We aimed to investigate the distribution of exercise capacity across the spectrum of adult congenital heart disease (ACHD) using own data and the published experience and to provide diagnosis, gender-, and age- specific reference values. Publications describing exercise capacity in ACHD patients using cardiopulmonary exercise testing (CPET) were identified (n = 2286 patients in 23 papers). In addition, we included 2129 patients who underwent CPET at our own institution. The majority of patients (80%) had reduced peak oxygen uptake (peak VO(2)) compared with normal values (defined as <90% of predicted peak VO(2)). There were significant differences in peak VO(2) between subgroups of patients, with the lowest values seen in patients with Eisenmenger syndrome and complex heart disease. However, even in patients with simple lesions, peak VO(2) was on average significantly reduced compared with normal values. Based on a large number of observations we herewith provide gender- and age-specific peak VO(2) centile plots for the most common lesions (Tetralogy of Fallot, systemic right ventricle, Ebstein anomaly and Fontan-palliation) and relate disease-specific exercise capacity to that required for specific activities of daily life, sports, and occupations. We provide age-, gender-, and diagnosis-specific data on peak VO(2) levels across the spectrum of ACHD allowing to compare the exercise capacity of individual patients with that of their peer patients. These data should be helpful in interpreting CPET results, guiding therapy, and advising patients on activities of daily life, sports participation, and choice of occupation.
