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Cardiac tumors are rare in children, with an incidence ranging between 0.01% and 0.32% ([1][1]). Although most cardiac tumors are benign, some of them may lead to serious complications. Early diagnosis is crucial because treatment is dependent on the type of tumor. Because of their rarity, centers

Ventriculocoronary arterial connections are typically found in patients with pulmonary atresia with an intact ventricular septum. This report describes a case of ventriculocoronary arterial connections in a patient with pulmonary atresia with a ventricular septal defect. Our case supports recent data suggesting a primary coronary artery developmental anomaly in pulmonary atresia. (Level of Difficulty: Advanced.), Graphical abstract, Ventriculocoronary arterial connections are typically found in patients with pulmonary atresia with an intact ventricular septum. This report…

Highlights • Exercise capacity in patients with CHD is lower than in the general population. • Non-invasive relaxation therapy may be effective in patients with dyspnoea. • Evidence based-medicine on relaxation therapy in the CHD population is poor. • This trial will assess the impact of Sophrology on exercise capacity in CHD patients., Background Recent advances in the field of congenital heart disease (CHD) have significantly improved the overall prognosis. Now more attention is being given to health-related quality of life (HRQoL) and promotion of physical activity. Non-invasive relaxation therapy may be effective in cardiac patients concerned with exercise-induced dyspnoea. The SOPHROCARE randomised trial aims to assess the impact of Caycedian Sophrology on cardiopulmonary fitness in adolescents and young adults with CHD. Methods The SOPHROCARE trial is a nationwide, multicentre, randomised, controlled study in CHD patients aged from 13 to 25 years old. Patients will be randomised into 2 groups (8 Sophrology group sessions vs. no intervention). The primary outcome is the change in percent predicted maximum oxygen uptake (VO2max) between baseline and 12-month follow-up. A total of 94 patients in each group is required to observe a significant increase of 10% in VO2max with a power of 80% and an alpha risk of 5%. The secondary outcomes are: clinical outcomes, cardiopulmonary exercise test parameters (VE/VCO2 slope, ventilatory anaerobic threshold, oxygen pulse, respiratory response to hypercapnia), health-related quality of life score (PedsQL), physical and psychological status. Conclusion After focusing on the survival in CHD, current research is opening on secondary prevention and patient-related outcomes. We sought to assess in the SOPHROCARE trial, if a Sophrology program, could improve exercise capacity and quality of life in youth with CHD. Trial registration Clinicaltrials.gov (NCT03999320).

Summary Background Coarctation of the aorta (CoA) is still challenging to diagnose in neonates with patent ductus arteriosus (PDA). Speckle tracking echocardiography allows reliable analysis of myocardial deformation in newborns and seems to provide important insides into regional changes in patients with left ventricular (LV) outflow tract obstruction. Aims To assess the interest of LV global longitudinal strain (GLS) measurement for predicting CoA in neonates with PDA and prenatal suspicion. Methods Prospective single-center study. Twenty-two newborns with prenatal suspicion of CoA were included. All newborns were evaluated in the first 12 hours of life. To assess the feasibility and the reproducibility of GLS, 14 healthy full-term newborns with PDA (group 3) were screened. CoA was diagnosed when DA closed, according to usual echocardiographic criteria. Results Six neonates developed CoA after DA closure (group 1) whereas 16 did not (group 2). Mean gestational age and birth weight were not different between the groups. GLS measurements were possible in 100%. Intra- and inter-observer variability of strain measurements was acceptable. GLS values were significantly lower in neonates who developed CoA (P = 0.015). To predict CoA, cut-off value of −17.42% gave the best compromise for sensitivity (83%) and specificity (72%). Aortic arch dimensions were modestly correlated with strain values. The presence of a bicuspid aortic valve was not associated with significant lower GLS values. Conclusion LV GLS analysis is a feasible and reproducible echocardiographic technique in newborns with PDA. Newborns who will develop CoA seem to have lower values of GLS than healthy neonates. Further studies are needed to confirm these preliminary results.

BACKGROUND: Mitral valve anomalies in children are rare but frequently severe, recalcitrant and not often amenable to primary repair, necessitating mechanical mitral valve replacement (M-MVR). This study examines outcomes of a cohort undergoing 1(st) M-MVR at 90 days post-M-MVR, transplant-free survival was 76% at 20 years follow-up (median follow-up: 16.6, IQR: 11.9-21.3). Adjusted analysis in those who survived >90 days showed elevated risk of death/transplant for males (HR 1.5; 95% CI:1.0-2.3), age at M-MVR

Neonates undergoing congenital heart surgery require highly specialized, resource-intensive care. Location of care and degree of specialization can vary between and within institutions. Using a multi-institutional cohort, we sought to determine whether location of admission is associated with an increase in health care costs, resource use and mortality.We retrospectively analyzed admission for neonates (30 days) undergoing congenital heart surgery between 2004 and 2013 by using the Pediatric Health Information Systems database (44 children's hospitals). Multivariate generalized estimating equations adjusted for center- and patient-specific risk factors and stratified by age at admission were performed to examine the association of admission intensive care unit (ICU) with total hospital costs, mortality, and length of stay.Of 19,984 neonates (60% male) identified, 39% were initially admitted to a cardiac ICU (CICU), 48% to a neonatal ICU (NICU), and 13% to a pediatric ICU. In adjusted models, admission to a CICU versus NICU was associated with a $20,440 reduction in total hospital cost for infants aged 2 to 7 days at admission (P = .007) and a $23,700 reduction in total cost for infants aged 8 to 14 days at admission (P = .01). Initial admission to a CICU or pediatric ICU versus NICU at15 days of age was associated with shorter hospital and ICU length of stay and fewer days of mechanical ventilation. There was no difference in adjusted mortality by admission location.Admission to an ICU specializing in cardiac care is associated with significantly decreased hospital costs and more efficient resource use for neonates requiring cardiac surgery.

Aims. To investigate the healing process and nickel release of the Hyperion occluder (Comed BV, Netherlands), as compared to the Amplatzer septal occluder (ASO) (St. Jude Medical Inc., St. Paul, MN, USA) in a chronic swine model. Background. Some long-term complications occurring after percutaneous atrial septal defect (ASD) closure may be partially associated with an inappropriate healing of the device and increased nickel release. There is no direct comparative study of different occluders for healing and nickel release. Methods. After percutaneous ASD creation, 12 pigs were implanted with 15 mm Hyperion (n = 6) and 15 mm ASO (n = 6) devices. After 1 month (n = 3 for each device) and 3 months (n = 3 for each device) of follow-up, device explantation was performed and healing was assessed using histopathological workup. Systemic and tissular nickel release was performed. Results. Implantation was successful in 100% without complications. Device coverage was observed as early as 1 month after implantation and was almost complete after 3 months. A granulation tissue with a predominantly mononuclear inflammatory reaction was observed in contact with nitinol wires while an inflammatory reaction was seen in contact with textile fibers. We found no statistically significant difference between the 2 devices whether for histological grading scores or systemic nickel release, regardless to follow-up duration. Conclusions. In this preclinical study, we demonstrated that Amplatzer septal occluder and Hyperion occluder were not significantly different for device healing and nickel release processes.

Levosimendan is a calcium-sensitizing drug with positive inotropic properties. As an inodilator, this molecule also has a vasodilation effect. While its efficacy has been demonstrated in the adult in the context of cardiac surgery, its pediatric use is still not widespread. Many studies have shown its safety of use in children, including in the newborn. Across the world, a growing number of teams use levosimendan to treat both acute and chronic heart failure. Through a review of the literature, we describe its pharmacodynamic effects, its current applications, and its perspectives of use in children.

Hypertension is an important and under-recognized cause of acquired cardiomyopathy in newborns. Half the etiologies of neonatal hypertension are undetermined. We present a rare case of severe neonatal hypertension with ventricular dysfunction secondary to substance exposure during pregnancy. This case report emphasizes the importance of a well-focused prenatal history on maternal medications and substance use.

As for any preclinical development of new implantable device, bench testing has been followed by experimental studies on large animal models for the development of atrial septal defect closure devices. Various models have been used according to studied species (porcine, ovine or canine model) and whether the septal defect was percutaneously or surgically created. Animal models of percutaneous atrial septal defect closure aim to assess the healing process and device endothelialisation, as well as the development of magnetic resonance imaging guided procedures, the short-term effects of volume overload on right ventricular contractility through haemodynamic studies and the understanding of other complications such as nickel hypersensitivity. Each technique has its own advantages and drawbacks, and leads to different punch-related, acute septal injuries that could have an effect on the healing process after device implantation. It has been suggested that some long-term, major device-related complications such as thrombosis or infective endocarditis may be associated with an inappropriate healing process or insufficient endothelialisation of the device, leading industrial companies to pay a great deal of attention to the healing process. Tissue reactions in animal models were shown to adequately reproduce the healing response after device implantation in humans, with an endothelial device coverage observed as early as 30 days after implantation and complete after 3 to 6 months. Research perspectives may evaluate both animal models and in-vitro studies in parallel with a view to clarify the endothelialisation process using human endothelial cells through in-vitro experiments. Self-sensing device for detecting the presence of endothelial cells on the surface of intracardiac occluders and high-resolution imaging techniques that could non-invasively assess the complete endothelialisation of a device would also be promising tools which would need large animal models studies before their clinical application.

Injury of the left main coronary artery may sometimes complicate a pulmonary valve replacement (PVR). We present the case of a 39-year-old man, with post-operative distortion of the left anterior descending coronary artery, which caused onset of ventricular arrhythmia associated with electrocardiographic signs of myocardial ischemia. A coronary angiogram revealed the cause of the lesion. Stenting the coronary artery restored normal coronary blood flow and improved the patient's condition. Through this case, we describe a new life-threatening complication after PVR. © 2015 Wiley Periodicals, Inc.

In ductus-dependent congenital heart disease, preserving the blood flow through the ductus arteriosus (DA) is vital before surgery. We present the cases of three full-term neonates with ductus-dependent congenital heart disease for whom near-infrared spectroscopy (NIRS) monitoring was performed. We recorded cyclical drops in regional oxygen saturation, both cerebral and renal, that corresponded to constrictions of the DA. These findings appeared either simultaneously or previous to SpO2 drops and were corrected by prostaglandin infusion. Through these cases, we assume that cyclical constrictions of ductal cells participate in the DA closure process in its early phase.

Summary Background Children with dilated cardiomyopathy in advanced heart failure may spend a long time awaiting heart transplantation. Consequently, mechanical circulatory support is sometimes required as a bridge to transplantation. Levosimendan, a positive inotropic agent, has been reported to be safe and efficient for the treatment of paediatric heart failure. Aims To report our experience with levosimendan in children with decompensated dilated cardiomyopathy. Methods Paediatric patients with dilated cardiomyopathy on the transplant waiting list and with criteria for mechanical support were included in this single-centred retrospective study. Each patient received at least one 24-hour infusion of levosimendan before mechanical circulatory support was considered. Biological and echocardiographic data were analysed. Results Six patients were included over a 24-month period. The median age was 25.5 months (7.7–34.2 months); 82 infusions were performed. Median B-type natriuretic peptide concentration decreased significantly between days 0 and 2 (2443 ng/L [1458–3819 ng/L] vs 1358 ng/L [1025–2534 ng/L]; P = 0.003). While only a trend was noted in left ventricular ejection fraction improvement ( P = 0.054 by Simpson's method and P = 0.068 by the Teicholz method), the subaortic velocity time integral rose significantly between days 0 and 8 (12.8 cm/s [10–14.5 cm/s] vs 15.3 cm/s [14.3–16.9 cm/s]; P = 0.041). Conclusions Levosimendan seems to improve haemodynamics in children with decompensated dilated cardiomyopathy; repeated infusions may delay the need for mechanical circulatory support while awaiting heart transplantation. This therapeutic agent should be systematically considered in this setting, in addition to conventional inotropic drugs.

We present the case of a 4-month-old child with atrioventricular canal associated with severe left atrioventricular valve dysfunction who previously underwent 3 surgical valve reconstructions without significant improvement. A Hybrid Melody valve (Medtronic, Minneapolis, MN) insertion was planned. Because of the low weight, the risk of left ventricular outflow tract obstruction was significant and therefore evaluated preprocedurally using a cardiac computed tomography-derived 3-dimensional printed model. In vitro tests showed good anchorage of the valve without subaortic obstruction and the procedure was then achieved with an excellent clinical result.

In this article we report the first case of Q fever endocarditis in a 13 years old child with a percutaneous pulmonary Melody

International audience; Thiamine deficiency is recognized in varied parts of the world. In Asia, it remains an important public health problem where highly polished rice is the major staple food and where other primary dietary sources of thiamine are in short supply. Beriberi, or clinically apparent thiamine deficiency, may present a variety of syndromes including myocardial dysfunction or wet beriberi, dry beriberi with neurological symptoms, and the more severe form Shoshin beriberi with cardiac failure and lactic acidosis. Infantile thiamine deficiency is a very rare condition in developed countries today. It occurs mainly in breastfed infants of mothers who have inadequate intake of thiamine. Clinical symptoms in such infants include gastrointestinal symptoms, cardiac failure, and lactic acidosis. We report the case of a 10-week-old girl, admitted with diarrhea, vomiting, acidosis, and cardiac failure. After excluding other etiologies of cardiomyopathy, biochemical thiamine deficiency confirmed the diagnosis of beriberi in an infant of a thiamine-deficient mother from Reunion Island, a French island where recently, with Mayotte Island, epidemic cases of beriberi have been described. This case is important to highlight the manifestations in young infants and to alert physicians to the possibility of thiamine deficiency in developed countries.

Introduction Congenital heart defects (CHD) are the most common congenital malformations, with a prevalence of nearly 8 per 1000 live births [1] . Less than 20% of these abnormalities can be attributed to a chromosomal or monogenic anomaly, but in the majority of cases, the cause remains unknown [2] . Some recent studies have identified environmental exposures as possible risk factors for the occurrence of CHD [3] , [4] . A critical period during which a woman or a man exposed to an agent is most at risk of congenital malformation in their fetus is now recognized [5] , [6] . The ARTEMIS Center, at the Bordeaux University Hospital, is dedicated to the evaluation of environmental exposures in patients with fertility disorders, pregnancy pathologies and congenital malformations. Objectives We aimed to present the prevalence of exposure to reproductive risk factors at the time of critical developmental period in patients managed at the ARTEMIS Center for the presence of a cardiac malformation in their child or fetus. Results Environmental exposures assessment of 45 mothers and 42 spouses were realized (from 2017 to February 2018). Regarding occupational exposures during the periconceptional period (3 months before, 3 months after the conception) some potential factors were identified: physical strain (9 mothers, 20.9%), drugs (4 mothers, 9.3%, and 3 spouses, 7.1%), organic solvants (3 mothers, 7.0%), pesticides (3 mothers, 7.0%, and 7 spouses, 16.7%), polycyliv aromatimatic hydrocarbons (4 spouses). Regarding non-occupational exposures, cleaning and maintenance products (12 mothers, 26.7% and 12 spouses, 28.5%), pesticides (8 mothers, 17.8% and 8 spouses, 19.0%), the DIY products (3 mothers, 6.7 and 3 spouses, 7.1%) were identified as well ( Table 1 ). Conclusion Environmental exposures with potential malformative risk have been identified, and prevention measures proposed. Further large studies evaluating the imputability of these exposures on the occurrence of CHD are necessary.

Because the classification of congenital bicuspid aortic valve (BAV) is of importance to predict a possible valvular dysfunction, we sought to assess the feasibility, the reproducibility, and the accuracy of three-dimensional transthoracic echocardiography (3D-TTE) to accurately depict the morphology of the leaflets in a BAV.Seventy-two consecutive children, who were suspected of having a BAV on two-dimensional transthoracic echocardiography (2D-TTE), were included in this prospective study. 2D-TTE and 3D-TTE views of a BAV were recorded by the same investigator, and then were analysed separately by two confirmed paediatric cardiologists. For each of these two imaging techniques, the spatial position of cusps and raphes was noted for each patient. Intra-observer concordance and inter-observer concordance were evaluated to assess the reproducibility of the techniques. Feasibility of 3D-TTE was 100%. Median acquisition time of 3D-TTE was 117 (98.5-176.8) s. Image quality seemed to be better with 3D-TTE compared with 2D-TTE. When using 3D-TTE, the diagnosis was reconsidered for 12 patients (17%). Only 44.4% of uncertain BAV cases identified by 2D-TTE were confirmed by 3D-TTE. Furthermore, 3D-TTE seems to provide a better visualization of the leaflet morphology, leading to reclassification for 34.4% (95% CI 22.9-47.3) of the patients. Agreement for the BAV classification between 2D-TTE and 3D-TTE was therefore moderate (κ = 0.46). Both inter-observer concordance and intra-observer concordance were good (κ = 0.91 and κ = 0.93, respectively) for 3D-TTE.3D-TTE is feasible and provides accurate description of a BAV in children. Compared with 2D-TTE, 3D-TTE seems to enable a better visualization of the structural geometry of the leaflets.

BackgroundThe association between long QT interval and sudden infant death syndrome has been clearly established. Several studies have been conducted to determine the evolution of the QT interval in childhood from birth, but only in full-term newborns. However, data on the QT interval in pre-term infants are extremely scarce. The objective was to describe the development of the QT interval in premature infants.Material and methodsIn a prospective monocentric study in a neonatal intensive care unit, pre-term newborns born before 37 weeks of gestation without congenital heart disease, family history of long QT, unstable haemodynamic status, or administration of drugs inducing QT interval prolongation were included with parental consent. An electrocardiogram was recorded in similar conditions weekly until discharge in each child. The corrected QT was calculated with Bazett's formula.ResultsIn all, 309 echocardiograms were recorded in 87 children, with gestational age ranging from 24–36 weeks. QT first increased after birth in very premature infants – less than 30 weeks of gestation – and then started to decrease, whereas it only decreased in more mature infants. When plotted against postmenstrual age, QT first increased, and then decreased after 32 weeks.DiscussionOur data suggest that the QT interval varies with postmenstrual age in very premature infants, reaching a peak at 32 weeks. These developmental changes may induce specific vulnerability to QT-lengthening medications in premature infants. This study underlines the need for specific pharmacological studies in this population.

Background: Three-dimensional echocardiography (3DE) allows accurate estimation of left ventricular (LV) volumes and function in children. Because of the peculiar morphology of the right ventricle (RV), RV volumes and function assessment remain challenging. 3D software adapted for RV morphology is a promising noninvasive method. Aims: The aims of this study were to evaluate the feasibility of 3D RV analysis in a normal pediatric population and to compare 3D RV to 3D LV measurements. Patients and methods: 3D transthoracic echocardiography was performed in 50 patients (mean age 9.5 years ± 5.1) with normal cardiac anatomy and function. Measurements were performed with the X3-1 or the X7-2 matrix probe (iE33, Philips Medical Systems, Andover, MA, USA). Ventricular volumes were analyzed with dedicated analysis software (TomTec Imaging Systems, Munich, Germany). Results: Measurements were possible in 100% of LVs and 94% of RVs. The stroke volumes of the two ventricles correlated well (r = 0.81 [95% CI, 0.68–0.89], P < 0.0001) with a mean difference of 3.6 mL (±9.2). Compared to the LV, the RV had higher mean end-diastolic volume (53 mL [±29] versus 46 mL [±46], P = 0.020) and end-systolic volume (29 mL [±17] versus 17 mL [±12], P < 0.0001) but lower mean ejection fraction (49%[±8] versus 67%[±10], P < 0.0001). Conclusion: 3DE using dedicated software is a feasible and reproducible method for RV volume quantification in children with normal hearts. Further studies are needed to validate the accuracy of the measurements. (Echocardiography 2012;29:492-501)

SummaryCongenital malformations of the mitral valve may be encountered in isolation or in association with other congenital heart defects. Each level of the mitral valve complex may be affected, according to the embryological development, explaining the fact that these lesions are sometimes associated with each other. As a perfect preoperative assessment is of importance, good knowledge of both normal and abnormal anatomy is required in order to guide the surgeon accurately. This review presents the different embryological, anatomical and echocardiographic aspects of the congenital mitral anomalies.

RésuméL’échocardiographie 3D transthoracique a longtemps été freinée dans son développement en raison de conditions techniques d’acquisition compliquées et de qualité d’images médiocres. L’avènement des sondes matricielles permet au 3D en devenant temps réel d’entrer dans la pratique clinique courante. Si la voie œsophagienne a permis au 3D de trouver ses lettres de noblesses par ses descriptions anatomiques uniques des valves et des septa, l’échocardiographie transthoracique peut désormais se décliner en modes 2D, Doppler et 3D. Ses applications dans la cardiologie congénitale et pédiatrique sont multiples : description anatomique précise des défauts septaux auriculaires et ventriculaires, classification des bicuspidies aortiques et analyse du mécanisme de sténose. Ainsi, l’échocardiographie 3D permet-elle de sélectionner de façon non invasive les patients, de guider et de juger du résultat d’un cathétérisme interventionnel. L’imagerie 3D est un excellent moyen de communication entre l’imageur et le cardiologue interventionnel mais aussi de délivrer des informations claires au patient et à la famille avant et après un cathétérisme.SummaryThree-dimensional echocardiography has improved dramatically due to technical advances in probe design and computer processing. The introduction of real time 3D echocardiography has led to its use in everyday clinical practice. Congenital heart disease demands a detailed understanding of the spatial relationships of cardiac structures to plan treatment. The introduction of new transthoracic 3D probes has extended the applications to real-time guidance of catheter procedures. Prominent among the cardiac lesions which have been studied are: atrial septal defects, ventricular septal defects and stenotic bicuspid aortic valves. Its values should be decisive in many congenital cardiac lesions requiring interventional catheterisation. 3D echocardiography is an easy way to communicate to the patient and its family about the pathology.

We report the case of a newborn with pulmonary atresia with intact ventricular septum and right ventricle-dependent coronary circulation. He died several weeks after a Blalock-Taussig procedure because of a progressive stenosis of the main coronary artery. We present echocardiographic and dual-source CT images of the stenosis, with autopsy correlation. To our knowledge, CT images of this quality have never been reported in a newborn. This case illustrates the extreme difficulty in prognosticating the outcome for these patients and underlines the need for a detailed neonatal coronary mapping to assess right ventricle-dependent coronary circulation.

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.

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

Pulmonary valve replacement (PVR) often is performed in adults with repaired tetralogy of Fallot (TOF). For patients who have tricuspid regurgitation (TR), tricuspid valve (TV) repair associated to PVR is still debated.We sought to evaluate perioperative factors related to TV repair when performed at the time of PVR in patients with repaired TOF.We retrospectively reviewed 104 patients with repaired TOF (or its equivalent) who underwent PVR (2002-2014).Mean age at initial complete correction and at PVR was 20.1 ± 17.2 months and 26.3 ± 9.5 years, respectively. Forty-one patients had significant preoperative TR: 24 were moderate (group M) and 17 were severe (group S). A total of 16 TV repair were performed (8 for each group). Moderate and severe tricuspid regurgitation observed in the first year following the initial complete repair were significantly associated with severe TR at PVR (P .001). In group M patients, TR was improved regardless of TV repair, whereas, in group S, residual significant TR was reported in 7 patients who did not have TV repair. No cases were observed for patients who underwent concomitant TV repair (P = .002). Among these patients with residual significant TR, 2 needed a tricuspid valve replacement. The functional status (New York Heart Association classification) of group S patients was significantly improved by concomitant TV repair.In adults with repaired TOF, TV repair is a safe procedure when performed at the time of PVR. If, at mid-term follow-up, there is probably no benefit of TV repair when preoperative TR is moderate, TV repair may improve both tricuspid valve function and functional status of the patients in case of severe preoperative TR.

Background Levosimendan is an inodilator agent belonging to the family of calcium sensitizer agents. Its positive inotropic and vasodilating properties make it interesting for the treatment of heart failure. Although this inotropic drug is increasingly used in children, a special license is still needed to obtain it in several countries. Aims To report our experience of levosimendan and to analyse its effects in children. Methods It was a single-centre retrospective study. From January 2010 to December 2014, all paediatric patients who received at least one perfusion of levosimendan were included. Levosimendan was administered as continuous infusions at a dose of 0.2 µg/kg/min for 24 hours without any loading dose. Medical records were analyzed for each patient. Results More than 200 patients received levosimendan during the period and more than 300 infusions were performed. No side effect was reported. Levosimendan significantly improved BNP level. Indications of levosimendan infusion were: 1) treatment of postoperative hemodynamic deterioration, 2) preoperative conditioning, 3) treatment of acute heart failure (myocardial ischemia, myocarditis, decompensated cardiomyopathies), 4) treatment of chronic heart failure (cardiomyopathies and failing Fontan). Conclusion Because of the long half-life of its active metabolite, levosimendan has a prolonged effect lasting for up to 7 to 9 days after discontinuation of a 24-hour infusion. Using this drug, the potential of arrhythmia is reduced as total intracellular calcium levels are not raised. This inodilator agent is safe and effective for the treatment of heart failure in children and must be part of the conventional therapeutic arsenal.

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

Left ventricular (LV) dysfunction may complicate paediatric cardiac surgery with cardiopulmonary bypass, notably after long aortic cross-clamping (ACC). Assessment of occult myocardial injury by conventional echocardiographic variables may be difficult in the postoperative period.To evaluate the feasibility of two-dimensional (2D) strain in the postoperative period, and to assess the effect of ACC duration on this variable.Thirty-three paediatric patients (age18years) with congenital heart disease undergoing cardiac surgery with cardiopulmonary bypass were included in this prospective single-centre study. Daily echocardiography was performed from the day before surgery to the fifth postoperative day. LV ejection fraction and LV 2D strain were measured. The cohort was divided into three groups according to ACC duration (group 1:30minutes; group 2: 30-80minutes; group 3:80minutes).Mean age and weight were 4.2±2.5years and 15.1±5.2kg, respectively. Feasibilities of longitudinal, circumferential and radial strains were good, and quite similar to conventional variables. Compared with conventional variables, intra- and interobserver agreements regarding 2D strain were better (r=0.916, P0.001 and r=0.855, P0.001 for longitudinal strain versus r=0.156, P=0.54 and r=0.064, P=0.80 for LV ejection fraction by Simpson's method). Postoperative evolution of longitudinal and circumferential strains was significantly different between the three groups (P0.001), whereas there was no difference using conventional variables.Postoperative LV 2D strain is a feasible and reproducible method. Strain measurements seem to indicate correlation with ACC duration.

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

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.

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.

Transoesophageal echocardiography is a useful tool for the intra-operative assessment of surgical repair in children with congenital cardiac malformations. We report the case of a subaortic membrane removal in a nine-year-old boy. Three-dimensional transoesophageal echocardiography revealed a mitral perforation created during the procedure.

We report the case a 14-year-old male presented with a perimembranous ventricular septal defect. 2D echocardiography showed both left ventricular to right ventricular and atrial shunt. Left and right ventricular 3D "en face" views of the defect were obtained. 3D echocardiography delineated the morphologic nature of the ventricular septal defect, its shape, and the direction in which the defect extends into the ventricular septum. Other anatomic structures such as the tricuspid valve leaflet, the right ventricular outflow tract, and the aortic valve can be displayed in their realistic spatial distribution. These findings have clinical implications when surgical repair is planned.

Bacterial endocarditis is rare in children. We report the case of streptococcal B endocarditis in a newborn whose mother had asymptomatic bacteriuria during pregnancy. This report emphasizes the importance of maternal intrapartum antibiotic therapy when there is a major risk of neonatal infection and underlines the diagnostic value of echocardiography in case of prolonged fever.

We report the case of a 1-month-old boy with an unusual association of supraventricular tachycardia and anomalous origin of the left coronary artery from the pulmonary artery. Although signs of infarction were visible on the first electrocardiogram, the presence of an arrhythmia did not immediately suggest a coronary anomaly. Echocardiography allowed the diagnosis, thus leading to appropriate care.

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