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.