant decrease in VPB in 5 pts. Mean VPB/24h was 17800 ± 8600 before and 3410 ± 8150 after and 125 ± 230 at 11 ± 6 months follow-up (p=0,0005). EF increase from 41 ± 8 to 53 ± 7% (p Conclusions RF ablation of PVB may be indicated if highly symptomatic, for electrical storm or for suspected tachycardiomyopathy with good although imperfect success rate

IntroductionSustained ventricular tachycardia (VT) in structural heart disease (SHD) is usually an indication for ICD implantation. Whether successful RF ablation of VT in this setting could avoid the need of ICD implantation is unknown.MethodsWe report our initial experience of VT ablation in 27 successive non-implanted pts (22 men, 62±18 yo) presenting with sustained monomorphic VT and SHD. An ICD was not implanted either because of debilitating or end stage extra cardiac pathology, advanced age or because the VT was well tolerated and EF was preserved. Amiodarone failed to control VT in 33% and beta-blockers in 73%.Results33 procedures were performed (1.2/pt). Seventeen pts had ischemic heart disease, four had ARVD, 2 dilated cardiomyopathy, 1 congenital and three had non-categorized SHD. Mean EF was 44±13% (5 pts with EF < 0,35%). The end-point was elimination of post-systolic potentials by RF. VT was inducible in 71% with 1 to 4 different morphology (1.8±1). Acute success was defined by lack of any inducible monomorphic sustained VT and was achieved in 78%. The only complication was an arterial dissection managed with angioplasty. One patient died after 10 days due to intractable heart failure associated with relapse of electrical storm. Patients were discharged on beta-blockers and amiodarone was interrupted except when otherwise indicated. Mean follow-up was 13±9 months. Four patients died from non arrhythmic cardiac or non cardiac causes. There was one late sudden death in a patient with low EF (incomplete procedure due to ongoing vascular complication). VT recurred in 24% without compromise of the vital status. RF ablation was performed in again in 5 pts and VT further recurred in two. Final VT recurrence rate including redo precedures was 12%.ConclusionsRF ablation for VT occurring in pts with SHD without ICD appears relatively safe and efficient and may be considered in some cases when ICD therapy is not desired because of associated morbidity, advanced age or when VT is well tolerated and does not occur in the context of advanced heart disease.

Introductionearly repolarization (ER) in Brugada or short QT syndrome is common and has been associated with a less favourable outcome. Even if apparently paradoxical, ER can also be seen in long QT (LQT) but prevalence and correlations to other variables are unknown.MethodsECG of 105 LQT patients (44 men, 36±21 yo) and 269 age and gender matched controls (135 men, 36±18 yo) were reviewed. LQT was diagnosed by a positive genetic testing (n=71) or by showing abnormal T wave and long QT interval spontaneously or during epinephrin infusion in pts without discovered genetic mutation (n=34). ER was defined by > 1mm J point elevation in the inferior or lateral leads with notch or slurring pattern.ResultsQT in lead II was 433±68 msec in LQT patients and 338±41 in controls (p

Introduction T wave morphological changes before onset of ventricular arrhythmia are poorly known. ICD-stored intracardiac electrograms (EGM) present a unique opportunity for detecting temporal changes in repolarisation before initiation of VT/VF. Methods 57 implanted patients with St Jude Medical ICDs (45 men, 64 ± 12 yo, mean EF 34 ± 15%) were prospectively enrolled. Eleven different T wave parameters were extracted from EGM (T amplitude, T peak time, T end time, T duration between baseline crossing and between points of maximal slopes, T peak to T end, maximal ascending and descending slopes, timing of points of maximal slopes and T wave area). Values of each parameter in recordings prior to VT/VF were compared to control recordings sharing comparable QRS and T wave morphology and similar heart rate in the same pts. Results 23 VT/VF episodes (24 ± 13 beats) and 13 baseline (25 ± 9 beats) (ns) were analyzed in 12 pts (1,9 episode/pt). Mean heart rate was 71 ± 17 bpm for episodes and 66 ± 16 bpm for baseline recordings (ns). Significant differences were found in T wave amplitude, T wave maximal slopes, T peak time and timing of the T wave maximal ascending slope (although ns when corrected by the heart rate). Conclusions T wave amplitude is higher, T wave slopes are steeper and initial parts of the T wave are earlier before ventricular arrhythmia compared to baseline. Detection of T wave changes prior to VT/VF might be useful in predicting imminent arrhythmia occurrence. Download : Download full-size image Example of T wave changes before VT

Background The mechanisms of action of cardiac resynchronisation therapy (CRT) and the predictive factors to CRT's effect are not well known. The mechanical left intra-ventricular dysynchrony (LIVD) has a important physiopathological role in severe left ventricular dysfunction with wide QRS. Several studies compared LIVD before and just after CRT implant but few data are available about LIVD in the mid to long term. Methods and Results We included prospectively the 42 consecutive patients (younger than 75 yo) implanted in the University Hospital of Toulouse with ACC/AHA/ESC indications for CRT. Characteristics of the population were: male sex 88%, ischemic cardiomyopathy 45%, mean LVEF 22%, mean QRS duration 160 ms, CRT-D 98%. The echocardiographic (Pulsed Doppler and Tissue Doppler Imaging) parameters of cardiac dysynchrony were obtained at baseline and six month (or more) after CRT implant. We defined the responder group as an improvement in 1 NYHA class and no hospitalisation for heart failure (HF) during the follow up (mean duration 15 month). 62% (n = 26) were clinical responders. The echocardiographic assessment was done at 11 (6 to 20) month. There was no statistically significant difference between the “residual” mechanical dysynchrony of the responders versus the non-responders. Considering only the responders, there is no reduction of LIVD at six month after CRT. A baseline aortic pre-ejection delay > 140 ms, a non ischemic cardiomyopathy and the left ventricular activation time measured before implantation with a method described recently by Sweeney et al are the only factors of response to CRT. Conclusions The clinical response to CRT is not well correlate with the “residual” mechanical LIVD such as we measured by echocardiography (DTI and PD).

Background and purposeWe studied short term effect of CRT implantation on renal function and focused especially on the role of contrast agent dose.Method: Acute renal failure (ARF) was defined as a decrease of more than 25% of the clearance of creatinine (Cl Cr) calculated with MDRD method within four days following implantation.Results: We considered 141 patients referred for CRT implantation: male: 79%, mean age: 68 +/−12, diabetes: 71%, HTA: 38%, Cl Cr: 48 mL +/−19, median contrast dose: 48 mL (IR: 30/80), median BNP variation: −30% (IR:-58/-8), Haemoglobin (Hb) variation: −6.3% +/−11.2.ARF occurred in 19 patients (13.5%), 3 of them died and one was lost. Renal function of 14 out of the 15 remaining completely recovered.In-hospital stay longer than 10 days was more frequent in ARF group (OR = 5.18, p

IntroductionMonomorphic sustained ventricular tachycardia (MSVT) in structural heart disease (SHD) carries poor prognosis and usually needs ICD implantation. Whether successful RF ablation of VT in this setting could avoid the need of ICD implantation is largely unknown.Methods105 successive with SHD (88 men, age 64±16) were followed after RF ablation of MSVT. An ICD was not implanted either because of end stage extra cardiac pathology, advanced age or well-tolerated MSVT and preserved EF. 33% were on amiodarone and 51% on beta-blockers.Results128 procedures were performed (1,2/pt) in 4 centers. Coronary artery disease was present in 54%, right ventricular dysplasia in 10%, dilated cardiomyopathy in 10% and various SHD in 26%. Mean EF was 48±12% (14% with EF

Background The success rate of left ventricular (LV) lead implantation for cardiac resynchronization therapy (CRT) is high. Congenital abnormalities of the coronary sinus (CS) are rare but can be responsible for unsuccessful implantation. Method and result A 64-year-old male with standard indication for CRT was referred to our institution for a CRT-D implantation. Unfortunately, intubation of the CS was not possible. TEE showed a congenital CS anomaly with complete drainage of the CS into the left atrium. Because of contra-indication for general anaesthesia and thus for surgical epicardial implantation, we proposed to implant the LV lead by a transseptal approach. After the patient had given his consent, a transseptal puncture was performed via the right femoral vein. A conventional screw-in lead was implanted at the laterobasal segment of the LV using a deflectable catheter guide introduced via the left subclavian vein through the transseptal puncture. Post-implantation parameters of the LV lead were acceptable: pacing threshold 0.7 V-0.4 ms, impedance 435 ohms, R wave amplitude 5 mvolts. Right ventricular and right atrium leads were then implanted (fig 1) A post-implant CT scan confirmed the ectopic location of the CS ostium without persistent superior vena cava (fig 2). Download : Download full-size image Figure 1 Download : Download full-size image Figure 2 The patient was discharged on anticoagulation with a targeted international normalized ratio between 3 and 4. At the one- and three-month follow-up, no complication had occurred. He was in class 2 of the NYHA and had not been hospitalised. Electrical parameters remained steady. Conclusion Complete drainage of the CS in the left atrium without persistent vena cava is a rare congenital abnormality. When cardiac resynchronization therapy is needed, transseptal implantation could be a feasible alternative.

Introduction post-ischemic electrical storm (PIES) occurring shortly after the acute phase of myocardial infarction (MI) is a rare but potentially lethal phenomenon. Clinical and therapeutic options are poorly known. Methods 14 pts (12 men, 61 ± 13 years old) underwent RF ablation for intractable PIES. PIES occurred 10 ± 5 days after MI (4 months in one pt). Mean EF was 28 ± 7%. Fast monomorphic VT (10 pts), FV (8 pts) or torsades de pointes (5 pts) recurred in each pt despite maximal anti-arrhythmic therapy (mean 22 ± 13 episodes). MI was inaugural anterior MI in each but 3 pts (subendocardial in 2, inferior in 1), with one vessel disease (left descending artery) in 8 pts (2 or 3 vessels in 6). When performed (4 pts), new PTCA did not control PIES. RF ablation was performed as a last chance solution, targetting ventricular premature beats arising from the left septal Purkinje network (in all patients the arrhythmia was of Purkinje origin, defined by a sharp spike Purkinje-potential preceding ventricular activation during sinus rhythm as well as during arrhythmia). Results Abolition of Purkinje potentials and of Purkinje premature beats led to resumption of electrical storm in 12/14 pts with 1 procedure (2 in one pt). The two pts with unsuccessful RF ablation died within a few hours from intractable heart failure and recurring arrhythmias. All other pts were implanted with an ICD (two pts had already been implanted before). During a 18 ± 19 months follow-up, 3 pts present with recurring isolated VT episodes found in ICD memories. Seven pts died from cardiac or non cardiac causes (7 ± 10 months). Conclusions PIES is a rare but potentially lethal event occurring mainly in inaugural large anterior acute MI. RF ablation of initiating Purkinje-premature beats may be an efficient therapeutic solution despite the poor long-term prognosis of such patients.

IntroductionDC shock is commonly used for external cardioversion of cardiac arrhythmias. Incidence, characteristics and predictive factors of transient ST-segment changes after DC shock are poorly known.Methods91 consecutive pts referred for external cardioversion of atrial fibrillation (AFib) (61 men, 69±10 yo) were prospectively included. Duration and amplitude of ST elevation or depression were quantified on12 lead-ECG immediately after the first DC shock. Correlations with DC shock characteristics clinical variables, underlying heart disease, echocardiographic parameters, biological parameters, medications, anesthesic drugs as well with morphological features were made.Results18 and 20 pts underwent 200 J or 300 J monophasic and 53 pts 200 J biphasic DC shocks. Immediate success rate was 95%.We found an incidence of 48% for ST segment changes: 35% for ST elevation (0,81±0,44 mV) and 13% for ST depression (0,2±0,07 mV, p

IntroductionVery narrow QRS has been described whose prevalence and prognosis relevance in the normal population is unknown.Methods546 healthy men between 50 and 60 yo (group 1) and 373 similar patients with coronary artery disease (368 men, EF < 50% in 40%) (group 2) underwent signal averaged ECG allowing precise measurement of QRS duration. All cause mortality was determined after 17±3 years follow-up.ResultsMean QRS duration was 97±13ms for group 1 and 103±16ms for group 2. 85 group 1 subjects (16%) had QRS < 85ms and 23 (4%) had QRS>120ms. 44 group 2 patients (12%) had QRS < 85ms and 44 (12%) had QRS>120ms. QRS were larger in case of lower EF, lack of previous angioplasty and multivessel disease.All cause mortality in group 1 was 10,4% (57/546): 6/85 in case of QRS120ms (p=ns compared to normal QRS duration). HR for all-cause mortality in case of QRS < 85ms was 0,75 (95% CI 0.32-1.76, p = 0,52) and 0,86 (95% CI 0.21-3.53, p = 0,84) for QRS >120ms.All cause mortality in group 2 was 29% (109/373): 7/44 in case of QRS 120ms (p=0.002 when compared to normal QRS duration). Adjusted HR for all-cause mortality in case of QRS < 85ms was 0,65 (95% CI 0.29-1.45, p = 0,29) and 1.73 (95% CI 1.02-2.94, p = 0,05) for QRS > 120ms.All cause mortality in group 2 was 29% (109/373): 7/44 in case of QRS 120ms (p=0.002 when compared to normal QRS duration). Adjusted HR for all-cause mortality in case of QRS < 85ms was 0,65 (95% CI 0.29-1.45, p = 0,29) and 1.73 (95% CI 1.02-2.94, p = 0,05) for QRS > 120ms.Late potentials (LP) were present in SA-ECG in 116 group 1 subjects (21%). LP were present in 100 group 2 patients (27%) and were significantly related to multivessel disease, altered EF, lack of revascularization or of angioplasty. LP were more frequently observed in case of QRS > 120ms in both groups. LP were nor related to all-cause mortality in both groups.ConclusionQRS “narrower than normal” (< 85ms) can be observed in a significant porpotion of healthy males between 50 and 60 years old and in similar patients with ischemic heart disease. In opposition to QRS > 120 msec which are independantly related to a higher all-cause mortality in coronary artery disease patients, QRS

Introduction Both type 1 Myotonic Dystrophy (Steinert disease) and Brugada syndrome may be complicated by conduction disturbances and sudden death. ST elevation in the right precordial leads is the hallmark of Brugada syndrome but may be seen in some myopathies. Mutations in DMPK gene in Steinert pts may lead to cytosolic accumulation of muted toxic RNA or altered alternate splicing of some RNA potentially causing sodium channel dysfunctions. The prevalence of Brugada ECG pattern in Steinert disease is unknown. Methods we perform ajmaline challenge test (1 mg/kg over 5 min) during electrophysiological (EP) testing in a population of 44 Steinert disease pts (27 men, 41±15 years old) without ST elevation at baseline. Left ventricular EF was normal in each case. The presence of type 1 ST elevation (> 2 mm J elevation with coved ST and negative T wave) after ajmaline challenge was correlated to clinical, ECG and electrophysiological variables. Results 8 pts (18%) present type 1 ST elevation in the right precordial leads after ajmaline infusion. Brugada pattern was more often seen in men: 7/27 (26%) vs 1/17 (6%) (p=0.09). Patients with negative ajmaline test presented more often with fascicular block: 13/35 (27%) versus none (p=0.03). Brugada pattern was not correlated to age, symptoms, PR interval, QRS, QT or QTc durations, HV interval (at baseline or after ajmaline), presence of bundle branch block, of late potentials at SA-ECG or inducibility of ventricular arrhythmias at EP study. Nine pts were implanted with a pace maker and four with an ICD. Significant or symptomatic bradycardia did not happen in any non implanted pts, while only one pt presented with malignant ventricular arrhythmias during the 6.3±2.6 years follow-up (ventricular fibrillation with hypokaliemia in an ajmaline negative pt). Conclusion Brugada ECG pattern can be elicited by class 1 drug in 18% of Steinert disease pts and especially in men. Presence of type 1 ST elevation under class 1 drug in Steinert disease do not seem to have some significant clinical or ECG correlations.

Introduction The Epicor system ® is based on high intensity focused ultrasound (HIFU) energy used for creating a wide circumferential linear left atrial lesion encircling both left atrial posterior wall and pulmonary veins (box lesion) and provides long-term cure in patients with atrial fibrillation undergoing heart surgery. Whether if acute complete disconnection of the box lesion is achieved by application of HIFU is unknown. Methods bipolar pacing and detection into the box lesion was studied in 9 pts (5 men, 77 ± 18 yo) undergoing heart surgery (5 aortic valve replacement, 3 mitral valve repair or replacement and one coronary by-pass) using bipolar electrophysiological catheter and a real time telemetry (Medtronic CareLink ® programmer), just after completion of the ablation process on the beating heart prior to initiation of extracorporeal circulation. Sinus rhythm was present or obtained using internal cardioversion in each before the ablation process. Results Entrance block was absent in 7 (1 to 1 conduction from sinus rhythm inside the box lesion), undetermined in one and present in one (dissociated slow local rhythm). Exit block was lacking in 6 (capture of the cardiac rate by pacing inside the box lesion) and present in 3 (dissociated sinus rhythm from the paced area). Conclusion Acute complete block of the Epicor ® HIFU induced box lesion is lacking in the vast majority of pts despite completion of the energy deliverance according to the automated ablation process. Whether block later happens, or whether supplementary applications would increase the electrophysiological and clinical success rate is unknown.

Background Cardiac Resynchronization Therapy (CRT) improves morbidity and mortality in systolic heart failure. Elderly patients are under represented in large clinical trials although incidence of heart failure is high in this population. Method we considered 146 consecutive patients 75 years old with standard indication for CRT implanted between 2005 and 2007. Mean age was 79 yo, 72% males, EF = 27%, ischemic heart disease (IHD) 46%, Atrial Fibrillation 54%, CRT-D: 16%. Minimal follow-up was 12 months. Total mortality, clinical improvement (gain > 1 NYHA class without hospitalization for congestive heart failure) and combined criteria (mortality and clinical improvement) were retrospectively assessed. Results Mean follow-up is 25 +/-12 months. Data are complete for 137 patients (94.2%). Survival rate is 65%, clinical improvement is achieved in 51% and combined criteria in 45%. Independent factors of non response (p Causes of deaths are: terminal heart failure: 35 pts, sudden cardiac death: 1 pt, non cardiac death: 13 pts and unknown: 2 pts. Conclusion CRT is effective in elderly patients. Factors of non response are similar to those observed in younger population. CRT-P appears to be the most suitable device for this population. (Voir tableau ci-dessous) Multivariate analysis OR (IC 95%) Combined criteria Total Mortality Clinical improvement NYHA IV 4,1 (1.5–12.4) 3.1 (1.2–8.5) 2.5 (1–6.6) IHD 2,2 (1–4.8) 3.4 (1.5–7.9) NS CRT-D NS 3.2 (1.2–8.8) NS Atrial fibrillation 2,7 (1.3–5.8) 2.2 (1–5.1) NS No ACEI/ARB 2,7 (0.9–8.8) NS NS Renal failure (creatinine > 10μm/l) 1,1 (1–1.2) NS 1.2 (1.1–1.3) Doses of diuretics NS 1.03 (1–1.1) NS