Prevalence, clinical profile and outcome of children with cardiomyopathy

Background: Cardiomyopathies are myocardial disorders in which the heart muscle is structurally and functionally abnormal, thereby affecting the ventricular systolic function and/or diastolic function. Comprehensive approach to management warrants recognizing the clinical profile in order to strategize to improve outcomes. Aim: To evaluate the types of cardiomyopathies, presentation and outcome among children admitted into a tertiary hospital in Sokoto. Methods. A hospital based prospective study of children aged below 15 years who were seen over an 8.5-year period. A proforma was used to collect the information on their socio-demographics, type of cardiomyopathy, associated presentations and outcome. Data was analysed using IBM SPSS version 25. Results: 71 out of 78,321 children seen that period (0.1%) were diagnosed with CM giving a hospital prevalence of 90.7/100,000. They comprised 40 (56.3%) males and 31 (43.7%) females. Age ranged from 1 month to 15 years (6.2 ± 3.8 years). Dilated CM (DCM) was the commonest in 63 (88.7%), restrictive CM (endomyocardial fibrosis-EMF) in 5 (7.0%) and hypertrophic (HCM) in 3 (4.2%). Forty-six (73%) of DCM had severe systolic dysfunction. DCM also occurred in 2 siblings of 2 patients on follow up. Two of the patients with EMF had recurrent pericardial effusions and all the patients with HCM died in infancy. Thirty (42.2%) of the total patients were lost to follow-up while 27 (38.0%) died. Conclusion: Dilated cardiomyopathy followed by restrictive type are the commonest types seen among children in Sokoto. It is associated with high morbidity and mortality.