Morbidity and mortality in adults with congenital heart defects in the third and fourth life decade

OBJECTIVES: The population of adults with congenital heart defects (ACHD) is continuously growing. Data on morbidity and mortality of ACHD are limited. This longitudinal observational study examined a group of ACHD with surgically corrected or palliated congenital heart defects (CHD) during a 15-year period. METHODS: ACHD that had participated in the initial study were invited for a follow-up examination. Mortality and hospitalization data were compared with a healthy control group. RESULTS: From 05/2017 to 04/2019 a total of 249/364 (68%) ACHD participated in the follow-up study: 21% had mild, 60% moderate and 19% severe CHD. During the observational period, 290 health incidents occurred (cardiac catheterization 37%, cardiovascular surgery 27%, electrophysiological study/ablation 20%, catheter interventional treatment 14%, non-cardiac surgery 3%). Events were more frequent in ACHD with moderate (53%) and severe (87%) compared to those with mild CHD (p < 0.001). 24 individuals died at a median age of 43 years during the observation period. 29% of them had moderate and 71% severe CHD corresponding to a mortality rate of 0%, 0.29% and 1.68% per patient-year in ACHD with mild, moderate and severe CHD. Long-term survival was significantly reduced in patients with severe CHD in comparison to individuals with mild and moderate CHD (p < 0.001). CONCLUSION: After correction or palliation of CHD, there was remarkable ongoing morbidity and mortality in ACHD patients over the 15-year observation period, particularly in individuals with moderate and severe CHD when compared with the general population. Thus, life-long special care is required for all surgically corrected or palliated ACHD patients. GRAPHICAL ABSTRACT: [Image: see text]