Olufemi Jaiyesimi1, Mohamed Kasem. 1. Department of Paediatrics, Regional Referral Hospital, Nizwa, Oman. efjay@omantel.net.om
Abstract
OBJECTIVE: 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. RESULTS: 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. CONCLUSION: 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.
OBJECTIVE: 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. RESULTS: 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. CONCLUSION: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.