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[Dilated myocardiopathy in children]
A Cabrera1, E Hernáez, N Clerigue
1Servicio de Cardiología Pediátrica, Hospital Infantil Cruces, Vizcaya.
Insights
Dilated cardiomyopathy in children is a moderately frequent condition. Its incidence is decreasing, and prognosis has improved with vasodilator treatment, making it assessable with non-invasive methods.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Research
- Clinical Medicine
Context:
- Dilated cardiomyopathy (DCM) is a significant cause of heart failure in children.
- Studied incidence and clinical course of DCM in pediatric patients from 1971-1988.
- Investigated diagnostic findings and treatment outcomes for childhood DCM.
Purpose:
- To analyze the incidence, clinical presentation, and outcomes of dilated cardiomyopathy in children.
- To evaluate the effectiveness of non-invasive diagnostic methods and vasodilator therapy.
- To assess changes in disease incidence and prognosis over time.
Summary:
- 46 children with dilated cardiomyopathy (DCM) were studied between 1971-1988, with a median age of 17 months.
- Heart failure was the most common presentation (98%). Echocardiography revealed dilated left ventricles and reduced ejection fraction in affected children.
- Incidence decreased over the study period, and vasodilator treatment improved prognosis, with all 6 treated patients surviving.
Impact:
- Highlights the importance of early diagnosis and intervention for pediatric dilated cardiomyopathy.
- Demonstrates the value of non-invasive echocardiography in assessing DCM.
- Suggests a positive impact of vasodilator therapy on the survival rates of children with DCM.
Abstract:
Between 1971-1988 we have studied 46 children with dilated cardiomyopathy. Twenty were males and 18 females, with a median age of 17 months (range from 1 day to 11 years). The incidence of 3.4 cases a year during the first nine years dropped to 1.3 cases a year in the following years. Two patients were siblings. Seventeen patients were studied with Eco-Doppler, cardiac catheterization and angiocardiography. The most frequent presentation was heart failure, present in 45 cases (98%). By bidimensional echocardiography it was observed that all patients had a dilated left ventricle, with a left atrial/aorta ratio of 1.5 +/- 0.3; the ejection fraction was diminished in 12 patients (11-36%) and the length of the left ventricle in the long parasternal axis view 4.2 +/- 0.7. All the patients studied had an elevated telediastolic pressure in the left ventricle, four with pulmonary artery pressure of 96 +/- 11 and seven with right atrial pressure of 24 +/- 5. There was mitral insufficiency in 4 patients and tricuspid insufficiency in three. Clinical course was favorable in 18 patients with median age at present of 112 months (range: 11 months to 15 years). The last 6 patients, treated with vasodilators, are living. In conclusion, dilated cardiomyopathy is a moderately frequent disease, with trend to diminish in incidence prognosis improved after treatment with vasodilator was introduced, and easily assessed with non-invasive methods.