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Idiopathic dilated cardiomyopathy in children: prognostic indicators and outcome
A Arola1, J Tuominen, O Ruuskanen
1Department of Pediatrics, University of Turku, Turku, Finland.
Insights
The outcome for Finnish children with idiopathic dilated cardiomyopathy (IDCM) remains poor, with 50% mortality. Infants and adolescent males with specific indicators like endocardial fibroelastosis face the worst prognosis, necessitating close monitoring and timely intervention.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Research
- Pediatric Oncology
Background:
- Idiopathic dilated cardiomyopathy (IDCM) in children presents a significant clinical challenge.
- Understanding prognostic factors is crucial for managing pediatric IDCM.
- Previous studies have highlighted varying outcomes in pediatric IDCM.
Purpose of the Study:
- To determine the long-term outcome of Finnish children and adolescents diagnosed with IDCM.
- To identify clinical, diagnostic, and therapeutic factors predicting the prognosis of pediatric IDCM.
Main Methods:
- A retrospective analysis of 62 Finnish pediatric patients with IDCM diagnosed between 1980 and 1991.
- Evaluation of clinical presentation, medical history, serial diagnostic tests (ECG, echocardiography, chest X-ray), histology, and treatment modalities.
- Multivariate analysis to identify significant predictors of outcome.
Main Results:
- During a mean follow-up of 3.9 years, 16% recovered, 27% had residual disease, 6.4% underwent heart transplantation, and 50% died.
- Infants and adolescent males with progressive left ventricular failure showed poorer outcomes.
- Histologic endocardial fibroelastosis, right ventricular failure at presentation, and need for anticoagulation were significant predictors of poor outcome.
Conclusions:
- The overall outcome for pediatric IDCM remains poor, emphasizing the need for improved management strategies.
- A subset of patients, particularly infants, can achieve full recovery.
- Adolescent males and infants with endocardial fibroelastosis and persistent heart failure symptoms require intensive monitoring and may benefit from early heart transplantation consideration.
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.