As an increasing number of patients with congenital heart disease reach adu
lthood, more information is needed regarding outcomes. The first signs of i
mpaired heart function may appear during exercise testing. The aim of the p
resent study was to establish mean values for maximal oxygen uptake in adul
ts with various congenital heart diseases. Patients from 6 major diagnostic
groups were identified, including patients with atrial septal defect (ASD,
n = 93), transposition of the greet arteries corrected with the Mustard pr
ocedure (n = 84), congenitally corrected transposition of the great arterie
s (CCTGA, n = 41), Tetralogy of Fallot (n = 168), Ebstein's anomaly (n = 37
), and Modified Fontan procedure (n = 52). Diminished maximal oxygen uptake
was found in all diagnostic groups across age compared with healthy subjec
ts. A significant decrease in maximal oxygen uptake with aging was found in
those with ASD (p <0.0001), CCTGA (p = 0.01), and Tetralogy of Fallot (p <
0.0001). There was no significant decline, however, in Ebstein's anomaly (p
= 0.270), Fontan procedure (p = 0.182), and in the Mustard patients (p = 0
.188). All patients achieved significantly lower heart rates than predicted
(mean for all groups, p <0.0001). Forced vital capacity values (3.51 L, me
an SD +/- 1.02) were lower than predicted values (4.10 L, mean SD +/- 0.90,
p <0.0001) for all patients groups except those with ASD. Mean values, how
ever, were within the accepted 20% range of variance. This study showed dim
inished aerobic capacity in all diagnostic groups when compared with a heal
thy population. The maximal oxygen uptake values across age groups can be u
sed as reference values in patients with similar diagnoses and as the basis
for further research. (C) 2001 by Excerpta Medica, Inc.