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This paper introduces a new software model and new tools for managing indoor smart environments (smart home, smart building, smart factories, etc.) thanks to MR technologies. Our fully-integrated solution is mainly based on a software modelization of connected objects used to manage them independently from their actual nature: these objects can be simulated or real. Based on this model our goal is to create a continuum between a real smart environment and its 3D digital twin in order to simulate and manipulate it. Therefore, two kinds of tools are introduced to leverage this model. First, we introduce two complementary tools, an AR and a VR one, for the creation of the digital twin of a given smart environment. Secondly, we propose 3D interactions and dedicated metaphors for the creation of automation scenarios in the same VR application. These scenarios are then converted to a Petri-net based model that can be edited later by expert users. Adjusting the parameters of our model allows to navigate on the continuum in order to use the digital twin for simulation, deployment and real/virtual synchronization purposes. These different contributions and their benefits are illustrated thanks to the automation configuration of a room in our lab., ICAT-EGVE 2019 - International Conference on Artificial Reality and Telexistence and Eurographics Symposium on Virtual Environments, Design and Programming, 97, 104, Jeremy Lacoche, Morgan Le Chenechal, Eric Villain, and Anthony Foulonneau, CCS Concepts: Human-centered computing --> Interactive systems and tools; HCI theory, concepts and models; Virtual reality

Background— β-Blocker efficacy in long-QT syndrome type 1 is good but variably reported, and the causes of cardiac events despite β-blocker therapy have not been ascertained. Methods and Results— This was a retrospective study of the details surrounding cardiac events in 216 genotyped long-QT syndrome type 1 patients treated with β-blocker and followed up for a median time of 10 years. Before β-blocker, cardiac events occurred in 157 patients (73%) at a median age of 9 years, with cardiac arrest (CA) in 26 (12%). QT-prolonging drugs were used by 17 patients; 9 of 17 (53%) had CA compared with 17 of 199 nonusers (8.5%; odds ratio, 12.0; 95% confidence interval, 4.1 to 35.3; P P P =0.001). None of the 26 patients with CA before β-blocker had CA/sudden death on β-blockers. Conclusions— β-Blockers are extremely effective in long-QT syndrome type 1 and should be administered at diagnosis and ideally before the preteen years. β-Blocker noncompliance and use of QT-prolonging drug are responsible for almost all life-threatening “β-blocker failures.” β-Blockers are appropriate therapy for asymptomatic patients and those who have never had a CA or β-blocker therapy. Routine implantation of cardiac defibrillators in such patients does not appear justified.

Resume Les fœtus ou les enfants des femmes porteuses d’un anticorps anti-SSA/Ro et/ou anti-SSB/La peuvent developper un « lupus erythemateux neonatal » comportant une atteinte cutanee, hematologique, hepatique et/ou un bloc auriculo-ventriculaire congenital (BAVc) survenant sur un cœur indemne de cardiopathie malformative majeure. La frequence du BAVc chez les femmes ayant une connectivite associee a un anticorps anti-SSA/Ro est estimee a 1 a 2 % et le risque de recurrence est de 10 a 17 %. Le BAVc complet est definitif et est associe a une morbidite (necessite d’implanter un pace maker dans 2/3 des cas) et une mortalite (16 a 19 %) faisant toute la gravite de ce syndrome. Une atteinte myocardique peut etre associee d’emblee ou apparaitre tardivement. Les autres atteintes sont egalement discutees. Lorsqu’une femme enceinte est porteuse d’un anticorps anti-SSA/Ro, la surveillance echographique obstetricale doit etre realisee tous les 15 jours entre 16 et 24 SA, voire toutes les semaines si elle a un antecedent de BAVc lors d’une grossesse precedente. Un electrocardiogramme est pratique de principe chez tous les enfants en periode neo-natale pour depister des BAVc incomplets. Le traitement curatif in utero du BAVc fait appel aux corticoides fluores, en privilegiant la betamethasone. Leur efficacite est inconstante.

Objectives: To investigate the clinical profile, natural history, and optimal management of persistent or permanent junctional reciprocating tachycardia (PJRT) in children. Methods and results: 85 patients meeting the ECG criteria for PJRT were enrolled in a retrospective multicentre study. Age at diagnosis varied from birth to 20 years (median 3 months). Follow up ranged from 0.1 to 26.0 (median 8.2) years. At the time of referral, 24 of 85 patients (28%) had congestive heart failure that was resolved with medical treatment in all patients. Eighty three patients received drug treatment initially. Amiodarone and verapamil were the most effective with a success rate of 84–94% alone or in association with digoxin. Radiofrequency ablation of the accessory pathway was performed in 18 patients. There was a trend for a relation between age at ablation and the result of the procedure, failures being more common in younger patients (three of six procedures in younger and 15 of 18 in older children were successful; p = 0.14). Two patients with persistent left ventricular dysfunction on echocardiography but with no symptoms of congestive heart failure died suddenly one month and three years after diagnosis. PJRT resolved spontaneously in 19 patients (22%). Age at diagnosis of PJRT was not a predictor of spontaneous resolution. Conclusions: PJRT is a potentially lethal arrhythmia in children with tachycardia induced cardiomyopathy. Spontaneous resolution of tachycardia is not uncommon. Antiarrhythmic treatment is often effective. Radiofrequency ablation should be performed in older children or when rate is not controlled, especially in patients with persistent left ventricular dysfunction.

Objective: To study the fetal response to prenatal therapy in non-hydropic fetuses with supraventricular tachycardia (SVT) as a function of fetal haemodynamic status at presentation. Study Design: Retrospective study. Material and Methods: Between 1990 and 2000, 40 non-hydropic fetuses presented with SVT. Twenty-eight had reciprocating SVT and 12 had atrial flutter. Ten fetuses had significant tricuspid valve regurgitation. All fetuses were treated prenatally. The main outcome measurement was fetal response to therapy as assessed by the rate of prenatal SVT reduction and by the mean time interval to sinus rhythm restoration. Results: The mean gestational age at presentation was 29 ± 4.9 weeks. Overall, there were 39 liveborns and 1 intrauterine death. Reduction of SVT was achieved prenatally in 32 cases (80%). Among the 30 cases without tricuspid regurgitation, prenatal conversion to sinus rhythm was achieved in 27 cases (90%) with a mean time interval of 7 days. Among the 10 fetuses presenting with tricuspid regurgitation, the rate of prenatal conversion was significantly lower (5/10) and the mean time interval to conversion was significantly longer (24 days; p = 0.04, Mann-Whitney test). In the subgroup treated by digoxin as first-line therapy (n = 32), the interval to sinus rhythm restoration was also significantly higher in the presence of tricuspid regurgitation, with a slightly but not significantly lower reduction rate. Conclusion: The response to prenatal therapy may be poorer in cases presenting with tricuspid regurgitation.

Smith-Magenis syndrome (SMS) is a clinically recognisable contiguous gene syndrome ascribed to interstitial deletions of chromosome 17p11.2. Patients have a phase shift of their circadian rhythm of melatonin with a paradoxical diurnal secretion of the hormone. Serum melatonin levels and day-night behaviour were studied in nine SMS children (aged 4 to 17 years) given acebutolol, a selective β1-adrenergic antagonist (10 mg/kg early in the morning). Cardiac examination, serum melatonin, motor activity recordings, and sleep diaries were monitored before and after drug administration. The present study shows that a single morning dose of acebutolol suppressed the inappropriate secretion of melatonin in SMS. A significant improvement of inappropriate behaviour with increased concentration, delayed sleep onset, increased hours of sleep, and delayed waking were also noted. These results suggest that β1-adrenergic antagonists help to manage hyperactivity, enhance cognitive performance, and reduce sleep disorders in SMS. Keywords: Smith-Magenis syndrome; circadian rhythms; melatonin

Isolated complete atrio-ventricular (AV) block in children occurs in anatomically normal hearts in the absence of any known predisposing factor likely to have caused the block. This study aimed to define the current prognosis of these conduction disorders.135 patients with isolated complete AV block, diagnosed before the age of 15 years, were included in this monocentric, retrospective study.52.6% of the cases were diagnosed in the antenatal or neonatal period, demonstrating their congenital nature. The disease was heralded by symptoms in only 6.7% of cases. A search for maternal anti-Ro/La antibodies was performed in 111 of the patients. It was positive in 56 cases, and was associated with an early diagnosis, a short delay in cardiac pacemaker implantation, and the occurrence of cardiomyopathy (16 cases), the latter representing a critical point in the progression of the disease (37.5% mortality at 6 years). Among the 55 cases not associated with antibodies, 2 cases were familial and no etiology was discovered in the others. The diagnosis was therefore made later, and no progression to cardiomyopathy was noted. A cardiac pacemaker was implanted in 122 patients (66 epicardial, 56 endocardial).Isolated complete AV block in children is not a homogenous entity: 'immunological' blocks are genuinely congenital and their prognosis remains grave, owing to the risk of cardiomyopathy, which is sometimes diagnosed late. The other types of block are diagnosed much later in childhood, and their mechanism is still unknown. They have a good prognosis as long as the indications for pacing the child are respected.

Hypertrophic cardiomyopathy (HCM) carries an increased risk of sudden death, especially in children and young adults. We investigated heart rate variability (HRV) in children with HCM, in order to evaluate its prognostic value. Seventeen patients (9 males and 8 females) with non-obstructive HCM were prospectively enrolled in the study. The diagnosis of HCM had been made between the ages of 1 month and 13 years (mean (SD) 71 (54) months). At the time of the study their ages ranged from 16 months to 16.5 years (mean 123 (70) months). HCM was defined by the presence of a hypertrophied, non-dilated left ventricle, in the absence of underlying cardiac, systemic disease or syndromic conditions. Four patients had a family history of HCM and two had a family history of premature (less than 50 years) sudden cardiac death of a first degree relative. At the time of the study, no patients were receiving treatment. Seven patients complained of moderate exertional dyspnoea; no patient had a history of chest pain or syncope. Standard 12 lead ECG showed that seven patients had abnormal repolarisation. On 24 hour monitoring, all patients were in stable sinus rhythm, and no arrhythmia was recorded. Ten patients underwent exercise testing and none had hypotension, arrhythmias or ischaemia during exercise. Five patients …

Prior to putting in place a percutaneous device, the assessment of the atrial septal defect anatomy is mandatory. The 3D transthoracic echocardiography is a non-invasive method bringing an imaging of the septal defect surface and its borders. Fifty-two patients ageing from 3 to 16 years old had a rotational 3D transthoracic echocardiography. Thirty-six (69%) were selected for a percutaneous closure (borders7 mm and a septal surface/atrial septal defect ratio2). Thirty-two of these selected patients (89%) benefited from the Amplatz prosthesis implantation with success. The maximal 3D diameter of the septal defect was 20 + 4 mm (14-30) compared to the mean size of prosthesis at 22 mm (18-30). Four of the 36 patients (aneurismal septum or a borderline septal surface/atrial septal defect ratio) were secondarily oriented to surgeons. Sixteen patients (31%) were selected directly to a surgical closure. The lack of borders or septal surface were confirmed by the surgical view. The 3D transthoracic echocardiography allows to define new criteria for the selection of patients prior to a percutaneous closure of atrial septal defects. This prospective study demonstrates that 9 out of 10 patients who had the 3D criteria had a percutaneous closure of the septal defect.

OBJECTIVETo evaluate the quality of life in patients with univentricular heart and to determine the impact of sociodemographic and clinical characteristics.DESIGN AND SETTINGRetrospective, cross sectional study conducted in a regional paediatric cardiology centre.PATIENTSThe health records of 89 survivors with univentricular heart (median age 21 years; range 17–49 years) were reviewed. Sixty seven answered the Duke questionnaire. Sociodemographic and clinical variables were similar in the responders and non-responders. The impact of sociodemographic and clinical variables on individual Duke's measures was assessed.RESULTSThe Duke scores of adults with univentricular heart were similar to the normal population. Cyanosis predicted a worse score for physical (p = 0.05) and perceived health measures (p = 0.02). A higher educational level predicted a better score for physical (p = 0.004), mental (p = 0.01), and general health measures (p = 0.02). Orthopaedic problems worsened the social score (p = 0.05). Psychosocial problems worsened the pain score (p = 0.04). In comparison with the other anatomical types, mitral atresia worsened the perceived health score (p = 0.02). Patients younger than 23 years scored better for almost all health and dysfunction measures.CONCLUSIONSDespite repeated interventions and other disease related everyday stresses, a selected group of adults with univentricular heart had a satisfying quality of life.