The study sought to analyze late causes of death after congenital cardiac surgery by era and defect severity.
We obtained data from a nationwide pediatric cardiac surgery database and Finnish population registry regarding patients who underwent cardiac surgery at <15 years of age at 1 of 5 universities or 1 district hospital in Finland from 1953 to 2009. Noncyanotic and cyanotic defects were classified as simple and severe, respectively. Causes of death were determined using International Classification of Diseases diagnostic codes. Deaths among the study population were compared to a matched control population.
Overall, 10,964 patients underwent 14,079 operations, with 98% follow-up. Early mortality (<30 days) was 5.6% (n = 613). Late mortality was 10.4% (n = 1,129). Congenital heart defect (CHD)–related death rates correlated with defect severity. Heart failure was the most common mode of CHD-related death, but decreased after surgeries performed between 1990 and 2009. Sudden death after surgery for atrial septal defect, ventricular septal defect, tetralogy of Fallot, and transposition of the great arteries decreased to zero following operations from 1990 to 2009. Deaths from neoplasms, respiratory, neurological, and infectious disease were significantly more common among study patients than controls. Pneumonia caused the majority of non–CHD-related deaths among the study population.
CHD-related deaths have decreased markedly but remain a challenge after surgery for severe cardiac defects. Premature deaths are generally more common among patients than the control population, warranting long-term follow-up after congenital cardiac surgery.