Long-term prognosis of congenital heart defects: a systematic review

In the large and expanding population of adults with congenital heart disease, little is known about their long-term outcome. By means of a systematic literature search, we aimed to assess the quantity and quality of data on long-term survival and morbidity in adults with common congenital heart defects.

All literature on MEDLINE from January 1980 to January 2007 was searched, using a broad range of keywords for atrial septal defect, ventricular septal defect, pulmonary stenosis, tetralogy of Fallot, aortic coarctation, and transposition of the great arteries. After study selection using pre-specified criteria and quality assessment, data were extracted and weighed according to number of patients. Pooled estimates were obtained.

We identified 322 articles. Selection yielded 35 articles comprising 7894 patients. Data on survival were mainly available up until 40 years of age. In this young population, survival varied from 87.4% in aortic coarctation to 99.6% in atrial septal defect. Data on morbidity were sparse. Based on these data, morbidity was substantial.

Until the age of 40 years, survival is decreased in patients with congenital heart defects, albeit most pronounced among patients with complex heart defects. Moreover, morbidity is considerable in all defects. Sufficient data on long-term survival and morbidity beyond the age of 40 years are lacking, yet crucial for optimal clinical care.