This Article
Right arrow Full Text
Right arrow Full Text (PDF)
Right arrow P3Rs: Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when P3Rs are posted
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow E-mail this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My File Cabinet
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via CrossRef
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Arola, A.
Right arrow Articles by Jokinen, E.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Arola, A.
Right arrow Articles by Jokinen, E.
Related Collections
Right arrow Heart & Blood Vessels

PEDIATRICS Vol. 101 No. 3 March 1998, pp. 369-376

Idiopathic Dilated Cardiomyopathy in Children: Prognostic Indicators and Outcome

Received Mar 31, 1997; accepted Aug 27, 1997.

Anita Arola*, Juhani TuominenDagger , Olli Ruuskanen*, and Eero Jokinen*

From the Departments of * Pediatrics and Dagger  Biostatistics, University of Turku, Turku, Finland.

Objective.  To determine the outcome of Finnish children and adolescents with idiopathic dilated cardiomyopathy (IDCM) and factors that might be useful as prognostic indicators.

Methodology.  The clinical profile and course of 62 Finnish children and adolescents (median age, 13 months; range, 1 day to 20 years) with IDCM in 1980 to 1991 were evaluated to detect factors that might predict outcome. Factors studied included age, gender, family history, previous viral illness, and symptoms and signs at presentation. Furthermore, data on serial electrocardiographic, echocardiographic, and chest x-ray examinations, histologic findings, and treatments were analyzed.

Results.  During a mean (±SD) follow-up of 3.9 ± 4.5 years (range, 1 day to 25 years), 10 patients (16%) recovered, 17 (27%) had residual disease, 4 (6.4%) underwent heart transplantation, and 31 (50%) died. Infants (<1 year of age) and adolescent (>= 15 years of age) male patients with progressing symptoms of left ventricular failure after initiation of medical therapy tended to have the poorest outcome. However, in multivariate analysis, only histologic evidence of endocardial fibroelastosis, clinical signs of right ventricular failure at presentation, and the need for anticoagulative therapy during follow-up, the last an expression of a severely impaired left ventricular systolic function, appeared to be significant predictors of long-term outcome.

Conclusions.  Our study confirms that the outcome of children with IDCM still remains poor. However, a group of patients, mainly infants, make a full recovery. Adolescent male patients as well as infants suffering from endocardial fibroelastosis with persisting symptoms of congestive heart failure after initiation of medical therapy tend to have the poorest outcome. These patients need a careful follow-up at short time intervals and, in the case of lacking response to medical treatment with resulting growth failure and/or poor quality of life, should be offered urgent heart transplantation.

Key words: cardiomyopathy, congestive, child, adolescent, prognosis.




This article has been cited by other articles:


Home page
 CirculationHome page
R. E. Andrews, M. J. Fenton, D. A. Ridout, M. Burch, and on Behalf of the British Congenital Cardiac Associ
New-Onset Heart Failure Due to Heart Muscle Disease in Childhood: A Prospective Study in the United Kingdom and Ireland
Circulation, January 1, 2008; 117(1): 79 - 84.
[Abstract] [Full Text] [PDF]

Home page
 CirculationHome page
P. E. F. Daubeney, A. W. Nugent, P. Chondros, J. B. Carlin, S. D. Colan, M. Cheung, A. M. Davis, C.W. Chow, R. G. Weintraub, and on behalf of the National Australian Childhood Car
Clinical Features and Outcomes of Childhood Dilated Cardiomyopathy: Results From a National Population-Based Study
Circulation, December 12, 2006; 114(24): 2671 - 2678.
[Abstract] [Full Text] [PDF]

Home page
 J Am Coll CardiolHome page
A. E. Tsirka, K. Trinkaus, S.-C. Chen, S. E. Lipshultz, J. A. Towbin, S. D. Colan, V. Exil, A. W. Strauss, and C. E. Canter
Improved outcomes of pediatric dilated cardiomyopathy with utilization of heart transplantation
J. Am. Coll. Cardiol., July 21, 2004; 44(2): 391 - 397.
[Abstract] [Full Text] [PDF]

Home page
 Physiol. GenomicsHome page
C. T. Maguire, H. Wakimoto, V. V. Patel, P. E. Hammer, K. Gauvreau, and C. I. Berul
Implications of ventricular arrhythmia vulnerability during murine electrophysiology studies
Physiol Genomics, September 29, 2003; 15(1): 84 - 91.
[Abstract] [Full Text] [PDF]

Home page
 NEJMHome page
S. E. Lipshultz, L. A. Sleeper, J. A. Towbin, A. M. Lowe, E. J. Orav, G. F. Cox, P. R. Lurie, K. L. McCoy, M. A. McDonald, J. E. Messere, et al.
The Incidence of Pediatric Cardiomyopathy in Two Regions of the United States
N. Engl. J. Med., April 24, 2003; 348(17): 1647 - 1655.
[Abstract] [Full Text] [PDF]

Home page
 CirculationHome page
C. I. Berul, B. K. McConnell, H. Wakimoto, I. P.G. Moskowitz, C. T. Maguire, C. Semsarian, M. M. Vargas, J. Gehrmann, C. E. Seidman, and J. G. Seidman
Ventricular Arrhythmia Vulnerability in Cardiomyopathic Mice With Homozygous Mutant Myosin-Binding Protein C Gene
Circulation, November 27, 2001; 104(22): 2734 - 2739.
[Abstract] [Full Text] [PDF]

Home page
 J. Thorac. Cardiovasc. Surg.Home page
R. D. Mainwaring, R. M. Healy, J. D. Murphy, and W. I. Norwood
Norwood/Batista operation for a newborn with dilated myopathy of the left ventricle
J. Thorac. Cardiovasc. Surg., September 1, 2000; 120(3): 612 - 615.
[Full Text] [PDF]

Home page
 NeoReviewsHome page
D. N. Rosenthal
Cardiomyopathy in Infants: A Brief Overview
NeoReviews, August 1, 2000; 1(8): e139 - 145.
[Full Text]

Home page
 J. Thorac. Cardiovasc. Surg.Home page
P. J. del Nido
EDITORIAL: PARTIAL LEFT VENTRICULECTOMY FOR DILATED CARDIOMYOPATHY IN CHILDREN
J. Thorac. Cardiovasc. Surg., May 1, 1999; 117(5): 918 - 919.
[Full Text] [PDF]