Clinical and echocardiographic patterns and outcomes of Sudanese children with cardiomyopathy.

Abstract

Cardiomyopathy (CMP) constitutes a group of diseases of heart muscle that lead to significant mortality and morbidity, with limited data in Africa. This study aims to describe clinical and echocardiographic (echo) patterns and short-term outcomes of Pediatric CMP. The study was carried at Gaafar Ibnauf Children's Hospital and included all children 0-18 years with an echo diagnosis of CMP. A retrospective part of the study was carried from January 2013 to 2019 and a prospective part from January to June 2019. Clinical and echo data were collected on presentation and on at least one follow up. A total of 146 patients were diagnosed with CMP during the study period. Most patients (48%) presented at 1-6 years of age, neonatal CMP was present in 4%. Familial incidence was detected in 11% of cases. Heart failure was the most common mode of presentation in 96% of patients; 65% needed hospitalisation; a third of them needed intensive care unit admission. The most common type of CMP was the dilated type (67%). Others included noncompaction CMP (19%), restrictive CMP and hypertrophic CMP (each of them in 6% of patients). Hospital mortality was 20% for the whole cohort and 100% for neonates. Other complications occurred in 21% of patients including arrhythmias, cerebrovascular accidents and end stage renal disease. Echo follow up showed that most patients (52%) remained the same, 26% worsened and 21% improved. Genetic and metabolic studies that could help to improve management and outcomes of CMP are needed.