Cardiomyopathies (CMP) are uncommon during childhood thus there are only a few reports of children in the developed or developing world. We performed a retrospective analysis of our experience at the National Institute of Cardiovascular diseases at Karachi Pakistan, of children less than 12 years of age admitted during 1988-1999 with an echocardiographic diagnosis of CMP The purpose was to determine the clinical features and outcome during follow up. There were 82 children with CMP and records were available in 72. Hypertrophic cardiornyopathy (HCMP) was the largest group, containing 31 of 72 (43%) followed by endocardial fibroebstosis EFE 18 of 72(25%). Restrictive cardiomyopathy (RCMP) had 10 (13.9%) and dilated DCMP 7 (9.7%) while familial CMP comprised 4 (5,6%). Two were suspected to have storage disease. The mean age of HCMP was 8.2±3.3 years in 21 and less than I year in 10 while EFE were a mean of 6.5±2.3 months in 12 and greater than 12 months in 5, one was I day old. The mean age of RCMP was 8.4±4.6 years and FCMP were mean 7.6±2.5 yeas and DCMP 5.3±2.1 years old and storage disease group was 7 months and 8 year age. Clinical and echocardiographic features were distinctive for each hemodynamic type. Twenty six of 72 (30%) were lost of follow up after discharge from the hospital and 46 (63.9%) were followed for a mean of 26 months (0.3-180 months). 19.6% died 13% in HCMP, 33.3% in EFE, 25% RCMP and 25% in DCMP Most deaths occurred early after admission. The cause of death was severe left ventricular dysfunction in majority. RCMP children were severely symptomatic with CHF and pulmonary hypertension. It is concluded that clinical features and echocardiography could be used to diagnose most of the CMP types during childhood and the prognosis was best for older children with HCMP.
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