Clinical profiles and outcomes for Omani children with dilated cardiomyopathy seen in a regional referral hospital

Abstract

To provide an account of paediatric dilated cardiomyopathy as seen in a region of Oman, analysing the data from 32 consecutive children who received care in our unit between January, 1999, and August, 2007. The patients, of whom 17 were male, were aged between 5 weeks and 8 years at presentation, with a median of 7 months. The disease was deemed to be myocarditis-induced in one-third, and idiopathic in half. Cardiac failure, seen in almost four-fifths, was the most frequent presenting feature. Correspondingly, the cardiothoracic ratios were increased, to a mean of 68% in 20 infants, and to 65% in 8 older children, and the left ventricular ejection fraction depressed, to a mean of 41%, in the 23 patients in whom it could be evaluated. Patients in cardiac failure received various combinations of diuretics, inotropes, and captopril. In addition, 6 received carvedilol, and 3 intravenous immunoglobulin. Death occurred in 2 patients shortly after admission, one left the hospital against medical advice, and the remaining 29 were followed-up for a mean of 37 months, with a range from 2 to 102 months. Recovery was noted in one-third of the patients, with one-quarter showing improvement but still requiring anti-failure medications. Slightly over two-fifths died. Of those with the idiopathic form, 40% died, with death occurring in 46% of those deemed to have myocarditis-induced disease, in half of those presenting in infancy, and in 57% of those who presented in cardiac failure. Dilated cardiomyopathy was often severe in our patients, albeit that the cause was frequently uncertain, and the response to standard anti-failure treatment unsatisfactory. Efforts should be intensified for unravelling its aetiology and improving medical treatment.