Dilated cardiomyopathy in children: Review of all presentations to a children's hospital over a 5-year period and the impact of family cardiac screening

Abstract

Aim: This study aims to report the aetiology and outcome of dilated cardiomyopathy (DCM) in children from New Zealand and to assess the outcome of protocolised family cardiac investigation introduced in 2007.

Methods: Retrospective review of all patients with DCM, aged <15 years, presenting to the children's hospital between 01/01/2007 and 01/03/2012. Oncology patients were excluded. Family screening results were assessed.

Results: Thirty-five patients were identified. Median age 1 year: 26/35 (74%) <5 years. Ethnicity: New Zealand European 14/35 (40%), Maori 11/35 (31%), Pacific Island 5/35 (14%) and other 5/35(14%). Excluding the four cases with biopsy-proven myocarditis, family cardiac screening was documented in 15/31 (48%). Of these, 7/15 (47%) had a positive family screen. Six previously undiagnosed DCM cases were found in these families. Five of the seven familial DCM cases had evidence of viral infection at presentation. Final aetiology: Myocarditis, 15/35 (43%), with Parvovirus being the commonest virus identified 10/15(67%). Familial, seven (20%), Idiopathic, seven (20%), Others, six (17%). Overall outcome was death 10/35(28%), ongoing dysfunction 16/35 (46%) and normalisation in 9/35 (26%). The median time to death from diagnosis was 1.5 months (3 days to 3 years). Death occurred in 4/7 with familial DCM (57%) compared with 6/28 (21%) among the others (ns). Recovery occurred in 0/7 with familial DCM versus 4/4 with biopsy proven myocarditis (P < 0.05).

Conclusions: Almost half of families screened had evidence of familial DCM, and these children carried a worse prognosis. Maori and Pacific Island children seem to be over-represented. Family investigation led to the identification of many pre-symptomatic individuals even when viral aetiology was suspected initially.

Keywords: familial; myocarditis; paediatric dilated cardiomyopathy; screening.