پیگیری و سرنوشت 115 کودک مبتلا به کاردیومیوپاتی اتساعی اولیه

    Background & Aim: In childhood, dilated cardiomyopathy has a prevalence of 36 in 100000. Its prognosis is one of the most important matters. The chance of 5-year survival is reported to be around 50% and 10-year one around 25%. We aimed to study the prognosis, treatment, and mortality of dilated cardiomyopathy patients in Iran. Patients & Method: In this descriptive study, the medical records of all infants and children with dilated cardiomyopathy hospitalized between 1990 and 2004 were reviewed. Results: Among 115 patients, 67(58%) were boys and 48(42%) were girls, with a mean age of 8.4 years(ranging from 3 months to 16 years) and mean weight of 21.5 Kg. The most common clinical manifestation was respiratory distress in 71.3% and congestive heart failure(CHF) in 62% of the patients. Ninety-four(82%) were discharged after medical therapy with a combination of digitalis, furosemide and angiotensin-converting enzyme inhibitor(captopril). Twenty-one patients(18%) died in the picture of severe CHF and dysrhythmias during the first admission. A mean follow-up of 6 years(ranging from 0 to 13 years) revealed that 56% were still alive. Conclusion: The prognosis is still poor especially for those under 2 years of age or with CHF or dysrhythmias, but new attempts have been made to use different therapeutic modalities like L-carnitine, growth hormone, intravenous immunoglobulin, cardiac transplantation, and new surgical approaches in pediatric patients.