Regular evaluation of physical capacity takes a crucial part in long-term follow-up in patients with congenital heart disease (CHD). This study aims to examine the accuracy of self-estimated exercise capacity compared to objective assessments by cardiopulmonary exercise testing in patients with CHD of various complexity.

We conducted a single centre, cross-sectional study with retrospective analysis on 382 patients aged 8–68 years with various CHD who completed cardiopulmonary exercise tests. Peak oxygen uptake was measured. Additionally, questionnaires covering self-estimation of exercise capacity were completed. Peak oxygen uptake was compared to patient’s self-estimated exercise capacity with focus on differences between complex and non-complex defects.

Peak oxygen uptake was 25.5 ± 7.9 ml/minute/kg, corresponding to 75.1 ± 18.8% of age- and sex-specific reference values. Higher values of peak oxygen uptake were seen in patients with higher subjective rating of exercise capacity. However, oxygen uptake in patients rating their exercise capacity as good (mean oxygen uptake 78.5 ± 1.6%) or very good (mean oxygen uptake 84.8 ± 4.8%) was on average still reduced compared to normal. In patients with non-complex cardiac defects, we saw a significant correlation between peak oxygen uptake and self-estimated exercise capacity (spearman-rho −0.30, p < 0.001), whereas in patients with complex cardiac defects, no correlation was found (spearman-rho −0.11, p < 0.255).

The mismatch between self-estimated and objectively assessed exercise capacity is most prominent in patients with complex CHD.

Registration number at Charité Universitätsmedizin Berlin Ethics Committee: EA2/106/14.

Transposition of the great arteries is the most common cyanotic cardiac lesion in newborns. Transposition of the great arteries without surgical correction is fatal during the first year of life. Contemporary outcome studies have shown that survival rates after surgery are excellent and most patients live to adulthood.

Woman with complex transposition of the great arteries with atrial and ventricular septal defects and subvalvular and valvular pulmonary stenosis, who has survived until the age of 31 years without surgery. The diagnosis was made by echocardiography and cardiac magnetic resonance. She underwent successful corrective surgical treatment after this age, by means of a Jatene operation.

In transposition of the great arteries patients, a high index of cases dies in the first month of life. Our case represents a natural history of the complex transposition of the great arteries. Non-invasive imaging studies are very useful for the diagnosis and follow-up of patients with transposition of the great arteries, especially echocardiography and cardiac magnetic resonance. In our case, the multimodality approach and the corrective surgery allowed her to survive.