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International audience; To investigate the feasibility and accuracy of cardiac multidetector computed tomography (MDCT) prosthesis sizing prior to ostium secundum atrial septal defect (ASD) percutaneous closure. Seventy consecutive patients were included in this retrospective bicentric study between May 2012 and June 2018. All underwent cardiac MDCT (primarily performed to rule out abnormal venous pulmonary return and coronary anomaly) and transesophageal echocardiography (TEE) before transcatheter closure: dimensions of the defect and peripheral rims were measured. Measurements of the defect obtained at TEE and MDCT were compared to prosthesis size. Our primary objective was the comparison of ASD maximal diameter obtained at MDCT (CT-Dmax) to prosthesis size. Intraclass correlation coefficient (ICC), Bland Altman plots and linear regression were calculated. Intra- and inter-observer agreements were calculated for MDCT defect measurements. Forty-three patients were finally included for defect measurements: 17 patients did not undergo transcatheter closure, and 10 had incomplete data. For CT-Dmax, ICC was 0.88 (CI 95% = [0.78-0.93]; p = 0.06); mean difference was - 0.8 ± 5.7 mm; regression linear equation was 0.9 × + 3.2 (p < 0.001). For maximal diameter at TEE versus prosthesis size, ICC was 0.46 (CI 95% = [0.21-0.61]; p = 0.003); mean difference was-6.0 ± 8.2 mm; regression linear equation was 0.91 × + 7.6 (p < 0.001). Intra- and inter-observer agreement for CT-Dmax were 0.97 (CI 95% = [0.95-0.98]) and 0.86 (CI 95% = [0.73-0.93]) respectively. MDCT is a reliable tool for sizing the defect of ostium secundum ASD, making it a complement or even an alternative to pre-procedural TEE.

Objectives This study sought to investigate patient intermediate-term outcomes after transcatheter pulmonary valve replacement (TPVR) with Edwards SAPIEN valve. Background The Edwards SAPIEN valve, initially designed for percutaneous aortic valve replacement, has been approved for TPVR in patients with dysfunctional right ventricular outflow tracts (RVOT), but only short-term follow-up has been reported. Methods From 2011 to 2016, 62 patients undergoing successful TPVR using the SAPIEN XT valve were consecutively included into the study. Primary efficacy and safety endpoints were defined as freedom from valve-reintervention and freedom from infective endocarditis at last follow-up, respectively. Results The primary efficacy outcome was met for 87.1% patients after a mean follow-up of 4.6 ± 1.8 years, corresponding to a freedom of reintervention at 5 years of 89% (95% CI 74.8-95.6%). Reinterventions were exclusively due to recurrent obstruction, no significant valvular regurgitation was observed. One case of infective endocarditis was reported, corresponding to a rate of 0.35% per patient-year (95% CI 0.01-2.00%). At 5 years, freedom from infective endocarditis was 98.4% (95% CI 89.1-99.8%). Six patients died or were transplanted due to advanced cardiac failure, without relationship with TPVR. In univariate analysis, reintervention was associated with young age, a smaller tube-graft, a higher pulmonary valve gradient after the procedure and a ratio of largest implanted stent diameter to invasive balloon conduit diameter over 1.35. Conclusions This study documents the mid-term safety and efficacy of the Edwards SAPIEN XT valve in patients with dysfunctional RVOT, and identifies a patient profile associated with an uncertain benefit-risk balance.

This paper aims to provide a paediatric cardiac computed tomography angiography expert panel consensus based on the opinions of experts from the Societe francaise d'imagerie cardiaque et vasculaire diagnostique et interventionnelle (SFICV) and the Filiale de cardiologie pediatrique congenitale (FCPC). This expert panel consensus includes recommendations for indications, patient preparation, computed tomography angiography radiation dose reduction techniques and postprocessing techniques. We think that to realize its full potential and to avoid pitfalls, cardiac computed tomography angiography in children with congenital heart disease requires training and experience. Moreover, paediatric cardiac computed tomography angiography protocols should be standardized to acquire optimal images in this population with the lowest radiation dose possible, to prevent unnecessary radiation exposure. We also provide a suggested structured report and a list of acquisition protocols and technical parameters in relation to specific vendors.

Background: Mitral valve prolapse (MVP) is a frequent disease that can be complicated by mitral regurgitation (MR), heart failure, arterial embolism, rhythm disorders, and death. Left ventricular (LV) replacement myocardial fibrosis, a marker of maladaptive remodeling, has been described in patients with MVP, but the implications of this finding remain scarcely explored. We aimed at assessing the prevalence, pathophysiological and prognostic significance of LV replacement myocardial fibrosis through late gadolinium enhancement (LGE) by cardiac magnetic resonance in patients with MVP. Methods: Four hundred patients (53±15 years of age, 55% male) with MVP (trace to severe MR by echocardiography) from 2 centers, who underwent a comprehensive echocardiography and LGE cardiac magnetic resonance, were included. Correlates of replacement myocardial fibrosis (LGE+), influence of MR degree, and ventricular arrhythmia were assessed. The primary outcome was a composite of cardiovascular events (cardiac death, heart failure, new-onset atrial fibrillation, arterial embolism, and life-threatening ventricular arrhythmia). Results: Replacement myocardial fibrosis (LGE+) was observed in 110 patients (28%; 91 with myocardial wall including 71 with basal inferolateral wall, 29 with papillary muscle). LGE+ prevalence was 13% in trace-mild MR, 28% in moderate MR, and 37% in severe MR, and was associated with specific features of mitral valve apparatus, more dilated LV and more frequent ventricular arrhythmias (45% versus 26%, P P =0.009) and moderate-severe MR (odds ratio, 2.28 [95% CI, 1.21–4.31], P =0.011). LGE+ was associated with worse 4-year cardiovascular event–free survival (49.6±11.7 in LGE+ versus 73.3±6.5% in LGE–, P P =0.002) were associated with poor outcome. Conclusions: LV replacement myocardial fibrosis is frequent in patients with MVP; is associated with mitral valve apparatus alteration, more dilated LV, MR grade, and ventricular arrhythmia; and is independently associated with cardiovascular events. These findings suggest an MVP-related myocardial disease. Last, cardiac magnetic resonance provides additional information to echocardiography in MVP.

Objectives This study sought to investigate patient intermediate-term outcomes after transcatheter pulmonary valve replacement (TPVR) with Edwards SAPIEN valve. Background The Edwards SAPIEN valve, initially designed for percutaneous aortic valve replacement, has been approved for TPVR in patients with dysfunctional right ventricular outflow tracts (RVOT), but only short-term follow-up has been reported. Methods From 2011 to 2016, 62 patients undergoing successful TPVR using the SAPIEN XT valve were consecutively included into the study. Primary efficacy and safety endpoints were defined as freedom from valve-reintervention and freedom from infective endocarditis at last follow-up, respectively. Results The primary efficacy outcome was met for 87,1% patients after a mean follow-up of 55 ± 21 months, corresponding to a freedom of reintervention at 5 years of 89% (95% CI 74.8–95.6%). Reinterventions were exclusively due to recurrent obstruction, no significant valvular insufficiency was observed. One case of infective endocarditis was reported, corresponding to a rate of 0.35% per patient-year (95% CI 0.01–2.00%). At 5 years freedom from infective endocarditis was 98.4% (95% CI 89.1–99.8%) ( Fig. 1 ). Six patients died or were transplanted due to advanced cardiac failure, without relationship with TPVR. In univariate analysis, reintervention was associated with young age, a smaller tube-graft, a higher pulmonary valve gradient after the procedure and a ratio of largest implanted stent diameter to invasive balloon conduit diameter over 1.35. Conclusions This study confirms the mid-term safety and efficacy of the Edwards SAPIEN XT valve in patients with dysfunctional RVOT, and identifies a patient profile associated with an uncertain benefit-risk balance.

The long-term prospective multi-centre nationwide (French) observational study FRANCISCO will provide new information on perimembranous ventricular septal defect with left ventricular overload but no pulmonary hypertension in children older than 1 year. Outcomes will be compared according to treatment strategy (watchful waiting, surgical closure, or percutaneous closure) and anatomic features of the defect. The results are expected to provide additional guidance about the optimal treatment of this specific population, which is unclear at present. Background The management of paediatric isolated perimembranous ventricular septal defect (pmVSD) with left ventricle (LV) volume overload but no pulmonary arterial hypertension (PAH) remains controversial. Three therapeutic approaches are considered: watchful waiting, surgical closure, and percutaneous closure. We aim to investigate the long-term outcomes of these patients according to anatomic pmVSD characteristics and treatment strategy. Methods The Filiale de Cardiologie Pediatrique et Congenitale (FCPC) designed the FRANCISCO registry, a long-term prospective nationwide multi-centre observational cohort study sponsored by the French Society of Cardiology, which enrolled, over 2 years (2018–2020), patients older than 1 year who had isolated pmVSD with LV volume overload. Prevalent complications related to pmVSD at baseline were exclusion criteria. Clinical, echocardiographic, and functional data will be collected at inclusion then after 1, 5, and 10 years. A core lab will analyse all baseline echocardiographic data to depict anatomical pmVSD features. The primary outcome is the 5-year incidence of cardiovascular events (infective endocarditis, sub-aortic stenosis, aortic regurgitation, right ventricular outflow tract stenosis, tricuspid regurgitation, PAH, arrhythmia, stroke, haemolysis, heart failure, or death from a cardiovascular event). We plan to enrol 200 patients, given the 10% estimated 5-year incidence of cardiovascular events with a 95% confidence interval of ±5%. Associations linking anatomical pmVSD features and treatment strategy to the incidence of complications will be assessed. Conclusions The FRANSCICO study will provide the long-term incidence of complications in patients older than 1 year with pmVSD and LV volume overload. The results are expected to improve guidance for treatment decisions.

International audience; The population of patients with congenital heart disease (CHD) is continuously increasing, and a significant proportion of these patients will experience arrhythmias because of the underlying congenital heart defect itself or as a consequence of interventional or surgical treatment. Arrhythmias are a leading cause of mortality, morbidity and impaired quality of life in adults with CHD. Arrhythmias may also occur in children with or without CHD. In light of the unique issues, challenges and considerations involved in managing arrhythmias in this growing, ageing and heterogeneous patient population and in children, it appears both timely and essential to critically appraise and synthesize optimal treatment strategies. The introduction of catheter ablation techniques has greatly improved the treatment of cardiac arrhythmias. However, catheter ablation in adults or children with CHD and in children without CHD is more technically demanding, potentially causing various complications, and thus requires a high level of expertise to maximize success rates and minimize complication rates. As French recommendations regarding required technical competence and equipment are lacking in this situation, the Working Group of Pacing and Electrophysiology of the French Society of Cardiology and the Affiliate Group of Paediatric and Adult Congenital Cardiology have decided to produce a common position paper compiled from expert opinions from cardiac electrophysiology and paediatric cardiology. The paper details the features of an interventional cardiac electrophysiology centre that are required for ablation procedures in adults with CHD and in children, the importance of being able to diagnose, monitor and manage complications associated with ablations in these patients and the supplemental hospital-based resources required, such as anaesthesia, surgical back-up, intensive care, haemodynamic assistance and imaging. Lastly, the need for quality evaluations and French registries of ablations in these populations is discussed. The purpose of this consensus statement is therefore to define optimal conditions for the delivery of invasive care regarding ablation of arrhythmias in adults with CHD and in children, and to provide expert and - when possible - evidence-based recommendations on best practice for catheter-based ablation procedures in these specific populations.

Purpose The purpose of this study was to assess the precision of four-dimensional (4D) phase-contrast magnetic resonance imaging (PCMRI) to measure mean flow and peak velocity (Vmax) in a pulsatile flow phantom and to test its sensitivity to spatial resolution and Venc. Material and methods The pulsatile flow phantom consisted of a straight tube connected to the systemic circulation of an experimental mock circulatory system. Four-dimensional-PCMR images were acquired using different spatial resolutions (minimum pixel size: 1.5 × 1.5 × 1.5 mm3) and velocity encoding sensitivities (up to three times Vmax). Mean flow and Vmax calculated from 4D-PCMRI were compared respectively to the reference phantom flow parameters and to Vmax obtained from two-dimensional (2D)-PCMRI. Results 4D-PCI measured mean flow with a precision of −0.04% to + 5.46%, but slightly underestimated Vmax when compared to 2D-PCMRI (differences ranging from −1.71% to −3.85%). 4D PCMRI mean flow measurement was influenced by spatial resolution (P Conclusion Using an experimental pulsatile flow model 4D-PCMRI is accurate to measure mean flow and Vmax with better results obtained with higher spatial resolution. We also show that Venc up to 3 times higher than Vmax may be used with no effect on these measurements.

Objectives The aim of this study was to describe and analyze data from patients treated in France with the Edwards SAPIEN transcatheter heart valve (Edwards Lifesciences LLC, Irvine, California) in the pulmonary position. Background The Edwards SAPIEN valve has recently been introduced for percutaneous pulmonary valve implantation (PPVI). Methods From April 2011 to May 2017, 71 patients undergoing PPVI were consecutively included. Results The median age at PPVI was 26.8 years (range 12.8 to 70.1 years). Primary underlying diagnoses were conotruncal malformations (common arterial trunk, tetralogy of Fallot and variants; n = 45), Ross procedure (n = 18), and other diagnoses (n = 8). PPVI indication was pure stenosis in 33.8% of patients, pure regurgitation in 28.1%, and mixed lesions in 38.1%. PPVI was successfully implemented in 68 patients (95.8%). Pre-stenting of the right ventricular outflow tract was performed in 70 patients (98.6%). Early major complications occurred in 4 subjects (5.6%), including 1 death, 1 coronary compression, and 2 pulmonary valve embolizations. Three of the 4 major complications occurred in the first 15 operated patients. No significant regurgitation was recorded after the procedure. Transpulmonary gradient was significantly reduced from 34.5 to 10.5 mm Hg (p Conclusions Early results with the Edwards SAPIEN valve in the pulmonary position demonstrate an ongoing high rate of procedural success.

Objectives This study sought to report the specific considerations and implications of percutaneous edge-to-edge repair in one case of severe systemic tricuspid regurgitation (STR). Background In congenitally-corrected transposition of great arteries (ccTGA) tricuspid valve results spontaneously in systemic position. In this situation, progressive STR and systemic right ventricular (SRV) dysfunction are frequently observed, leading ultimately to altered functional capacities and pulmonary edema. In this setting surgical valve repair or replacement is challenging given the underlying SRV failure, therefore heart transplant is usually preferred. However, recent studies have underlined the reliability of edge-to-edge repair for secondary severe tricuspid and mitral regurgitation. Few data are currently available concerning the percutaneous management of STR. Case management We report about the case of a 59 year-old man presenting with dyspnea and mild peripheral edema. He was known for non-operated ccTGA with SRV ejection fraction (EF) of 25%, and severe STR, defined as an effective regurgitant orifice area of 40 mm2. The patient was in theory eligible for heart transplant but a percutaneous approach was first attempted. The procedure was performed under general anesthesia, transesophageal echocardiography guidance and through a femoral vein route. A MitraClip XTR device (Abbott Vascular, Santa Clara, CA) was successfully implanted between the septal and the anterior leaflets, without any complication ( Fig. 1 ). After the procedure the STR improved dramatically from grade IV to I. After 2 years follow-up the patient is still asymptomatic and free from severe STR, concomitantly SRV EF improved up to 32% under optimal medical treatment. Conclusions Percutaneous edge-to-edge therapy of STR in ccTGA seems to be feasible, safe and effective to reduce regurgitation grade, to limit SRV dilatation and to improve patient symptoms.

Introduction Computed tomography angiography (CTA) is a reliable imaging tool to evaluate children congenital heart disease (CHD) but requires radiation exposure. This study aims to investigate image quality and radiation dose with a prospective electrocardiogram (ECG)-gated 80 multi-slice CTA, in 182 consecutive children examinations with CHD. Materials and methods In total, 182 consecutive examinations performed with a prospective ECG-gated 80 multi-slice CTA from March 2016 to December 2017 in Nantes university hospital were retrospectively analyzed. Radiation dose was assessed by dose length product (DLP) and effective dose (E). Objective quality image was assessed by contrast to noise ratio (CNR). Subjective quality image was assessed by two radiologists with a 10-point scale evaluating visualization of coronary arteries. Correlation between quality image, radiation dose, age, weight, heart rate was analyzed. Results DLP and E were 29.3 ± 15.5 mGy.cm and 0.6 ± 0.2 mSv, respectively. Agreement between radiologists in assessment of subjective image quality was 0.79. Subjective image quality score was significantly better (P Conclusion Prospective ECG-gated 80 multi-slice CTA is performing for the evaluation of children CHD with good image quality and low radiation dose. Visualization of small structures is better when age and weight increase, heart rate decreases and when radiation dose is more important. CNR evolves in the opposite side to subjective image quality score. It seems less appropriate to assess image quality from clinical viewpoint of the radiologist.

Cardiac catheterization has greatly contributed to the progress made in the management of congenital heart diseases (CHD). Initially used in diagnosis, it allowed the understanding of heart diseases, their anatomy and hemodynamics. Gradually, the development of interventional cardiology has played a major role in the management of these malformations (Patent ductus arteriosus [PDA] and atrial septal defect [ASD] closure, pulmonary dilatation, percutaneous pulmonary valve implantation…). The development of such technology was made possible through the concomitant development of imaging techniques: fluoroscopy, ultrasound, MRI and CT. Imaging should provide an accurate view of the lesions, the surrounding cardiac structures, as well as medical devices and catheters used. Here we address the field of fusion images. The principle of image fusion is based on the association by superposition of several imaging techniques: real-time fluoroscopy and slice imaging performed offline, or ultrasound imaging performed simultaneously. The goal is to improve the overall view of the organ, its surrounding structures and as the consequence to help the interventional cardiologist.

International audience; This article was designed to provide a pediatric cardiac computed tomography angiography (CCTA) expert panel consensus based on opinions of experts of the Société Française d'Imagerie Cardiaque et Vasculaire diagnostique et interventionnelle (SFICV) and of the Filiale de Cardiologie Pédiatrique Congénitale (FCPC). This expert panel consensus includes recommendations for indications, patient preparation, CTA radiation dose reduction techniques, and post-processing techniques. The consensus was based on data from available literature (original papers, reviews and guidelines) and on opinions of a group of specialists with extensive experience in the use of CT imaging in congenital heart disease. In order to reach high potential and avoid pitfalls, CCTA in children with congenital heart disease requires training and experience. Moreover, pediatric cardiac CCTA protocols should be standardized to acquire optimal images in this population with the lowest radiation dose possible to prevent unnecessary radiation exposure. We also provided a suggested structured report and a list of acquisition protocols and technical parameters in relation to specific vendors.

Aims Several data suggest that acute myocarditis could be related to genetic variants involved in familial cardiomyopathies, particularly arrhythmogenic cardiomyopathy, but the management of patients with acute myocarditis and their families regarding their risk for having an associated inherited cardiomyopathy is unclear. Methods and results Families with at least one individual with a documented episode of acute myocarditis and at least one individual with a cardiomyopathy or a history of sudden death were included in the study. Comprehensive pedigree, including genetic testing, and history of these families were analysed. Six families were included. Genetic analysis revealed a variant in desmosomal proteins genes in all the probands [five in desmoplakin (DSP) gene and one in desmoglein 2 gene]. In the five families identified with a DSP variant, genetic testing was triggered by the association of an acute myocarditis with a single case of apparently isolated dilated cardiomyopathy or sudden death. Familial screening identified 28 DSP variant carriers; 39% had an arrhythmogenic left ventricular (LV) cardiomyopathy phenotype. Familial histories of sudden death were frequent, and a remarkable phenotype of isolated LV late gadolinium enhancement on contrast‐enhanced cardiac magnetic resonance without any other structural abnormality was found in 38% of asymptomatic mutation carriers. None of the DSP variant carriers had imaging characteristics of right ventricle involvement meeting current Task Force criteria for arrhythmogenic right ventricular cardiomyopathy. Conclusions Comprehensive familial screening including genetic testing in case of acute myocarditis associated with a family history of cardiomyopathy or sudden death revealed unknown or misdiagnosed arrhythmogenic variant carriers with left‐dominant phenotypes that frequently evade arrhythmogenic right ventricular cardiomyopathy Task Force criteria. In view of our results, acute myocarditis should be considered as an additional criterion for arrhythmogenic cardiomyopathy, and genetic testing should be advised in patients who experience acute myocarditis and have a family history of cardiomyopathy or sudden death.

To demonstrate that the use of step-and-shoot (SAS) mode in paediatric cardiac CT angiography (CCTA) is possible at heart rates (HR) greater than 65 bpm, allowing low-dose acquisition with single-source 64-slices CT.We retrospectively included 125 paediatric patients (0-6 years). CCTA was performed with SAS at diastolic phase in 31 patients (group D, HR 65 bpm), at systolic phase in 45 patients (group S, HR ≥ 65 bpm) and with non-gated mode in 49 patients (group NG). Effective dose (ED) and image quality using a 3-grade scoring scale (1, excellent; 2, moderate; 3, insufficient) of group S were compared with group D for coronary examinations and group NG for entire thorax vascular anatomy.For coronary indications, median ED was 0.6 mSv in group D versus 0.9 mSv in group S (p 0.01). For whole thorax indications, median ED was 2.7 mSv in group NG versus 1.1 mSv in group S (p 0.001). The mean image quality score was (1.4 ± 0.6) points in group D, (1.4 ± 0.7) in group S for coronary indications (p = 0.9), (1.3 ± 0.6) in group S for whole thorax indications and (2.0 ± 0.0) in group NG (p 0.001).SAS mode is feasible in children with HR greater than 65 bpm allowing low-dose CCTA. It provided comparable image quality in systole, compared to diastole. SAS at the systolic phase provided better image quality with less radiation dose compared to non-gated scans for whole thorax examinations.

Objectives The short-term efficacy and safety of transcatheter closure (TCC) for the management of coronary artery fistulas (CAFs) was investigated in pediatric patients. Background CAFS are rare with potentially severe complications and their management is still a matter of debate. Percutaneous closure appears to be the treatment of choice in anatomically suitable vessels but limited data are available in the pediatric population. Methods This retrospective, observational, multicenter, national study included patients under 16 years of age who underwent TCC of a congenital CAF. Patients with additional congenital heart defect were excluded. Results 61 patients (36 girls, 25 boys) with a median age of 0.6 year [0–15.4] at diagnosis and 3.9 years [0–16] at procedure were included. The CAF was large in 48 patients (79%); it was distal in 23 (38%) and proximal in 22 (36%). Most patients (77%) were asymptomatic at diagnosis. Clinical signs of congestive heart failure were present in seven patients (11%). Perioperative complications included three cases of ST elevation myocardial infarction (exclusively during attempted closure of a distal CAF), three devices migrations, and one case of leg ischemia. One patient died after surgical closure of a large distal CAF that could not be closed by TCC. Follow-up data were collected for 43 patients (70%) for a median of 91 days [0–4,824]. The Kaplan-Meyer estimate for complete occlusion at 2 years was 73 ± 7.6%. Conclusion TCC in the pediatric population appears to be effective and associated with few complications. © 2015 Wiley Periodicals, Inc.

The aim of this study was to describe and analyze data from patients treated in France with the Edwards SAPIEN transcatheter heart valve (Edwards Lifesciences LLC, Irvine, California) in the pulmonary position.The Edwards SAPIEN valve has recently been introduced for percutaneous pulmonary valve implantation (PPVI).From April 2011 to May 2017, 71 patients undergoing PPVI were consecutively included.The median age at PPVI was 26.8 years (range 12.8 to 70.1 years). Primary underlying diagnoses were conotruncal malformations (common arterial trunk, tetralogy of Fallot and variants; n = 45), Ross procedure (n = 18), and other diagnoses (n = 8). PPVI indication was pure stenosis in 33.8% of patients, pure regurgitation in 28.1%, and mixed lesions in 38.1%. PPVI was successfully implemented in 68 patients (95.8%). Pre-stenting of the right ventricular outflow tract was performed in 70 patients (98.6%). Early major complications occurred in 4 subjects (5.6%), including 1 death, 1 coronary compression, and 2 pulmonary valve embolizations. Three of the 4 major complications occurred in the first 15 operated patients. No significant regurgitation was recorded after the procedure. Transpulmonary gradient was significantly reduced from 34.5 to 10.5 mm Hg (p 0.0001). No patient died during a 1-month follow-up period. At 1-year follow-up, the death rate was 2.9%, and 3 patients had undergone surgical reintervention (44%).Early results with the Edwards SAPIEN valve in the pulmonary position demonstrate an ongoing high rate of procedural success.

Objective Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart disease usually diagnosed during the first months of life. Without surgical treatment, ALCAPA carries a high mortality risk and disease presentation in adulthood is rare. We describe the diagnosis and management of patients presenting with ALCAPA in adulthood. Methods This multicenter French nationwide retrospective study included adult patients diagnosed from 1980 to 2014. Results 11 adult patients (mean age: 38 ± 1 7 years) were analyzed. All patients were symptomatic, presenting with chest pain, palpitations, heart failure, and/or syncope. Electrocardiogram was abnormal in 8 (73%) patients. Echocardiogram showed a mildly depressed left ventricular ejection fraction of 50 ± 13%, kinetic abnormalities in 5 (45%) patients and significant mitral regurgitation in 8 (73%) patients. Coronary angiography was performed in 10 (91%) patients and confirmed the diagnosis. Computerized tomography-scan, magnetic resonance imaging and myocardial scintigraphy were performed when deemed necessary. Ten patients underwent reconstructive surgery but one patient was not operated because of age. Four patients experienced post-operative complications including cardiogenic shock, heart failure, renal failure, and/or additional surgery. After a median follow-up of 2.5 years, all 10 operated patients were alive and asymptomatic and the non-operated patient had died at the age of 70 from syncope related to ventricular tachycardia. Conclusions ALCAPA may be diagnosed in adults. Although complications may occur post-operatively, long-term outcome is favorable in adult patients undergoing surgical correction. Surgery should be discussed as first-line therapy in adults with ALCAPA.

Cardiac catheterization has greatly contributed to the progress made in the management of congenital heart diseases (CHD). Initially used in diagnosis, it allowed the understanding of heart diseases, their anatomy and hemodynamics. Gradually, the development of interventional cardiology has played a major role in the management of these malformations (Patent ductus arteriosus [PDA] and atrial septal defect [ASD] closure, pulmonary dilatation, percutaneous pulmonary valve implantation…). The development of such technology was made possible through the concomitant development of imaging techniques: fluoroscopy, ultrasound, MRI and CT. Imaging should provide an accurate view of the lesions, the surrounding cardiac structures, as well as medical devices and catheters used. Here we address the field of fusion images. The principle of image fusion is based on the association by superposition of several imaging techniques: real-time fluoroscopy and slice imaging performed offline, or ultrasound imaging performed simultaneously. The goal is to improve the overall view of the organ, its surrounding structures and as the consequence to help the interventional cardiologist.

Background Coronary angiography is more complex in patients with coronary artery bypass grafts ( CABG ). Image fusion is a new technology that allows the overlay of a computed tomography ( CT ) three‐dimension (3D) model with fluoroscopic images in real time. Methods and Results This single‐center prospective study included 66 previous CABG patients undergoing coronary and bypass graft angiography. Image fusion coronary angiographies (fusion group, 20 patients) were compared to conventional coronary angiographies (control group, 46 patients). The fusion group included patients for whom a previous chest CT scan with contrast was available. For patients in this group, aorta and CABG were reconstructed in 3D from CT acquisitions and merged in real time with fluoroscopic images. The following parameters were compared: time needed to localize the CABG ; procedure duration; air kerma ( AK ); dose area product ( DAP ); and volume of contrast media injected. Results are expressed as median. There were no significant differences between the 2 groups in patient demographics and procedure characteristics (access site, number of bypass to be found, and interventional cardiologist's experience). The time to localize CABG was significantly shorter in the fusion group (7.3 versus 12.4 minutes; P =0.002), as well as the procedure duration (20.6 versus 25.6 minutes; P= 0.002), AK (610 versus 814 mGy; P= 0.02), DAP (4390 versus 5922.5 cGy·cm 2 ; P =0.02), and volume of iodinated contrast media (85 versus 116 cc; P =0.002). Conclusions 3D image fusion improves the CABG detection in coronary angiography and reduces the time necessary to localize CABG , total procedure time duration, radiation exposure, and volume of contrast media.

SummaryCardiac catheterization has contributed to the progress made in the management of patients with congenital heart disease (CHD). First, it allowed clarification of the diagnostic assessment of CHD, by offering a better understanding of normal cardiac physiology and the pathophysiology and anatomy of complex malformations. Then, it became an alternative to surgery and a major component of the therapeutic approach for some CHD lesions. Nowadays, techniques have evolved and cardiac catheterization is widely used to percutaneously close intracardiac shunts, to relieve obstructive valvar or vessel lesions, and for transcatheter valve replacement. Accurate imaging is mandatory to guide these procedures. Cardiac imaging during catheterization of CHD must provide accurate images of lesions, surrounding cardiac structures, medical devices and tools used to deliver them. Cardiac imaging has to be ‘real-time’ with an excellent temporal resolution to ensure ‘eyes–hands’ synchronization and ‘device–target area’ accurate positioning. In this comprehensive review, we provide an overview of conventional cardiac imaging tools used in the catheterization laboratory in daily practice, as well as the effect of recent evolution and future imaging modalities.

Introduction Outflow tract obstruction is thought to be involved in left ventricular (LV) remodeling in sarcomeric hypertrophic cardiomyopathy (HCM). Objective To determine the influence of obstruction to biological parameters of remodeling and global longitudinal strain (GLS) in HCM. Material and methods Patients with HCM who accepted to participate to this study were included. Transthoracic echocardiography at rest and exercise with GLS measurement was carried out. Blood was sampled before and early after exercise. Patients were divided into 3 groups according to the presence of an obstruction at rest (OHCM), effort (Latent obstruction: LOHCM) or none (HCM). Results We included 40 HCM patients, 7 OHCM, 19 LOHCM and 14 NOHCM. Troponin-T and NTproBNP levels at rest were significantly higher in OHCM compared to NOHCM (19.5 ± 10 vs 10 ± 5.8 ng/L, P = 0.017 and 1184 ± 389 vs 520 ± 388 ng/L, P = 0.005). Troponin-T and NTproBNP levels at rest were correlated with the outflow tract peak gradient ( r = 0.49, P = 0.001 and r = 0.41, P = 0.005, respectively) and with the alteration of GLS ( r = 0.51, P = 0.001 and r = 0.46, P = 0.003, respectively). GLS at rest was significantly more altered in OHCM. After exercise ST2, IL6, GDF15, Troponin-T and NTproBNP increased significantly in OHCM + LOHCM while only NTproBNP increased in NOHCM. In multivariate analysis, postexercise Troponin-T ( β = 0.57, P Fig. 1 ). Conclusion In HCM, biological parameters of remodeling are correlated to obstruction and GLS. GLS is itself correlated with the outflow peak gradient. Exercise in obstructive forms of HCM induces a significant increase in multiple biological parameters of remodeling. Troponin-T level is a strong predictor of GLS alteration both at rest and exercise.

The short-term efficacy and safety of transcatheter closure (TCC) for the management of coronary artery fistulas (CAFs) was investigated in pediatric patients.CAFS are rare with potentially severe complications and their management is still a matter of debate. Percutaneous closure appears to be the treatment of choice in anatomically suitable vessels but limited data are available in the pediatric population.This retrospective, observational, multicenter, national study included patients under 16 years of age who underwent TCC of a congenital CAF. Patients with additional congenital heart defect were excluded.61 patients (36 girls, 25 boys) with a median age of 0.6 year [0-15.4] at diagnosis and 3.9 years [0-16] at procedure were included. The CAF was large in 48 patients (79%); it was distal in 23 (38%) and proximal in 22 (36%). Most patients (77%) were asymptomatic at diagnosis. Clinical signs of congestive heart failure were present in seven patients (11%). Perioperative complications included three cases of ST elevation myocardial infarction (exclusively during attempted closure of a distal CAF), three devices migrations, and one case of leg ischemia. One patient died after surgical closure of a large distal CAF that could not be closed by TCC. Follow-up data were collected for 43 patients (70%) for a median of 91 days [0-4,824]. The Kaplan-Meyer estimate for complete occlusion at 2 years was 73 ± 7.6%.TCC in the pediatric population appears to be effective and associated with few complications.

A 44-year-old man was referred to our hospital 3 years after heart transplantation for systematic endo-myocardial biopsies. The initial physical examination and transthoracic echocardiography were normal. The right internal jugular vein was punctured under local anaesthesia. Three heart biopsies of the right ventricular septum were obtained. One hour after this …

The coronary angiography among patients with coronary artery bypass graft (CABG) are more complex procedures with more X-ray exposure, longer compared to conventional coronary angiography. Image fusion is a new technology allowing a fusion in real-time of a 3D model obtained by reconstruction of computed tomography (CT) images with fluoroscopic images. To evaluate the contribution of image fusion in coronary angiography for the detection of CABG. A prospective study over 9 months, single center comparing the use of image fusion to conventional coronary angiography. All patients coming for angiographic control of CABG were included in the study. Patients who had previously (for another indication) a chest CT with injection of contrast media were the fusion image group, patients without prior injected chest CT constituted the control group. For patients in the fusion group, the aorta, the CABG, sternotomy sutures, the bronchi and a vertebra were reconstructed in 3D from CT acquisitions and these images were incorporated in real-time on fluoroscopic images. The following parameters were reported in both groups and compared: search time of CABG, procedure time, X-Ray exposure and the amount of contrast. 59 patients were included, 13 in the fusion image group, 46 in the control group. There were not significant differences between the 2 groups in terms of general characteristics patients and procedures. The search time of CABG was significantly shorter in the fusion group of images in the control group (16.04 min±13.2 min vs 6.4 min±4.7 min, p=0.002), as well as procedure time (31.5 min±15.2 min vs 19.4 min± 6.8 min, p = 0.007), fluoroscopy time (14.76 min±8.3 min vs 9.3 min±4.2 min, p = 0.04), Air KERMA (822.9 mGy±475.5 mGy vs 569.1 mGy±242.4 mGy, p = 0.01) and amount of iodinated contrast (121 cc ± 43.5 cc vs 88.2 cc±30.8 cc, p = 0.01). 3D reconstruction of CABG from CT with real-time fusion on coronary angiography images may shorten the search time of CABG, procedure time, X-Ray exposure and the use of iodinated contrast compared to standard coronary angiography for diagnostic purposes in patients with CABG. Download full-size image Abstract 0498 – Figure: 3D picture merged with the real-time fluoroscopic

International audience; BACKGROUND: Preclinical studies and pilot clinical trials have shown that high-dose erythropoietin (EPO) reduces infarct size in acute myocardial infarction. We investigated whether a single high-dose of EPO administered immediately after reperfusion in patients with ST-segment elevation myocardial infarction (STEMI) would limit infarct size. METHODS: A total of 110 patients undergoing successful primary coronary intervention for a first STEMI was randomized to receive standard care either alone (n = 57) or combined with intravenous administration of 1,000 U/kg of epoetin β immediately after reperfusion (n = 53). The primary end point was infarct size assessed by gadolinium-enhanced cardiac magnetic resonance after 3 months. Secondary end points included left ventricular (LV) volume and function at 5-day and 3-month follow-up, incidence of microvascular obstruction (MVO), and safety. RESULTS: Erythropoietin significantly decreased the incidence of MVO (43.4% vs 65.3% in the control group, P = .03) and reduced LV volume, mass, and function impairment at 5-day follow-up (all P < .05). After 3 months, median infarct size (interquartile range) was 17.5 g (7.6-26.1 g) in the EPO group and 16.0 g (9.4-28.2 g) in the control group (P = .64); LV mass, volume, and function were not significantly different between the 2 groups. The same number of major adverse cardiac events occurred in both groups. CONCLUSIONS: Single high-dose EPO administered immediately after successful reperfusion in patients with STEMI did not reduce infarct size at 3-month follow-up. However, this regimen decreased the incidence of MVO and was associated with transient favorable effects on LV volume and function.

Un homme de 67 ans, en bon etat general, sans antecedent particulier hormis un accident de la voie publique (AVP) severe a l’âge de 20 ans, consulte aux urgences ophtalmologiques pour cecite brutale de l’œil gauche. Le diagnostic retenu est une thrombose de l’artere centrale de la retine. Le patient beneficie d’une echographie cardiaque qui met en evidence une masse d’allure kystique (centre hypoechogene et corticale hyperechogene) situee a proximite de l’anneau mitral, juste en arriere de la valve posterieure. La valve mitrale est continente. Le bilan biologique est normal. Les serologies hydatidoses sont negatives. Un scanner thoracoabdominal confirme la presence d’une masse calcifiee au niveau du sillon auriculoventriculaire gauche. Une IRM cardiaque est realisee. Celle-ci met en evidence une masse en isosignal T1, hypersignal T2, non rehaussee apres injection de gadolinium.

Un homme de 72 ans beneficie d’une echographie cardiaque dans le bilan d’un trouble visuel d’apparition brutale qui s’avere etre une thrombose de la veine centrale de la retine. L’echographie met en evidence une masse intraventriculaire gauche de petite taille implantee a proximite du troisieme segment du feuillet posterieur de la valve mitrale. Cette masse n’a pas pu etre responsable de l’accident visuel recent, mais une indication operatoire est retenue compte tenu du risque emboligene. Un coroscanner est programme dans le bilan preoperatoire (fig. 1). Il confirme la masse pediculee intraventriculaire gauche et montre un reseau coronaire sain.

A l’occasion d’un episode viral, on decouvre, chez une jeune fille de 12 ans, un souffle cardiaque a l’auscultation. Une echographie cardiaque est realisee et montre une masse intraventriculaire droite, situee dans l’infundibulum, mobile a chaque systole. En IRM, cette masse unique est pediculee, implantee sur le septum interventriculaire, et obstrue partiellement l’infundibulum a chaque systole.

We describe an atypical myxoma in the right ventricular outflow tract of a 12-year old girl. After identification using echocardiography, we assessed its precise location and attachments by magnetic resonance imaging. Subsequent to surgical removal, histology confirmed the myxomatous nature of the tumour.

SummaryComputed tomography (CT) technology is acquiring a key role in the diagnostic process of complex cardiac congenital anomalies. Recent advances and improvements in spatial and temporal resolution and radiation dose are encouraging the use of CT scanning in children. Paediatric cardiologists should have a good knowledge of the potential of CT techniques and their limitations to plan and properly perform CT examinations without forgetting radiation concerns. In this paper, we will discuss the principal indications for CT scans in newborns and children in our clinical practice. We will also outline the most-used strategies for dose reduction. Basic knowledge about the various CT techniques is crucial, not only to perform, but also to interpret CT results, thus helping the medical and surgical management of patients.

Introduction The link between acute myocarditis (AM) and familial cardiomyopathies (CM) remains unclear. Purpose To assess the clinical significance of AM in families with CM. Methods We describe the pedigree of 6 families with at least one case of AM and a familial history of CM or sudden death (SD). AM was defined as an infarct-like clinical presentation with normal coronary arteries and myocardial inflammation (MI) documented by cardiac magnetic resonance (CMR), or as an autopsy proven AM. Results In family 1 to 5, genetic testing was triggered by the association of a documented case of AM with an index case of CM or early SD. In this setting, all genetic testing revealed a mutation in the desmoplakin (DSP) gene. In family 1, patient II.1 (15 y.o) was diagnosed with AM 6 months after his 12 y.o sister's SD. In family 2, patient II.4 (17 y.o) was diagnosed with AM. His mother had a DCM, with a CMR revealing the presence of MI. In family 3, patient IV.3 (22 y.o) died suddenly from an AM, attested by post-mortem autopsy. Her aunt had a DCM. In family 4, patient II.4 (41 y.o) had an AM, progressing toward a DCM. Her mother had died suddenly at the age of 39, and her niece had a DCM. In family 5, patient V.16 (9 y.o) presented 4 recurrent episodes of AM. Her cousin's mother had a DCM. In family 6, patient IV.3 had 3 episodes of AM, his father had previously been diagnosed with an arrythmogenic right ventricular cardiomyopathy (ARVC) with a desmoglein 2 (DSG2) mutation ( Table 1 , Fig 1). Conclusion AM is strongly associated to desmosomal mutations when a familial history of cardiomyopathy is present, particularly in DSP gene. In these families, DCM phenotype and SD are frequent, and a notable proportion of isolated LGE suggestive of myocardial fibrosis is present in asymptomatic relatives. These results highlight the need for a comprehensive familial screening in case of AM.

Coronary angiography are more complex in patients with coronary artery bypass grafts (CABG). These procedures are longer leading to more X-ray exposure, compared to conventional coronary angiography. Image fusion is a new technology allowing a fusion in real-time of a 3D model obtained by