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SummaryBackgroundMany stents are used “off-label” during the management of congenital heart diseases (CHD).AimsTo describe indications for, results of, and adverse events associated with stenting in CHD in current practice.MethodsParticipation in this study was proposed to all catheterization laboratories that specialize in CHD in France (M3C network). All paediatric and adult CHD cases with stent implantation in 2013 were included retrospectively.ResultsOverall, 207 stents were implanted in 151 patients across 11 centres. Median age was 13.7 years (range, 5 days to 70.1 years). Main procedure indications were branch pulmonary artery angioplasty (n=46, 29.1%), aortic (re)coarctation stenting (n=43, 27.2%), percutaneous pulmonary valve implantation (n=32, 20.2%) and ductus arteriosus stenting (n=14, 8.9%). The main stents implanted were the CP Stent™ (n=61, 29.5%), the Max™ LD stent (n=43, 20.8%), the Valeo® stent (n=28, 13.5%) and valved stents (n=30, 14.5%). Procedures were considered successful in 96.8% of cases (95% confidence interval [CI] 92.8–99.0%). Adverse events were observed in 23 procedures (14.7%, 95% CI 9.5–21.0%). Ductus arteriosus stenting (odds ratio 12.4, 95% CI 2.0–77.5; P

Chemotherapy-induced cardiotoxicity is a major cause of morbidity and mortality in cancer survivor. The most clinically evident and best known cardiotoxicity is the anthracycline adverse effect with heart failure. Many cardiovascular adverse effects appear after cancer therapy: heart congestive failure, myocardial ischemia, hypertension, thromboembolic complications, arrhythmias and conduction disturbances. There are potential strategies to mitigate the risks of cardiac complications for cancer patients with physical examination, echocardiography and electrocardiogram. The management of the cardiotoxicity is variable.

IntroductionCardiopulmonary bypass can be responsible for post-operative left ventricular dysfunction. As conventional echographic parameters are not reliable in the post-operative period, we assessed left ventricular function by 2D strain. The main objective of this study was to evaluate the peri-operative feasibility and reliability of 2D strain. The second objective was to assess the impact of aortic cross-clamp duration on echographic parameters.Methods33 pediatric patients (80 min for group 3).ResultsMean age and weight were 4 years [2-6] and 15kg [10-20]. Mean duration of aortic cross-clamp was respectively 22 min, 56 min and 101 min in groups 1, 2 and 3. Longitudinal, circumferential and radial strains were feasible in 91%, 95% and 95% of the cases respectively, which was similar to conventional parameters (97%). The reliability of 2D strain measurements was better than the one of conventional parameters (intra and inter-observer correlation coefficients: 0.916, p

Background— Although dual-chamber pacing improves cardiac function in patients with complete congenital atrioventricular block (CCAVB) by restoring physiological heart rate and atrioventricular synchronization, the long-term detrimental effect of asynchronous electromechanical activation induced by apical right ventricular pacing (RVP) has not been well clarified. Methods and Results— Twenty-three CCAVB adults (24±3 years) with a DDD transvenous pacemaker underwent conventional echocardiography before implantation and, after at least 5 years of RVP, an exercise test and echocardiography coupled with tissue Doppler imaging and tissue tracking. They were compared with 30 matched healthy control subjects. After 10±3 years of RVP, CCAVB adults had significantly higher values versus controls in terms of intra-left ventricular (LV) asynchrony (respectively, 59±18 versus 19±9 ms, P P P P =0.05) and was higher than in controls (1±0.1, P P P P P Conclusions— Prolonged ventricular dyssynchrony induced by long-term endovenous RVP is associated with deleterious LV remodeling, LV dilatation, LV asymmetrical hypertrophy, and low exercise capacity. These new data highlight the importance of the ventricular activation sequence in all patients with chronic ventricular pacing.

Background Patients after repaired tetralogy of Fallot (TOF) frequently have right ventricular (RV) dysfunction and prolonged duration (QRSd), the latter is considered as a sudden death risk factor. It has been suggested that QRSd mainly reflects abnormalities of the RV outflow tract (RVOT) rather than the RV body itself. We aimed to better understand the RV electrical activation pattern in these patients using activation mapping. Methods 63 adults (36,3± 14 yo, median QRS duration 159ms IQR 142- 170) referred for either catheter ablation or pulmonary valve replacement late after TOF repair underwent a MRI, with fibrosis analysis, and an invasive RV activation mapping (201±32 sites per patient; Carto 3 – Biosense Webster). RV total activation time (RVTAT) was defined as the duration between the first and the last RV EGM. Results The delay between QRS onset and earliest RV EGM was 28± 23 ms traducing the absence of RV purkinje activation and the left to right ventricle activation. We observed in all patients a single RV breakthrough (mid-septal in 86%, septo-basal in 7% and apico-septal in 7%) followed by 2 spreads of activation: a first wave from the septum to the RV anterior wall through the RVOT with fragmented EGM in the infundibulum; a second wave from the septum to the RV free wall through the apex with slow conduction. The RV free wall was the latest activated in all the patients (Figure). RVTAT (145±20 ms) was correlated to QRS duration (r=0,72 ; p Conclusion RV delayed activation in patients with repaired TOF traduces a homogeneous activation pattern that is not only the consequence of an infundibular disease but also reflects a slow conduction in the RV free wall. The author hereby declares no conflict of interest Download : Download full-size image Figure . anterior and posterior views of RV activation maps (right side) and RV voltage maps (left side) in the same patient. RVOT: Right ventricular outflow tract; VSD: Ventricular septal defect; IVS: Interventricular septum; TA: Tricuspid annulus; PA: Pulmonary annulus.

Objectives The importance of the left atrial appendage (LAA) on left atrial (LA) hemodynamics is unknown. We sought to evaluate the effect of LAA percutaneous closure (LAAPC) on left atrial remodeling in patients with paraxysmal atrial fibrillation (AF) and permanent AF. Methods All patients refered for LAAPC with Amplatzer Cardiac Plug (ACP) and Watchman device were enrolled. Cardiac computed tomography (CT) for LA volume measurement and transthoracic echocardiography (TTE) for diastolic function assessment were performed at baseline and 3 months after LAAPC. An average of 3 consecutives measurements were performed for TEE parameters in all patients. Results Sixty-three patients (mean age 73±9 years) were included. 38% (n=24) in sinus rythm (SR) at baseline and 55% (n=35) in permanent AF. Patients in SR at baseline and permanent AF at 3 months were excluded (n=4,7%).The mean CHA2DS2-VASc score was 4,3±1,3. There was non significant difference in the functionnal status and BNP level (155,6±107 vs ±150,7pg/mL; p=0,85) between baseline and 3 months follow-up. Left atrial volume excluding the LAA (145 ±55cm 3 baseline vs 144±50cm 3 at 3 months; p=0,30) showed no significant change after 3 months in overall population, neither in the SR (99,7±19,1 vs 103,8±21cm 3; p=0,32) or the permanent AF groups (173,2±54 vs 171,7±48,6cm 3; p=0,59). MV peak E-wave (84,2±22,7 vs 86,7±26cm/s; p=0,62) and A-wave velocities (65,4±14,4; 68,5±22,2cm/s; p=0,66) dit not differ between baseline and follow-up but E/E’ ratio was increased in the overall population after LAAPC (7,9±2,1 vs 9,1±3,6cm/s; p=0,038) and there was trend to higher E/E’ ratio in the SR group (7,7±1,6 vs 9±3,3cm/s, p=0,46). Conclusions There’s no evidence for early LA remodeling after LAAPC, but diastolic function might be negatively influenced by LAAPC suggesting the potential role of LAA in atrial function. Further studies are warranted to confirm the prelimary results.

This report describes the case of a 6-month-old girl with a large cardiac fibroma in the right ventricle. Ventricular tachycardia associated with the fibroma was successfully treated with amiodarone. At the age of 3 years, surgical resection was indicated because of right ventricular outflow tract obstruction caused by progression of the tumor. The fibroma was successfully resected, and further follow-up evaluation was uneventful.

Background Pulmonary valve replacement (PVR) for patients with repaired Tetralogy of Fallot (TOF) and pulmonary regurgitation (PR) improves functional status without definitive improvement of peak VO2 despite correction of the haemodynamic lesion. We aim to study cardiorespiratory and peripheral profiles during exercise test before and after PVR to understand this improvement. Methods Patients with TOF and severe PR performed a cardiopulmonary exercise testing (CPET) and a cardiac MRI for the measurement of ventricular volumes and pulmonary regurgitation fraction (FR) before and after PVR. Results Fifteen patients (mean age 33 ± 13 years) were included. CPET was performed 6 months before and 25 ± 2 months after PVR. Following PVR: significant improvement in NYHA functional class (P = 0,006) without significant improvement in peak VO2 (26 ± 6 to 25 ± 6,9 mL/kg/min, P = 0,36) nor maximal workload (114 ± 30 to 118 ± 30 Watt, P = 0,31) with unchanged maximal heart. Patients had a moderate hyperventilation without pejorative criterion (VE/VCO2 nadir 31 ± 3 to 32 ± 7, P = 0,61) without pulmonary limitation (maximal breathing reserve 31 ± 23 to 21 ± 17%, P = 0,27) despite a restrictive pattern in spirometric results. Nevertheless, we found a significant improvement in peak oxygen pulse (9,9 ± 2,5 to 11 ± 4, P = 0,04) and a significant postponed ventilatory anaerobic threshold (VAT) after PVR (05:28 ± 2:00 to 6:57 ± 1:53 min, P = 0,002; 61 ± 20 to 75 ± 18 Watt, P = 0,006). PR (FR: 46 ± 14 to 5,6± 9,7%, P Conclusion Despite the lack of improvement in the peak VO2, hemodynamic improvement was observed by the significant improvement in oxygen pulse and peripheral capacity associated with an improvement in functional capacity. It can be assumed that the peak VO2 is not the best marker for the evaluation of aerobic capacity and that our evaluation could be based on the VAT in these patients.

Background Supraventricular tachycardia (SVT) is the most common arrhythmia in children. Isolated SVT inducing cardiogenic shock is rare and factors leading to cardiac dysfunction are unknown. We aim to determine the predictive factors of cardiogenic shock in children with SVT. Methods In total, 109 children under the age of 1 [median age 10 days (1 d; 30 d)], who were referred for SVT to our pediatric cardiologic intensive care unit were included. Cardiogenic shock was defined as a systemic ventricular ejection fraction under 50% and the need for inotropes use. Types of SVT, antenatal diagnosis of arrhythmia, demographic data, clinical and biological characteristics and the therapeutic management were reviewed. Results Seventeen percent of patients had cardiogenic shock [median age 12 days (3.5 d; 17.5 d)]. Heart failure was significantly more frequent in case of flutter (54.5% in flutter; 13.3% in other SVT; P Conclusion The occurrence of cardiogenic shock is not uncommon in children with SVT and concerns mainly patients with flutter. Faster heart rate and the ineffectiveness of adenosine are associated with potentially severe outcomes. These predictive factors of shock will help identify higher risk patients and improve the therapeutic management.

OBJECTIVES Total cavopulmonary connection (TCPC) performed in the second decade of life has rarely been studied. Thus, we investigated (bicentric study) early and late morbidity and mortality following completion of TCPC in these patients. METHODS From January 1999 to June 2014, 63 patients (14.5 ± 2.9 years) underwent TCPC (extracardiac conduit). Palliation before completion was an isolated bidirectional cavopulmonary shunt (BCPS) in 3 patients or BCPS associated with additional pulmonary blood flow (APBF) that was either antegrade (Group 1) in 38 (63%) or retrograde (Group 2) in 22 (37%). Preoperative and perioperative data were reviewed retrospectively. RESULTS Mean pulmonary arterial and ventricular end-diastolic pressures were 12.2 and 9.2 mmHg, respectively. Mean Nakata index was 279 ± 123 and 228 ± 87 mm 2 /m 2 in Groups 1 and 2, respectively ( P = 0.01). Aortic cross-clamping was performed in 22 from Group 1 and 8 from Group 2 ( P = 0.04). Mean follow-up was 4.57 years [0.8-15]. Nine patients had prolonged stays in the intensive care unit (>6 days). There were 1 early and 2 late deaths (non-cardiac related). Actuarial survival was 96% at 4 years. At last follow-up, single-ventricle function remained normal or improved in all patients (Group 1) compared to 82% in Group 2 ( P = 0.02). New York Heart Association (NYHA) class had improved in both groups: 47 patients were NYHA class II and 16 class III preoperatively vs 50 class I and 10 class II postoperatively ( P < 0.001). CONCLUSIONS Single-ventricle palliation with BCPS and APBF allowed completion of TCPC in the second decade of life, with encouraging mid-term results. However, BCPS with retrograde APBF was associated with single-ventricle dysfunction: thus, this technique needs to be used cautiously as long-lasting palliation.

SummaryEosinophilia may be responsible for cardiac injuries of widely varying severity, from acute myocarditis to endomyocardial fibrosis. In this review, we present both the molecular mechanisms that are responsible for these lesions and their clinical and paraclinical aspects. Numerous aetiologies can lead to severe eosinophilia, but these are mainly represented by hypersensitivity reactions, rheumatological diseases and hypereosinophilic syndrome. Because cardiac involvement may be extremely severe, echocardiography should be always performed in the context of eosinophilia and appropriate therapeutics should be started rapidly in order to limit the progression of the disease.

We describe a family with suspicion of genetic arrhythmia that has benefited from a wide genetic exploration. The eldest of the siblings presented syncope at age 5.5 years and cardiac explorations were normal. A few months later, her elder sister presented a sudden death at age 4.5 years, while she was playing in the garden. The cardiac explorations showed a heart of normal structure but presence of polymorphic premature ventricular complexes. Isoprenaline test was positive. Treatment with beta-blockers (nadolol 50 mg/m 2 ) was introduced. There was no family history of sudden death or other cardiac defects. Because of these two serious rhythmic events occurring in two young children, a genetic study was initiated by next generation sequencing of 42 genes involved in cardiac arrhythmias (long QT, Brugada, cathecholaminergic ventricular tachycardia). Two heterozygous mutations (c.613C > T/p.Gln205* and c.22 + 29 A > G) were identified in the Triadin gene, coding for a protein of the calcium release complex, recently involved in cathecholaminergic ventricular tachycardia in two families (Roux-Buisson et al., 2012). The parents of our two cases were each carriers of a heterozygous mutation and had no cardiac symptoms. Their cardiac assessment did not show any abnormality (ECG Holter, exercise test, Isoprenaline test). The nonsense p.Gln205* mutation was present in one of the published families; however the splicing mutation in intron 1 had never been identified. Minigene experiments helped to confirm its pathogenicity. Presymptomatic testing was then proposed to the third child of the family (age 3), finding the two pathogenic mutations. She was therefore put under the same treatment as her sisters. This is the second report of an autosomal recessive cathecholaminergic ventricular tachycardia due to the Triadin gene. This case illustrates the interest of Next Generation Sequencing exploring simultaneously several candidate genes, in cases of sudden death of unknown origin.

An asymptomatic 8-year-old boy was addressed to our echolab for the exploration of a systolic cardiac murmur. ECG demonstrated a right heart volume overload and a right bundle branch block. Chest X-ray showed a slight enlargement of right-sided heart chambers. TTE and TEE showed a prolapse of the anterior leaflet of the tricuspid valve (TV; Panels A and B ; see Supplementary data online, Video S1 ). Because of a lack of coaptation, this prolapse was responsible for a severe tricuspid insufficiency ( Panels C and D ). The width of the vena contracta was estimated at …

SummaryPercutaneous pulmonary valve implantation now has a key role in the setting of dysfunctional right ventricle-to-pulmonary artery conduits or failing bioprosthetic pulmonary valves. However, despite the excellent results obtained with the two devices available currently (the Melody® valve [Medtronic Inc., Minneapolis, MN, USA] and the Edwards SAPIEN® valve [Edwards Lifesciences, Irvine, CA, USA]), many patients eligible for pulmonary valve replacement remain unsuitable for percutaneous pulmonary valve implantation, mainly because of large native outflow tracts. Accordingly, one of the major challenges for the future is to expand percutaneous pulmonary valve implantation to a broader population of patients. Moving forward, there is important ongoing research that is intended to improve patient outcomes, expand percutaneous pulmonary valve implantation therapy and continue to reduce the number of open-heart surgeries in this population. In this review, we underline the limitations and issues associated with the devices available currently, and we focus on the development of new strategies (such as hybrid approaches or magnetic resonance-guided procedures), new devices (such as right ventricular outflow tract reducers or the novel Native Outflow Tract valved stent from Medtronic) and new technologies (such as tissue-engineered valves), which may help to take up these challenges and represent the future of transcatheter valve implantation.

Background The experience with the implantable cardiac defibrillator (ICD) in patients with transposition of the great arteries (TGA) and history of atrial switch surgery remains limited. Methods Retrospective evaluation aiming to assess characteristics and outcomes of consecutive TGA patients with history of atrial switch surgery implanted with an ICD between January 2005 and June 2012 in four French centers. Results Of the 12 patients (median 34years [28, 40]; 67% male), 4 patients (33%) were implanted for secondary prevention after symptomatic documented sustained ventricular tachycardia or sudden cardiac arrest. ICDs were implanted for primary prevention in 8 patients (67%), including cardiac resynchronization in 3 patients; severe systemic ventricle dysfunction was present in all cases (median ejection fraction 27% [20, 40]). Overall, one patient died during the ICD implantation secondary to refractory cardiac arrest after defibrillation testing. Over a median follow-up of 19months [10, 106], 6 patients out of 11 (54%) experienced worsening of congestive heart failure, including 5 who were eventually transplanted. Overall, 3 patients (27%) experienced significant ICD-related complications, whereas only one patient (primary prevention indication) developed appropriate ICD therapy (successful anti-tachycardia pacing without shock). Half of the patients presented with at least one episode of sustained (≥5min) atrial arrhythmia during follow-up. Conclusions Our findings underline the key role of progressive heart failure in dictating outcomes among TGA patients with prior atrial switch repair. Our results also underline the need of better risk-stratification for sudden cardiac death in those patients.

Tramadol is a common analgesic, widely prescribed because of its efficiency and safety. We report the case of a 7-year-old child admitted in cardiac intensive care unit for cardiogenic shock due to tramadol intoxication. Without any past history, the child was admitted at emergency room for generalised convulsion, followed by respiratory distress. Cardiogenic shock was suspected after clinical examination and chest X-ray and confirmed by transthoracic echocardiography showing low left ventricular ejection fraction (30 %) with pulmonary hypertension. No evidence of cardiac infarction or myocarditis was found. Tramadol intoxication was suspected because of empty tramadol tablets found near the child and later confirmed by toxicologic analysis showing high blood concentration of tramadol (1 mg/L) and O-desmethyltramadol (1.5 mg/L). Hemodynamic support by inotropic drug infusion and diuretics was necessary. Left ventricular function normalised after 2 days of treatment allowing drug infusion weaning. Cardiac magnetic resonance imaging performed 3 days after admission confirmed normal left ventricular ejection fraction and volumes without evidence of late gadolinium enhancement. Cardiogenic shock due to tramadol intoxication is rare but exists. Negative inotropic effect of high doses of tramadol has been suspected. Quick recovery is possible.

Ventricular arrhythmias are frequent in patients with repaired tetralogy of Fallot but their underlying mechanisms remain unclear. In this study, ventricular electrical and structural remodelling was assessed in an animal model that mimics postoperative tetralogy of Fallot.Piglets underwent a tetralogy of Fallot repair-like surgery (rTOF N=6) or were sham-operated (Sham N=5). Following cardiac function assessment in vivo by MRI 3–4 months after surgery, pigs were euthanized and their hearts rapidly excised. Electrophysiological properties of right (RV) and left ventricles (LV) were obtained by optical mapping. Fibrosis was assessed histologically.RV dysfunction was evident while LV function remained unaltered in rTOF pigs. LV action potential duration (APD) was significantly longer on the epicardium (Sham 280±50ms; rTOF 390±76ms) and endocardium (Sham 301±20ms; rTOF 403±34ms) of rTOF animals (P

Ventricular arrhythmias are frequent in patients with repaired tetralogy of Fallot but their underlying mechanisms remain unclear. In this study, ventricular electrical and structural remodelling was assessed in an animal model that mimics postoperative tetralogy of Fallot. Piglets underwent a tetralogy of Fallot repair-like surgery (rTOF N=6) or were sham-operated (Sham N=5). Following cardiac function assessment in vivo by MRI 3-4 months after surgery, pigs were euthanized and their hearts rapidly excised. Electrophysiological properties of right (RV) and left ventricles (LV) were obtained by optical mapping. Fibrosis was assessed histologically. RV dysfunction was evident while LV function remained unaltered in rTOF pigs. LV action potential duration (APD) was significantly longer on the epicardium (Sham 280±50ms; rTOF 390±76ms) and endocardium (Sham 301±20ms; rTOF 403±34ms) of rTOF animals (P

Stents have contributed to the development of the interventional catheterization of congenital heart diseases (CHD). The aim of this study was to describe current indications and results. Methods Participation to this study was proposed to all catheterization laboratories specialized in CHD in France (M3C network). All cases with stents implantation in 2013 in pediatric and adult CHD were retrospectively included. Adverse events were assessed using the Bergersen classification. Risk markers were searched using univariate and multivariate analysis. Results 174 stents were implanted in 127 patients. Patients were aged 15.7±15.1 year old (min 5 day-old - max 70 year-old). Main indications were transcatheter pulmonary valve replacement (prestenting and revalvulation; n=29 patients, 23.0%), pulmonary artery branches angioplasty (n=34, 27.0%), aortic coarctation or recoarctation stenting (n=37, 29.4%) and ductus arteriosus stenting (n=11, 8.7%). 18 Patients (14.3%) were under one-year old and 35 (27.8%) were over 18 year-old. Main pathologies were tetralogy of Fallot and variants (ACC-CHD 8.3, n=45, 35.7%), and aortic coarctation (ACCCHD 9.2, n=36, 28.6%). More than one stent was implanted in 32 procedures (24.4%, max. 5 stents). Main stents implanted were the CP stent (33.0%), EV3 LD max (22.0%), Valeo (16.2%) and valved stents (15.0%). 98.5% Procedures were considered as successful. Adverse events were observed in 16.7% procedures (stent related in 12.1% procedures). Serious adverse events were observed in 12.9%. After uni- and multivariate adjustment, only procedure type was related to the risk of total adverse events but not to stent related adverse events. Age, weight, center, type of stents, genetic disorder and type of CHD were not significantly related to the risk of adverse events. Conclusion Stents are used in various CHD catheterization procedures, from infancy to adult age. Adverse events rate is significant and seems related to the type of procedure.

ObjectivesTo assess health-related quality of life (QoL) in patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) and correlations with clinical status.MethodsThis cross-sectional study included PAH-CHD patients in 14 centers in France. QoL was self-reported with a generic questionnaire (SF-36) and a PH-specific questionnaire (CAMPHOR). Patients filled out the Hospital Anxiety and Depression Scale (HADS) questionnaire. Main clinical data were collected.Results200 patients were included (mean age 43±SD15.7 years, range 15 to 86 years, 70% female), and were classified as Eisenmenger syndromes (73.5%), PAH associated with systemic to pulmonary shunts (13%), PAH associated with small defects (3.5%) and PAH after corrective cardiac surgery (10%). At inclusion, 76.5% patients were receiving PAH-specific treatments. All component scores of both QoL questionnaires showed degradation vs healthy population standard scores. QoL scores were lower in females than in males for all dimensions of both questionnaires (p

Evaluate the incidence, diagnosis, and outcome of coronary compression (CC) during right-ventricular outflow tract interventions.The incidence, risk factors, diagnosis, and outcomes of CC during percutaneous pulmonary valve implantation are poorly defined.One-hundred consecutive patients (May 2008 to January 2012) undergoing transcatheter right-ventricular outflow tract treatment in two institutions were studied.CC occurred in six patients (6%) with a right ventricular outflow conduit stenosis, at a median age of 24.5 (13-49) years. It involved the left main coronary artery in four and the right coronary artery originating from the left anterior descending coronary artery in two patients. Conduit types were homograft (n = 3), bioprosthesis (n = 2), and a pericardial patch (n = 1). Median diameter was 23 (17-24) mm at surgical implantation. CC was diagnosed through a selective coronary angiogram during balloon dilation of the conduit in the first three patients and through an aortic root angiogram for the three next cases because we recognized that proximal compression could be masked during coronary artery cannulation. It was suspected on pre-procedure imaging (magnetic resonance imaging and/or computed tomography) in three cases. Patients with abnormal coronary anatomy tend to be at increased risk of CC (P = 0.0504). One institution had a higher incidence of CC (P = 0.04). CC resolved after balloon deflation. No patient underwent conduit stenting. Four patients underwent surgical reconstruction of right ventricular outflow tract.CC is accurately diagnosed during right-ventricular outflow tract interventions. We recommend an aortic root angiogram during dilation with a non-compliant balloon matching the diameter and length of the intended conduit implant.

PurposeEvaluation of the right ventricle (RV) using echography is challenging in patients with repaired Tetralogy of Fallot (rTOF). The objective of this study was to evaluate the feasibility and the accuracy of a multiparametric echographic approach including 2D strain and 3D for RV volumes and function assessment, in comparison with MRI.Methods and resultswe performed an echographic study including 2D TAPSE, S’TDI, Tei indice, Fractional area change (FAC)), 2D strain and 3D, and an MRI in 26 consecutive patients with rTOF before PVR and one year after surgery. TAPSE, S’ TDI and 2D strain parameters were poorly correlated with MRI regarding RV function assessment. FAC was well correlated with REVF before and after PVR (r=0.70, p

Atrial fibrillation aged patients present a greater risk of cardioembolic events and major bleeding, LAAC could an attractive alternative for these patients however its safety and efficacy have not been reported previously in the subgroup of patients ≥85 years old. The objective of the study was

BackgroundIntra-aortic balloon pump (IABP) is a commonly used method of temporary circulatory support in adults. Despite the availability of paediatric size balloons, the use of IABP in children is currently not widespread.AimsTo describe our practice of IABP in children and to review its indications.MethodsSingle-centre retrospective study. We reviewed the medical records of all paediatric patients (

PurposePeridevice leaks after left atrial appendage (LAA) closure with the Amplatzer® Cardiac Plug (ACP) have been reported using trans oesophageal Echocardiography (TOE), but this evaluation is challenging, and the few available data are inconsistent. There are currently few data regarding the evaluation of cardiac CT (CT) for peridevice leaks detection. We aimed to assess the incidence and the related factors of ACP peridevice leaks using CT.MethodsCardiac-CT using a multi-phase protocol and a second-generation dual-source-CT-system was performed in 45 patients with non-valvular atrial fibrillation 24h before the procedure and 3 months after LAA closure with ACP. Peridevice leak was defined as a presence of opacification of the LAA by the contast agent.ResultsFourty three patients were included, aged 73±9.4 years. ACP size compared to mean diameter of the landing zone measured by CT was oversized by 2.7±2.2mm. Peridevice leaks were found on CT in 18 patients (41.9%). The rate of peridevice leaks was lower among patients in whom the ACP oversizing was3mm, 23.5% and 55% respectively, p=0.052. The size of the LAA landing zone did not correlate significantly with the presence of peridevice leak (20.6±3.7 vs 21.5±4.2mm, p=0.47). The shape of the LAA was not associated with peridevice leaks (p=0.39). Clinical outcome with a mean follow-up 14.7 months was similar in both groups with 1 stroke in the peride-vice leak group and 2 in the other group (5.5% and 8% respectively).ConclusionsCardiac CT scan is an accurate technique for peridevice leaks detection. Important oversizing of the device is not associated with absence of residual leaks. There is a trend toward more residual leaks in the biggest LAAs. No adverse outcome were related with a residual leak.

Background— The origin of congenital or childhood nonimmune isolated atrioventricular (AV) block remains unknown. We hypothesized that this conduction abnormality in the young may be a heritable disease. Methods and Results— A multicenter retrospective study (13 French referral centers, from 1980–2009) included 141 children with AV block diagnosed in utero, at birth, or before 15 years of age without structural heart abnormalities and without maternal antibodies. Parents and matched control subjects were investigated for family history and for ECG screening. In parents, a family history of sudden death or progressive cardiac conduction defect was found in 1.4% and 11.1%, respectively. Screening ECGs from 130 parents (mean age 42.0±6.8 years, 57 couples) were compared with those of 130 matched healthy control subjects. All parents were asymptomatic and in sinus rhythm, except for 1 with undetected complete AV block. Conduction abnormalities were more frequent in parents than in control subjects, found in 50.8% versus 4.6%, respectively ( P P P P SCN5A mutation screening identified 2 mutations in 2 patients among 97 children. Conclusions— ECG screening in parents of children affected by idiopathic AV block revealed a high prevalence of conduction abnormalities. These results support the hypothesis of an inheritable trait in congenital and childhood nonimmune isolated AV block.

Background Patients treated by atrial redirection surgery (Senning or Mustard procedure) for transposition of the great arteries (TGA) have an important risk for heart failure caused by dysfunction of the systemic right ventricle. Conventional nonsystemic ventricular pacing (non-systVP) may even further increase this risk. Objective We investigated the effects of endocardial non-systVP, biventricular pacing (BiVP), and single-site systemic ventricular pacing (systVP) on systolic cardiac pump function in patients with TGA and status post atrial redirection surgery (SenningMustardTGA). Methods During clinically indicated catheterization in 9 patients with SenningMustardTGA, endocardial ventricular stimulation (overdrive DDD mode; 80–90 beats/min) was applied with temporary pacing leads at the nonsystemic and the systemic ventricle. Acute changes in the maximal rate of pressure rise (dP/dt max ) and systolic pressure of the systemic ventricle, as induced by non-systVP, systVP, and BiVP compared to reference, were assessed with a pressure wire within the systemic ventricle. Reference was AAI pacing with a similar heart rate (n = 7) or non-systVP at a lower heart rate than that during stimulation at experimental sites (85 beats/min vs 90 beats/min; n=2). Results Systemic dP/dt max and systolic ventricular pressure were significantly higher during systVP (+15.6% and +5.1%, respectively) and BiVP (+14.3% and +4.9%, respectively, compared with non-systVP). In 6 of 7 patients, systemic dP/dt max was higher during BiVP and systVP than during AAI pacing. Conclusions In a population of patients with SenningMustardTGA, acute hemodynamic effects of endocardial systVP and BiVP were significantly and equally better than those of non-systVP. In some patients, systVP and BiVP might even be better than ventricular activation by the intrinsic conduction system.

International audience; AIMS: The natural history of congenital or childhood non-immune, isolated atrioventricular (AV) block is poorly defined. METHODS AND RESULTS: We retrospectively studied 141 children with isolated, non-immune AV block diagnosed in utero, or up to 15 years of age, at 13 French medical centres, between 1980 and 2009. Patients with structural heart disease or maternal antibodies were excluded. Atrioventricular block was asymptomatic in 119 (84.4%) and complete in 100 (70.9%) patients. There was progression to complete AV block in 29/41 (70.7%) patients with incomplete AV block over 2.8 ± 3.4 years (1-155 months), but all patients with incomplete AV block may not have been included in the study. Narrow QRS complex was present in 18 of 26 patients (69.2%) with congenital, and 106 of 115 (92.2%) with childhood AV block. Pacemakers were implanted in 112 children (79.4%), during the first year of life in 18 (16.1%) and before 10 years of age in 90 (80.4%). The mean interval between diagnosis of AV block and pacemaker implants was 2.6 ± 3.9 years (0-300 months). The pacing indication was prophylactic in 70 children (62.5%). During a mean follow-up of 11.6 ± 6.7 years (1-32 years), no patient died or developed dilated cardiomyopathy (DCM). The long-term follow-up was uncomplicated in 127 children (90.1%). CONCLUSION: In this large multicentre study, the long-term outcome of congenital or childhood non-immune, isolated AV block was favourable, regardless of the patient's age at the time of diagnosis. No patient died or developed DCM, and pacemaker-related complications were few.

Total cavopulmonary connection (TCPC) has not been studied in adults. We investigated early and midterm morbidity and mortality in adults undergoing TCPC and assessed risk factors for mortality.Between June 1994 and October 2010, 30 adults (21.3 ± 5.5 years) underwent TCPC (extracardiac conduit). Twenty-two patients who had palliated single ventricles underwent TCPC completions and 8 patients underwent TCPC conversions. Preoperative and perioperative data were reviewed retrospectively.Six of 9 patients with preoperative atrial flutter or fibrillation or intraatrial reentry tachycardia were treated in the catheterization room. An aortic cross-clamp was necessary in 12 patients, and 16 TCPCs were fenestrated. Mean follow-up was 51 months (range, 4-198 months). Early mortality was 10%: 2 of 8 conversions and 1 of 22 completions. There was 1 late conversion death (at 56 months postoperatively). Postoperatively, 4 patients required pacemakers and 1 patient required long-term antiarrhythmic medication, but no heart transplantations were necessary. Risk factors for early mortality were arrhythmia (p = 0.02), aortic cross-clamp (p = 0.054), and extracorporeal circulation in hypothermia (p = 0.03). Risk factors for overall mortality were conversion (p = 0.047), absence of fenestration (p = 0.036), surgery before January 2006 (p = 0.036), aortic cross-clamp (p = 0.018), extracorporeal circulation in hypothermia (p = 0.008), and arrhythmia (p = 0.005). New York Heart Association functional class had improved at the last follow-up: preoperatively, 17 patients were in class II and 12 patients were in class III versus 18 patients in class I and 9 patients in class II postoperatively (p0.001). At the last clinical visit, systemic ventricular function was maintained, and no late supraventricular arrhythmia was found.Early and midterm TCPC results for adults are encouraging for completion but are disappointing for conversion. Identified risk factors for mortality should improve patient selection for TCPC.

International audience; Dilated Cardiomyopathy (DCM) is one of the leading causes of heart failure with high morbidity and mortality. More than 30 genes have been reported to cause DCM. To provide new insights into the pathophysiology of dilated cardiomyopathy, a mutational screening on 4 DCM-causing genes (MYH7, TNNT2, TNNI3 and LMNA) was performed in a cohort of 105 unrelated DCM (64 familial cases and 41 sporadic cases) using a High Resolution Melting (HRM)/sequencing strategy. Screening of a highly conserved arginine/serine (RS)-rich region in exon 9 of RBM20 was also performed. Nineteen different mutations were identified in 20 index patients (19%), including 10 novels. These included 8 LMNA variants in 9 (8.6%) probands, 5 TNNT2 variants in 5 probands (4.8%), 4 MYH7 variants in 3 probands (3.8%), 1 TNNI3 variant in 1 proband (0.9%), and 1 RBM20 variant in 1 proband (0.9%). One proband was double-heterozygous. LMNA mutations represent the most prevalent genetic DCM cause. Most patients carrying LMNA mutations exhibit conduction system defects and/or cardiac arrhythmias. Our study also showed than prevalence of mutations affecting TNNI3 or the (RS)-rich region of RBM20 is lower than 1%. The discovery of novel DCM mutations is crucial for clinical management of patients and their families because pre-symptomatic diagnosis is possible and precocious intervention could prevent or ameliorate the prognosis.

Risk factors and rates of reoperation, arrhythmias, systemic right ventricular dysfunction (RVD), and late death after a Senning procedure were investigated.One-hundred thirty-two patients underwent a Senning operation between 1977 and 2004 (105 simple and 27 complex transpositions of the great arteries). Mean follow-up time was 19.5 ± 6.6 years. Surviving patients were evaluated by transthoracic echocardiography and electrocardiography. Right ventricular function was assessed in 70 patients by isotopic ventriculography or magnetic resonance imaging.Operative and late mortality were 5.3% (7/132) and 9.6% (12/125), respectively. Nine patients were reoperated for left ventricular outflow tract obstruction or baffle stenosis. Survival rate was 91.5%, 91%, 89%, and 88% at 1, 5, 10, and 20 years, respectively. Probability of maintaining permanent sinus rhythm was 80%, 65%, 55%, and 44%. Twelve patients required pacemaker implantation. Probability of no supraventricular tachycardia, atrial flutter/fibrillation or ventricular tachycardia was 95.5%, 91.5%, 88%, and 75%, respectively. These parameters were similar for simple and complex transposition. Probability of right ventricular ejection fraction40% was 100% at 5 and 10 years, and 98% at 20 years for simple transposition, and 100%, 92%, and 58% for complex transposition. This difference was statistically significant. Risk factors for RVD were complex transposition (p0.001), body weight (p = 0.008), no cardioplegia (p0.001), and tricuspid valve regurgitation (p = 0.004).Senning procedure results in very good long-term survival out to 20 years. Both RVD and baffle stenosis were rare, but there was a concerning incidence of arrhythmia over time suggesting careful long-term surveillance.

Introduction Levosimendan (Lev) is a new calcium sensitizer and K-ATP channel opener. The documentation regarding this drug is one of the largest ever on the safety and efficacy of a new pharmacological agent in acute heart failure syndromes in adult population. Its use in paediatric is limited to successful weaning from biventricular mechanical support in case reports or small trials conducted in the immediate postoperative period. We report our experience of using (Lev) during refractory cardiogenic shock (RCS) in infants. Patients and Methods Four infants aged 2–24 months and suffering from hypokinetic dilated cardiomyopathy) were included in this study. All presented with uncontrolled RCS (LVEF Results Mean LVEF before and 8 days after administration of Lev significantly increase from 19.75% +/− 1.7 to 33 +/− 2.95 (p Conclusion During the refractory cardiogenic shock of the child with hypokinetic dilated cardiomyopathy, levosimendan may improve myocardial function allowing weaning of conventional inotropes and circulatory support. Re-injection may also be necessary. Randomized studies with larger number of patients are needed to confirm these very encouraging results.

IntroductionRight heart failure is a common feature in patients with repaired tetralogy of Fallot (TOF), right ventricular (RV) dysfunction and right bundle branch block (RBBB). Biventricular pacing (BVP) has been described as a potentially useful therapeutic in some cases. We aimed to investigate the clinical outcome and dyssynchrony echocardiographic characteristics of patients with repaired TOF and BVP.MethodsA systematic retrospective study of all of the patients of CHU de Bordeaux with repaired TOF and BVP was realized. Clinical NYHA status and exercise test performance were retrieved before and 6 months after BVP. All patients benefited from an echocardiography with dyssynchrony measures in spontaneous rhythm, RV pacing and BVP.Results10 patients (7 male, 36,6±13 years old) were retrieved from our database. Surgical repair had occurred at the age of 7,4±5,8 years. BVP was effective since 18±10 months.After 6 months of BVP were noted a significant improvement in NYHA class (1,3±0,4 vs 1,8±0.6, p = 0.05) and exercise test capacity (93±22W vs 78±14W, p

Objectives Senning procedure was performed for the first time in France at Bordeaux University Hospital in 1965. Follow up have come out focusing on failing systemic right ventricle and rhythmic complication. The aim of this work is to identify prognosis factors of reoperation, arrhythmias and right ventricular dysfunction. Methods This single institution study is a retrospective analysis of 132 patients, with simple (105 patients) and complex (27 patients) transposition of the great arteries, after a Senning procedure between 1977 and 2004. The mean follow-up time was 19.5 ± 6.6 years. Conventional follow up and testing were performed in the same unit. An isotopic ventriculography or MRI was done for 70 patients to investigate the systemic right ventricular function. Results Operative mortality was 5.3%. Late mortality was 9.6%. 9 patients underwent a single reoperation and one needs heart transplantation. Actuarial survival rate was 91.5%, 91%, 89% and 88% at respectively 1, 5, 10, and 20 years. There is no statistically difference between simple and complex transposition for actuarial survival rate, maintaining permanent sinus rhythm or arrhythmias occurrence. After 20 years of 98% of patients in simple transposition group have ejection fraction > 40% versus 58% in complex transposition (p Conclusion Imaging systemic right ventricular dysfunction (FEVD

t a e t o v i o c Introduction Among congenital heart defects causing cyanosis, tetralogy of Fallot (TOF) is the most common. Major progress has been made since 1954, when Lillehei performed the first surgical repair, such that most children operated today reach adulthood. The follow-up of adults presenting with various conenital heart diseases and management of the specific compliations they have are challenging. While, in the case of TOF, he shortand intermediate-term surgical results are generally xcellent, the longer term clinical course may be complicated y right heart failure, life-threatening ventricular tachyarrhythias, major impulse propagation disorders, and sudden eath. Right heart failure is due to the combined effects of pressure and volume overloads and of the myocardial lesions inflicted during and after the operation. Right bundle branch block (BBB) almost invariably develops after surgical repair of TOF. Furthermore, a wide QRS is prognostically important, and QRS duration and risk of developing ventricular arrhythmias are closely correlated. The hemodynamic consequences of right BBB might play a predominant role in the long-term clinical outcomes of these patients, and a relationship probably exists between electrical and mechanical dysfunction, as well as links between myocardial remodeling, rhythm disturbances, and electromechanical dyssynchrony. Depressed contractility and remodeling may be promoted by mechanical dyssynchrony, which, therefore, may be a desirable therapeutic target. Emulating studies of left ventricular (LV) stimulation in patients with left heart failure and left BBB, a few cases of resynchronization in patients with congenital

The purpose of this study was to assess the potential of late positive functional remodeling after percutaneous pulmonary valve implantation (PPVI) in right ventricular outflow tract dysfunction.PPVI has been shown to impact acutely on biventricular function and exercise performance, but the potential for further late functional remodeling remains unknown.Sixty-five patients with sustained hemodynamic effects of PPVI at 1 year were included. Patients were divided into 2 subgroups based on pre-procedural predominant pulmonary stenosis (PS) (n = 35) or predominant pulmonary regurgitation (PR) (n = 30). Data from magnetic resonance imaging and cardiopulmonary exercise testing were compared at 3 time points: before PPVI, within 1 month (early) and at 12 months (late) after PPVI.There was a significant decrease in right ventricle end-diastolic volume early after PPVI in both subgroups of patients. Right ventricle ejection fraction improved early only in the PS group (51 ± 11% vs. 58 ± 11% and 51 ± 12% vs. 50 ± 11%, p0.001 for PS, p = 0.13 for PR). Late after intervention, there were no further changes in magnetic resonance parameters in either group (right ventricle ejection fraction, 58 ± 11% in the PS group and 52 ± 11% in the PR group, p = 1.00 and p = 0.13, respectively). In the PS group at cardiopulmonary exercise testing, there was a significant improvement in peak oxygen uptake early (24 ± 8 ml/kg/min vs. 27 ± 9 ml/kg/min, p = 0.008), with no further significant change late (27 ± 9 ml/kg/min, p = 1.00). In the PR group, no significant changes in peak oxygen uptake from early to late could be demonstrated (25 ± 8 ml/kg/min vs. 25 ± 8 ml/kg/min vs. 26 ± 9 ml/kg/min, p = 0.48).In patients with a sustained hemodynamic result 1 year after PPVI, a prolonged phase of maintained cardiac function is observed. However, there is no evidence for further positive functional remodeling beyond the acute effects of PPVI.

Reliability and Reproducibility of QRS Duration. Background: A QRS >120 ms remains the recommended criterion for the selection of cardiac resynchronization therapy (CRT) candidates. However, the reproducibility of this measurement has not been studied thoroughly. Methods: QRS duration was measured by 3 experienced cardiologists and by automatic measurement on 228 electrocardiograms (ECGs) randomly collected from 188 subjects, including neonates, healthy adults, patients with complete and incomplete bundle branch block, and CRT candidates. All ECGs were recorded at a 25 mm/s sweep speed. Forty recordings were duplicated and 50 ECGs were recorded at both 25 and 50 mm/s. Results: Significant interobserver differences (P < 0.001) were found between each combination of paired observers, with an up to 50-ms absolute variability between cardiologists and low concordance with computerized measurements. Intraobserver absolute variability was also significant (P < 0.01) for the 3 observers. These significant differences persisted (P < 0.01) when focusing our interest on the ECGs in the 100–140 ms range (defined as at least one out of the 4 measures in this range). Considering the 120 ms limit, 22 (27.5%) ECGs were differently classified by at least one of the cardiologists. We observed similar interobserver differences between each combination of paired observers with a 50 mm/s sweep speed. Conclusion: Manual QRS duration measurements were associated with significant inter- and intraobserver variability and low concordance with computerized measurements. The measurement of QRS is, therefore, operator-dependent and a reevaluation of the measurement methods may be essential to develop clinical and investigative standards. (J Cardiovasc Electrophysiol, Vol. 21, pp. 890-892, August 2010)