Hostname: page-component-76fb5796d-wq484 Total loading time: 0 Render date: 2024-04-26T11:40:01.464Z Has data issue: false hasContentIssue false
  • English
  • Français

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: joanam@gmail.com
Get access Rights & Permissions [Opens in a new window]

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 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1. Lipshultz, SE, Sleeper, LA, Towbin, JA, et al. The incidence of paediatric cardiomyopathy in two regions of the United States. N Engl J Med 2003; 348: 1647–1655.Google Scholar
2. Maron, BJ, Towbin, JA, Thiene, G, et al. American Heart Association Council on Clinical Cardiology, Heart Failure and Transplantation, Council on Clinical Cardiology, Heart Failure and Transplantation Committee, Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups, and Council on Epidemiology and Prevention. Contemporary definitions and classifications of the cardiomyopathies: an American Heart Association scientific statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; and Council on Epidemiology and Prevention. Circulation 2006; 113: 1807–1816.Google Scholar
3. Boucek, MM, Waltz, DA, Edwards, LB, et al. Registry of the International Society for Heart and Lung Transplantation: ninth official paediatric heart transplantation report. J Heart Lung Transplant 2006; 25: 893–903.Google Scholar
4. Towbin, JA, Lowe, AM, Colan, SD, et al. Incidence, causes, and outcomes of dilated cardiomyopathy in children. JAMA 2006; 296: 1867–1876.CrossRefGoogle ScholarPubMed
5. Daubeney, PE, Nugent, AW, Chondros, P, et al. National Australian Childhood Cardiomyopathy Study. Clinical features and outcomes of childhood dilated cardiomyopathy: results from a national population-based study. Circulation 2006; 114: 2671–2678.CrossRefGoogle ScholarPubMed
6. Baughman, KL. Diagnosis of myocarditis: death of Dallas criteria. Circulation 2006; 113: 593–595.Google Scholar
7. Nugent, A, Daubney, PE, Chondros, P, et al. National Australian Childhood Cardiomyopathy Study. The epidemiology of childhood cardiomyopathy in Australia. N Engl J Med 2003; 348: 1639–1646.CrossRefGoogle Scholar
8. Bowles, NE, Ni, J, Kearney, DL, et al. Detection of viruses in myocardial tissues by polymerase chain reaction: evidence of adenovirus as a common cause of myocarditis in children and adults. J Am Coll Cardiol 2003; 42: 466–472.Google Scholar
9. Schultz, JC, Hilliard, AA, Cooper, LT Jr, Rihal, CS. Diagnosis and treatment of viral myocarditis. Mayo Clin Proc 2009; 84: 1001–1009.Google Scholar
10. Kühl, U, Pauschinger, M, Seeberg, B, et al. Viral persistence in the myocardium is associated with progressive cardiac dysfunction. Circulation 2005; 112: 1965–1970.Google Scholar
11. Mahrholdt, H, Wagner, A, Deluigi, CC, et al. Presentation, patterns of myocardial damage, and clinical course of viral myocarditis. Circulation 2006; 114: 1581–1590.CrossRefGoogle ScholarPubMed
12. Kindermann, I, Kindermann, M, Kandolf, R, et al. Predictors of outcome in patients with suspected myocarditis. Circulation 2008; 118: 639–648.Google Scholar
13. Cox, GF. Diagnostic approaches to paediatric cardiomyopathy of metabolic genetic etiologies and their relation to therapy. Prog Pediatr Cardiol 2007; 24: 15–25.CrossRefGoogle ScholarPubMed
14. Wilkinson, JD, Landy, DC, Colan, SD, et al. The paediatric cardiomyopathy registry and heart failure: key results from the first 15 years. Heart Fail Clin 2010; 6: 401–413; vii.Google Scholar
15. Friedman, RA, Moak, JP, Garson, A. Clinical course of idiopathic dilated cardiomyopathy in children. J Am Coll Cardiol 1991; 18: 152–156.Google Scholar
16. Arola, A, Tuominen, J, Ruuskanen, O, et al. Idiopathic dilated cardiomyopathy in children: prognostic indicators and outcome. Paediatrics 1998; 101: 369–376.Google Scholar
17. English, RF, Janosky, JE, Ettedgui, JA, et al. Outcomes for children with acute myocarditis. Cardiol Young 2004; 14: 488–493.Google Scholar
18. Feingold, B, Webber, SA. Acute myocarditis and cardiomiopathies. In: Munoz R, Morell V, Cruz Ed, Vetterly C (eds) Critical Care of Children with Heart Disease: Basic Medical and Surgical Concepts, 1st edn. Springer, London, 2010, pp. 507–520.Google Scholar
19. Alvarez, JA, Wilkinson, JD, Lipshultz, SE. Outcome predictors for paediatric dilated cardiomyopathy: a systematic review. Prog Pediatr Cardiol 2007; 23: 25–32.Google Scholar
20. Hsu, DT, Pearson, GD. Heart failure in children: part II: diagnosis, treatment, and future directions. Circ Heart Fail 2008; 2: 490–498.Google Scholar