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Background: Arrhythmogenic left ventricular cardiomyopathy (ALVC) is characterized by fibrofatty myocardial replacement demonstrated on cardiac magnetic resonance by late gadolinium enhancement (LGE) mainly involving the subepicardium. The aims of this study were to describe the layer-specific strain (LSS) echocardiography phenotype of ALVC and to compare it with LGE features., Methods: All consecutive ALVC pathogenic genetic variant carriers and noncarrier relatives were separated into four prespecified groups (overt ALVC [group 1], isolated LGE [group 2], pathogenic genetic variant carrier without ALVC phenotype [group 3], and no genetic variant carrier [group 4]) and studied accordingly using cardiac magnetic resonance and LSS echocardiography., Results: Eighty-five individuals were included. Endocardial global longitudinal strain (GLS)-epicardial GLS (GLSepi) gradient was altered predominantly in group 1, illustrating transmural strain alteration in overt ALVC (3.8 ± 1.1 in group 1, 4.3 ± 2.2 in group 2, 5.2 ± 1.2 in group 3, and 5.4 ± 1.6 in group 4; P = .0017), whereas GLSepi was impaired predominantly in group 2 (endocardial GLS and GLSepi were 15.0 ± 4.1% and 11.2 ± 3.3%, respectively, in group 1; 20.5 ± 2.8% and 16.2 ± 5.5% in group 2; 23.4 ± 3.3% and 18.2 ± 2.7% in group 3; and 24.6 ± 2.8% and 19.2 ± 1.9% in group 4; P < .0001 for all). GLSepi was able to detect subepicardial LGE in genetic variant carriers without overt ALVC with an area under curve of 0.84 (95% CI, 0.73-0.95). However, segmental epicardial and endocardial strain behaved similarly and showed comparable diagnostic values for segmental LGE detection (areas under the curve, 0.72; [95% CI, 0.69-0.76] and 0.73 [95% CI, 0.70-0.76], respectively, P = .40)., Conclusions: LSS alteration in ALVC progresses from the epicardium to the endocardium along with disease severity. Irrespective of LSS analysis, which did not provide incremental diagnostic value for the detection and localization of LGE, strain echocardiography was shown to be a potential surrogate marker of LGE, including in apparently healthy individuals with isolated LV fibrosis., (Copyright © 2024 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.)

Aims: Aortic valve calcification (AVC) of surgical valve bioprostheses (BPs) has been poorly explored. We aimed to evaluate in vivo and ex vivo BP AVCs and its prognosis value., Methods and Results: Between 2011 and 2019, AVC was assessed using in vivo computed tomography (CT) in 361 patients who had undergone surgical valve replacement 6.4 ± 4.3 years earlier. Ex vivo CT scans were performed for 37 explanted BPs. The in vivo CT scans were interpretable for 342 patients (19 patients [5.2%] were excluded). These patients were 77.2 ± 9.1 years old, and 64.3% were male. Mean in vivo AVC was 307 ± 500 Agatston units (AU). The AVC was 562 ± 570 AU for the 183 (53.5%) patients with structural valve degeneration (SVD) and 13 ± 43 AU for those without SVD (P < 0.0001). In vivo and ex vivo AVCs were strongly correlated (r = 0.88, P < 0.0001). An in vivo AVC > 100 AU (n = 147, 43%) had a specificity of 96% for diagnosing Stage 2-3 SVD (area under the curve = 0.92). Patients with AVC > 100 AU had a worse outcome compared with those with AVC ≤ 100 AU (n = 195). In multivariable analysis, AVC was a predictor of overall mortality (hazard ratio [HR] and 95% confidence interval = 1.16 [1.04-1.29]; P = 0.006), cardiovascular mortality (HR = 1.22 [1.04-1.43]; P = 0.013), cardiovascular events (HR = 1.28 [1.16-1.41]; P < 0.0001), and re-intervention (HR = 1.15 [1.06-1.25]; P < 0.0001). After adjustment for Stage 2-3 SVD diagnosis, AVC remained a predictor of overall mortality (HR = 1.20 [1.04-1.39]; P = 0.015) and cardiovascular events (HR = 1.25 [1.09-1.43]; P = 0.001)., Conclusion: CT scan is a reliable tool to assess BP leaflet calcification. An AVC > 100 AU is tightly associated with SVD and it is a strong predictor of overall mortality and cardiovascular events., Competing Interests: Conflict of interest: T.L.T. and J.-Christ R. received a basic research grant from Abbott-St Jude company dedicated to a mitral valve prolapse project. The other authors have no disclosure., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.)

Purpose: The purpose of this study was to evaluate the percentage of coronary angiography that can be securely avoided by the interpretation of coronary arteries on pre transcatheter aortic valve implantation CT (TAVI-CT), using CT images obtained with deep-learning reconstruction and motion correction algorithms., Material and Method: All consecutive patients who underwent TAVI-CT and coronary angiography, from December 2021 to July 2022 were screened for inclusion in the study. Patients who had previous coronary artery revascularization or who did not undergo TAVI were excluded. All TAVI-CT examinations were obtained using deep-learning reconstruction and motion correction algorithms. On TAVI-CT examinations, quality and stenosis of coronary artery were analyzed retrospectively. When insufficient image quality and/or when diagnosis or doubt of one significant coronary artery stenosis, patients were considered as having possible coronary artery stenosis. The results of coronary angiography were used as the standard of reference for significant CAS., Results: A total of 206 patients (92 men; mean age, 80.6 years) were included; of these 27/206 (13%) had significant coronary artery stenosis on coronary angiography and were referred for potential revascularization. Sensitivity, specificity, negative predictive value, positive predictive value, and accuracy of TAVI-CT to identify patients requiring coronary artery revascularization was 100% (95% confidence interval [CI]: 87.2-100%), 100% (95% CI: 96.3-100%), 54% (95% CI: 46.6-61.6), 25% (95% CI: 17.0-34.0%) and 60% (95% CI: 53.1-66.9%) respectively. Intra- and inter observer variability was substantial agreement for quality and decision to recommend coronary angiography. Mean reading time was 2 ± 1.2 (standard deviation) min (range: 1-5 min). Overall, TAVI-CT could potentially rule out indication for revascularization for 97 patients (47%)., Conclusion: Analysis of coronary artery on TAVI-CT using deep-learning reconstruction and motion correction algorithms can potentially safely avoid coronary angiography in 47% of patients., Competing Interests: Declaration of Competing Interest The authors have no conflicts of interest related to this work to declare., (Copyright © 2023 Société française de radiologie. Published by Elsevier Masson SAS. All rights reserved.)

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., (© 2021 Wiley Periodicals LLC.)

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 Congénitale (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.

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 <0.0001). In trace-mild MR, despite the absence of significant volume overload, abnormal LV dilatation was observed in 16% of patients and ventricular arrhythmia in 25%. Correlates of LGE+ in multivariable analysis were LV mass (odds ratio, 1.01 [95% CI, 1.002-1.017], 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 <0.0001). In a stepwise multivariable Cox model, MR volume and LGE+ (hazard ratio, 2.6 [1.4-4.9], 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.

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., (© 2020 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of the European Society of Cardiology.)

This paper aims to provide a paediatric cardiac computed tomography angiography expert panel consensus based on the opinions of experts from the Société française d'imagerie cardiaque et vasculaire diagnostique et interventionnelle (SFICV) and the Filiale de cardiologie pédiatrique congénitale (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., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

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., (Copyright © 2020 Société française de radiologie. Published by Elsevier Masson SAS. All rights reserved.)

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 (V max ) 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: 1.5×1.5×1.5mm 3 ) and velocity encoding sensitivities (up to three times V max ). Mean flow and V max calculated from 4D-PCMRI were compared respectively to the reference phantom flow parameters and to V max obtained from two-dimensional (2D)-PCMRI., Results: 4D-PCI measured mean flow with a precision of -0.04% to+5.46%, but slightly underestimated V max when compared to 2D-PCMRI (differences ranging from -1.71% to -3.85%). 4D PCMRI mean flow measurement was influenced by spatial resolution (P<0.001) with better results obtained with smaller voxel size. There was no effect of Venc on mean flow measurement. Regarding V max , neither spatial resolution nor Venc did influence the precision of the measurement., Conclusion: Using an experimental pulsatile flow model 4D-PCMRI is accurate to measure mean flow and V max with better results obtained with higher spatial resolution. We also show that Venc up to 3 times higher than V max may be used with no effect on these measurements., (Copyright © 2018 Soci showét showé françaises de radiologie. Published by Elsevier Masson SAS. All rights reserved.)

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 < 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%)., Conclusions: Early results with the Edwards SAPIEN valve in the pulmonary position demonstrate an ongoing high rate of procedural success., (Copyright © 2018 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objective: 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., Methods: 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., Results: 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)., Conclusion: 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., (Copyright © 2018 Associazione Italiana di Fisica Medica. Published by Elsevier Ltd. All rights reserved.)

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., (© 2016 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley Blackwell.)

Computed 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., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

Cardiac 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., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

Unlabelled: Renovascular hypertension accounts for 5-10 % of hypertension cases in children; there is currently no consensus on treatment. Here, we report on our clinical experience with this disease and outline the different pathways in which to investigate it. We report retrospectively on ten children diagnosed with renovascular hypertension at the University Hospital of Nantes from 2001 to 2012. The main findings were obtained by fortuitous screening of children aged 2 months to 14 years old with neurofibromatosis (n = 2) and fibromuscular dysplasia (n = 8). The hypertension was always severe yet asymptomatic. Lesions were complicated in nine out of ten cases and included bilateral, multiple, mid-aortic syndrome and aneurysm. Doppler ultrasound associated with computed tomography allowed for a precise diagnosis in seven out of ten cases. Where ambiguities persisted, they were highlighted by arteriography, the gold standard investigation. Medical treatment was insufficient, leading to invasive procedures in nine out of ten children: 2 nephrectomies, 2 autotransplantations, and 21 repetitive percutaneous transluminal angioplasties. After invasive procedures, blood pressure control improved in four cases and was resolved in three., Conclusion: Arteriography remains to be the gold standard technique for renovascular hypertension in children and can be combined with angioplasty when medical treatment is rendered obsolete. The role of computed tomography is controversial. Despite the heterogeneity of the children studied, we present a general medical and therapeutic management pathway for the treatment of this disease.

Background: Percutaneous mitral valve repair (MVR) using the MitraClip(®) is a new option for severe mitral regurgitation (MR)., Aim: To describe initial French experience regarding short-term and mid-term safety and efficacy., Methods: A multicentre cohort reported experience of percutaneous MVR using the MitraClip(®) in French centres from December 2010 to September 2012. All patients were judged inoperable or at high surgical risk. Short-term and mid-term safety and efficacy results are presented., Results: Sixty-two patients (72.7±11.4years; 71.7% men; 81.0% New York Heart Association [NYHA] class III or IV; logistic Euroscore 18.7±13.1%; 93.3% MR≥grade 3; 73.8% secondary MR) underwent percutaneous MVR using the MitraClip(®) under general anaesthesia. Procedural success was 95.2% (83.1% of patients received one clip; 16.9% received two clips). At discharge, 88.2% of implanted patients had a residual MR≤grade 2. Transthoracic echocardiography showed a significant decrease in ejection fraction (39.9±14.8% pre vs. 36.2±14.3% post), end-diastolic diameter (63.8±10.6mm vs. 61.4±12.3mm, respectively) and systolic pulmonary pressure (52.1±13.9mmHg vs. 44.7±10.9mmHg, respectively). The in-hospital mortality rate was 3.2%. The survival rate at 6-month follow-up was estimated at 83.1%, with 90.9% of patients in NYHA class I or II and residual MR≤grade 2 in 80% of cases., Conclusion: This initial French experience, despite being in its learning phase, showed promising results in patients considered ineligible for surgery, as observed in more experienced centres. Randomized studies are mandatory to confirm these preliminary data., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

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., (Copyright © 2012 Mosby, Inc. All rights reserved.)

Background and Purpose: Patients harboring nongiant cerebral aneurysms may rarely present with an ischemic infarct distal to the aneurysm. The aim of this case series was to report clinical and radiologic characteristics of these patients, their management, and outcome., Materials and Methods: We undertook a single-center retrospective analysis of consecutive patients admitted during an 8-year period with an acute ischemic stroke revealing an unruptured nongiant (<25 mm) sacciform intracranial aneurysm. Clinical, radiologic, therapeutic, and follow-up data were analyzed., Results: Nine patients were included. The mean size of aneurysms was 9.6 ± 6 mm, and 5 were partially or totally thrombosed. Two patients had a fatal SAH within 3 days after stroke-symptom onset, whereas asymptomatic meningeal bleeding was diagnosed or suspected in 2 others. Most of the patients with unthrombosed aneurysms were successfully treated by endovascular coiling in the acute phase. Thrombosed aneurysms were usually treated with antithrombotics, and most recanalized secondarily, requiring endovascular treatment or surgical obliteration. No recurrence of an ischemic event or SAH was observed during the 31 ± 12 months of follow-up (from 4 to 53 months)., Conclusions: In this single-center series, the frequency of early SAH in patients with ischemic stroke distal to an unruptured intracranial aneurysm was high. Acute management should be undertaken with care regarding antithrombotic use, and early endovascular coiling should be considered.

The right ventricular function is difficult to assess owing to its complex morphology, structure and function. The right ventricle (RV) comprises three compartments, the inlet, the apex, and the outlet contracting with a peristaltic motion from the inflow to the outflow chamber and is tightly linked to left ventricular (LV) function through the pulmonary circulation, the interventricular septum and the myocardium inside the pericardial envelop. The relation of RV function to symptom occurrence, exercise capacity and prognosis in a wide variety of cardiac diseases emphasizes the usefulness of its routine assessment. The evaluation of the RV is largely carried out by echocardiography in daily clinical practice despite important limitations inherent to two-dimensional imaging. Multiple views and numerous parameters allow clinicians to integrate the RV function in the clinical decision-making process. Recent modalities of echocardiography such as myocardial deformation and three-dimensional imaging or exercise echocardiography are promising tools for the assessment of the RV. Cardiac magnetic resonance imaging provides the unique opportunity to image the RV in motion and in three dimensions without the limitation of echogenicity. Therefore, cardiac magnetic resonance imaging is taking a growing place in the assessment of the RV in a wide variety of cardio-pulmonary diseases as pulmonary hypertension, ischemia, arrhythmogenic right ventricular cardiomyopathy, hypertrophic cardiomyopathy, heart failure or congenital heart diseases. Integrating the complex interplay between both ventricles and the pulmonary circulation, this review will discuss the latest results of standard and novel techniques allowing the assessment of RV function by echocardiography and cardiac magnetic resonance imaging, and will provide to the clinicians, facing therapeutic challenges, a comprehensive overview of right heart function.

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.