0346: Neonatal arterial switch operation: the sooner the better!

ObjectiveTo evaluate the impact of preoperative management on in-hospital post-operative outcomes after an arterial switch operation (ASO) in newborns with transposition of the great arteries (TGA)MethodsIn this retrospective monocentric study, we included all newborns with TGA±ventricular septal defect who underwent an ASO between 2008 and 2014. Collected data included demographics, clinical and anatomic characteristics, and preoperative management (Rashkind, prostaglandin infusion, mechanical ventilation, age at surgery). Univariate and multivariate analyses were performed to study the impact of preoperative characteristics on time to extubation, a surrogate marker of postoperative morbidity.ResultsAmong the 59 included newborns, 47 (79%) had an antenatal diagnosis, 31 (52%) received prostaglandins (median duration: 96 h; range 0-192h), and 38 (65%) underwent a Rashkind procedure. Mean age at surgery was 5±2 days. The mortality rate was 5% (n=3). Younger age at surgery significantly increased the probability to be extubated within 2 days after surgery, both in univariate analysis (Odd ratio 0.64; confidence interval 0.44-0.92) (p=0.01) and after adjustment for preoperative characteristics and management (OR 0.61; CI 0.39-0.95) (p=0.03). The presence of coronary anomalies tended to increase time to extubation, whereas Rashkind procedure and prostaglandin treatment had no impact.ConclusionOur study shows that younger age at surgery is associated with lower postoperative morbidity in newborns undergoing an ASO. These findings suggest that early surgery is preferable to delay with palliative management such as Rashkind and/or Prostaglandins

SCN5A mutations in 442 neonates and children: genotype-phenotype correlation and identification of higher-risk subgroups.

Aims To clarify the clinical characteristics and outcomes of children with SCN5A-mediated disease and to improve their risk stratification. Methods and results A multicentre, international, retrospective cohort study was conducted in 25 tertiary hospitals in 13 countries between 1990 and 2015. All patients ≤16 years of age diagnosed with a genetically confirmed SCN5A mutation were included in the analysis. There was no restriction made based on their clinical diagnosis. A total of 442 children {55.7% boys, 40.3% probands, median age: 8.0 [interquartile range (IQR) 9.5] years} from 350 families were included; 67.9% were asymptomatic at diagnosis. Four main phenotypes were identified: isolated progressive cardiac conduction disorders (25.6%), overlap phenotype (15.6%), isolated long QT syndrome type 3 (10.6%), and isolated Brugada syndrome type 1 (1.8%); 44.3% had a negative electrocardiogram phenotype. During a median follow-up of 5.9 (IQR 5.9) years, 272 cardiac events (CEs) occurred in 139 (31.5%) patients. Patients whose mutation localized in the C-terminus had a lower risk. Compound genotype, both gain- and loss-of-function SCN5A mutation, age ≤1 year at diagnosis in probands and age ≤1 year at diagnosis in non-probands were independent predictors of CE. Conclusion In this large paediatric cohort of SCN5A mutation-positive subjects, cardiac conduction disorders were the most prevalent phenotype; CEs occurred in about one-third of genotype-positive children, and several independent risk factors were identified, including age ≤1 year at diagnosis, compound mutation, and mutation with both gain- and loss-of-function

Arecibo PALFA survey and Einstein@Home: Binary pulsar discovery by volunteer computing

We report the discovery of the 20.7ms binary pulsar J1952+2630, made using the distributed computing project Einstein@Home in Pulsar ALFA survey observations with the Arecibo telescope. Follow-up observations with the Arecibo telescope confirm the binary nature of the system. We obtain a circular orbital solution with an orbital period of 9.4hr, a projected orbital radius of 2.8lt-s, and a mass function of f = 0.15 M ⊙ by analysis of spin period measurements. No evidence of orbital eccentricity is apparent; we set a 2σ upper limit e ≲ 1.7 × 10 -3 . The orbital parameters suggest a massive white dwarf companion with a minimum mass of 0.95 M ⊙ , assuming a pulsar mass of 1.4 M ⊙ . Most likely, this pulsar belongs to the rare class of intermediate-mass binary pulsars. Future timing observations will aim to determine the parameters of this system further, measure relativistic effects, and elucidate the nature of the companion star. © 2011. The American Astronomical Society. All rights reserved

Rhumatisme articulaire aigu

Le rhumatisme articulaire aigu représente un modèle d’auto-immunité acquise liée à la présence d’anticorps dirigés contre l’endocarde valvulaire cardiaque et qui se développent au décours d’une infection pharyngée à streptocoque. Les lésions peuvent évoluer à bas bruit vers des séquelles valvulaires (cardiopathie rhumatismale), parfois sévères et compliquées d’insuffisance cardiaque. Cette pathologie a été éradiquée des pays industrialisés grâce à l’amélioration du niveau de vie et aux programmes de prévention primaire (traitement des angines) et secondaire (antibiothérapie au long cours). Mais elle demeure un problème de santé publique majeur dans les pays en voie de développement. L’utilisation de l’échographie cardiaque a récemment montré son utilité pour détecter les formes mineures de la maladie permettant de traiter précocement les enfants affectés

Volatile chemical release by bethylid wasps: identity, phylogeny, anatomy and behaviour

International audienceThe structures of volatile chemicals released by parasitic wasps in the family Bethylidae are shown to correspond to the subfamily to which the species belong. Species in the Epyrinae release skatole (3-methylindole) and species in the Bethylinae release a spiroacetal (2-methyl-1,7-dioxaspiro [5.5]undecane): these compounds are chemically very different. The enantiomeric composition of the spiroacetal differs between congeneric species. Chemical release is a discrete event under the active control of both male and female wasps. Structural differences between the mandibular glands and intramandibular glands suggest the mandibular glands to be the source of released volatiles. Real-time mass spectrometry shows that the spiroacetal is released by Goniozus nephantidis females during dyadic resource contests, with release more common during more aggressive interactions. Chemical tagging with deuterium further shows that the volatile is released by the loser of an agonistic interaction and not the winner. The function of spiroacetal and skatole release by bethylids is discussed