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Direct communication between the right pulmonary artery and the left atrium in the presence of intact atrial septum is a rare congenital entity. A 12-year-old boy presenting with dyspnea at exertion was fortuitously found to have low arterial oxygen saturation at rest. Imaging studies showed a large tortuous vessel originating in the distal right pulmonary artery, and ending into the left atrium via the right upper pulmonary vein. The patient underwent successful elective videothoracoscopic stapling and division of the fistula.

Introduction Echocardiographic assessment of right ventricle (RV) function has become essential in pediatric cardiology. Objective The aim of our study was to determine the most appropriate mathematical model for RV function reference values in children. Method Normal children aged 0 to 18 years referred to the pediatric cardiology consultation were screened in this prospective cross-sectional study. Each RV parameters (S’, E’, A’ waves, TEI index, TAPSE) was modeled with four explanatory variables: age, size, weight and body surface area. Four different mathematical models were applied: a linear model, a polynomial model of degree 2, a linear regression model by spline with free knot, and a polynomial regression model of degree 2 by spline with a free knot. The choice of the best explanatory variable and modelization method was made on the basis of the model's coefficient of determination (R2). Results We included 314 normal children aged 2 days to 18 years (46% female, 88 infants, 26 neonates, 226 children). The modelization of the RV variables over all ages did not follow a linear model, with the lowest coefficients of determination in the four explanatory variables. The quadratic spline model best adjusted the modelization of all RV variables. No model accurately explained the TEI index in any of the four explanatory variables. The S’ wave was best modelized by the quadratic spline model with the weight. The knot at 6.86 kg corresponded to the curves’ break. The E’ wave was best modelized by the quadratic spline model with the age. The knot at 0.29 years corresponded to the curve's break. The A’ wave was best modelized by the quadratic model with the size. The knot at 81 cm corresponded to the break of the curve. The TAPSE was best modelized by the quadratic spline model with the weight. The knot at 9.04 kg corresponded to the curve's break. Conclusion The quadratic spline model best applies to echocardiographic RV reference values in pediatrics.

Backgroundpeak oxygen uptake (VO2) correlates with health related quality of life (QoL) in adults with heart failure. Cardio-pulmonary exercise test (CPET) is recommended in the follow-up of adults with congenital heart diseases (CHD). Few data is available as regards correlation between CPET and QoL among children with CHD.Methods and results202 CHD children aged 8 to 18 performed a CPET (treadmill n=96, cycle-ergometer n=106). CHD severity was stratified into 4 groups. All children and parents filled out the Kidscreen QoL questionnaire. Peak VO2, anaerobic threshold (AT), oxygen pulse followed a downward significant trend with increasing CHD severity and conversely for VE/VCO2 slope. Self-reported and parent-reported physical well-being QoL scores correlated with peak VO2 (respectively r=0.27, p

In the presence of new surgical techniques, the treatment of congenital valvular aortic stenosis is under debate. We reviewed the results and late outcomes of all 93 patients aged 1 day to 18 years, treated with balloon valvuloplasty (BAV) as first-line therapy for congenital aortic valve stenosis in our center from January 1991 to May 2012. Mean age at procedure time was 2.4 years; 37 patients underwent BAV at age ≤30 days (neonates), 29 patients at age ≥1 month and