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[Clinical aspects of dilated cardiomyopathy in pediatric age]
Insights
Dilated cardiomyopathy in children has unpredictable outcomes. Reduced fractional shortening is a key indicator of poor prognosis, aiding in identifying children who may benefit from cardiac transplant.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Research
Background:
- The natural history and prognostic factors of pediatric dilated cardiomyopathy are poorly understood.
- Disease progression varies significantly, with some children improving, some remaining stable, and others rapidly deteriorating.
Purpose of the Study:
- To identify prognostic factors for poor outcomes in pediatric dilated cardiomyopathy.
- To determine risk factors for impending death to guide cardiac transplant decisions.
Main Methods:
- A cohort of 22 children (median age 5 years) with dilated cardiomyopathy was followed for 40 months.
- Clinical data and laboratory studies including ECG, Holter, echocardiography, and chest X-ray were analyzed.
- Comparison of clinical and laboratory findings between survivors and non-survivors was performed.
Main Results:
- At follow-up, 31.8% improved, 31.8% remained unchanged, and 36% worsened.
- Mortality was 40.9% (9 out of 22), with survival rates of 72% at 12 months and 59.3% at 60 months.
- Reduced fractional shortening was the only statistically significant predictor of poor outcome (p < 0.025).
Conclusions:
- Pediatric dilated cardiomyopathy exhibits variable natural history.
- Fractional shortening is a critical prognostic indicator in pediatric dilated cardiomyopathy.
- Identifying high-risk patients is crucial for timely therapeutic interventions like cardiac transplantation.
Abstract:
Natural history and prognostic factors of dilated cardiomyopathy in pediatric age are not well identified so far. The course of the disease, often, presents "odd" characteristics: some of the patients show clinical improvement, someone remains unchanged and some of them have a negative evolution in a very short time. It is necessary to identify the group of patients having a poor outcome in order to define prognostic factors of impending death so that a cardiac transplant could be offered to them as a reasonable therapeutic choice. Twenty-two children, 9 males and 13 females (median age 5 +/- 5 years) with dilated cardiomyopathy were studied. Their natural history was followed for a period of 40 +/- 30 months. Clinical data, laboratory studies (electrocardiogram, Holter monitoring, echocardiogram M-mode, 2-D, Doppler and chest X-ray) were taken into consideration. At the end of follow-up period 7 patients showed improvement in cardiac status (31.8%), 7 remained unchanged (31.8%) and 8 (36%) got worse; 9 died. The survival curve showed a rate of 72% at 12 months and of 59.3% at 60 months. Of 9 died patients, 2 died suddenly and seven for congestive heart failure. Clinical findings and laboratory investigations of 13 survived patients (Group I) and 9 died patients (Group II) were compared to establish the risk factors of poor outcome. The fractional shortening is the only significant factor of poor outcome (p less than 0.025). Other negative factors were identified more in Group II than in Group I.(ABSTRACT TRUNCATED AT 250 WORDS)