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Background A new three-dimensional echocardiographic (3DE) automated software (HeartModel) is now available to quantify left heart chamber. Our aims were to assess the feasibility of this software in children and its correlation with manual 3DE and cardiac magnetic resonance (CMR). Methods Ninety-two children were prospectively included in 2 separate protocols. In protocol 1, 73 healthy children (8.8 ± 3.0 years) underwent 2D and 3D transthoracic echocardiography (EPIQ 7 C). LV end-diastolic volume (LVEDV), LV end-systolic volume (LVESV), LVEF and LA volume at ventricular end-systole (LAV) obtained with automated 3DE were compared with the manual 3DE measurements. In protocol 2, automated 3DE measurements from 19 children with cardiopathy (12.8 ± 2.9 years) were compared with CMR values. Intra and inter-observer variability were assessed. Results Automated 3DE was feasible in 77% of datasets and reduced significantly time required for indices analysis compared with manual 3DE (20 vs. 125 seconds). There was excellent correlation for LVEDV, LVESV and LAV between automated and manual 3DE (R = 0.95) but less for LVEF (R = 0.57). Compared with manual 3DE, automated 3DE slightly overestimated LVEDV, LVEF and LAV and slightly underestimated LVESV. There were excellent correlation for LV volumes between automated 3DE and CMR (R = 0.94) but the correlation for LVEF was moderate (R = 0.46). Compared with CMR, automated 3DE slightly underestimated LVEDV and LVESV (biase-7.5%), underestimated LAV (− 31.6%), and had a negligible bias for LVEF (1.0%). Intraobserver and interobserver variability for automated 3DE measurements were low (< 12% (Fig. 1)). Conclusions HeartModel is promising software for fast assessment of left heart chamber volume and function. Its feasibility in children aged more than 5 years is good, with high reproducibility. The automated 3DE measurements were comparable to manual 3DE. Compared with CMR, LVEDV and LVEF measured by automated 3DE seem interesting in clinical practice. [ABSTRACT FROM AUTHOR]

Introduction New 3D automatic software (Heart model (HM), Philips Healthcare, Andover, Massachusetts) is now available to quantify left heart chamber size and function. Its feasibility and accuracy in children have not been reported. The aim of this study was to assess feasibility and reproducibility of HM in healthy children, and to compare LV indices obtained by this technique with classic 2D biplane method. Methods Sixty consecutive healthy children aged more than 5 years were included (mean age 6.5 years, mean weight 20.8 kg). All infants underwent TTE (Epic 7, X5-1, Philips Healthcare, Andover, Massachusetts). Left ventricle (LV) end-systolic and end-diastolic volumes, LV ejection fraction (EF) and left atrium (LA) end-systolic volume were obtained by biplane Simpson method. 3D dataset was obtained from apical 4-chamber view and analyzed using HM software. LV indices obtained by the two methods were compared. Results Feasibility of HM was 80%, in 30 cases (62.5%) with contour adjustment and 18 cases (37.5%) without contour adjustment. Intraobserver variability was (0%, 4% and 8% for HM without contour adjustment, HM with contour adjustment, and biplane method respectively). Interobserver variability was (6% and 12% for HM with contour adjustment and biplane method respectively). Test- retest variability for HM was 6.5%. Correlation between HM and biplane measurements was strong ( r = 0.85 to 0.94). LVEF obtained by HM was lower than that obtained by biplane method (bias −8%). However, LV volumes and LA end-systolic volume were larger (bias +12 and +8 respectively) ( Fig. 1 ). Conclusion HM is a promising software for assessment of left heart chamber volume and function. Its feasibility in infants aged more than 5 years is good with excellent reproducibility. Working is going on to validate this technique in children with congenital heart disease and to compare it with MRI measurement as the gold standard. [ABSTRACT FROM AUTHOR]

The pathophysiology of ventricular septal defect (VSD) is determined by the size of the defect and the state of the pulmonary vascular resistance. We assessed the morphology of VSD using 3D transthoracic echocardiography (3D-TTE) and the ability to estimate the pathophysiology. Methods Forty-eight children with isolated unique muscular (77%) and membranous VSD (23%) were included. Severity of the VSD was rated according to their pathophysiology. (Type 1: minor left-to-right (L-R) shunt; 2a: significant L-R shunt (left ventricular end diastolic diameter (LVEDD) Z-score>2); 2b: VSD associated with pulmonary hypertension). 3D VSD measurements were obtained after a multi-planar reconstruction of a TTE 3D full volume (X5-1 or X7-2 matrix probes, ie33, Philips). Diagnosis properties of the ratio of 3D VSD area to aortic annulus area (3DA/AAA), 3D VSD area to body surface area (3DA/BSA) and 2Dmax diameter to BSA (2DD/BSA) were compared. Results Median age was 8.5 month-old (min 1 max 123). LVEDD Z-score was>2 in 19 children (40%), (12 (25%) had pulmonary hypertension). 3D-VSD systolic area, 3D and 2D max. diameters were correlated with LVEDD. (r=0.71, r=0.52, r=0.55, P<0.05). Systolo-diastolic variation of 3D VSD area was higher in muscular than in membranous VSD (Median 54%vs27%, P=0.0001). VSD were asymmetric with a mean ratio of maximal to minimal 3D diameters of 2.1±1.3 in membranous VSD and 3.2±1.5 in muscular VSD (P=0.01). VSD severity was correlated with LVEDD Z-score, 3DA/AAA and 3DA/BSA ratio (r=0.52, r=0.63, r=0.60, P<0.05), but not with the 2DD/BSA ratio. Ability to diagnose type 2b VSD was higher with the 3DA/AAA or 3DA/BSA ratio than the 2D/BSA ratio (ROC area 0.97 and 0.96 vs 0.85). A 3DA/AAA ratio>0.39 has a sensitivity of 92% and a specificity of 97% to diagnose a 2b-VSD. Conclusion 3D-TTE allows a morphological and a quantitative assessment of muscular and membranous VSD. The 3DA/AAA ratio is an accurate diagnostic tool to assess the pathophysiology of the VSD. [ABSTRACT FROM AUTHOR]

Objectif: Discuter l’intérêt du défibrillateur automatique implantable (DAI) en cas de troubles du rythme ventriculaires graves inauguraux chez l’enfant atteint de myocardiopathie spongieuse. Sujet: La non compaction du ventricule gauche est une cardiopathie rare sous diagnostiquée. Elle peut être isolée ou associée à d’autres anomalies cardiaques. La présentation clinique est extrêmement variable. Ses complications sont la dysfonction ventriculaire gauche, les troubles du rythme cardiaque, les accidents thrombo-emboliques systémiques et la mort subite. La fréquence des arythmies est de 20 % chez l’enfant. Nous rapportons ici le cas d’un enfant de 33 mois, originaire du bénin, né de parents consanguins, chez qui le diagnostic de non compaction du ventricule gauche isolée a été porté au décours de 3 arrêts circulatoires sur fibrillation ventriculaire. Un DAI a été posé en association avec un traitement par cordarone et aspirine. Ce cas s’intègre dans une forme familiale (3 enfants atteints sur 5). Conclusion: La pose d’un DAI n’est pas fréquente chez le jeune enfant. Les troubles du rythme ventriculaires, dans le cadre du myocarde non compacté, peuvent cependant apparaître comme une indication formelle y compris chez le jeune enfant. [ABSTRACT FROM AUTHOR]

Résumé Le syndrome de Loeys-Dietz est une forme rare de maladie du tissu conjonctif dont les symptômes sont apparentés au syndrome de Marfan mais dont le phénotype peut être plus fruste. Mise en évidence récemment, cette affection demeure méconnue, alors que ses conséquences peuvent être dramatiques, notamment en raison des atteintes cardiovasculaires. Nous rapportons le cas d’une enfant de 4 ans suivie pour une luxation congénitale de la hanche chez laquelle un examen systématique a mis en évidence une hyper-élasticité cutanée et une luette bifide, faisant évoquer une pathologie du tissu conjonctif. Les signes squelettiques étaient frustes, la taille étant normale pour l’âge. Il n’y avait pas d’antécédents familiaux, cardiologiques ou non, en faveur d’une pathologie des tissus conjonctifs. Les explorations cardiologiques ont permis de découvrir un anévrysme de l’aorte ascendante au niveau du sinus de Valsalva (26 mm, Z-score + 4,24). L’analyse génétique a mis en évidence une mutation du gène transforming growth factor-beta receptor type II ( TGFβR2 ), posant le diagnostic de syndrome de Loeys-Dietz. Des atteintes squelettiques associées à une bifidité de la luette ou un hypertélorisme et un anévrysme de l’aorte ascendante doivent orienter l’enquête génétique vers la recherche d’une vasculopathie par anomalie de la voie du TGFβ telle que le syndrome de Loeys-Dietz. Summary Loeys-Dietz syndrome is a rare form of connective tissue disorder, whose clinical features can resemble those of Marfan syndrome, but with a more unpolished appearance. Recently brought out, this pathology remains little known; however, its consequences may be dramatic. We report on the case of a 4-year-old girl followed for a congenital hip dislocation, in which a systematic exam found increased cutaneous elasticity and a bifid uvula, suggesting a connective tissue disorder. Symptoms were unpolished, as the child's height was normal, without any positive cardiac, rheumatological, or ophthalmological family history. Cardiovascular tests found a thoracic aortic aneurysm at the Valsalva sinus (26 mm, Z-score = +4.24). A genetic investigation found a TGFβR2 gene mutation, leading to the diagnosis of Loeys-Dietz syndrome type 2. Skeletal damage associated with bifid uvula and/or hypertelorism and an aneurysm of the ascending aorta should guide the genetic investigation to the search for TGF-β vasculopathy such as Loeys-Dietz syndrome. [ABSTRACT FROM AUTHOR]

Résumé Les fistules artério-veineuses pulmonaires sont des vaisseaux anormaux reliant l’artère aux veines pulmonaires. Elles entraînent un shunt extracardiaque droite-gauche à l’origine d’une hypoxémie réfractaire à l’oxygénothérapie. Nous rapportons le cas d’une fillette de 7 ans présentant une volumineuse fistule artério-veineuse (FAV) pulmonaire avec une hypoxémie réfractaire découverte à l’occasion d’une anesthésie générale pour adénoïdectomie. Cette FAV a été diagnostiquée par l’angioscanner thoracique. Une occlusion percutanée a été réalisée en deux temps au cours d’un cathétérisme cardiaque interventionnel. Ce fait clinique illustre les circonstances de découverte, la démarche diagnostique face à une hypoxémie réfractaire de l’enfant et les avantages et inconvénients de la fermeture percutanée de FAV pulmonaire. Summary Pulmonary arteriovenous fistulas are abnormal vessels joining the right pulmonary artery to the pulmonary veins. They lead to an extracardiac right-to-left shunt with refractory hypoxemia. We report the case of a 7-year-old girl with a large pulmonary arteriovenous fistula discovered with refractory hypoxemia diagnosed during general anesthesia for adenoidectomy. Radio-opacity was observed on the upper lobe of the right lung. The diagnosis was made using thoracic angiotomography. The proximal arterial vessel arose from the lobar pulmonary artery. The fistula had developed in the entire right upper lobe and drained into two veins flowing into the right superior pulmonary artery. Given the marked hypoxemia, the potential risks of pulmonary hemorrhage and pulmonary infection, an occlusion of the fistula was indicated. After discussion between surgeons and interventional cardiologists, catheterization was indicated. The occlusion of the fistula was successful at the second attempt after release of a vascular plug in the main proximal vessel. This case illustrates the clinical circumstances of diagnosis of arteriovenous fistula, the diagnostic algorithm for refractory hypoxemia and the therapeutic options, with discussion of the benefits and drawbacks of a catheterization procedure. [ABSTRACT FROM AUTHOR]