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G80 Presence and Progression of Idiopathic Dilated CaRdiomyopathy

Aim Idiopathic Dilated cardiomyopathy (iDCM) is an uncommon presentation in children and young people but with significant and quite often poor prognosis. We aim to look at the presence and progression of iDCM. Material and Methods 12 year retrospective analysis of data in a single centre; all patients with the term “DCM” were reviewed from the cardiac database (36 patient). A diagnosis was made on clinical and echocardiographic criteria. We excluded any patient where a cause was identified, analysed sequential echocardiograms and ECGs, and evaluated their progression and outcome. Results There were 24 patients with iDCM; 15 boys and 9 girls. Presentation age range was Birth – 16 years years (mean: 5 years 8 months). Children/adolescents were followed up for an average of 80-months (range: 6 – 143 months). We measured LV dimensions and ventricular function and “z” scores calculated to track growth along with valvar lesions. 22 were on medication; b-blockers in 16, and 21 had an ACE inhibitor/ARB. 6 had additional medication in the form of Spironolactone and (12 ) diuretics. Anti-platelet (12)) use was also studied. 3 underwent transplant and 2 were referred for a transplant and adult cardiology review. 3 patients who had CRT. Ventricular function was tracked to the point of the last review. Clinical improvement was noted on NYHA criteria. We had an adverse outcome (death/transplant) in 17%, death in 4 with 1 going on to have a transplant within this period. Of the survivors, we tracked their NYHA grade at the last review – (I, through IV). The fractional shortening was (7 – 28%) at presentation, (mean 18%), and 10 – 34% at discharge (mean 25%). Of those who died, the mean FS was 17%, and 28% in the survivors. LV diastolic dimension (LVDd, z scored) similarly was noted at presentation and at last review. Conclusion We found that there was an adverse outcome in 17%. Actuarial survival was 100% at 1 year and 8.3% at 5 years with the best outcomes noted in younger age groups and in those where there was an improvement in systolic function.