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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.

References

1. Elliott, P, Andersson, B, Arbustini, E, et al. Classification of the cardiomyopathies: a position statement from the European society of cardiology working group on myocardial and pericardial diseases. Eur Heart J 2008; 29: 270–276.CrossRefGoogle Scholar
2. Soler, R, Rodríguez, E, Remuiñán, C, Bello, J, Díaz, A. Magnetic resonance imaging of primary cardiomyopathies. J Comput Assist Tomogr 2003; 27: 724–734.CrossRefGoogle ScholarPubMed
3. Arola, A, Jokinen, E, Ruuskanen, O, et al. Epidemiology of idiopathic cardiomyopathies in children and adolescences. A nationwide study in Finland. Am J Epidemiol 1997; 146: 385–393.Google Scholar
4. Cox, GF, Sleeper, LA, Lowe, AM, et al. Factor associated with establishing a causal diagnosis for children with cardiomyopathy. Pediatrics 2006; 118: 1519–1531.Google Scholar
5. Caso, P, Cioppa, C, Musto, B, De Leva, F, Vitale, D, Calabrò, R. Aspetti clinici della cardiomiopatia dilatativa in età pediatrica. Cardiologia 1990; 35: 839–844.Google Scholar
6. Bostan, OM, Cil, E. Dilated cardiomyopathy in childhood: prognostic features and outcome. Acta Cardiol 2006; 61: 169–174.Google Scholar
7. Helton, E, Darragh, R, Francis, P, et al. Metabolic aspect of myocardial disease and a role for L-carnitine in the treatment of childhood cardiomyopathy. Pediatrics 2000; 106: 623.Google Scholar
8. Allen, HD, Gutgesell, HP, Clark, EB, Driscoll, DJ. Moss and Adams. Heart Disease in Infants, Children and Adolescents Including the Fetus and Young Adult. Williams and Wilkins; 2008.Google Scholar
9. McMahon, CJ, Nagueh, SF, Eapen, RS, et al. Echocardiographic predictors of adverse clinical events in children with dilated cardiomiopathy: a prospective clinical study. Heart 2004; 90: 908–915.CrossRefGoogle Scholar
10. Talercio, CP, Seward, JB, Driscoll, DJ, Fisher, LD, Gersh, BJ, Tajik, AJ. Idiopathic dilated cardiomyopathy in the young: clinical profile and natural history. J Am Coll Cardiol 1985; 6: 1126–1131.Google Scholar
11. Griffin, ML, Hernandez, A, Martin, TC, et al. Dilated cardiomyopathy in infants and children. J Am Coll Cardiol 1988; 11: 139–144.CrossRefGoogle ScholarPubMed
12. Burch, M, Siddiqi, SA, Celemajer, DS, Scott, C, Bull, C, Deanfield, JE. Dilated cardiomyopathy in children: determinants of outcome. Br Heart J 1994; 72: 246–250.CrossRefGoogle ScholarPubMed
13. Towbin, JA, Lowe, AM, Colan, SD, et al. Incidence, causes, and outcomes of dilated cardiomyopathy in children. JAMA 2006; 296: 1867–1876.CrossRefGoogle ScholarPubMed
14. Badertscher, A, Bauersfeld, U, Arbenz, U, Baumgartner, MR, Schinzel, A, Balmer, C. Cardiomyopathy in newborns and infants: a broad spectrum of aetiologies and poor prognosis. Acta Paediatr 2008; 97: 1523–1528.CrossRefGoogle ScholarPubMed
15. Debray, FC, Lambert, H, Chevalier, J, et al. Long-termoutcome and clinical spectrum of 73 pediatric patients with mitochondrial diseases. Pediatrics 2007; 119: 722–733.CrossRefGoogle ScholarPubMed
16. Scaglia, F, Towbin, JA, Craigen, WJ, et al. Clinical spectrum, morbidity and mortalità in 113 patients with mitochondrial disease. Pediatrics 2004; 114: 925–931.CrossRefGoogle Scholar
17. Daubeney, PEF, Nugent, AW, Chondros, P, et al. Clinical features and outcomes of childhood dilated cardiomypathy. Results from a national population-based study. Circulation 2006; 114: 2671–2678.CrossRefGoogle Scholar
18. Webber, SA, Boyle, GJ, Jaffe, R, Pickering, RM, Beerman, LB, Fricker, FJ. Role of right ventricular endomyocardial biopsy in infants and children with suspected or possible myocarditis. Br Heart J 1994; 72: 360–363.Google Scholar