This study tested the hypothesis that patients with a congenital heart disease are sensitive regarding heartbeat perception, reflected in enhanced attention for heartbeat, estimation of own heart rate, and a vulnerability to become anxious by listening to heartbeat sounds. Twenty adults with a congenital heart disease, and 20 healthy controls conducted 3 experimental tasks: a concentration task during distraction by heartbeat sounds, own heart rate estimation, and exposure to different patterns of heartbeat sounds. The results showed that patients were more distracted by heartbeat, and were also worse at estimating heart rate than controls. However, heartbeat sounds did not evoke anxiety. In conclusion, patients with a congenital heart disease may differ from controls in heartbeat perception, but there was no support for obsessive monitoring for heartbeat or excessive reactions to heartbeat sounds.