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Prediction of postnatal clinical course in primary congenital dilated cardiomyopathy
Hidenori Yamamoto1, Yoshie Fukasawa1, Naoki Ohashi2
1Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan.
Insights
Primary congenital dilated cardiomyopathy (PCDCM) has a poor prognosis. Fetal echocardiography and birth factors predict heart failure onset but not survival in PCDCM patients.
Area of Science:
- Pediatric Cardiology
- Fetal Medicine
- Neonatology
Background:
- Primary congenital dilated cardiomyopathy (PCDCM) is a severe condition affecting newborns.
- Understanding its prognosis and incidence is crucial for clinical management.
Purpose of the Study:
- To predict postnatal prognosis of PCDCM using fetal and perinatal data.
- To estimate the incidence of PCDCM in the population.
Main Methods:
- A multicenter retrospective study analyzed fetal/perinatal data against postnatal outcomes in eight PCDCM patients.
- Incidence was calculated via a population-based study.
Main Results:
- Heart failure onset occurred by a median of 8 days; 75% of patients died or needed advanced support by 67 days.
- Cardiothoracic area ratio, Apgar score, and birth weight standard deviation correlated with heart failure onset timing.
- No predictors for survival were identified. The cumulative incidence of PCDCM was 1.21 per 100,000 births.
Conclusions:
- PCDCM carries a grave prognosis.
- Fetal cardiothoracic area ratio, birth weight, and Apgar score are valuable for predicting heart failure onset.
- These indicators can aid in peri- and postnatal management strategies for PCDCM.
Background:
The aim of this study was to investigate the prediction of postnatal prognosis using fetal and perinatal data in patients with primary congenital dilated cardiomyopathy (PCDCM), and to estimate the incidence of this disease.
Methods:
We examined correlations between fetal or perinatal data and postnatal clinical course in a multicenter retrospective study of eight patients with PCDCM. Incidence was calculated in a population-based study.
Results:
All patients developed heart failure at a median of 8 days (range, 0-43 days), and six patients died or required extracorporeal artificial heart therapy at a median of 67 days (range, 0-92 days). The cardiothoracic area ratio from fetal echocardiography, the Apgar score, and the standard deviation of birth weight correlated significantly with the date at onset of heart failure. However, no data correlated with survival. Cumulative incidence of PCDCM was calculated as 1.21 per 100 000 total births (95% confidence interval, 0.37 to 2.06).
Conclusions:
Primary congenital dilated cardiomyopathy has a poor prognosis, but cardiothoracic area ratio from fetal echocardiography, body weight at birth, and Apgar score correlate with the timing of the onset of heart failure, and these indicators might therefore be useful for peri- and postnatal management.
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