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Funding Acknowledgements Type of funding sources: None. Introduction In a subset of patients with idiopathic ventricular fibrillation (IVF), the cause is identified as short-coupled premature ventricular complexes (ScPVCs), which mostly originate from Purkinje cells, and are often short-living and undetected. There is no consistent test allowing identification of patients vulnerable to this malignant arrhythmia. We aimed at evaluating sodium channel blocker (NaBl) testing in patients who survived IVF associated with SCPVCs. Methods We retrospectively reviewed data of 112 patients at 3 centers, with the diagnosis of IVF associated with spontaneous and documented scPVCs. No patient from the previous report was included. We analyzed all patients who underwent NaBl tests during evaluation of IVF. An arrhythmogenic NaB test was defined as the occurrence of ≥10 scPVCs and/or ≥ 1 repetitive form (couplet, triplet, run) during the test, without spontaneous scPVC at baseline. We defined scPVCs as an R-on-T pattern PVC. We evaluated the occurrence of scPVC during NaB infusion, the arrhythmia pattern and we compared the characteristics of the arrhythmogenic versus non-arrhythmogenic response groups. Data regarding anti-arrhythmic drug treatment and follow-up were collected at each center. Results In total, 48 patients were included (Table). The NaB tests were performed using ajmaline 1mg/kg/5 or 10min, or flecainide 2mg/kg/10min. Overall, an arrhythmogenic NaB test occurred in 23 of 48 (48%) patients. Isolated ScPVCs were observed in 11 patients, couplet/triplets in 10, and runs in 6. The arrhythmogenic response was reproduced in 5/5 patients who had a second NaB test during scPVC mapping before catheter ablation. There were no significant differences between patients with arrhythmogenic or non-arrhythmogenic NaB tests in all baseline clinical variables tested including VF burden (Table). All patients were followed at least 1 year after initial diagnosis and NaB test. Under hydroquinidine (600 or 900mg/day), appropriate defibrillator shocks occurred in 7 of 8 patients with arrhythmogenic NaB test; whereas shocks occurred only in 2 of 11 patients with non-arrhythmogenic NaB test (P=0.02). Conclusion Sodium blockers unravel scPVCs in up to 50% of patients with IVF and may therefore represent a valuable tool to detect a potential cause for sudden cardiac death (SCD) or unexplained syncope. Additionally, the arrhythmogenic response to NaB may be associated with the success of hydroquinidine in preventing IVF recurrence, a finding that suggests the existence of distinct scPVCs phenotypes and that may have important therapeutic implications. Further functional and genetical investigations are warranted to explore the mechanisms underlying this association.

Funding Acknowledgements Type of funding sources: None. Background Ventricular tachycardias(VT) are life-theatening cardiac arrhythmias. Catheter ablation, the standard approach to VT is relying heavily on maps depicting intracardiac signals. The current mapping approach was based on bipolar electrograms, which are massively influenced by the direction of the underlying tissue activation. The omnipolar (OT) electrogram method was recently proposed to generate orientation-independent electrograms and has shown improved results in recent publications (1-2). We describe the first cohort of patients ablated for VT with the help of an OT-enabled catheter. Purpose To evaluate acute and mid-term follow-up of patients ablated for VT using an OT enabled mapping system. Methods Eighteen patients (age 65 ± 19; 2 females, 32% nonischemic, median ejection fraction 40), underwent catheter ablation for scar-related VT. 50% (9) n patients had previous ablation, 4 of which underwent epicardial access. High-density omnipolar maps were obtained with an HD-grid catheter after which ablation was performed with an irrigated-tip catheter. In all patients the ablation was primarily substrate-based with the support of CT scan scar visualization. In 18 patients local abnormal ventricular activities (LAVA) ablation was performed (3), while one patient showed no LAVA. In 7 patients VT mapping was possible due to stable blood pressure during the tachycardia. The median radiofrequency application duration was 36 min. While all patients except one were inducible at the beginning of the procedure, only 3 patients were inducible at the end with a protocol using up to 3 extrastimuli. Follow-up was available in 13 patients (7.1 ± 2.5 months) with 66% freedom from VT recurrence (60% in redo cases and 71% in first procedures). Conclusion OT is a reliable method to visualize signals and perform a substrate-based ablation in with good acute and mid-term outcomes. Future studies will evaluate the impact on long term outcomes.

BACKGROUND: The prevalence of atrial fibrillation(AF) and diabetes mellitus is rising to epidemic proportions. We aimed to assess the impact of diabetes on the management and outcomes of patients with AF.METHODS: The EORP-AF General Long-Term Registry is a prospective, observational registry from 250 centres across 27 European countries. Outcomes of interest were as follows: i)rhythm control interventions; ii)quality of life; iii)healthcare resource utilisation; and iv)major adverse events.RESULTS: Of 11,028 patients with AF, the median age was 71 (63-77) years and 2537 (23.0%) had diabetes. Median follow-up was 24 months. Diabetes was related to increased use of anticoagulation but less rhythm control interventions. Using multivariable analysis, at 2-year follow-up, patients with diabetes were associated with greater levels of anxiety (p = 0.038) compared to those without diabetes. Overall, diabetes was associated with worse health during follow-up, as indicated by Health Utility Score and Visual Analogue Scale. Healthcare resource utilisation was greater with diabetes in terms of length of hospital stay (8.1 (±8.2) vs. 6.1 (±6.7) days); cardiology and internal medicine/general practitioner visits; and emergency room admissions. Diabetes was an independent risk factor of major adverse cardiovascular event (MACE; HR 1.26 [95% CI, 1.04-1.52]), all-cause mortality (HR 1.28 [95% CI, 1.08-1.52]), and cardiovascular mortality (HR 1.41 [95% CI, 1.09-1.83]).CONCLUSION: In this contemporary AF cohort, diabetes was present in 1 in 4 patients and it served as an independent risk factor for reduced quality of life, greater healthcare resource utilisation and excess MACE, all-cause mortality and cardiovascular mortality. There was increased use of anticoagulation therapy in diabetes but with less rhythm control interventions.

Multiple endocrine neoplasia (MEN) are genetic predisposition syndromes to endocrine tumors including MEN1, MEN2 and exceptionally MEN4. MEN are transmitted in an autosomal dominant fashion with a high penetrance. Classically, there is no genotype/phenotype correlation for NEM1 whereas this is the case for NEM2. Patients with NEM1, linked to an inactivating mutation of the menin gene, may present with: primary hyperparathyroidism, pituitary adenoma, duodeno-pancreatic neuroendocrine tumors (NETs), bronchial tumors with an increased risk of thymoma, adrenal cortical tumors, an increased risk of breast cancer and characteristic skin involvement such as collagenomas, lentiginomas and an increased risk of skin cancer. These patients require at least annual follow-up. Screening of children is proposed from the age of 5 years. Patients with NEM2, linked to an activating mutation of the RET proto-oncogene, all present with medullary thyroid carcinoma (MTC) at a variable age depending on the genotype. Some patients present a pheochromocytoma (50 %) and hyperparathyroidism (20 %). Pediatric forms with aggressive CMT, ganglioneuromatosis and marfanoid syndrome exist (rare NEM2B). Some mutations are associated with a risk of aggressive CMT, justifying prophylactic thyroidectomy before 6 months of age. The age of genetic testing depends on the mutation subtype in the NEM2 parent. NEM4, related to a mutation in the CDKN1B gene, are rare, with a less well-known pathogenesis and their follow-up is not well codified.

Resume But de l’etude Une etude realisee en IRM cardiaque suggere que l’exposition chronique a un pheochromocytome « stable » induit des plages de fibrose et de myocardite ventriculaires gauches. Ces anomalies etant classiquement associees a une alteration de la deformation systolique longitudinale, nous emettons l’hypothese que cette deformation est alteree chez les patients porteurs d’un pheochromocytome. Patients et methode Cette etude cas-temoin retrospective a ete realisee a partir des patients de la base de donnees du CHU de Bordeaux, inclus entre 2008 et 2016. Nous avons compare le global longitudinal strain (GLS), les autres parametres de deformation systolique et les parametres echocardiographiques classiques entre un groupe de patients porteurs de pheochromocytome et un groupe de temoins apparies pour l’âge, le sexe, l’indice de masse corporelle et la pression arterielle systolique moyenne. Resultats L’analyse a porte sur 47 patients et 47 temoins correctement apparies. Nous n’avons pas retrouve de difference statistiquement significative entre les 2 groupes en termes de GLS (−20,7 ± 2,4 % vs −20,2 ± 2,7 %, p = 0,40), de deformation radiale, de masse ventriculaire gauche ou de fonction diastolique. La fraction d’ejection ventriculaire gauche et la deformation circonferentielle etaient significativement plus elevees chez les patients que chez les temoins, le diametre telediastolique significativement plus bas. Conclusion Aucune alteration significative du GLS n’a ete identifiee chez nos patients porteurs d’un pheochromocytome. Plusieurs hypotheses peuvent expliquer ces resultats. La presence de foyers de fibrose et de myocardite etant associes a un pronostic cardiologique pejoratif, une IRM cardiaque systematique pourrait se discuter, dans l’attente d’etudes complementaires.

BACKGROUND: Cardiac troponins (cTn) have been reported to be predictors for adverse outcomes in atrial fibrillation (AF), patients, but their actual use is still unclear.AIM: To assess the factors associated with cTn testing in routine practice and evaluate the association with outcomes.METHODS: Patients enrolled in the ESC-EHRA EORP-AF General Long-Term Registry were stratified into 3 groups according to cTn levels as (i) cTn not tested, (ii) cTn in range (≤99th percentile), (iii) cTn elevated (>99th percentile). The composite outcome of any thromboembolism /any acute coronary syndrome/cardiovascular (CV) death, defined as Major Adverse Cardiovascular Events (MACE) and all-cause death were the main endpoints.RESULTS: Among 10 445 AF patients (median age 71 years, 40.3% females) cTn were tested in 2834 (27.1%). cTn was elevated in 904/2834 (31.9%) and in-range in 1930/2834 (68.1%) patients. Female sex, in-hospital enrollment, first-detected AF, CV risk factors, history of coronary artery disease, and atypical AF symptoms were independently associated with cTn testing. Elevated cTn were independently associated with a higher risk for MACE (Model 1, hazard ratio [HR] 1.74, 95% confidence interval [CI] 1.40-2.16, Model 2, HR 1.62, 95% CI 1.28-2.05; Model 3 HR 1.76, 95% CI 1.37-2.26) and all-cause death (Model 1, HR 1.45, 95% CI 1.21-1.74; Model 2, HR 1.36, 95% CI 1.12-1.66; Model 3, HR 1.38, 95% CI 1.12-1.71).CONCLUSIONS: Elevated cTn levels were associated with an increased risk of all-cause mortality and adverse CV events. Clinical factors that might enhance the need to rule out CAD were associated with cTn testing.

Resume La glycation est un processus physiologique accru par l’hyperglycemie chronique. En cas de diabete, la glycation et l’oxydation induisent la formation de produits de la glycation avancee (Advanced Glycation End-products, AGEs), deleteres pour les tissus. Du fait de leurs proprietes optiques, il est possible de mesurer l’autofluorescence cutanee (AF), correlee aux concentrations tissulaires des AGEs. Cette mesure est de realisation facile, et non invasive. L’AF est un marqueur de la memoire metabolique, associe aux valeurs anciennes de l’HbA1c. Cependant, l’âge, la fonction renale et l’heredite influencent les valeurs d’AF. Elle est associee aux complications micro- et macroangiopathiques du diabete quel que soit leur type. En definitive, l’AF pourrait etre utilisee comme marqueur predictif precoce des complications du diabete. Meme si son interpretation reste delicate, l’utilisation de l’AF en pratique courante serait possible afin d’optimiser la prise en charge du patient vers une medecine personnalisee.

La fibrilacion auricular (FA) y el aleteo auricular (AA) son las taquicardias mas comunes en la auricula. Afectan al 2% de la poblacion general, y su prevalencia aumenta de forma considerable despues de los 60 anos. Constituyen dos presentaciones de arritmia de un mismo mecanismo de reentrada. Ademas de los sintomas a veces incapacitantes (palpitaciones, cansancio, mareos), confieren un mayor riesgo de episodios cardioembolicos y de insuficiencia cardiaca, cuya prevencion es esencial. Asi, los anticoagulantes se inician si la puntuacion CHA2DS2-VASc es alta, y mantener el ritmo sinusal es un objetivo fundamental. Aunque los farmacos se recomiendan como terapia de primera linea, la ablacion es mucho mas eficaz, sin los efectos nocivos de un antiarritmico a largo plazo. Por lo tanto, la ablacion se considerara al principio del tratamiento: en la practica, tan pronto como fracase el primer farmaco antiarritmico. El principio de este procedimiento, que tiene una tasa de riesgo baja, pero cuyo impacto es potencialmente grave, debe discutirse durante una consulta con un especialista en arritmologia. La taquicardia auricular focal (TAF) es infrecuente, benigna y afecta a personas jovenes. Por lo tanto, solo se tratan las formas sintomaticas o responsables de cardiopatia arritmica.

Funding Acknowledgements Type of funding sources: None. Background Atrial tachycardias (ATs) are often seen in the context of AF ablation. Objectives We evaluated the role of the Marshall bundle (MB) network in left atrial (LA) ATs using high-density high-spatial resolution 3D mapping. Methods 199 post-AF ablation LA tachycardias were mapped in 140 consecutive patients (112 (80%) males, mean age: 61.8 years); 133 (66.8%) were macro-reentrant and 66 (33.2%) were scar-related re-entry. MB-dependent perimitral AT (PMAT) was diagnosed where the difference between the post pacing interval and the tachycardia cycle length (PPI-TCL) was 20ms in areas bypassed by the VOM (the distal coronary sinus (CS), the posterior LA and the mitral isthmus). MB-related re-entry was diagnosed by PPI-TCL 20ms in the septal annulus. Typically, in MB-dependent localized re-entry, the earliest activation was found along the MB-LA endocardial connection or MB-CS epicardial connection. Results The MB network was found to participate in 60 (30.2%) re-entrant ATs, 31 PMATs and 29 localized re-entries. High-frequency multiphasic fragmented electrograms with long duration were often recorded endocardially or epicardially at the MB-LA or MB-CS connections. The amplitude and duration of these signals were 0.5 ± 0.79 mV and 65 ± 40 ms for MB-PMATs and 0.26 ± 0.28mV and 122 ± 67 ms for MB-localized re-entries. Unipolar EGMs at the site of endocardial-epicardial breakthrough had a rS pattern in all MB-related ATs. Of 60 MB-related ATs, 49 (81.6%) terminated with RF ablation, 44 (73.3%) at the MB-LA junction and 5 (8.3%) at the MB-CS junction, while 9 (15%) terminated after 2.5-5 cc of alcohol infusion inside the vein of Marshall (VOM). Of the 31 MB-related macroreentrant ATs, 17 (54.8%) terminated at the MB-LA junction, 5 (16.1%) at the MB-CS junction and 7 (22.6%) with alcohol infusion inside the VOM. Two macroreentries (6.5%) using the MB did not terminate with RF energy either endocardially at the MB-LA junction or epicardially at the MB-CS junction, and we were unable to identify or cannulate the VOM for ethanol infusion. Of the 29 localized re-entrant ATs using the MB, 27 (93.1%) terminated at the MB-LA junction, none terminated at the MB-CS junction and 2 (6.9%) terminated after alcohol infusion. After a mean follow up of 12 months, only 4 patients (6.7%) had AT recurrence. Conclusions MB re-entrant ATs accounted for up to 29% of the left ATs after AF ablation. Ablation of the MB-LA or CS-MB connections or alcohol infusion inside the VOM is required to treat these arrhythmias. Abstract Figure.

Objectif Le caractere mini-invasif d’une procedure et la rapidite de son execution conditionnent la rehabilitation postoperatoire des patients. Dans le cadre de la mise en place d’un protocole de surrenalectomie partielle nous avons cherche a simplifier la technique chirurgicale tout en satisfaisant au respect des preceptes carcinologiques et fonctionnels. L’objectif de cette video est d’illustrer les points cles de la technique de surrenalectomie partielle pour pheochromocytome. Methodes Les etapes cles de l’intervention, realisees avec le robot chirurgical Da Vinci, modele Si (Intuitive Surgical) a l’aide de 3 bras operateurs, un optique de 0° et 2 trocarts de 5 mm et 12 mm pour l’aide operatoire, sont presentees. Resultats La patiente, 29 ans, presentait un pheochromocytome de la surrenale gauche de 18 mm de grand axe, dans le cadre d’une NEM2. La premiere etape consistait en un abord tumoral direct avec exposition limitee du rein. La tumorectomie dans le plan de l’enucleation a ete realisee sans clampage de la veine surrenalienne. Realisation de l’hemostase du lit d’exerese. La duree operatoire etait de 90 minutes. Les pertes sanguines peroperatoires etaient negligeables. La patiente est restee stable sur le plan tensionnel au decours de l’intervention, et est sortie a j1. L’analyse anatomopathologique a confirme un pheochromocytome potentiellement agressif, et le caractere sain des marges d’exerese. Il s’agit de la troisieme surrenalectomie partielle pour pheochromocytome realisee dans notre centre, sans signe de recidive a respectivement 31, 8 et 5 mois. Conclusion La technique de surrenalectomie partielle avec abord tumoral direct permet la conservation du parenchyme sain et d’eviter le recours a la corticotherapie postoperatoire, sans augmenter la morbidite perioperatoire. Malgre un faible nombre de donnees dans la litterature, cette approche peut etre recommandee pour les tumeurs surrenaliennes de petite taille chaque fois qu’elle est possible.

Objective Adrenocortical carcinoma (ACC) has an aggressive but variable clinical course. Prognostic stratification based on the European Network for the Study of Adrenal Tumours stage and Ki67 index is limited. We aimed to demonstrate the prognostic role of a points-based score (S-GRAS) in a large cohort of patients with ACC. Design This is a multicentre, retrospective study on ACC patients who underwent adrenalectomy. Methods The S-GRAS score was calculated as a sum of the following points: tumour stage (1–2 = 0; 3 = 1; 4 = 2), grade (Ki67 index 0–9% = 0; 10–19% = 1; ≥20% = 2 points), resection status (R0 = 0; RX = 1; R1 = 2; R2 = 3), age (2D statistic. Results We included 942 ACC patients. The S-GRAS score showed superior prognostic performance for both PFS and DSS, with best discrimination obtained using the individual scores (0–9) (C-index = 0.73, R2D = 0.30, and C-index = 0.79, R2D = 0.45, respectively, all P < 0.01vs each component). The superiority of S-GRAS score remained when comparing patients treated or not with adjuvant mitotane (n = 481 vs 314). In particular, the risk of recurrence was significantly reduced as a result of adjuvant mitotane only in patients with S-GRAS 4–5. Conclusion The prognostic performance of S-GRAS is superior to tumour stage and Ki67 in operated ACC patients, independently from adjuvant mitotane. S-GRAS score provides a new important guide for personalised management of ACC (i.e. radiological surveillance and adjuvant treatment).

Pheochromocytoma is a rare disease, which may manifest as severe cardiac complications. Apart from these situations, the "chronic" cardiac impact is not clearly defined. A cardiac MRI study suggests that these patients are carrying areas of fibrosis and foci of left ventricular myocarditis. Since these abnormalities are usually associated with altered left ventricular longitudinal systolic strain, we hypothesize that this strain is altered in patients with a "chronic" pheochromocytoma.This retrospective case-control study was performed using patients from the Bordeaux University Hospital database, included between 2008 and 2016. We compared the left ventricular global longitudinal strain (GLS), radial and circumferential systolic strain and classic echocardiographic parameters between patients with pheochromocytoma and controls matched for age, sex, body mass index and systolic blood pressure.The analysis included 47 patients and 47 correctly matched controls. There were no statistically significant differences between the 2 groups in terms of GLS (-20.7±2.4% vs. -20.2±2.7%, P=0.40), radial strain, left ventricular mass or diastolic function. Left ventricular ejection fraction and circumferential strain were significantly higher in patients than in controls, with a significantly lower telediastolic diameter.No significant changes in GLS were observed in our pheochromocytoma patients, compared with controls. Several hypotheses may explain these results. The presence of fibrosis foci and areas of left ventricular myocarditis being associated with a poor cardiological prognosis, a systematic cardiac MRI could be discussed in these patients, until further studies are performed.

Aims To compare the arrhythmic response to isoproterenol and exercise testing in newly diagnosed arrhythmogenic right ventricular cardiomyopathy (ARVC) patients. Methods and results We studied isoproterenol [continuous infusion (45 µg/min) for 3 min] and exercise testing (workload increased by 30 W every 3 min) performed in consecutive newly diagnosed ARVC patients. Both tests were evaluated with regard to the incidence of (i) polymorphic premature ventricular contractions (PVCs) and couplet(s) or (ii) sustained or non-sustained ventricular tachycardia (VT) with left bundle branch block [excluding right ventricular outflow tract VT]; and compared to a control group referred for the evaluation of PVCs without structural heart disease. Thirty-seven ARVC patients (63.5% male, age 38 ± 16 years) were included. The maximal sinus rhythm heart rate achieved during isoproterenol testing was significantly lower compared to exercise testing (149 ± 17 bpm vs. 166 ± 19 bpm, P

Traitement des troubles bipolaires, depressifs ou schizoides (FDA 1970), le lithium est un cation similaire au sodium et au potassium affectant le transport ionique. Les principales complications du traitement sont le syndrome de neurotoxicite irreversible au lithium (SILENT) lie a une toxicite aigue et des complications thyroidiennes, parathyroidiennes et renales. Les complications thyroidiennes, plus frequentes chez les femmes, correspondent a une hypothyroidie, un goitre, voire une hyperthyroidie. En raison du transport actif Na+/I-, le lithium s’accumule dans la thyroide ou il inhibe la secretion hormonale et stimule la proliferation des follicules. L’augmentation du volume thyroidien est precoce. La prevalence des goitres est 4 fois superieure a la population generale et l’hypothyroidie (8-20 %) plus frequente en cas d’auto-immunite thyroidienne pre-existante. Des hyperthyroidies susceptibles d’aggraver l’humeur sont decrites dans 5 % des cas. Par ailleurs, le lithium peut constituer un traitement adjuvant des hyperthyroidies resistantes aux antithyroidiens. Une augmentation de la calcemie et de la PTH survient dans 30 % des cas car le lithium stimule la proliferation des cellules parathyroidiennes par activation de la voie Wnt. Le risque d’hyperparathyroidie par adenome mais surtout par hyperplasie, est 5 fois superieures a la population generale, avec cette particularite d’une frequente hypocalciurie par action sur le CaSR. Les complications renales incluent un risque d’insuffisance renale aigue ou chronique et de diabete insipide nephrogenique. Generateur de deshydratation avec hypernatremie, ce dernier est generalement reversible apres l’arret du lithium. Une surveillance reguliere de la TSH, de la calcemie, du ionogramme et de la creatinine est conseillee sous lithium.

Introduction Micro Computed tomography has emerged as a powerful imaging tool to study the micro-structure of cardiac tissue of small mammals. However, conventional techniques in whole organs of large mammals, is compromised due to the use of high molecular weight contrast agents and the relative thickness of samples. Objective To develop a tissue preparation method to optimize microCT imaging of whole hearts with sizes including those of humans. Methods Male pig hearts (N = 5) were treated by: hydrated samples with conventional contrast agent (iodine, 0.5%) or dehydrated in ethanol and air-dried under hexamethyldisilazane (HMDS). Human hearts (N = 3) were treated using air-drying method. Hearts were imaged at 21.7 μm isotropic resolution by microCT (SkyScan 1276 Bruker). Images were quantified with Amira software using a 3D cylinder correlation technique. Results The X-ray attenuation of air-dried samples was better optimized for the bandwidth of the X-ray detector, indicated by reduced x-ray attenuation (pig 65%, human 55), compared to almost complete signal attenuation from hydrated samples (93%). Voxel intensity histograms showed two populations corresponding to the tissue and the background media and sample support structures. Yet, the separating minima of histogram intensities were always observed at lower intensities for air-dried hearts ( Fig. 1 A) than hydrated hearts, increasing the dynamic range for tissue-specific signals ( Fig. 1 B). Imaging air-dried pig or human hearts permitted a simple threshold-based segmentation to isolate microstructures of the heart, including vessels, myocardial fibers and Purkinje from the background (1C). Fiber orientation quantification could be performed directly on unfiltered images. Fiber orientation was validated against subsequent histology of the same samples. Conclusion Air drying with HMDS enables microCT imaging of human-sized hearts while preserving micro-structure and the identification of individual cardiac muscle fibers.

Background Embolic event is one of the complications of VT ablation. This may be due to the presence of intra-cardiac thrombus before ablation. However, there is no clear consensus on how to rule out thrombus before the procedure. Objective We sought to examine the prevalence and risk factors of intra-cardiac thrombus with cardiac computed tomographic (CT) scan in patients undergoing scar-related VT ablation. Methods In absence of contra-indication, all patients undergoing scar-related VT ablation at our institution underwent contrast-enhanced cardiac CT within one week before ablation. 324 consecutive patients (292 male, 59±16 yo) have been included in this study. The etiology was ischemic cardiomyopathy (CMP) (n=191), arrhythmogenic right ventricular CMP (ARVC) (n=37), congenital CMP (n=11) or other CMP (n=85). LVEF was Results Intra-cardiac thrombus was diagnosed in 29 (9%) patients: in the left atrium (n=8), in the right atrium (n=1), in the left ventricle (n=15), in the right ventricle (n=3), in right and left atrium (n=1), and in left atrium and right ventricle (n=1). Moreover in 2, a bilateral pulmonary embolism was identified. The population with thrombus was older (65±12 vs 58±16 years, p=0,005), with more permanent atrial fibrillation (AF) (28% vs 8%; p=0.005). Patients with left ventricular (LV) aneurysm were at higher risk of thrombus 50% vs 3% (p Conclusion CT scans help eliminating intra-cardiac thrombus before VT ablation procedure. A high proportion of thrombus (9%) was identified. Whereas LV thrombus should systematically be ruled out before scar related VT ablation, in patients with AF, a LAA thrombus should also be eliminated as well as RV thrombus in patients with ARVC. Acknowledgement/Funding ANR-10-IAHU-04

Background Complex ATs with a complicated circuit have been increasing because of the native or iatrogenic scars in atrium. However, development of resolution in the EAM allows us to reach the correct diagnosis. The purpose of this study was to reassess the relationship between characteristics of ATs and cycle length based on the correct diagnosis with high-resolution mapping system. Method Among 85 consecutive AT patients treated with with high-resolution mapping system “Rhythmia”, patients with at least one mappable AT during procedure were prospectively included in this study. Maximal, minimal, and variation of ATCL was calculated from the decapolar in the coronary sinus (CS) during 2minutes, and the relation between CL and respiration was measured on the Rhythmia (Figure 1). These values were compared to AT types. Results 90 ATs in 65 patients (mean 59±13 yrs, 16 females, 55 post-AF ATs) including 4 focal-ATs, 23 localized-AT, and 63 macroreentrant ATs were analyzed. As shown in the table 1, the maximum and minimum CL was similar between focal-AT, localized-AT, and, macroreentrant-AT. However, both absolute CL-variation and ratio of CL-variation were significantly smaller in macroreentrant ATs (P=0.009 and P=0.0012, respectively). Table 2 described the comparison between 66 left-ATs and 24 right-ATs. Although the maximum and minimum CL were similar, both absolute CL-variation and ratio of CL-variation were significantly larger in right ATs (P=0.001 and P=0.007, respectively). Additionally, the correlation of CL to the respiration was significantly more frequently seen in the right-ATs (3.0% in left-ATs and 62.5% in right-ATs, P Table 1, 2 and Figure 1 Conclusions CL-variation of ATs may be significantly smaller in macroreentrant ATs and significantly larger in right ATs. CL-variation correlated to the respiration may suggest right-ATs. These information may be helpful to diagnose ATs before mapping.

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.

Introduction La survenue d’une hypercalcemie au cours d’une pneumocystose est un phenomene rare mais de plus en plus regulierement decrit. Une etiologie granulomateuse avec synthese accrue de calcitriol est suspectee, sans preuve directe de cette hypothese. Nous proposons une description des pneumocystoses hypercalcemiques et recherchons une correlation entre intensite des lesions pulmonaires et anomalies ioniques. Materiels et methodes Etude monocentrique et retrospective dans un CHU (de 01/2008 a 11/2018). Les patients etaient identifies par un double codage « pneumocystose » et « hypercalcemie » lors d’une recherche PMSI. Les mesures de densites pulmonaires, cotees en unites Hounsfield (UH), etaient realisees avec le logiciel Syngo.Via® sur les scanners thoraciques disponibles. Des analyses de correlation etaient realisees avec le logiciel GraphPad®. Les valeurs de calcemie etaient corrigees sur la protidemie. Resultats Au total, 931 patients ont ete hospitalises pour une pneumocystose sur le CHU durant la periode etudiee. Vingt-deux patients presentaient une hypercalcemie (10 hommes, 12 femmes, âge moyen 62 ans), soit une frequence de 2,3 %. Vingt patients etaient des greffes renaux. La calcemie moyenne etait de 2,94 mmol/L. Aucun dosage de parathormone (PTH) n’etait eleve, la vitamine D etant en moyenne a 34,5 ng/mL. On observait une nette decroissance des taux de PTH au cours de la pneumocystose pour l’ensemble des patients, en comparaison a leur dernier bilan de suivi de greffe. Douze patients sur quatorze presentaient un calcitriol eleve (1,97 fois la normale superieure en moyenne), les 2 autres patients presentant un calcitriol normal, les 8 derniers n’ayant pas de dosage disponible dans le bilan etiologique de leur hypercalcemie. Il n’existait pas de correlation entre les valeurs de calcemie et calcitriol, mais une correlation negative entre calcitriol et PaO2 (r = −0,59, p = 0,045). Seize scanners ont ete relus. La densite pulmonaire moyenne etait de −697 UH(±66). Il n’existait pas de correlation entre l’intensite des lesions pulmonaires, les valeurs de calcitriol (p = 0,52), de calcemie (r = 0,435, p = 0,092) ou de PaO2 (p = 0,75) mais avec les valeurs de PCO2 (r = 0,592, p = 0,0425). Conclusion Nous rapportons la plus importante serie de pneumocystoses hypercalcemiques decrite a ce jour. Cette forme est principalement observee chez les greffes renaux. La decroissance des taux de PTH plaide pour une origine extra-parathyroidienne et les valeurs elevees de calcitriol pour une synthese granulomateuse. Les raisons pour lesquelles certaines pneumocystoses se compliquent de granulomes sont inconnues, mais la responsabilite de certains genotypes de Pneumocystis jirovecii est suspectee.

Introduction La RIV des TE avec du 177Lu DOTATATE est d’une efficacite remarquable. L’Agence Europeenne du Medicament recommande une surveillance renforcee. Selon les etudes, 2 a 10 % des patients auront des effets secondaires graves, hematologiques. On connait les limites pour l’extrapolation de ces resultats dans la vraie vie et nous n’avons pas de facteurs predictifs clairement identifies a ce jour. L’utilisation du 177Lu DOTATATE requiert donc une vigilance et une formation des praticiens. En outre, les TE sont des maladies heterogenes. Pour certaines formes avancees, mais lentement evolutives, la question du rapport benefice-risque du traitement se pose. Les connaissances radiobiologiques sont moins etoffees pour la RIV que pour la radiotherapie externe. Il est donc necessaire d’approfondir la recherche dans ce domaine. Comment allier evaluation dans la vraie vie, encadrement de pratiques, tout en favorisant la recherche ? Methode Nous proposons de creer un registre qui se distingue d’une base de donnees et d’une cohorte par un recueil exhaustif, non biaise et non limite dans le temps. Avec le soutien de la SFMN, du CNP de medecine nucleaire et le support technique et juridique de la Federation des Specialites Medicales, nous avons cree le registre Epilunet (Epidemiologie-Lutetium-NeuroEndocrine-Tumor). Il s’inspire du registre Epithor qui, avec des milliers de procedures de chirurgie thoracique enregistrees, ameliore la securite des soins, participe a la formation medicale (reconnue par l’HAS), tout en permettant une valorisation scientifique et economique des donnees. Resultats Epilunet est reconnu par la CNIL. La saisie, assistee par des menus deroulants, permet une ouverture rapide des dossiers qui sont compiles via internet dans un registre national. Chaque centre disposera de l’acces a ses donnees. L’utilisation de la base nationale pour des projets de recherche sera possible sur accord du comite scientifique (a condition de citer l’origine). Discussion–conclusion Ce registre pourrait prefigurer celui pour la 177Lu PSMA therapie. Nos confreres espagnols publient deja les resultats dans la vraie-vie de 125 patients inclus dans leur registre national (SEPTRALU Registry). Si, et seulement si, Epilunet est renseigne de maniere exhaustive et reguliere, alors nous pourrons savoir quels malades nous traitons, pour quel benefice et avec quelle toxicite ? Le defi collectif est donc de parvenir a remplir ce registre ; y arriverons-nous. ?

Resume Les progres de la biologie moleculaire ont permis ces dernieres annees des avancees considerables dans le determinisme genetique et physiopathologique des pheochromocytomes et des adenomes de Conn. Par contre la physiopathologie moleculaire des hypercortisolismes ACTH-independants demeurait inconnue dans la plupart des cas. L’annee 2014 a vu une explosion de nouveaux travaux qui revolutionnent nos connaissances et concepts physiopathologiques en matiere d’hyperplasie macronodulaire des surrenales ACTH-independantes (AIMAH) ou d’adenome cortisolique. Enfin, le retentissement cardiovasculaire de l’hypercortisolisme infraclinique des adenomes cortisoliques infracliniques (ACIC) decouverts fortuitement etait jusqu’ici discute car reposant exclusivement sur des etudes transversales. Deux grandes etudes de suivi ont permis de preciser l’histoire naturelle de ces lesions. Toutes ces donnees temoignant de la richesse de la recherche clinique et moleculaire de ces dernieres annees dans l’hypercortisolisme ACTH-independant ont ete exposees et discutees au congres de l’Endocrine et font l’objet de cet article.

Introduction Drug-refractory electrical storm due to VF or polymorphic VT is rare but has poor prognosis. Case reports have suggested temporary pacing could be useful to suppress recurrent arrhythmia. Methods All consecutive patients admitted to the Cardiac Intensive Care Unit of our institution from 2009 to 2017 for VF electrical storm refractory to medical therapy in whom temporary pacing was used at the acute phase were included. Results Ten patients met inclusion criteria. Nine men and one woman mean age was 67 ± 21. Two patients were already equipped with an ICD in secondary prevention. Mean number of arrhythmic events during electrical storm was 28.7 ± 43.5. Electrical storm was related to a recent ischemic event (6 patients), Brugada syndrome with fever episode (1), or Flecainide intoxication (1). Cause remained unknown in 2 patients. Mean LVEF was 36 ± 15%. Medical therapy using Amiodarone (6), B-blockers (4), and Lidocaine (5) for ischemic patients or Isoproterenol for Brugada syndrome had failed in all patients. Rapid pacing (99.0 ± 20.8 bpm) at the atrial (4), ventricular (5), or both (1) levels was initiated at this stage. Pacing therapy was ineffective with the Flecainide intoxication and the idiopathic VF patient, reduced the number of VF episodes with the Brugada syndrome patient and completely suppressed arrhythmias in all other patients. Atrial and ventricular pacing were equally effective. Patients with effective temporary pacing underwent ablation (2), or were managed medically (3). There was no serious pacing-related complication. Conclusion Temporary pacing seems to be a quick and safe therapeutic option in VF electrical storm. It's particularly effective in patients with recent ischemic events in which it can be used as a bridge to ablation or to await medical therapy optimization.

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.

Introduction Ventricular Fibrillation (VF) is one of the most frequent causes of sudden cardiac death. It is known that Purkinje fibers (PF) play an important role of arrhythmia trigger and recent studies suggest a potential role of these fibers as a substrate maintaining VF. Objective The goal of this study was to define the role of Purkinje-muscle junctions (PMJ) in maintaining VF and to determine the effect of catheter ablation of these junctions. Method Optical mapping experiments were performed in coronary-perfused LV wedge preparations from sheep (n = 8), stained with Di-4-ANBDQBS and using LED illumination at 660 nm. CMOS camera (MiCAM, BrainVision) was used to obtain endocardial recordings. PMJ were localized through PF stimulation and were compared to late activation zones (LAZ). Endocardial burst pacing (10 Hz) was applied to trigger 10 minutes of VF, before and after PMJ ablation (30 W, 15 s), and recordings were acquired every 2 minutes. Phase mapping was used to quantify rotor and focal dynamics. Results No dominant frequency differences between PMJ and LAZ were found for all VF episodes, before (P = 0.81) and after (P = 0.79) PMJ ablation. No change in focal activity was observed during this initial phases of VF. However, a tendency for colocalization for distances between PMJs and rotors was observed before ablation. Distances increased significantly (20 ± 7 mm vs. 24 ± 7 mm; P = 0.009) after ablation. Moreover, a decrease in mean rotor duration and a significant increase in rotor number for all VF episodes (6 ± 4 vs. 12 ± 11; P = 0.026) were also found after ablation. Conclusion These results suggest that the PMJ is involved in maintaining VF by playing a role in rotor dynamics.

The mainstay of treatment for atrial fibrillation (AF) remains pharmacological, however, catheter ablation, since an early attempt in 1994 has undergone many evolutions up to the present day whereby it has taken an increasing place in the management of this arrhythmia. In paroxysmal AF, the most recent studies report a success rate of more than 80% at 1 year of follow-up after a single procedure (free of symptoms without antiarrhythmic drugs). In persistent AF the technique continues to evolve with a success rates between 70% and 95% even if several long and complex procedures are often needed, which are not without risk, to achieve these results. With constant improvement in this field catheter ablation has become a valuable tool in the management strategy of AF.

The development of molecular biology tools has allowed major advances in the genetic determinism and the pathophysiology of pheochromocytomas and Conn's adenomas. However, the molecular pathophysiology of ACTH-independent Cushing's Syndrome was mostly unknown until recently. In 2014, major new insights concerning the physiopathology of ACTH-independent macronodular adrenal hyperplasias (AIMAH) and the cortisol-secreting adenomas have been published. Elsewhere, the cardiovascular consequences of the subclinical hypercortisolism due to adrenal incidentalomas, was described only in some cross-sectional studies. The natural history of these lesions has been documented in two large follow-up studies. These new data presented during the Endocrine Congress are summarized herein.