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Aims: High-sensitive troponin T (hs-TnT), N-terminal pro-B-type natriuretic peptide (NT-proBNP), and C-reactive protein (CRP) are established prognostic biomarkers for cardiovascular (CV) morbidity and mortality and frequently used in symptomatic and/or hospitalized adults with congenital heart disease (ACHD). Their prognostic value in clinically stable ACHD has not yet been well established. This study investigates the predictive value of hs-TnT, NT-proBNP, and CRP for survival and CV events in stable ACHD. Methods and results: In this prospective cohort study, 495 outpatient ACHD (43.9 ± 10.0 years, 49.1% female) underwent venous blood sampling including hs-TnT, NT-proBNP, and CRP. Patients were followed up for survival status and the occurrence of CV events. Survival analyses were performed with Cox proportional hazards regression analysis and Kaplan–Meier curves. During a mean follow-up of 2.8 ± 1.0 years, 53 patients (10.7%) died or reached a cardiac-related endpoint including sustained ventricular tachycardia, hospitalization with cardiac decompensation, ablation, interventional catheterization, pacer implantation, or cardiac surgery. Multivariable Cox regression revealed hs-TnT (P = 0.005) and NT-proBNP (P = 0.018) as independent predictors of death or cardiac-related events in stable ACHD, whilst the prognostic value of CRP vanished after multivariable adjustment (P = 0.057). Receiver-operator characteristic curve analysis identified cut-off values for event-free survival of hs-TnT ≤9 ng/L and NT-proBNP ≤200 ng/L. Patients with both increased biomarkers had a 7.7-fold (confidence interval 3.57–16.40, P < 0.001) higher risk for death and cardiac-related events compared with patients without elevated blood values. Conclusion: Subclinical values of hs-TnT and NT-proBNP are a useful, simple, and independent prognostic tool for adverse cardiac events and survival in stable outpatient ACHD. Registration: German Clinical Trial Registry DRKS00015248. Graphical Abstract [ABSTRACT FROM AUTHOR]

OBJECTIVES: Surgical septal myectomy is performed to relieve left ventricular outflow tract narrowing in severe drug-refractory obstructive hypertrophic cardiomyopathy. The objective of this study was to assess the perioperative and long-term clinical outcome of this procedure performed during infancy. METHODS: Clinical, transthoracic echocardiographic, electrocardiographic, 24-h Holter, cardiopulmonary exercise test and genetic data were extracted by medical record review. A subset of patients underwent additional prospective detailed clinical evaluation including cardiac magnetic resonance imaging with contrast. RESULTS: Surgery was performed in 23 paediatric patients between 1978 and 2015 at the German Heart Centre Munich. Twelve patients had undergone surgery during infancy (≤1 year) (Group A), 11 between 1 and 18 years of age (Group B). The underlying genetic diagnosis was Noonan syndrome spectrum and non-syndromic hypertrophic cardiomyopathy. As compared to Group B, patients in Group A showed more concomitant cardiac procedures and received more homologous transfusions. One perioperative death occurred in Group A, and none in Group B. Two patients in Group A but no patient in Group B required redo septal myectomy. The long-term clinical outcome was similar between the 2 groups. One patient in Group B required cardioverter-defibrillator/pacemaker implantation for higher degree atrioventricular block and none in Group A. There was no evidence of differences in myocardial fibrosis between groups on longterm follow-up magnetic resonance imaging. CONCLUSIONS: Surgical septal myectomy can be performed safely during infancy with favourable perioperative and long-term clinical outcome but with a trend towards a higher reoperation rate later in life. [ABSTRACT FROM AUTHOR]

OBJECTIVES In several former studies, adolescents and adults with congenital heart disease (CHD) had a reduced exercise capacity even with defects considered to be simple. Currently, children might get better medical management and less restrictions concerning an active lifestyle or sports activities. The exercise performance of this new generation of children with CHD has to be evaluated. METHODS In the year 2010, 88 children (12.7 years, 52 males), 11–14 years old, with various CHD performed a cardiopulmonary exercise test in our institution. These children were matched for age and gender with healthy subjects who underwent the same procedure at a school survey. RESULTS In comparison with healthy controls, children with CHD had a diminished peak oxygen uptake (CHD: 35.5 ml/min/kg vs controls: 42.4 ml/min/kg; P < 0.001) corresponding to 87.1% (CHD) and 99.5% (Controls) of the reference value, respectively. Peak oxygen uptake decreased with the severity of the heart defect (r = −0.410; P < 0.001). However, there was no difference in oxygen uptake at the ventilatory threshold (CHD: 20.6 ml/min/kg vs controls: 21.5 ml/min/kg; P = 0.68). CONCLUSIONS Currently, children with CHD are not limited in their submaximal exercise performance. However, there is still a reduction in peak oxygen uptake. [ABSTRACT FROM PUBLISHER]

Aims: Patients with congenital heart disease usually show diminished exercise capacity and quality of life. However, there is only little information about daily activity, a marker for lifestyle, exercise capacity, and the prevention of arteriosclerosis. This study investigated exercise capacity, quality of life, daily activity, and their interaction with univentricular heart physiology after total cavopulmonary connection (TCPC). [ABSTRACT FROM PUBLISHER]