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Paediatric dilated cardiomyopathy: clinical profile and outcome. The experience of a tertiary centre for paediatric cardiology

Published online by Cambridge University Press:  15 January 2014

Joana O. Miranda ,
Liane Costa ,
Esmeralda Rodrigues ,
Elisa L. Teles ,
Maria J. Baptista  and
José C. Areias
Joana O. Miranda*
Affiliation:
Department of Paediatric Cardiology, Centro Hospitalar São João, Porto, Portugal
Liane Costa
Affiliation:
Department of Pediatrics, Centro Hospitalar São João, Porto, Portugal
Esmeralda Rodrigues
Affiliation:
Department of Pediatrics, Metabolic Diseases Unit, Centro Hospitalar São João, Porto, Portugal
Elisa L. Teles
Affiliation:
Department of Pediatrics, Metabolic Diseases Unit, Centro Hospitalar São João, Porto, Portugal
Maria J. Baptista
Affiliation:
Department of Paediatric Cardiology, Centro Hospitalar São João, Porto, Portugal
José C. Areias
Affiliation:
Department of Paediatric Cardiology, Centro Hospitalar São João, Porto, Portugal
*
Correspondence to: J. O. Miranda, Department of Paediatric Cardiology, Centro Hospitalar São João. Alameda Prof. Hernâni Monteiro, 4202–451 Porto, Portugal. Tel: +351 225512100; Fax: +351225512273; E-mail: [email protected]
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Abstract

Dilated cardiomyopathy is the most common form of cardiomyopathy in the paediatric population and an important cause of heart transplantation in children. The clinical profile and course of dilated cardiomyopathy in children have been poorly characterised. A retrospective review of 61 patients (37 female; 24 male) diagnosed with dilated cardiomyopathy from January, 2005 to June, 2012 at a single institution was performed. The median age at diagnosis was 15 months. Heart failure was present in 83.6% of patients and 44.3% required intensive care. The most prevalent causes were idiopathic (47.5%), viral myocarditis (18.0%) and inherited metabolic diseases (11.5%). In viral myocarditis, Parvovirus B19 was the most common identified agent, in concurrence with the increasing incidence documented recently. Inherited metabolic diseases were responsible for 11.5% of dilated cardiomyopathy cases compared with the 4–6% described in the literature, which reinforces the importance of considering this aetiology in differential diagnosis of paediatric dilated cardiomyopathy. The overall mortality rate was 16.1% and five patients underwent heart transplantation. In our series, age at diagnosis and aetiology were the most important prognosis factors. We report no mortality in the five patients who underwent heart transplantation, after 2 years of follow-up.

Keywords

Dilated cardiomyopathyheart failurepaediatric cardiologyheart transplantmyocarditismetabolic disease
Type
Original Articles
Information
Cardiology in the Young , Volume 25 , Issue 2 , February 2015 , pp. 333 - 337
Copyright
© Cambridge University Press 2014 

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