Outcome Factors of Idiopathic Dilated Cardiomyopathy in Children - a Long-term Follow-up Review

Background: Idiopathic dilated cardiomyopathy in children has a high rate of mortality. Cardiac transplantation is the treatment of choice in those who fail to respond to therapeutics. Several studies have been carried out to determine unfavourable prognoses, and to provide an early indication for cardiac transplantation. Nevertheless, no consensus has been reached on the matter.

Objective: To propose predictors of death in children with idiopathic dilated cardiomyopathy.

Methods: We reviewed data extending over 22 years from 142 consecutive children with idiopathic dilated cardiomyopathy, of whom 36 died. The criteria for inclusion were the presence of congestive heart failure or cardiomegaly in a routine chest X-ray, confirmed by enlargement and hypo kinesis of the left ventricle in the echocardiogram. We included asymptomatic children in functional class I. Based on Cox's analysis of clinical and laboratory data, we sought any predictors of death.

Results: In univariate analysis, the predictors were functional class IV at presentation (p equal to 0.0001), dyspnoea (p equal to 0.0096), and reduced pedal pulses (p equal to 0.0413). In chest X-ray, they were maximal cardiothoracic ratio (p equal to 0.0001) and pulmonary congestion (p equal to 0.0072). In the electrocardiogram, right atrium overload (p equal to 0.0118), ventricular arrhythmias (p equal to 0.0148) and heart rate (p equal to 0.027). In the echocardiogram, mitral regurgitation of grade 3 to 4 (p equal to 0.002), the left atrial to aortic ratio (p equal to 0.0001), and left ventricle ejection fraction (p equal to 0.0266). In multivariate analysis, the independent predictors were maximum cardiothoracic ratio (p equal to 0.0001), left ventricle ejection fraction (p equal to 0.0013), mitral regurgitation of grade 3 or 4 (p equal to 0.0017), functional class IV at presentation (p equal to 0.0028), and ventricular arrhythmias (p equal to 0.0253).

Conclusion: Children, who have these predictors of death should be considered for early heart transplantation when no improvement is observed in clinical treatment.