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Idiopathic dilated cardiomyopathy in children: clinical profile and prognostic determinants
G Nogueira1, F F Pinto, A Paixão
1Serviço de Cardiologia Pediátrica-Hospital de Santa Marta. ip228442@ip.pt
Insights
Idiopathic dilated cardiomyopathy in children is serious, with high mortality. Clinical severity, poor left ventricular function, and severe arrhythmias indicate an unfavorable prognosis and guide heart transplant decisions.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Research
- Clinical Pediatrics
Background:
- Idiopathic dilated cardiomyopathy (IDCM) presents a significant mortality risk in pediatric populations.
- Understanding the clinical course and prognostic indicators of childhood IDCM is crucial for managing patients and identifying candidates for cardiac transplantation.
Purpose of the Study:
- To characterize the clinical evolution of idiopathic dilated cardiomyopathy in children.
- To identify prognostic factors associated with mortality and survival in pediatric IDCM patients.
Main Methods:
- Retrospective review of clinical records for 41 children diagnosed with IDCM between January 1985 and December 1997.
- Analysis of demographic, clinical, electrocardiographic, echocardiographic, and hemodynamic data.
- Comparison of parameters between survivors (Group I) and deceased patients (Group II).
Main Results:
- Of 34 eligible children, 11 (32.3%) recovered, 13 (38.2%) survived with left ventricular dysfunction, and 10 (29.4%) died.
- Mortality rates were 23.5% within the first year and 29.4% by five years.
- Unfavorable prognosis correlated with severe clinical presentation, lower left ventricular shortening fraction (10% vs. 13%), and severe arrhythmias (40% vs. 3.5%).
Conclusions:
- Clinical severity, reduced left ventricular function, and severe arrhythmias are key indicators of poor prognosis in pediatric idiopathic dilated cardiomyopathy.
- These factors can aid in identifying high-risk patients who may benefit from early consideration for heart transplantation.
Abstract:
Idiopathic dilated cardiomyopathy is a severe disease with a high mortality rate in childhood. Its clinical evolution and prognosis are important for the selection of cardiac transplantation candidates. In order to characterize its evolution and identify prognostic factors, the clinical records of 41 children with the diagnosis of idiopathic dilated cardiomyopathy, admitted from January 1985 to December 1997, were reviewed. Survivors (Group I) and deceased (Group II) were separately analyzed, according to the following parameters: age, sex, race, clinical severity, electrocardiographic, echocardiographic and haemodynamic findings. Seven children were excluded from the study: six of them were lost to follow-up and one died from a surgical complication. Of the remaining 34 children, 20 were male (M) and 14 were female (F) (M/F: 1.4). Age range at diagnosis was 7 days to 14 years (median: 1.5 years), and follow-up time was from 18 days to 10.5 years (median: 2.5 years). Eleven (32.3%) children fully recovered, 13 (38.2%) survived with left ventricular dysfunction, and ten (29.4%) died, half of them within the first three months of follow-up. Mortality was 23.5% (8 out of 34 children) during the first year of follow-up and 29.4% (ten out of 34 children) at five years. Unfavorable prognosis was more frequently associated to: 1) clinical severity at the time of presentation; 2) lower mean left ventricular shortening fraction (10 +/- 7% in group II and 13 +/- 5% in group I); 3) occurrence of severe arrhythmia (40% in group II and 3.5% in group I). In this series a group of higher mortality risk was identified, based on some of the analyzed parameters, which should be considered as selection criteria for early heart transplantation.