Marie Vincenti

Thoracic computed tomography in absent pulmonary valve syndrome management

A neonate with absent pulmonary valve syndrome (APVS) and features of tetralogy of Fallot presented respiratory distress. The prolonged need for mechanical ventilation was an indication for surgical repair. Preoperative thoracic CT imaging showed nearly complete closure of the distal trachea, compressed by the aneurysmal dilatation of the pulmonary arteries, and its reopening after ventilator pressure was increased. Management of APVS may require high‐pressure ventilation to prevent bronchial collapse due to compression by the pulmonary arteries. In this context, thoracic CT imaging is very useful for adjusting ventilation support so that surgical repair can be performed in optimal conditions.

Cardiopulmonary fitness in children with congenital heart diseases versus healthy children

Objective We aimed to compare the cardiopulmonary fitness of children with congenital heart diseases (CHD) with that of age-adjusted and gender-adjusted controls. We also intended to identify clinical characteristics associated with maximum oxygen uptake (VO2max) in this population. Methods and results We included in a cross-sectional multicentre study a total of 798 children (496 CHD and 302 controls) who underwent a complete cardiopulmonary exercise test (CPET). The association of clinical characteristics with VO2max was studied using a multivariate analysis. Mean VO2max in the CHD group and control represented 93%±20% and 107%±17% of predicted values, respectively. VO2max was significantly lower in the CHD group, overall (37.8±0.3vs 42.6±0.4 mL/kg/min, P<0.0001) and for each group (P<0.05). The mean VO2max decline per year was significantly higher in CHD than in the controls overall (−0.84±0.10 vs −0.19±0.14 mL/kg/min/year, P<0.01), for boys (−0.72±0.14vs 0.11±0.19 mL/kg/min/year, P<0.01) and for girls (−1.00±0.13 vs −0.55±0.21 mL/kg/min/year, P=0.05). VO2max was associated with body mass index, ventilatory anaerobic threshold, female gender, restrictive ventilatory disorder, right ventricle systolic hypertension, tricuspid regurgitation, the number of cardiac catheter or surgery procedures, and the presence of a genetic anomaly. Conclusions Although the magnitude of the difference was not large, VO2max among children with CHD was significantly lower than in normal children. We suggest performing CPET in routine follow-up of these patients. Trial registration number ClinicalTrials.gov NCT01202916; Post-results.

Cardiopulmonary exercise test in children with congenital heart diseases: correlation between ventilatory parameters and maximum oxygen uptake

Gavotto A. (1,2), Matecki S. (2, 5), Guillaumont S. (3), Picot M.C. (4), Amedro P. (1,5) Pediatric and Congenital Cardiology Department, University Hospital, Montpellier, France (1); Pediatric Functional Exploration Laboratory, Physiology Department, University Hospital, Montpellier, France (2); Pediatric Cardiology and Rehabilitation Unit, Saint-Pierre Institute, Palavas-Les-Flots, France (3); Epidemiology and Clinical Research Department, University Hospital, Montpellier, France (4);Physiology and Experimental Biology of Heart and Muscles Laboratory PHYMEDEXP, UMR CNRS 9214 – INSERM U1046, University of Montpellier, Montpellier, France (5)

0350: Correlation between cardio-pulmonary exercise test variables and health-related quality of life among children with congenital heart diseases

Background peak 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 results 202 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 Conclusions peak VO2 and anaerobic threshold are the two CPET variables which best correlated with self and parents-reported QoL in this large pediatric cohort. If QoL is involved as a “patient related outcome” in a clinical trial in pediatric cardiology, we suggest to use parents related QoL scores. Clinical Trial Registration ClinicalTrials.gov (number NCT01202916).