Objective: To analyze the prognostic value of cardiomegaly, pulmonary congestion and cardiothoracic ratio as indicators of death and survival in children with idiopathic dilated cardiomyopathy.
Methods: We carried out a retrospective review of 152 patients with idiopathic dilated cardiomyopathy diagnosed between September 1979 and March 2003. In the first 72 months, 722 exams were performed (100 in the first 15 days).
Statistical Analysis: chi-square, Student's t test, ANOVA and Kaplan-Meier curves. Alpha = 0.05; beta = 0.80.
Results: The mean age at presentation was 2.2+/-3.2 years. Idiopathic dilated cardiomyopathy incidence was higher in children younger than 2 years (76.3% - 95% CI = 68.7% to 82.8%) (p < 0.0001). Sex (p = 0.07) and color (p = 0.11) were not significant and mortality was not influenced by age (p = 0.73), sex (p = 0.78) or color (p = 0.20). Most patients were severely ill (84.2% - 95% CI = 77.4% to 89.6%; functional class III and IV; p < 0.0001). All 43 deaths occurred in this group (p = 0.0008). Cardiomegaly at presentation was observed in 94.1% (95% CI = 89.1% to 97.2%) (p < 0.0001), and pulmonary congestion in 75.6% (95% CI = 68.0% to 82.2%) (p < 0.0001). Pulmonary congestion and cardiomegaly were more frequent in functional class III/IV patients (RC = 8.03 - 95% CI = 2.85% to 23.1%) (p < 0.0001). Pulmonary congestion was a marker of death (RC = 3.16 - 95% CI = 1.06% to 10.07) (p = 0.0222), but not cardiomegaly (p = 0.1185). Survival was influenced by both cardiomegaly (p = 0.0189) and pulmonary congestion (p = 0.0050). Mean and maximum cardiothoracic ratio were higher in the death group (0.749+/-0.053 vs. 0.662+/-0.080) (p < 0.0001) and (0.716+/-0.059 vs. 0.620+/-0.085) (p < 0.0001). ANOVA revealed a progressive decrease in cardiothoracic ratio in the survival group (p < 0.0001).
Conclusions: In children with idiopathic dilated cardiomyopathy, the presence of pulmonary congestion at presentation and increased cardiothoracic ratio are associated with poor survival.
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