Predictive factors for long-term prognosis in adults with cyanotic congenital heart disease — Japanese multi-center study

Objective Adults with cyanotic congenital heart disease (CCHD) are associated with a significant incidence of morbid events and premature deaths that may be predicted during childhood. We aimed to identify predictive factors related to long-term prognosis through a Japanese multi-center cross-sectional study. Methods Data were collected from 253 adults with CCHD (126 men; age 28 (18 to 56) years) from 15 participating centers between 1998 and 2003. Laboratory data such as cardiothoracic ratio (CTR), percutaneous oxygen saturation (SpO2), hematocrit levels (Ht) and platelet counts (Pl-c) at the age of 15 years were collected for predictive factor analysis for death and cardiovascular and systematic events. Predictive factors were determined by multivariate Cox regression analysis. Results After a mean follow-up of 21 (0–42) years, 23 patients died with a median age of 29 (18–54) years (heart failure in 8, sudden in 6 and systematic complications in 9). Survival since 18 years of age was 91% and 84% at 10 and 20 years, respectively. Significant predictive factor for death was Pl-c < 130 × 109/l and for renal failure (n = 7) was Ht > 65%. 162 patients were hospitalized and predictors for hospitalization due to heart failure (n = 45) were common atrioventricular canal CTR > 60% and Pl-c < 100 × 109/l and that due to arrhythmias (n = 44) were systematic right ventricle and CTR > 60%. Conclusions This multi-center study provides an objective basis of assessing the long-term prognosis in patients with CCHD. These data are useful in making decisions regarding medical management and in favorably altering the non-operative course of the disease.