ABSTRACT
BACKGROUND: Health-related quality of life (HR-QoL) stands as a determinant "patient-related outcome" and correlates with cardio-pulmonary exercise test (CPET) in adults with chronic heart failure or with a congenital heart disease (CHD). No such correlation has been established in pediatric cardiology. 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 HR-QoL questionnaire. Peak VO2, anaerobic threshold (AT), and 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 HR-QoL scores correlated with peak VO2 (respectively r=0.27, p<0.0001 and r=0.43, p<0.0001), percentage of predicted peak VO2 (r=0.28, p=0.0001 and r=0.41, p<0.0001), and percentage of predicted VO2 at AT (r=0.22, p<0.01 and r=0.31, p<0.0001). Significant correlations were also observed between several HR-QoL dimensions and dead space to tidal volume ratio (VD/VT), oxygen uptake efficiency slope (OUES), oxygen pulse but never with VE/VCO2 slope. The strongest correlations were observed in the treadmill group, especially between peak VO2 and physical well-being for parents (r=0.57, p<0.0001) and self (r=0.40, p<0.0001) reported HR-QoL. CONCLUSIONS: Peak VO2 and AT are the two CPET variables that best correlated with HR-QoL in this large pediatric cohort, parents' reports being more accurate. If HR-QoL is involved as a "PRO" in a pediatric cardiology clinical trial, we suggest using parents related physical well-being HR-QoL scores. CLINICAL TRIAL REGISTRATION: ClinicalTrials.gov (number NCT01202916).
Subject(s)
Exercise Test/methods , Heart Defects, Congenital/physiopathology , Heart Defects, Congenital/psychology , Adolescent , Anaerobic Threshold/physiology , Child , Chronic Disease , Cross-Sectional Studies , Exercise Test/standards , Female , Heart Defects, Congenital/blood , Humans , Male , Oxygen/blood , Oxygen Consumption/physiology , Prognosis , Prospective Studies , Quality of Life , Self Report , Surveys and QuestionnairesABSTRACT
Aortic arch anomalies are relatively frequent and account for 15 to 20 % of all congenital cardiovascular malformations. They can be discovered in case of symptoms of airway or esophageal compression such as dysphagia, chronic cough, stridor, wheezing and recurrent respiratory infections. We report the cases of two children with encircling aortic arch anomaly. The 3-year-old boy had complained of chronic respiratory symptoms such as laryngitis and asthma since the age of 4 months. The 20-month-old boy presented with inter-mittent stridor, dysphagia and frequent vomiting. In both patients, the cervico-thoracic CT-scan showed a right aortic arch with retro-esophageal left subclavian artery and Kommerell's diverticulum. Surgery was curative. The understanding of normal and abnormal embryologic development of the aortic arch, and the knowledge of the different types of vascular compression and their clinical signs are mandatory for the early diagnosis and adequate treatment of such malformations.
Les anomalies de l'arc aortique, relativement fréquentes, représentent 15 à 20 % de toutes les maladies cardiovasculaires congénitales. Elles peuvent être découvertes face à des symptômes de compression oesophagienne et/ou des voies respiratoires, tels que dysphagie, toux chronique, stridor, wheezing, voire infections respiratoires à répétition. Nous rapportons deux cas d'anomalie encerclante des arcs aortiques. Le premier patient, âgé de 3 ans, présentait, depuis l'âge de 4 mois, des symptômes respiratoires avec multiples épisodes étiquetés de laryngites et d'asthme. Le deuxième, âgé de 20 mois, présentait un stridor intermittent, de la dysphagie ainsi que des vomissements fréquents. Chez ces deux patients, le scanner cervico-thoracique démontra un arc aortique droit avec une artère sous-clavière gauche rétro-oesophagienne au départ d'un diverticule de Kommerell. Dans les deux cas, la cure chirurgicale fut suivie de la disparition des symptômes. La compréhension du développement embryologique normal et anormal des arcs aortiques, ainsi que la connaissance des manifestations cliniques des compressions vasculaires sont indispensables à leur diagnostic précoce et traitement adéquat.
