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BACKGROUND: Congenital heart disease (CHD) entails a broad spectrum of malformations with various degrees of severity and prognosis. Consequently, new and specific healthcare needs are emerging, requiring responsive healthcare provision. Research on this matter is predominantly performed on population-based databases, to inform clinicians, researchers and policy-makers on health outcomes and economic burden of CHD. Most databases contain data either from administrative sources or from clinical systems. We describe the methodological design of the BELgian COngenital Heart Disease Database combining Administrative and Clinical data (BELCODAC), to investigate patients with CHD. METHODS: Data on clinical characteristics from three university hospitals in Belgium (Leuven, Ghent and Brussels) were merged with mortality and socio-economic data from the official Belgian statistical office (StatBel), and with healthcare use data from the InterMutualistic Agency, an overarching national organization that collects data from the seven sickness funds for all Belgian citizens. Over 60 variables with multiple entries over time are included in the database. RESULTS: BELCODAC contains data on 18,510 patients, of which 8926 patients (48%) have a mild, 7490 (41%) a moderately complex and 2094 (11%) a complex anatomical heart defect. The most prevalent diagnosis is Ventricular Septal Defect in 3879 patients (21%), followed by Atrial Septal Defect in 2565 patients (14%). CONCLUSIONS: BELCODAC comprises longitudinal data on patients with CHD in Belgium. This will help build evidence-based provision of care to the changing CHD population. ispartof: INTERNATIONAL JOURNAL OF CARDIOLOGY vol:316 pages:72-78 ispartof: location:Netherlands status: published

BACKGROUND: Impaired pulmonary function is an independent predictor of mortality in adult congenital heart disease (CHD), but has been scarcely studied in the paediatric CHD population. AIMS: To compare the pulmonary function of children with CHD to healthy controls, and evaluate its association with clinical outcomes, exercise capacity, and quality of life. METHODS: Cross-sectional multicentre study among 834 children (555 CHD and 279 control subjects) who underwent a complete spirometry and a cardiopulmonary exercise test (CPET). The 5th centile (Z-score = -1.64) was used to define the lower limit of normal. The association of clinical and CPET variables with spirometry was studied using a multivariate analysis. Children and their parents filled in the Kidscreen health-related quality of life questionnaire. RESULTS: Forced vital capacity (FVC) and forced expiratory volume in 1 s (FEV1) Z-scores values were lower in children with CHD than controls (-0.4 ± 1.5 vs. 0.4 ± 1.3, P < 0.001 and -0.5 ± 1.4 vs. 0.4 ± 1.2, P < 0.001, respectively), without any obstructive airway disorder. Restrictive pattern was more frequent in CHD patients than in controls (20% vs. 4%, P < 0.0001). FVC Z-scores were predominantly impaired in complex CHD, such as heterotaxy (-1.1 ± 0.6), single ventricle (-1.0 ± 0.2), and complex anomalies of the ventricular outflow tracts (-0.9 ± 0.1). In multivariate analysis, FVC was associated with age, body mass index, peak oxygen uptake, genetic anomalies, the number of cardiac surgery and cardiac catheter procedures. FVC and FEV1 correlated with self and proxy-related quality of life scores. CONCLUSION: These results suggest that pulmonary function should be monitored early in life, from childhood, in the CHD population. TRIAL REGISTRATION NUMBER: NCT01202916, post-results.

This study evaluated the prognostic value of postoperative cardiac troponin-I (cTnI) in predicting all-cause mortality up to 3 months after normothermic congenital cardiac surgery.Prospective observational study.University hospital.All children ages 0 to 10 years.None.cTnI was measured after the induction of anesthesia but before the surgery, at the pediatric intensive care unit arrival, and at 4, 12, and 24 hours postoperatively. Follow-up was extended up to 6 months. Overall, 169 children were analyzed, of whom 165 were survivors and 4 were nonsurvivors. cTnI levels were significantly higher in nonsurvivors only at 24 hours (p = 0.047). Children undergoing surgery with cardiopulmonary bypass (CPB) had significantly higher cTnI concentrations compared with those without CPB (p0.001). Logistic regression analysis was performed on the 146 children in the CPB group with the following predictive variables: CPB time, postoperative cTnI concentrations, the presence of a cyanotic malformation, and intramyocardial incision. None of the variables predicted mortality. Postoperative cTnI concentrations did not predict 6 months׳ mortality. Only cTnI at 24 hours predicted the length of stay in the pediatric intensive care unit.This study did not find that postoperative cTnI concentration predicted midterm mortality after normothermic congenital heart surgery. (ClinicalTrials.gov identifier: NCT01616394).

Objectives Previous research in adult congenital heart disease (ACHD) patients suggested better clinical and managerial outcomes following specialized over general cardiac follow-up. The impact of different care levels, such as shared care, remained uninvestigated. Methods Propensity score analyses were performed on the BELgian COngenital heart disease Database combining Administrative and Clinical data (BELCODAC). Adult patients who ever attended a specialized congenital cardiology clinic (N=6,579) were categorized in five care levels based on their cardiac follow-up pattern between 2006 and 2010. Medium-term (2011-2015) economic outcomes were medical costs and resource use (i.e., hospitalizations and emergency department visits). Results Respectively 51.5% and 80% of patients with moderate and mild lesions were categorized in the general cardiac care (GCC) or no cardiac care group (NCC). In patients with moderate lesions, highly specialized cardiac care (HSC; exclusive follow-up by ACHD specialists) and shared care with predominantly specialized cardiac care (SC+) were associated with significantly lower medical costs and resource use compared to shared care with predominantly general cardiac care (SC-) and GCC. In the patient population with mild lesions, HSC was associated with better economic outcomes than SC- and GCC, but SC+ was not. HSC was associated with fewer hospitalizations (-33%) and less pharmaceutical costs (-46.3%) compared to SC+. Patients with mild and moderate lesions in the NCC group had better economic outcomes than those in the GCC and SC- groups, but post-hoc analysis revealed that they had a different patient profile than patients under cardiac care. Conclusions More specialized care levels are associated with better economic outcomes in patients with mild or moderate lesions in cardiac follow-up. Shared care with strong involvement of ACHD specialists might be a management option to consider. Characteristics of patients without cardiac follow-up but good medium-term economic prospects should be further scrutinized

Background Impaired pulmonary function is an independent predictor of mortality in adult congenital heart disease (CHD), but has been scarcely studied in the paediatric CHD population. Aims To compare the pulmonary function of children with CHD to healthy controls, and evaluate its association with clinical outcomes, exercise capacity, and quality of life. Methods Cross-sectional multicentre study among 834 children (555 CHD and 279 control subjects) who underwent a complete spirometry and a cardiopulmonary exercise test (CPET). The 5th centile (Z-score = − 1.64) was used to define the lower limit of normal. The association of clinical and CPET variables with spirometry was studied using a multivariate analysis. Children and their parents filled in the Kidscreen health-related quality of life questionnaire. Results Forced vital capacity (FVC) and forced expiratory volume in one second (FEV1) Z-scores values were lower in children with CHD than controls (− 0.4 ± 1.5 vs. 0.4 ± 1.3, P Conclusion These results suggest that pulmonary function should be monitored early in life, from childhood, in the CHD population.

Resume Les progres therapeutiques en oncologie pediatrique ont permis de porter la survie a cinq ans pour tous types de cancer confondus a 80 %, permettant a un nombre croissant d’enfants d’etre gueris et d’atteindre l’âge adulte. Cependant, ces survivants de cancers sont a risque de developper des complications secondaires aux traitements. Les anthracyclines et la radiotherapie mediastinale sont potentiellement cardiotoxiques et contribuent a une morbidite et une mortalite significative a long terme. Cette revue resume brievement la physiopathologie des complications cardiaques liees aux anthracyclines et a la radiotherapie, leur incidence, ainsi que les facteurs de risque principaux. Les etudes les plus significatives sont exposees dans cette revue et toutes demontrent un risque accru de complications cardiaques par rapport a la population normale. Enfin, les differentes techniques actuellement disponibles ou emergentes pour le suivi cardiaque des patients, les moyens de prevention pour limiter la cardiotoxicite et les traitements de ces complications cardiaques une fois diagnostiquees seront developpes. Les pathologies cardiovasculaires etant la cause principale de deces chez ces survivants, le depistage d’une dysfonction cardiaque debutante reste la cle de voute du devenir de ces patients.

Cardiac MRI can predict the likely tumor type in the majority of children with a cardiac mass. A comprehensive imaging protocol is essential for accurate diagnosis. However, histologic diagnosis remains the gold standard, and in some cases malignancy cannot be definitively excluded on the basis of cardiac MRI images alone.

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