Pediatric idiopathic dilated cardiomyopathy: A single center experience.

CONTEXT: Idiopathic dilated cardiomyopathy (IDCM) is a severe illness with high mortality in the pediatric population.

AIMS: To highlight our experience about clinical course and outcome of IDCM.

SETTINGS AND DESIGN: Patients' files were reviewed retrospectively for diagnosed cases of IDCM in the pediatric cardiology unit of King Abdul Aziz University Hospital, Jeddah, Saudi Arabia, from Jan 2003 to Jun 2011.

MATERIALS AND METHODS: Data about full history, clinical examination and investigations were recorded and grouped according to outcome as alive and well (group 1), alive and symptomatic (group 2) and worsened or dead (group 3).

STATISTICAL ANALYSIS: Data was subjected to descriptive analysis. Chi-square and Student's paired t-test techniques were used where appropriate. Spearman rank correlation and survival analysis was done.

RESULTS: Eighty three patients were included with presenting age median (range), i.e.,14 (2 months-12 years) with females predominance 53 (63.9%). On presentation; cardiomegaly was noted in 72 (86.7%) with increased lung vascularity in 45 (54%). Sixty-one (74%) patients had ST segment and T-wave changes on electrocardiogram, while the same number had left ventricular hypertrophy, and 15 (18%) had arrhythmias. Echocardiography records on presentation and at last follow-up showed significant difference in several areas. Group 1 had 40 (48.2%), Group 2 had 23 (27.7%) while 20 (24.1%) were in Group 3 including nine cases who died. Survival rate over three years was 78%. Older the age, worse was the outcome (Spearman's rho = 0.3, P = 0.04).

CONCLUSION: Majority of subjects were presented during first year of life; the three year survival rate was 78%. Favorable outcome was correlated with younger age at presentation.