OBJECTIVES:
Adults with congenital heart defects (ACHD) are a continuously increasing and aging population. Up to now, there is limited data on long-term morbidity of these patients. The purpose of this study was to examine the long-term course of patients with surgically corrected or palliated congenital heart defects over a 15-year-period.

PATIENTS and METHODS:
The current study was based on a previous study at our tertiary pediatric cardiology and ACHD referral center from 2003 to 2004 on 364 patients with a corrected or palliated congenital heart defect (CHD). For the current study, all patients from our initial study were invited to participate at this follow-up examination.

RESULTS:
A total of 249 of the 364 (68%) individuals from the initial study could be recruited. Median age was 40.1±8.5 (range: 27-60) years and 105/249 (42%) were female. 52/249 (21%) individuals had a simple, 150/249 (60%) a moderate and 47/249 (19%) a severe CHD, respectively. Severity of CHD was as follows: 13/48 (27) simple, 73/142 (51%) moderate and 41/47 (87%) severe CHD. Within the 15-year period hospitalization for any cardiac reason was required in 127/249 (51%) patients. The hospitalization rate increased significantly from simple to moderate and to severe CHD (p<0.003, p<0.001, respectively). Most frequently, patients with Fontan circulation, d-transposition of the great arteries (dTGA) and tetralogy of Fallot (TOF) needed in-hospital treatment. Of the 127 patients, cardiac surgery was performed in 62%, catheterization in 83% with interventional procedures in 31% and electrophysiological study in 45%. As before, need for interventions was positively correlated with complexity of CHD (p<0.001). 4.8% of all study participants developed infective endocarditis, and again patients with moderate and severe CHD were most frequently affected. Furthermore, 3.7% of the study participants suffered from pulmonary arterial hypertension (PHT). During the observational period, the number of patients who had to take cardiac medication increasd significantly (from 42% to 68%; p<0.001) as well as the quantity of medication per patient (from 0.64 to 1.6; p<0.001). The number of patients on cardiac medication or anticoagulation doubled during the observational period. The three most frequently prescribed substances included ACE-inhibitors/ARB, ß-blockers and antiarrhythmics. The quantity of drugs had significantly increased in patients with moderate (p<0.001) and severe (P<0.001) CHD. 

CONCLUSIONS:
Over the 15-year study period on adults with corrected or palliated CHD, patients developed a remarkable morbidity, particularly in moderate and severe CHD. Number of patients requiring inpatient treatment for any cardiac causes increased with complexicity of CHD as well as the risk of catheter intervention or cardiac surgery, infective endocarditis and PHT. Nevertheless, even patients with simple CHD developed cardiac-related morbidity. According to these findings, lifelong special care is required for all surgically corrected or palliated ACHD patients.