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The relations between endocardial voltage mapping and the genetic background of patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) have not been investigated so far. A total of 97 patients with proved or suspected ARVC who underwent 3-dimensional endocardial mapping and genetic testing have been retrospectively included. Presence, localization, and size of scar areas were correlated to ARVC diagnosis and the presence of a pathogenic variant. A total of 78 patients (80%) presented with some bipolar or unipolar scar on endocardial voltage mapping, whereas 43 carried pathogenic variants (44%). Significant associations were observed between presence of endocardial scars on voltage mapping and previous or inducible ventricular tachycardia, right ventricular function and dimensions, or electrocardiogram features of ARVC. A total of 60 of the 78 patients (77%) with an endocardial scar fulfilled the criteria for a definitive arrhythmogenic right ventricular dysplasia diagnosis versus 8 of 19 patients (42%) without scar (p = 0.003). Patients with a definitive diagnosis of ARVC had more scars from any location and the scars were larger in patients with ARVC. In the 68 patients with a definitive diagnosis of ARVC, the presence of any endocardial scar was similar whether an ARVC-causal mutation was present or not. Only scar extent was significantly greater in patients with pathogenic variants. There was no difference in the presence and characteristics of scars in PKP2 mutated versus other mutated patients. The 3-dimensional endocardial mapping could have an important role for refining ARVC diagnosis and may be able to detect minor forms with otherwise insufficient criteria for diagnosis. The trend for larger scar extent were observed in mutated patients, without any difference according to the mutated genes.

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

Background Hybrid ablation for the treatment of atrial fibrillation is a single combined procedure consisting in a minimally thoracoscopic surgical ablation followed by a catheter ablation. This promising technique is recommended in persistent atrial fibrillation according to the current guidelines but is not routinely performed in France, mainly due to the absence of reimbursement by the French National Health Insurance. Aims The aim of this prospective and single-centre study was to analyse, for the first time in France, the feasibility, efficacy and complication rates of hybrid ablation in patients with persistent atrial fibrillation. Methods Hybrid ablation was performed in 15 consecutive patients (13 men, mean age 61 ± 6 years) with persistent (7 patients) or long standing persistent (8 patients) atrial fibrillation. Results Hybrid ablation was completed in 14/15 patients. Eleven patients returned in sinus rhythm during the procedure. Two patients (13%) had major per-procedural complications and 2 had minor complications. During an average follow-up of 25 ± 6 months, 6 patients (40%) underwent a redo catheter ablation because of atrial tachycardia, mainly peri-mitral atrial flutter. At 1 year follow up, 14/15 patients were in sinus rhythm, including 11 free of antiarrhythmic drugs.

Left ventricular assist device (LVAD) implantation has become an important therapeutic option in patients with end-stage heart failure, with many of them already having an implantable cardioverter-defibrillator (ICD) at the time of LVAD surgery. LVAD electromagnetic interference (EMI) with loss

Objectives This study sought to identify atrial tachycardia (AT) demonstrating atrial activation duration (AAD) lasting longer than the length of the tachycardia cycle (TCL); to assess AT prevalence; and to evaluate the mechanisms and characteristics associated with these AT episodes by using the Rhythmia system (Boston Scientific, Marlborough, Massachusetts). Background Ultra-high-density mapping allows very accurate characterization of mechanisms involved in AT. Some complex patterns may involve AAD which is longer than the tachycardia cycle length (TCL) which makes maps difficult to interpret. Prevalence and characteristics of such ATs are unknown. Methods A cohort of 100 consecutive patients undergoing ablation of 125 right (n = 21) or left (n = 104) ATs using ultra-high-density mapping were retrospectively included. Offline calculation of right or left AAD was compared to TCL. Results Mean TCL was 293 ± 65 ms, and mean AAD was 291 ± 74 ms (p = NS). AT mechanisms were macro-re-entry in 74 cases (59%), localized re-entry in 27 cases (22%), and focal AT in 21 cases (17%) (types were mixed in 3 cases). Fifteen ATs (12%) had AADs that were longer than the TCL (71 ± 45 ms longer, from 10 to 150 ms). TCL was equal to the AAD in 97 ATs (78%), whereas 13 ATs (10%) had AAD shorter than the TCL (focal AT in each case). There were no differences between right and left atria for prevalence of ATs with AADs that were longer than the TCLs. There were significant differences in AT mechanisms according to the AAD-to-TCL ratio (p Conclusions ATs with AAD lasting longer than the TCL were present in approximately 10% of the ATs referred for ablation, mostly in ATs caused by localized re-entry. Ultra-high-density mapping allows detection of these complex patterns of activation.

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.

Investigation and catheter ablation of cardiac arrhythmias are currently still based on optimal knowledge of arrhythmia mechanisms in relation to the cardiac anatomy involved, in order to target their crucial components. Currently, most complex arrhythmias are investigated using three-dimensional electroanatomical navigation systems, because these are felt to optimally integrate both the anatomical and electrophysiological features of a given arrhythmia in a given patient. In this article, we review the technical background of available three-dimensional electroanatomical navigation systems, and their potential use in complex ablations.

Background Cardiac resynchronization therapy (CRT) is an effective treatment of heart failure (HF), but is limited by a substantial proportion of nonresponders. We hypothesized that adding a second left ventricular (LV) lead to deliver a triple-site CRT (V3 CRT) may improve clinical status of CRT nonresponders. Objective We assessed the feasibility and safety of adding a second LV lead to CRT nonresponders and its clinical impact. Methods Eighty-four recipients of a CRT system and considered as nonresponders as per clinical composite score (CCS) were enrolled in this multicenter study. They were randomized to the V3 arm (implantation of an additional LV lead; n=43) or control arm (no change; n = 41). Implant success rate, incidence of severe adverse events, CCS, and secondary clinical and echocardiographic end points were evaluated at 12 and 24 months. Results Positioning of a second LV lead was successful at first (40 of 44 - 90.9%) or second (4 of 44 - 9.09%) attempt. The perioperative complication rate (infection, system explant, pneumothorax, and hematoma) was high (procedures or system-related complications for 9 patients- 20.4%). After 24 months, 35 systems (79.5%) were working properly. The multinomial logistic regression model showed that V3 treatment had no significant influence (P = .27) on the CCS, number of HF hospitalizations, time to first HF hospitalization, New York Heart Association class, and LV ejection fraction at 12 and 24 months. Conclusion Although addition of a second LV lead in CRT nonresponders is feasible with a high success rate, this approach is associated with a significant rate of severe adverse events and does not provide significant long-term clinical benefits (ClinicalTrials.gov Identifier No. NCT01059175).

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

Background Prevalence of early repolarisation ECG pattern (ER) has been repetitively determined in adults, but has not been reported in large unselected children populations so far. Methods ECG from 1000 successive healthy children from birth to 17years old were prospectively recorded and analyzed. ER was defined by ≥0.1mV J point elevation in at least two contiguous inferior or lateral ECG leads. Correlations with age, gender, ethnic origin, physical activity, Sokolow index and heart rate were performed. Results Prevalence of ER pattern was 23,6% (95% CI 21–26.2%) and increased after 8years of age. ER was independently correlated to a non Caucasian origin and a higher Sokolow index, but not to gender or level of physical activity. ER was independently correlated to an older age in children with heart rate Conclusion ER is present in a quarter of children of various age and is related to ethnic origin, an older age, a slower heart rate and a higher Sokolow index.

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.

Introduction The incidence of pacemaker-mediated tachycardia (PMT) varies as a function of patient characteristics, device programming and algorithm specificities. We investigated the efficacy of the Boston Scientific algorithm by reviewing PMT episodes in a large device population. Methods In this multicenter study, we included 328 patients implanted with a Boston Scientific device: 157 non-dependent patients with RYTHMIQ™ activated (RYTHMIQ group), 76 patients with permanent AV-conduction disorder (AV-block group) and 95 Cardiac Resynchronization Therapy patients (CRT group). For each patient, we reviewed the last 10 remote monitoring-transmitted EGMs diagnosed as PMT. Results We analyzed 784 PMT episodes across 118 patients. In the RYTHMIQ group, the diagnosis of PMT was correct in most episodes (80%) of which 69% was directly related to the prolongation of the AV-delay associated with the RYTHMIQ algorithm. The usual triggers for PMT were also observed (PVC 16%, PAC 9%). The remainder of the episodes (20%) in RYTHMIQ patients and most episodes of AV-block (66%) and CRT patients (74%) were incorrectly diagnosed as PMT during sinus tachycardia at the maximal tracking rate. The inappropriate intervention of the algorithm during exercise causes non-conducted P-waves, loss of CRT (sustained in six patients) and may have been pro-arrhythmogenic in one patient (induction of ventricular tachycardia). Conclusion Algorithms to minimize ventricular pacing can occasionally have unintended consequences such as PMT. The PMT algorithm in Boston Scientific devices is associated with a high rate of incorrect PMT diagnosis during exercise resulting in inappropriate therapy with non-conducted P-waves, loss of CRT and limited risk of pro-arrhythmic events.

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.

Objectif. Decrire les facteurs associes a la prescription d’anticoagulants oraux directs (AOD). Methode. Cohorte de patients de rythmologie du Centre Hospitalier Universitaire de Toulouse, traites par anticoagulants oraux dans le cadre d’une fibrillation atriale (FA). Une regression logistique a permis de decrire les facteurs associes a la prescription d’AOD et ceux associes a l’arret de l’anticoagulant. Resultats. Parmi les 140 patients inclus, 92 etaient traites par anti-vitamines K (AVK) et 48 par AOD. Les facteurs associes a la prescription d’AOD etaient un diagnostic de FA recent, la prise anterieure d’AVK et l’absence d’anti-agregants plaquettaires (AAP). L’arret de l’anticoagulant (n = 24) etait associe a la prise d’AOD. Conclusion. Les AOD sont moins prescrits que les AVK chez les patients sous AAP. L’ international normalized ratio (INR) est equilibre chez la majorite des patients sous AVK etant passes aux AOD. Une etude plus puissante permettrait de confirmer le profil de prescription des AOD.

Summary Background In recent decades, advances in surgery and therapeutic catheterization have steadily increased the life expectancy and prevalence of adults with congenital heart disease (CHD). Aims We assessed medical and psychosocial variables of adults with CHD, according to the disease complexity. Methods We included, from a single-centre observational cohort study, 135 consecutive adults with CHD (median age of 40 years, interquartile range: 28.0–51.0) followed in our cardiology unit, who answered a questionnaire assessing daily activity and psychosocial functioning. Disease complexity was classified according to the Bethesda conference. Results Cardiac malformation complexity was simple in 61 (45.2%), moderate in 50 (37.0%) and complex in 24 (17.8%) patients. Cardiac surgery had been performed in 86.5% of moderate and complex patients. Complications (such as heart failure, arrhythmia and pulmonary hypertension) were mainly observed in the complex group ( P = 0.003). Physical activity was lower in the complex group (no activity in 58.8%, but sport previously contraindicated in 50% of these; P = 0.03). Education level tended to be lower in the complex and moderate groups than in the simple group (respectively, 31.2% and 33.3% vs. 45.7% had passed the Baccalaureate; P = 0.47). The pass rate was lower in patients with complications ( P = 0.037) or more than one cardiac surgery ( P = 0.03). In the complex group, 56.3% of patients were unemployed ( P = 0.048). Conclusions Complexity of heart disease and medical history affect education level and employment of adults with CHD. Academic education of children with a complex defect and career counselling are important to prevent unemployment among adults with CHD.

A 84-year-old woman with a remote anterior myocardial infarction and a left ventricular ejection fraction of 20% was referred for well-tolerated chronic monomorphic ventricular tachycardia (VT). VT recurred during hospitalization despite beta-blockers and oral loading with amiodarone, and it then

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.

Despite isolated reports of Brugada syndrome (BrS) in the inferior or lateral leads, the prevalence and prognostic value of ST elevation in the peripheral electrocardiographic (ECG) leads in patients with BrS remain poorly known.To study the prevalence, characteristics, and prognostic value of type 1 ST elevation and ST depression in the peripheral ECG leads in a large cohort of patients with BrS.ECGs from 323 patients with BrS (age 47 ± 13 years; 257 men) with spontaneous (n = 141) or drug-induced (n = 182) type 1 ECG were retrospectively reviewed. Two hundred twenty-five (70%) patients were asymptomatic, 72 (22%) patients presented with unexplained syncope, and 26 (8%) patients presented with sudden death (12 patients) or appropriated implantable cardioverter-defibrillator therapies (14 patients) at diagnosis or over a mean follow-up of 48 ± 34 months.Thirty (9%) patients presented with type 1 ST elevation in at least 1 peripheral lead (22 patients in the aVR leads, 2 in the inferior leads, 5 in both aVR and inferior leads, and 1 in the aVR and VL leads). Patients with type 1 ST elevation in the peripheral leads more often had mutations in the SCN5A gene, were more often inducible, had slower heart rate, and higher J-wave amplitude in the right precordial leads. Twenty-seven percent (8 of 30) of the patients with type 1 ST elevation in the peripheral leads experimented sudden death/appropriate implantable cardioverter-defibrillator therapy, whereas it occurred in only 6% (18 of 293) of other patients (P.0001). In multivariate analysis, type 1 ECG in the peripheral leads was independently associated with malignant arrhythmic events (odds ratio 4.58; 95% confidence interval 1.7-12.32; P = .0025).Type 1 ST elevation in the peripheral ECG leads can be seen in 10% of the patients with BrS and is an independent predictor for a malignant arrhythmic event.

Early repolarization pattern (ERP) has recently been associated with idiopathic ventricular fibrillation and with cardiovascular mortality in the general population. We aimed to identify electrocardiographic tools to differentiate the "malignant" form of ERP from benign ERP in a population-based study. We retrospectively assessed the prevalence of ERP by recording electrocardiograms in 1,161 southwestern French subjects 35 to 64 years old. ERP was defined by an elevation of the J point ≥1 mm in 2 consecutive leads excluding leads V(1) through V(3). We categorized ERP as notching or slurring pattern as located in inferior and/or lateral leads and measured the J-point elevation amplitude. ST segment after ERP was categorized as ascendant or horizontal/nonascendant and T waves as negative or positive. Association of ERP with all-cause and cardiovascular mortalities was assessed by adjusted Cox proportional hazard models. ERP was found in 157 subjects (13.3%). During a mean follow-up of 14.2 ± 2 years, 77 subjects died (6.6%), of whom 24 (2.1%) died from cardiovascular causes. Subjects with ERP had an increased hazard ratios for all-cause mortality (2.45, 95% confidence interval [CI] 1.44 to 4.15, p = 0.001) and cardiovascular mortality (5.60, 95% CI 2.27 to 11.8, p = 0.001). The highest risk was found for notching ERP and ERP with a nonascendant/horizontal ST segment, yielding when associated increased hazard ratios of 3.84 (95% CI 2.14 to 6.92, p = 0.001) and 8.75 (95% CI 3.48 to 22.0, p = 0.001) for all-cause and cardiovascular mortalities, respectively. Conversely, a slurring ERP or ascendant ST segment was not associated with increased mortality. ERP localization, J-point elevation amplitude, or T-wave morphology did not distinguish benign from malignant forms of ERP. In conclusion, ERP with notching pattern and horizontal/descendant ST segments was associated with the highest risk of all-cause and cardiovascular deaths. These electrocardiographic patterns may be used for risk stratification in subjects with ERP.

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.

Summary Background Differences in the duration of the excitable gap along the reentry circuit during typical atrial flutter are poorly known. Aim To prospectively evaluate and compare the duration and composition of the excitable gap during typical counterclockwise atrial flutter in different parts of the circuit all around the tricuspid annulus. Methods The excitable gap was determined by introducing a premature stimulus at various sites around the tricuspid annulus during typical counterclockwise atrial flutter in 34 patients. Excitable gap was calculated as the difference between the longest resetting coupling interval and the effective atrial refractory period. Results The duration of the excitable gap, the effective atrial refractory period and the resetting coupling interval differed significantly along the tricuspid annulus. Duration of excitable gap was significantly longer at the low lateral right atrium (79 ± 22 ms) than at the cavotricuspid isthmus (66 ± 23 ms; P = 0.002). The effective atrial refractory period was significantly longer at the cavotricuspid isthmus (160 ± 26 ms) than at the high lateral right atrium (149 ± 29 ms; P = 0.004). Other locations, such as coronary sinus ostium, right atrial septum and atrial roof displayed intermediate values. Conclusion The duration of the excitable gap differed significantly along the tricuspid annulus, with a larger excitable gap at the lateral right atrium and a shorter excitable gap at the cavotricuspid isthmus, because of longer refractory periods at the isthmus.

Concealed retrograde activation of bundle branch has been often proposed for explaining the persistence of functional rate-dependent bundle-branch block, but direct proof of such a mechanism in man has rarely been demonstrated. We report intracardiac recordings compatible with a reversal of activation of the left basal interventricular septum during intermittent left bundle-branch block. In our case, however, retrograde activation into the left bundle-branch cannot be recordable, probably due to the concealment within the muscular septal activation, even if this may explain the perpetuation of bundle-branch block according to the linking phenomenon.

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.

We describe the case of a young boy with d-transposition of the great arteries (TGA) that was surgically corrected with an arterial switch operation, who presented with sustained ventricular tachycardia (VT) that was successfully ablated at the embryologic pulmonary artery–related sinus of Valsalva (SV). Although the VT mechanism is still uncertain, the presence of mid-diastolic potentials during VT and postsystolic potentials during sinus rhythm argue for a re-entrant mechanism.