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 (V̇O2 peak) after total cavopulmonary connection (TCPC), identify predictors of deterioration, and derive a disease-specific reference V̇O2 peak 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 V̇O2 peak 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 V̇O2 peak 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. V̇O2 peak 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 V̇O2 peak reference dataset may help the clinicians to grade the severity of exercise capacity impairment in individual TCPC patients.

• Klíčová slova
• Cardiopulmonary exercise testing, Exercise capacity, Fontan circulation, Oxygen consumption, Total cavopulmonary connection,
• MeSH
• bypass pravého srdce metody   MeSH
• dítě MeSH
• dospělí MeSH
• Fontanova operace * metody   MeSH
• kvalita života MeSH
• lidé MeSH
• longitudinální studie MeSH
• retrospektivní studie MeSH
• spotřeba kyslíku MeSH
• tolerance zátěže * fyziologie   MeSH
• vrozené srdeční vady * patofyziologie   rehabilitace   chirurgie   MeSH
• Check Tag
• dítě MeSH
• dospělí MeSH
• lidé MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• klinická studie MeSH
• práce podpořená grantem MeSH

• MeSH
• dítě MeSH
• kardiochirurgické výkony rehabilitace   MeSH
• lidé MeSH
• mladiství MeSH
• táboření * MeSH
• vrozené srdeční vady rehabilitace   chirurgie   MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• mladiství MeSH
• Publikační typ
• časopisecké články MeSH

A total of 338 patients aged 10 to 56 years, who had undergone various cardiac operations, were divided, on the basis of a spiroergometric examination, performed together with the determination of maximal oxygen intake (MOI), into 4 functional classes. Determination of functional aerobic impairment, i.e. the magnitude of deviation of the established MOI value from that theoretically anticipated in view of the patient's age and sex, made it possible to determine the degree of decrease in the reserve capacity of the patient's cardiac and respiratory system (CRS). Comprehensive assessment of the functional state of the CRS, while taking into account the patient's individual characteristics, is a prerequisite for a differentiated quantitative approach to the design of rehabilitation programmes.

• MeSH
• aerobióza MeSH
• dítě MeSH
• dospělí MeSH
• dýchání * MeSH
• fibrilace síní chirurgie   MeSH
• kardiochirurgické výkony rehabilitace   MeSH
• lidé středního věku MeSH
• lidé MeSH
• mitrální stenóza chirurgie   MeSH
• mladiství MeSH
• spotřeba kyslíku MeSH
• srdce patofyziologie   MeSH
• srdeční selhání chirurgie   MeSH
• vrozené srdeční vady metabolismus   rehabilitace   chirurgie   MeSH
• Check Tag
• dítě MeSH
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladiství MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• srovnávací studie MeSH