Reference values for exercise limitations among adults with congenital heart disease. Relation to activities of daily lifesingle centre experience and review of published data

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 VO2) compared with normal values (defined as 90 of predicted peak VO2). There were significant differences in peak VO2 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 VO2 was on average significantly reduced compared with normal values. Based on a large number of observations we herewith provide gender- and age-specific peak VO2 centile plots for the most common lesions (Tetralogy of Fallot, systemic right ventricle, Ebstein anomaly and Fontanpalliation) 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 VO2 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.