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Dilated cardiomyopathy presenting in childhood: aetiology, diagnostic approach, and clinical course*

Published online by Cambridge University Press:  20 September 2010

Valentina Gesuete ,
Luca Ragni ,
Daniela Prandstraller ,
Guido Oppido ,
Roberto Formigari ,
Gaetano D. Gargiulo  and
Fernando M. Picchio
Valentina Gesuete*
Affiliation:
Pediatric Cardiology Unit, S.Orsola-Malpighi Hospital, via Massarenti 9, 40138 Bologna, Italy
Luca Ragni
Affiliation:
Pediatric Cardiology Unit, S.Orsola-Malpighi Hospital, via Massarenti 9, 40138 Bologna, Italy
Daniela Prandstraller
Affiliation:
Pediatric Cardiology Unit, S.Orsola-Malpighi Hospital, via Massarenti 9, 40138 Bologna, Italy
Guido Oppido
Affiliation:
Pediatric Cardiac Surgery Unit, S.Orsola-Malpighi Hospital, via Massarenti 9, 40138 Bologna, Italy
Roberto Formigari
Affiliation:
Pediatric Cardiology Unit, S.Orsola-Malpighi Hospital, via Massarenti 9, 40138 Bologna, Italy
Gaetano D. Gargiulo
Affiliation:
Pediatric Cardiac Surgery Unit, S.Orsola-Malpighi Hospital, via Massarenti 9, 40138 Bologna, Italy
Fernando M. Picchio
Affiliation:
Pediatric Cardiology Unit, S.Orsola-Malpighi Hospital, via Massarenti 9, 40138 Bologna, Italy
*
Correspondence to: V. Gesuete, MD, Pediatric Cardiology Unit, S.Orsola-Malpighi Hospital, via Massarenti, 9, 40138 Bologna, Italy. Tel: (+39)-051-6363435, (+39)-333-4993760; Fax: (+39)-051-6363116; E-mail: [email protected]
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Abstract

Objective

To determine the outcome of dilated cardiomyopathy presenting in childhood and the features that might be useful for prognostic stratification.

Methods

Retrospective study of 41 consecutive children affected by dilated cardiomyopathy – aged 0–14 years; median 33.4 plus or minus 49.25 – between 1993 and 2008. We reviewed the medical history to determine age at diagnosis, family history, previous viral illness, aetiology, symptoms and signs at presentation, treatment, and outcome. The diagnosis was made on the basis of cardiomegaly and evidence of poor left ventricular function by echocardiography. We also carried out a metabolic evaluation including blood lactate, pyruvate, carnitine, amino acids, urine organic acids, assessment of respiratory chain enzymes, and analysis of histopathological material. Survival curves were constructed by the Kaplan–Meier method.

Results

Follow-up ranged from 10 days to 162 months – median 45.25 plus or minus 41.15 months. Freedom from death or cardiac transplantation was 68.3% at 5 years. The primary end-point of death/cardiac transplantation was associated with the need for intravenous inotropic support. A trend towards a poorer prognosis was found for age at diagnosis of more than 5 years and for a metabolic aetiology of dilated cardiomyopathy. For the children affected by cardiomyopathy as part of a multi-system involvement, mortality was 50%.

Conclusions

In children, dilated cardiomyopathy is a diverse disorder with outcomes that depend on cause, age, and cardiac failure status at presentation. Overt cardiac failure at presentation is a major prognostic factor for death or cardiac transplantation. Older age at presentation and metabolic aetiology may be associated with a poorer prognosis.

Keywords

Cardiomyopathycardiac failureoutcome
Type
Original Articles
Information
Cardiology in the Young , Volume 20 , Issue 6 , December 2010 , pp. 680 - 685
Copyright
Copyright © Cambridge University Press 2010

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Footnotes

*

Our experience with paediatric dilated cardiomyopathy.

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