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Abstract Aims Fontan palliation is a surgical strategy for patients with complex congenital heart disease, in whom biventricular circulation cannot be achieved. Long‐term survival is negatively affected by the absence of sub‐pulmonary ventricle and increased systemic venous pressure. Exercise capacity is a known predictor of overall survival and quality of life in congenital heart defects. We aim to track individual trends of peak oxygen uptake (V̇O2peak) after total cavopulmonary connection (TCPC), identify predictors of deterioration, and derive a disease‐specific reference V̇O2peak dataset. Methods and results A retrospective study of serial cardiopulmonary exercise testing (CPET) data, gathered from all patients who underwent TCPC in the Czech Republic between 1992 and 2016. Of 354 consecutive patients with TCPC, 288 (81.4%) patients underwent one or more CPETs yielding 786 unique V̇O2peak values used as a reference dataset. Longitudinal data were available in 206 (58.2%) patients, who underwent a median (inter‐quartile range) of 3.0 (2.0–5.0) CPETs over a mean (standard deviation) of 8.9 (5.5) years. The decline of exercise capacity with age was linear and not faster than in healthy peers (P = 0.47), but relative values of V̇O2peak in TCPC patients were 12.6 mL/min/kg lower. Single ventricular morphology and pulmonary artery size had no significant influence on the exercise capacity dynamics. V̇O2peak decline correlated negatively with the trend of body mass index z‐score (P = 0.006) and was faster in women than men (P = 0.008). Conclusions Total cavopulmonary connection patients have significantly reduced exercise capacity. The age‐related decline paralleled the healthy population and correlated negatively with the body mass index trend. The presented V̇O2peak reference dataset may help the clinicians to grade the severity of exercise capacity impairment in individual TCPC patients.

BackgroundThe aim of this study was to analyze the relation between the hepatic fibrosis markers, liver morphology and hemodynamics assessed by magnetic resonance imaging (MRI) after total cavopulmonary connection (TCPC).Materials and methodsAdult patients after TCPC performed in childhood between 1993 and 2003 are the subjects of this observational study. The follow-up protocol consisted of clinical and echocardiographic examination, liver elastography, cardiopulmonary exercise test, MRI hemodynamics and liver morphology assessment and direct enhanced liver fibrosis (ELF) test.ResultsThe cohort consisted of 39 patients (46% female) with a median age at study 26 (IQR 23–28) years and interval from TCPC 21 (IQR 20–23) years. There was no correlation between ELF test and any MRI variables, but procollagen III amino-terminal peptide (PIIINP), a single component of ELF test, correlated significantly with ventricular end-diastolic volume (r = 0.33; p = 0.042) and inferior vena cava flow (r = 0.47; p = 0.003). Fifteen (38%) patients with liver nodules had compared to other 24 patients higher end-diastolic volume (ml/m2) 102.8 ± 20.0 vs. 88.2 ± 17.7; p = 0.023, respectively. PIIINP correlated significantly with inferior vena cava flow (r = 0.56; p = 0.030) and with end-diastolic volume (r = 0.53; p = 0.043), but only in patients with liver nodules.ConclusionGradual progression of liver fibrosis, particularly hepatic arterialization caused by liver nodules formation, increases inferior vena cava flow and subsequent ventricular volume overload may further compromise single ventricle functional reserve in adult patients after TCPC.

Prevention of sport-related health issues is an important medical and social topic especially due to possible sudden death of athlete, which is most often caused by an adverse cardiac condition. Diagnoses that could cause sudden cardiac death differs between paediatric and adult population. Inherited conditions predominate in children and adolescents, acquired conditions are responsible for sudden cardiac death in majority of adult athletes. Nowadays there is general agreement about screening of risks of sudden cardiac death. In the Czech Republic, sports physicians, general practitioners or primary care paediatricians perform sudden cardiac death risk screening as a part of preparticipation screening. Nevertheless, many more medical conditions must be screened during preparticipation check-ups; some of them are directly related to growth and development of an individual. [ABSTRACT FROM AUTHOR]