Publication:

A study of clinical profile and outcome in children with dilated cardiomyopathy

Background Objective was to analyse the clinical profile of children diagnosed with Dilated cardiomyopathy and to correlate the same with the severity and functional status on 2-D echocardiography.

Methods It was a prospective, retrospective and observational study. A total of 150 subjects were selected from patients visiting the cardiology OPD, admitted in medical wards, paediatric Intensive Care Unit of a tertiary care referral centre. The demographic profile, symptomatology, heart functions and compliance were recorded. The cases were evaluated by detailed history, general and cardiovascular examination and laboratory investigations along with 2D echocardiography.

Results The demographic profile is significant as the age at diagnosis predicts the risk of mortality with older children having the worst outcome. A significant association observed between decreasing ejection fraction and increasing mortality with a p value (p=0.02). Signs of pedal edema and hepatomegaly are associated with unfavourable outcome with a significant p value of (p=0.03). The overall prognosis of children with Dilated cardiomyopathy steadily improved with timely use of appropriate medications. Only 1 patient in this study had undergone orthotopic heart transplant at the age of 15 years (case of chemotherapy induced Dilated cardiomyopathy).

Conclusions Dilated cardiomyopathy is a difficult entity to treat in children as most cases are idiopathic and the therapy remains mostly supportive. Hypovitaminosis-D and hypocalcemia are common treatable causes of dilated cardiomyopathy in children and must be ruled out in all cases of dilated cardiomyopathy. Patients refractory to medical therapy should be considered for ventricular assist devices and orthotopic heart transplantation.