Sophie Guillaumont

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AIMS The natural history of congenital or childhood non-immune, isolated atrioventricular (AV) block is poorly defined. METHODS AND RESULTS We retrospectively studied 141 children with isolated, non-immune AV block diagnosed in utero, or up to 15 years of age, at 13 French medical centres, between 1980 and 2009. Patients with structural heart disease or(More)
BACKGROUND The origin of congenital or childhood nonimmune isolated atrioventricular (AV) block remains unknown. We hypothesized that this conduction abnormality in the young may be a heritable disease. METHODS AND RESULTS A multicenter retrospective study (13 French referral centers, from 1980-2009) included 141 children with AV block diagnosed in utero,(More)
Secundum-type atrial septal defects (ASDII) account for approximately 10% of all congenital heart defects (CHD) and are associated with a familial risk. Mutations in transcription factors represent a genetic source for ASDII. Yet, little is known about the role of mutations in sarcomeric genes in ASDII etiology. To assess the role of sarcomeric genes in(More)
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).(More)
AIM To compare the haemodynamic status during high-frequency oscillatory ventilation and conventional mechanical ventilation in very preterm infants with respiratory distress syndrome. METHODS Thirty-two neonates of less than 30 wk gestation randomly assigned to high-frequency oscillatory ventilation (n = 15) or conventional mechanical ventilation (n =(More)
Nutritional status and chronic pulmonary hyperinflation can alter respiratory muscle function in cystic fibrosis (CF). This study investigated: 1) whether inspiratory muscle function is reduced in children with stable CF in comparison with healthy controls; and 2) the mechanisms leading to inspiratory muscle weakness, which probably predispose to(More)
Palliative treatment of the hypoplastic left heart syndrome is rarely practiced in France because of the poor prognosis of the first surgical stage of the Norwood procedure. The recent modification proposed by Sano seems to give more promising results. The authors report their experience with the Norwood-Sano procedure compared with the classical Norwood(More)
We report the short and mid-term results of the Norwood procedure (Stage one) in 20 patients with hypoplastic left heart syndrome or univentricular heart with aortic obstruction. Seven patients were prenatally diagnosed. Preoperatively there was obstruction to pulmonary venous return in 6 cases, a mild to moderate tricuspid regurgitation in 6 cases, and 11(More)
Nineteen asthmatic boys (aged 13.4 years, 25-75 percentile: 11.5-15.1 years) performed short bouts of maximal exercise (force-velocity test) to test their anaerobic fitness and tolerance of maximal anaerobic exercise. Fourteen healthy boys (aged 13.9 years, 25-75 percentile: 11.6-15.7 years) matched for anthropometric characteristics including lean body(More)
Congenital atrioventricular block is defined (Yater) as a documented conduction defect in a young subject with unquestionable bradycardia in the absence of a history of infection which might have caused the condition after birth. It is a rare condition (1 out of 20,000 births) and may be isolated or associated with another congenital malformation. Four(More)