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[Natural history and prognostic factors in primary endocardial fibroelastosis in infants]
M Jarrar1, G Vaksmann, F Godart
1Service de cardiologie infantile, Hôpital cardiologique, Lille.
Insights
Dilated cardiomyopathy in infants, often mimicking endocardial fibroelastosis, shows a high cure rate. However, family history and digitalis dosage significantly impact prognosis, with recurrences indicating a worse outcome.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Research
- Neonatal Medicine
Context:
- Dilated cardiomyopathy in patients under two years old.
- Clinical presentation mimicking primary endocardial fibroelastosis.
- Long-term outcomes in a pediatric cardiac unit.
Purpose:
- To analyze the long-term outcomes of infants diagnosed with dilated cardiomyopathy presenting as primary endocardial fibroelastosis.
- To identify factors influencing prognosis, including family history and treatment regimens.
- To evaluate the effectiveness of digitalis treatment and the impact of symptom recurrence.
Summary:
- A 33-year study of 127 infants with dilated cardiomyopathy showed a 74% cure rate.
- Factors like family history and symptom recurrence were linked to poorer outcomes.
- Prolonged high-dose digitalis treatment emerged as a critical prognostic factor.
Impact:
- Highlights the potential for high cure rates in neonatal dilated cardiomyopathy.
- Identifies key indicators for adverse prognosis, aiding in risk stratification.
- Suggests optimized digitalis therapy and vigilant monitoring for recurrences are crucial for improved patient management.
Abstract:
Over a 33 year period, 127 patients under 2 years of age with dilated cardiomyopathies and appearances compatible with the diagnosis of primary endocardial fibroelastosis were admitted to the paediatric cardiac unit of the CHRU of Lille. The average follow-up was 8.9 +/- 6.7 years. Ninety-four children (74%) were cured, 16 (13%) had persistent cardiomegaly and/or left ventricular dysfunction on echocardiographic examination, and only 17 (13%) patients died (10 in the year following their initial hospital admission including 5 in the first week). The outcome of patients was not related to age at diagnosis, sex, cardiothoracic index, initial shortening fraction of the left ventricle or the period at which the patients were seen for the first time. On the other hand, the presence of a family history of cardiomyopathy was associated with a significantly worse prognosis. A recurrence of symptoms was the factor most closely correlated with a bad prognosis: 12 of the 19 patients (63%) with this evolution died, and 4 others (21%) had persistent myocardial dysfunction at the end of the study. These recurrences were often observed after premature withdrawal or after use of ineffective dosages of digitalis. In the authors' experience, dilated cardiomyopathy in neonates with clinical features of primary endocardial fibroelastosis is associated with a relatively high number of cures. Prolonged treatment with high doses of digitalis seems a determining prognostic factor.(ABSTRACT TRUNCATED AT 250 WORDS)