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Introduction Patients with systemic right ventricle (mostly congenitally-corrected transposition of the great arteries or transposition of the great arteries corrected by atrial switch) commonly develop significant systemic tricuspid valve regurgitation and systemic right ventricular dysfunction in adulthood, both of which presenting a therapeutic dilemma for the care team. Percutaneous edge-to-edge repair could be a alternative to surgery. Methods Seven high-risk surgical patients with severe systemic tricuspid regurgitation undergoing a percutaneous repair were included between July 2020 and March 2021. Our study is a prospective analysis of short and mid-term clinical, biological, echocardiographic and MRI outcomes with an expected minimum follow-up of 2 years. Results The first data tend to show a significant benefit of the repair on clinical status (dyspnea severity, quality of life, test exercise performance), a decrease of BNP level and an improvement of tricuspid regurgitation and right ventricular volume and function measured by echocardiography and MRI. Besides, the rate of failure and complications seems to be very low. Discussion Percutaneous edge-to-edge repair of systemic tricuspid regurgitation might be a safe and effective therapeutic option in high-risk surgical adult patients.

Background Pulmonary homografts (PH) are used as a first-line treatment for surgical right ventricular outflow tract (RVOT) reconstruction in patients with congenital heart disease (CHD). Despite a better freedom from reintervention than prosthetic conduits, PH are not spared from failure and cases of early dysfunction are regularly described. Aims The aim of this study was to assess the rate of early PH dysfunction in patients of the Bordeaux University Hospital and to identify associated risk factors. Methods A monocentric retrospective study was conducted in children and adults with CHD and PH implantation for RVOT reconstruction. Clinical and echocardiographic data were collected during follow-up. PH dysfunction was defined as a peak of gradient greater than 50 mmHg and/or as pulmonary regurgitation greater than moderate. Early dysfunction was defined as occurring within two years postoperatively. Primary endpoint was the early PH dysfunction rate at 2 years. The dysfunction-free survival curve was calculated according to the Kaplan-Meier method. A logistic regression with univariate then multivariate analysis was performed to identify risk factors for early dysfunction. Results Between January 2002 and November 2020, 112 PH were implanted in 110 patients and 11 cases of homograft dysfunction were reported during the follow-up, including 9 cases of early dysfunction. The rate of early dysfunction was 9.4 [3.3–15.1] % and freedom from reintervention was 94.6 [90.0–99.0] % at two years. The only independent risk factor identified by the multivariate analysis was duration of extracorporeal circulation (P = 0.007) but the length of stay in intensive care unit (P = 0.088) and the initial maximum pulmonary transvalvular gradient (P = 0.06) were also close to significance in the multivariate analysis. Conclusion Although PH provide a durable substitute for RVOT reconstruction, a significant proportion of patients presents early PH dysfunction and requires premature reintervention. An inflammatory mechanism is suspected but dedicated studies should be conducted to validate this hypothesis.

Background Percutaneous paravalvular leak closure (PVLc) has emerged as an alternative to surgery. It remains a technically challenging procedure. We aim to compare outcomes after mitral and aortic PVLc. Methods We analyzed data from PVLc procedures performed over the 2 first years of inclusion in the FFPP (Fermeture de Fuite Periprothetique) study, a prospective observational industry-independent cohort study which started in January 2017. Results We analyzed 147 PVLc (99 mitral–48 aortic), performed in 127 patients (1 procedure in 109 patients, 2 in 16 and 3 in 2) included in 22 centers among 3 countries (France, Poland and Belgium). Age (69 ± 10 versus 69 ± 11 yo), left ventricular ejection fraction (56 versus 51%), Euroscore2 (9 versus 7.6) and rate of mechanical valve (56.7% versus 41.7%, P = 0.1) were not significantly different among mitral and aortic groups. All patients had heart failure and/or hemolytic anemia. Hemolysis was more common in mitral PVL (62 versus 43%, P = 0.04). A mean of 1.4 (min 1, max 3) and 1.8 (min 1 max 5) devices were respectively required for technically successful aortic and mitral PVLc. No complication was reported in procedures with failure of device implantation. Aortic PVLc were faster than mitral PVLc (1h18 versus 2h20), with a trend towards a higher rate of technical success (96% versus 87%, P = 0.1) and fewer rate of major adverse events (worsening hemolysis, stroke, life threatening events and deaths; 2% versus 14%, P = 0.02). At 1 month follow-up, events were reported in 2% of patients (blood transfusion for hemolysis) versus 26.2% (deaths 3.3%; hemolysis 14.8%; heart failure 3.3%; heart failure and hemolysis 4.9%) after successful aortic and mitral PVLc respectively, P = 0.6. Conclusion Mitral PVLc is a more complex procedure than aortic PVLc, with a lower success rate and a higher risk of peri-procedural and one-month severe adverse events. Longer follow-up data are expected and will be available from this on-going study.

Introduction We are facing an exponential request for PFO closure since the last recommendations of the French societies of vascular neurology and cardiology. Because of the lack of anesthesiologists resources in public institution to perform transoesophagial echocardiography (TEE)-guided procedures, we are performing PFO closure under intracardiac ultrasound guidance (ICE). Given the cost of this equipment, we conducted a medico-economic study to compare TOE and ICE guided procedure. Methods All the patients who underwent PFO closure between January and June 2018 were included. The overall cost of each procedure were analysed including hospital stay fees, procedure and device cost and anesthesia costs when applicable, and was compared with reimbursement data extracted from medicalized information system program. Results Fifty-eight patients stays were analyzed. Thirsty-six procedures were performed under local anesthesia with ICE guidance versus 24 under GA with TEE guidance. The overall cost for the FPO closure under GA was 2995.59 € (including an overall anesthesia procedure cost of 908.37 €) versus 4048.42 € for non-sedated procedure with ICE guidance. Associated costs excluding the price of the PFO device itself was 31 € for procedures under GA and 1531 € for non-sedated procedures (including the cost of the ICE catheter). Stays level of severity was level 1 severity in 74%. Average length of stay was to 2.74 days. The average income for the hospital was 2970.91 € for level 1 severity stays and 5164.52 € for level 2 severity stays. For level 1 severity stays, the use of an ICE catheter leads to a deficit of 917.34 €/stay, while a procedure under GA leads to a benefit ok 71.38 €/stay. Conclusion Decreasing the length of stay without compromising patients’ safety and defining the appropriate cost of ICE catheters with the industry and health authorities would permit to find safe and sustainable strategies for PFO closure.

Introduction 3D-printing have demonstrated improving in LAAC device sizing, a reduce procedure time and number of prostheses employed per patient. Otherwise, LAAC procedures may be at risk if unfavourable anatomy and performed in frailly patients. Method Three hundred patients are expected to be included in LAA-Print French registry. Procedure time, success rate, number of prosthesis per procedure and complications will be compared to patients without 3D printing simulation. Cardiac CT prior to LAAO. Industrial Manufacture of 3D-printed LAA models including all LA cavity. Simulation on the basis of 3D printing models using specifics sheaths and prosthesis (Boston TM and AbbottR). Check of transseptal puncture site in order to avoid a misalignment. Risk ranking for failure of procedure: low/moderate/high based on 3D printing simulation. Aim To improve the efficiency and safety of LAA closure. Results Nb of center: 15, 51 patients since January 2018. Procedure risk was classified: low risk in 60%, moderate 20% and high-risk 20%. Outcomes in low risk group: no prosthesis changes, operating time 35 (27–56 min), no leaks > 3 mm. In the group of moderated or high-risk: procedure failure occurred in 50%. Incidence of leaks > 5 mm was 40% and device misalignment occurred in 70%. The operating time was significantly prolonged 75 min (45–102). The rate of device change occurred in 60%. Factors that critically impacted the LAAO procedure's success were: retroversion of the LAA,marked elliptical shape of LAA ostium, protuberant pulmonary ridge, marked flat chicken wing shape, depth. Conclusion 3D printing simulation: leads to enhanced communication between all key players. Allows a better transseptal puncture planning. Permits a tailored selection of the appropriate device. We identified a group at risk according to LA anatomy. In this group, 3D printing may accurately guide the procedure by carefully checking the trans septal puncture site and/or using specific catheter shape.

Background Left and right ventricular (LV and RV) remodeling in repaired tetralogy of Fallot (TOF) is poorly understood. Objectives To identify correlates of focal scar and diffuse fibrosis in patients with history of TOF repair by using cardiac magnetic resonance (CMR). Methods Patients with prior TOF repair underwent CMR including cine imaging to assess ventricular volumes and ejection fraction (EF), T1 mapping to assess LV and RV diffuse fibrosis, and high resolution late gadolinium-enhanced (LGE) imaging to quantify scar size. Structural imaging data were related to clinical characteristics and functional imaging markers. In 40 patients, cine and T1 mapping results were compared to age- and sex-matched controls. Results In total, 103 patients were enrolled (age 28 ± 15 years, 36% women), including 36 with prior PV replacement. Compared to controls, TOF patients showed lower LV and RVEF and higher RV volume, RV wall thickness, and native T1 and ECV values on both ventricles. Scar size related to LVEF and RVEF while LV and RV native T1 related to RV dilatation. On multivariable analysis, scar size and LV native T1 were independent correlates of ventricular arrhythmia. Patients with history of PV replacement showed larger scar on RV outflow tract but LV and RV native T1 were shorter ( Fig. 1 ). Conclusions Focal scar and biventricular diffuse fibrosis are detected on CMR after TOF repair. Scar size relates to systolic dysfunction, and diffuse fibrosis to RV dilatation. Both may be implicated in ventricular arrhythmias. The finding of shorter T1 after PV replacement suggests that diffuse fibrosis may reverse with therapy.

Backgrounds Congestive hepatopathy usually appears and develops slowly after a Fontan operation, often without obvious clinical features, but it may lead to life-threatening complications. Objective We aim to assess the potential usefulness of the liver stiffness (LS) in the longitudinal follow up of Fontan patients. Material and method Patients were prospectively evaluated using clinical examination, laboratory tests, echocardiography and LS using transient elastography (TF). This work up was made annually or in case of clinical complication. Results Forty-six patients (22.1 ± 8,1 years of age and 9.7 ± 6.5 years post-Fontan) were enrolled. Mean time between first and last work up was 3.27 ± 1.9 years. During this period clinical complication occurred in 12/46 (26%) including 6 arrhythmias, 2 cirrhosis, 1 steato-hepatitis, 3 exudative enteropathies and 2 venous-collateral. Mean LS at baseline was 14 ± 7.4 kPa. LS was significantly higher in patient with complications compared to those who were free from any complication (17.2 ± 7.7 vs. 13.8 ± 5.9 kPa, P = 0.019). No significant change in stiffness was also observed with age (P = 0.73), time since Fontan (P = 0.64), presence fenestration (15 ± 6.8 vs. 15.1 ± 6.7 kPa, P = 0.82) or ventricular morphology (15 ± 13.1 vs. 16 ± 14.1 vs. 10.9 ± 11.6 kPa, P = 0.09). Conclusion Fibroscan appears to be a good tool for the non-invasive follow-up of FP. Indeed, a significate elevation of the LS is associated with the occurrence of complications.

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 paroxymal 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 TTE parameters in all patients. Results Sixty-three patients (mean age 73±9 years) were included. 38% (n=24) was in sinus rythm at baseline and 55% (n=35) in permanent AF. Patients in SR at baseline and permanent AF at 3 months were exclued (n=4, 7%). The mean CHA2DS2-VASc score was 4,3±1,3. The procedure was successful in all patients.There was no significant difference in the functionnal status (p=0,74) and BNP (155,6 ± 107 baseline vs 155±150,6 pg/ml at 3 months ; p=0,85). Left atrial volume excluding the LAA (14±55ml baseline vs 144±50 cm3 at 3 months; p=0,30) showed no significant change after 3 months in the overall population, neither in the SR (99,7 ± 19,1 baseline vs 103,8±21 cm3 at 3 months ; p=0,32) or the permanent AF groups (173,2±54 baseline vs 171,7±48,6 cm3 at 3 months ; p=0,59).MV peak E-wave (84,2 ± 22,7 ; 86,7±26 cm/s, p=0,62) and A-wave velocities (65,4±14,4 ; 68,5 ± 22,2 cm/s, p=0,66) did not differ between baseline and three months. E/E’ ratio was higher in the overall population and there was a trend to higher E/E’ ratio in the SR group. Conclusions There's no evidence for early LA remodeling after LAAPC. LV pressure loading might be slighlty more elevated after LAA closure suggesting the potential reservoir role of the LAA. Further studies are warranted to confirm these prelimary results. The author hereby declares no conflict of interest

BackgroundAlthough coarctation of the aorta could be suspected prenatally, the diagnosis is not certain before birth and remains difficult in the neonatal period especially when ductus arteriosus is open. 2D strain, a recent echocardiographic tool to assess myocardial performance, allows the early detection of abnormal myocardial deformation.ObjectiveThe aim of this study was to assess the accuracy of left ventricular 2D strain to predict aortic coarctation in neonates with patent ductus arteriosus.Patients and MethodsThis was a single centre prospective study. Neonates with patent ductus arteriosus and prenatal/postnatal suspicion of isthmic coarctation were included. Left ventricular (LV) 2D strain was performed for each patient. Patients were divided into three groups: those who developed coarctation (group 1, n=9), those who had normal aorta after ductal closure (group 2, n=10), and a control group of healthy neonates with patent ductus arteriosus (group 3, n=20).ResultsThe median age of gestation was 38+4 weeks of amenorrhea and the median birth weight was 3.088kg. The inter observer agreement was good for the assessment of LV 2D strain. Although radial strain was significantly decreased in group 1 (17.76 vs 40.19 in group 2, p

Background After congenital heart surgery, delayed sternal closure (DSC) can be used electively to aid in hemodynamic and respiratory stability in the initial postoperative period. Risk factors for DSC have been previously determined for neonates. The purpose of our study was to determine risk factors that may predict the need for DSC in children Patients and methods We retrospectively reviewed records of children Results Cardiopulmonary bypass (CPB) time was significantly longer in children who had DSC (189 min ± 68 versus 130 min ± 44, P Conclusion Small age at surgery, long CPB time and altered preoperative condition are highly at risk for DSC in children

Background Costello syndrome (CS) and Cardiofaciocutaneous syndrome (CFCS) are both RASopathies, a group of developmental disorders caused by a mutation in genes encoding in the RAS/MAPK pathway. RASopathies are prone to cardiac malformations and sudden cardiac death. Description of electrocardiographic findings in these patients is rare. The objective of this study was to describe electrocardiogram and correlation with echocardiographic findings in a cohort of CS and CFC. Method A 12-lead electrocardiogram (ECG) and a trans-thoracic echocardiography (TTE) were performed for all patients during the same period in July 2016, and were analyzed by two different cardiologists. Results Twenty-four patients were included, 14 with CFCS and 10 with CS. The mean age was 10.3 ± 7.0 years. In 14 patients (58%), at least one abnormality was noticed on the electrocardiogram: axis deviation in 11 patients (46%); atrial hypertrophy in 5 patients (21%); left ventricular hypertrophy in 2 patients (8%); incomplete bundle-branch block in 3 patients (13%) and a significant Q-wave in 5 patients (21%). All patients with left axis deviation and/or left ventricular hypertrophy at ECG had a hypertrophic cardiomyopathy (sub-aortic septal, apical or concentric hypertrophy) on TTE. The patients with a significant Q wave (among them 2 had an extreme right axis deviation) had in fact a minimal hypertrophic cardiomyopathy (septal thickness Conclusion Significant Q-wave or extreme right axis deviation on the ECG may suggest the potential presence of myocardial fibrosis. Detection of fibrosis by MRI may play an important role for the risk stratification of sudden cardiac death in these patients.

Background Liver stiffness (LS) assessed by Transient elastography (TE) is known to be elevated in patients with a Fontan circulation and is considered as a promising tool to evaluate hepatopathy in these patients. However, the longitudinal evolution of this parameter has never been studied. Methods We conducted a prospective ongoing study since 2007 including all patients with a Fontan circulation who underwent an annual TE assessment in addition to their systematic work-up and/or in case of a clinical complication. Results In this ongoing study, 20 patients were screened with TE and 17 had at least two LS evaluations. Median age at the first TE was 21 years [range 4–32], median duration between the Fontan surgery and the first TE was 4 years [range 1–12], and 6.6 years [range 3–15] at the last TE screening. The median LS at the first evaluation was 12.8 kPA [range 6–47] and 12.4 kPA [range 6–27] at the last evaluation. No significant modification of LS values was observed in our cohort, except for 3 who developed a failing Fontan and are currently waiting for cardiac transplant. Conclusion Liver stiffness assessed by TE is stable over time in Fontan patients but significantly increases in patients with a failing Fontan. This tool might be useful in the longitudinal follow-up of these patients for anticipating the decrease of cardiac status and to evaluate non-invasively the effects of targeted therapies before heart transplant.

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

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.

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.

Background Although ventricular septal defect (VSD) is one of the most common congenital heart disease, this defect is not so frequent in adults because of early surgery and spontaneous closure. Complications such as aortic insufficiency, infectious endocarditis or supraventricular arrhythmia are classically described. Purpose The main objective of this study was to determine the outcome of adult patients who were operated for VSD after childhood. Methods We conducted an observational, retrospective and multicenter study including patients operated for VSD from the age of 18 years, over a 15-year period (2000–2015). Overall survival and occurrence of adverse events were analyzed. Results Seventy-three patients were included. Mean age was 37.47 ± 2.1 years with a male predominance (62%). Type 2 VSDs were the most common type encountered. After surgery, overall survival was 92% with a mean follow-up of 6 years. Excluding events occurring during the early postoperative period, overall survival was significantly better in patients operated for isolated VSD than in patients operated for VSD associated with other cardiac injuries (95% versus 92%, P = 0.02). Early postoperative complications were found in 14% of patients (principally atrioventricular block requiring early postoperative permanent pacing). Aortic cross clamp and cardiopulmonary bypass time were significantly lower in patients operated for isolated VSD than in patients with associated lesion. Conclusion As morbi-mortality is low, surgical closure of VSD in adults is not a high-risk intervention. Survival of patients with isolated VSD is better than that of patients with associated cardiac lesions. This last finding advocates for early surgery before the apparition of complications.

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

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.

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

BackgroundAnimal studies showed that levosimendan protects cardiomyocytes against apoptotic cell death in acute viral myocarditis. In addition to its inotropic properties, levosimendan seems to prevent the detrimental effects of oxidative stress and to limit cardiomyocyte loss.AimTo analyze the outcome of children who received levosimendan for acute or fulminant viral myocarditis.Patients and methodsRetrospective single center study covering the period 2007- 2013. All children (55% (100% for the acute myocarditis group). 1 patient had heart transplantation 18 months later.ConlusionIn our experience, levosimendan was efficient for the inotropic support of acute and fulminant viral myocarditis. We believe that its cardioprotective effect enabled us to limit the use of mechanical assistance (only 11% of the fulminant forms) and allowed a recovering of the cardiac function for the majority of the patients. Although further clinical studies are needed to confirm these data, levosimendan should be systematically considered for the treatment of these critical patients.

Background The PROTECT AF trial previously demonstrated that left atrial appendage closure (LAAC) was non inferior to warfarin in patients with nonvalvular atrial fibrillation (NVAF). However, this trial included patients eligible for anticoagulation therapy who received warfarin for 6 weeks after device implantation. The purpose of the present study was to assess the safety and efficacy of LAAC for stroke patients with NVAF and contraindication for anticoagulation. Methods Consecutive patients with a previous ischemic or hemorrhagic stroke, NVAF and contraindication for anticoagulation underwent LAAC with the Amplatzer Cardiac Plug device between July 2010 and July 2013 in a French university hospital. Follow-up included clinical evaluation at 3 and 12 months, and a cardiac computed tomography (CT) at 3 months. Single-antiplatelet therapy was prescribed after the procedure for a minimum of 3 months and stopped if the control cardiac CT demonstrated complete LAA exclusion. RESULTS: 26 patients (age 73±8 years) were included. The mean CHA2DS2-VASc and HAS-BLED scores were 4±1.5 and 4±0.8, respectively. The main contraindications for anticoagulation were: intracerebral hemorrhage while receiving anticoagulation (62%), ischemic stroke with large hemorrhagic transformation (15%) and probable cerebral amyloid angiopathy (8%). The procedure was successful in 100%. Procedure-related complications were serious pericardial effusion (3.8%) and femoral bleeding (7.7%). During a mean follow-up of 8.6 (3-16) months, ischemic stroke occurred in 2 patients (7.7%), after antiplatelet therapy was stopped for one of them. One patient died of an intracranial hemorrhage. Conclusion LAAC followed by a single antiplatelet therapy could be a reasonable alternative for stroke patients with NVAF and contraindication for anticoagulation. Lifelong rather than short-term single antiplatelet therapy should be prescribed after the procedure for patients at high cardio-vascular risk.

Background Patients with corrected tetralogy of Fallot (TF) often suffer from symptomatic right ventricular failure always associated with right bundle branch block (RBBB) on resting ECG. Aims Our objective was to evaluate the mid-term safety and efficacy of cardiac resynchronization (CRT) in this population. Methods We collected retrospectively every adverse events due to biventricular pacing of patients with corrected tetralogy of Fallot implanted with CRT system in our department. We also compared clinical datas (NYHA score), stress tests, ECG, echocardiographic results before implantation, at 6 months of follow-up and at the last evaluation. Results From August 2005 to Septembre 2009, 9 patients were implanted with CRT system, 6 (66,7%) with transvenous leads and 3 (33,4%) with epicardic leads, mostly composed of men (6, 66,7%) with a mean age of 34,2±14,5 years, 4 (44,5%) had an Implantable Cardioverter Defibrillator (ICD) function. During a median follow-up of 65 months [50-80], no main adverse event was reported, we had 1 atrial lead dislogement, 3 (33%) lead disfunctions causing only 1 (11%) resynchronisation failure and 1 (11%) intermittent phrenic nerve stimulation. CRT were associated with lower NHYA score (1,4±0,52 then 1,3±0,8 vs 2±0,4, p Conclusion CRT in corrected tetralogy of Fallot anad right ventricule failure is safe and seems to be associated with an improvement of patient’s functional status. This first study on CRT in this population should be confirmed by a large prospective randomised multicentric clinical trial.

Introduction Transient elastography is a rapid, noninvasive and reproducible approach to assess liver fibrosis by measuring liver stiffness (LS). However, because the liver is enveloped by a capsule, any variation in parenchymal fluid content could theoretically affect LS. LS has been correlated to central venous pressure (CVP) in an animal model. We aimed to determine the correlation between LS and CVP in children and adults with congenital heart disease. Methods In this ongoing prospective study, all patients referred for right heart catheterization were included. Measurements of mean right atrial pressure were obtained under general anesthesia (Fi02=21%) using an Optitorque 5 French catheter. The patients underwent 10 LS measurements (median value taken as representive) by transient elastography (Fibroscanâ, Echosens, France) within the 24 hours before catheterization. The results of LS are expressed in kilopascals (kPa). Results Eleven children (mean age=8±6 yo, 54% male) and 6 adults (mean age=34±17 yo, 66% male) have been included so far. Catheterism indications were pulmonary angioplasty (n=5), Melody valve implantation (n=1), fenestration occlusion after a Fontan procedure (n=1) and pre operative assessement of a complex congenital heart defect (n=10). Mean right atrial pressure was 9,1±3,9 mmHg and mean LS was 8,4±4,2 kPa. Correlation between LS and mean right atrial pressure was excellent for these first 17 patients (r=0,87). Conclusion Liver stiffness is a new, rapid and reliable method to evaluate CVP in patients with congenital heart disease. This non invasive parameter could potentially be usefull for patients in whom CVP play a key role, especially in patients with a Fontan circulation.

Introduction Syncopes are frequent in the pediatric population. The majority is benign but, for a minority of children, a cardiac disease is the underlying cause and has to be recognized as it can be fatal. Syncope units developped in adult population have demonstrated major improvement in diagnostic process, hospitalisation reduction time, with favourable long-term outcome. We report our experience of syncope management in chidren and adolescents through a dedicated syncope unit. Methods In this ongoing prospective study, we enrolled 31 consecutive patients (13±3 yo, 65% male) between Jan 2011 and Mar 2012, referred for loss of consciousness (LOC) in a dedicated pediatric syncope unit involving a pediatric cardiologist, a nurse, a physiotherapist and a psychologist. All patients underwent initial evaluation including medical history assessment, physical examination, 12-lead ECG and echocardiography to exclude non-cardiogenic syncopes. If initial assessement was abnormal, they underwent targeted tests that differed according to suspected aetiology. Patients with neurocardiogenic syncope underwent specific physiotherapy training and a consultation with a psychologist. Results The most common cause of LOC was neurocardiogenic syncope − 22 patients (71%), psychogenic LOC − 7 patients (23%). One patient (3%) had a long QT syndrome and received beta blocker therapy. One patient had typical epileptic seizure and was transferred to neurologic departement. Mean hospitalization duration was 0,9±0,5 days. Head-up tilt testing was positive in 62% neurocardiogenic syncope. Echocardiograms and exercise tests were not contributive for diagnosis. After a mean follow up of 9±4 months, including physiotherapist and/or phychologist specific care, syncope reccurence occured in 4 patients (12%). Conclusion Syncope unit in pediatric population with dedicated team improves diagnostic process, reduces hospitalisation and decreases syncope recurrence when adapted follow up is proposed.

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 has been described as a potentially useful therapeutic in some cases. We aimed to investigate the changes in electrical epicardic activation using non invasive electrocardiographic imaging (ECGI) in different pacing configurations.Methodseight adults with TOF, clinical signs of RV dysfunction and RBBB underwent implantation of a BVP device. Electrocardiographic imaging (ECGI) was applied during intrinsic activation (IA), right ventricular (RV) stimulation and BVP.Results1) In spontaneous rhythm, the activation maps were compatible with a RBBB aspect with major dispersion of the activation of the right ventricle (RV activation time 114±38 ms).2) In RV pacing, right ventricular activation time is shorter but left ventricular activation is significantly delayed (110±27 vs 72±22ms, p

IntroductionPatients with repaired tetralogy of Fallot (TOF) represent a new category of patients referred to electrophysiology laboratory for ventricular arrhythmia (VA) mapping and ablation. Different anatomical regions have been identified as potentially responsible for reentry: ventricular septal defect (VSD) patch, surgical incisions, right ventricular outflow tract (RVOT) patch. We aimed to investigate electrophysiological substrate responsible for potential VA in patients with repaired TOF.MethodsAll patients with repaired TOF referred to the CHU de Bordeaux for VA evaluation from January 2008 to april 2010 underwent right ventricular (RV) 3D mapping. Sinus activation and voltage mapping was then performed before VA induction ± ablation.Results7 patients (4 male, 42,5±12 years old) underwent RV mapping during VA evaluation. Surgical repair of TOF had been realized 36±11 years before the procedure. All patients displayed a right bundle branch block on 12 lead electrocardiogram.Sinus rhythm RV activation begins in all patients in the septum and then activates the RV centrifugally with a zone of slow conduction with a double potential (100±30ms) going from the tricuspid annulus (TA) to the RVOT. Voltage maps (figure) show systematically the same pattern of a zone of low voltage (< 1.5mV) due to the VSD repair close to the RVOT scar area. This area fits with the slow conduction area. In the 2 patients with sustained ventricular tachycardia (VT), critical isthmus was located in this area.ConclusionsSpecific activation and voltage pattern was found in these Fallot patients. In the 2 patients with sustained VT, the critical isthmus was found between VSD repair patch and RVOT scar.

PurposePulmonary valve replacement (PVR) is commonly performed in aduts with repaired TOF to avoid late complications related to severe pulmonary regurgitation. However, few data are available concerning perioperative complications. The aim of this study was to evaluate the perioperative complications and to determine predictive factors of the Low Cardiac Output Syndrome (LCOS) in patients undergoing PVR.Methods and Results30 patients with TOF who underwent PVR between 2008 and 2009 were retrospectivelly enrolled. Mean age at valve surgery was 29.5 (range: 6.5–56.5). PVR was conducted with beating heart using a normothermic CPB (77+/−25min) in 16 pts. 14 pts underwent additional surgery requiring aortic cross clamp (CPB mean time was 113+/−21min). Survival rate was 97% at 90 days. Post operative complications were uncommon (VT in 6%, Mechanical Ventilation> 24 hours in 6%, renal dysfunction in 10%) except for the LCOS (46%). Prolonged CPB duration> 80min (p

BackgroundThe morphology of the LAA is extremely heterogeneous. Acurate sizing of the landing zone is crucial for the optimal choice of the device diameter. Cardiac CT has been shown to provide acurate measurement of the LAA. TOE and selective LAA angiography can provide discordant results during the procedure, and there is no consensus about the most reliable imaging modality.AimCompare the sizing of the LAA using a multimodality imaging approach.Methods and resultsWe retrospectively included 45 patients (aged 67.6±6.2 years) who underwent LAA occlusion using the Amplatzer® cardiac plug (ACP). Mean diameter of the landing zone was determined using the different imaging modalities: MPR for cardiac-CT and 3D TOE, multiple angle view at 0, 45, 90 and 120° using 2D TOE, and monoplanar projection (RAO30° cranial 20°) for angiography. High intra and inter observer reproductibility were found for diameter (r=0.93 and 0.87, p