Childhood Cardiomyopathies: A Study in Tertiary Care Hospital in Upper Egypt

INTRODUCTION: Cardiomyopathy (CMP) is defined by the World Health Organization (WHO) as a disease of the myocardium associated with cardiac dysfunction. An understanding of CMP is very important, as it is a common cause of heart failure in children, and the most common indication for heart transplantation in children older than one year, but data on CMP in Egypt are scarce. The aim of this study was to determine the number, risk factors, clinical presentation, complications and outcome of different types of childhood cardiomyopathies in Sohag University Hospital. METHODS: This cross-sectional hospital based study enrolled fifty children with Cardiomyopathy in Pediatric Departments, Neonatal Care Units, and Cardiac Outpatient Clinics in Sohag University Hospital from March 01, 2014 to February 28, 2015. RESULTS: Cases with Dilated Cardiomyopathy (DCMP) were 38 (76%), and those who had Hypertrophic Cardiomyopathy (HCMP) were 12 (24%). Dyspnea was the most common presenting complaint in 71% of cases. In cases with DCMP, the mean EF was 33.8, and FS was 17.11, while in cases with HCMP, the mean EF was 70.75, FS was 37. Fifty percent of cases were found to have moderate to severe PHT. Serum CK-MB was elevated in 3 (6%) cases, while serum Troponin I was elevated in 2 (4.2%) cases who diagnosed as having myocarditis. Viral myocarditis was the most common identified etiological agent responsible for 14 (37%) cases with DCMP. CONCLUSIONS: CMP represents a considerable percentage of children with cardiac disorders. DCMP is the most common type, usually presented with congestive heart failure, and the most common cause is myocarditis. L-Carnitine profile was normal in all cases, despite its routine use. Pediatricians need to raise their clinical suspicion to CMPs, as atypical presentations are not uncommon. To do screening for other family members, cardiac enzymes (CK-MB, Troponin I) have to be done in all newly diagnosed CMP cases, along with a revision of the routine prescription of L-Carnitine.