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Chylothorax Associated with Congenital Complete Atrioventricular Block
Shuhei Fujino1, Hidehiko Maruyama1, Keiko Tsukamoto1
1Division of Neonatology, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, National Center for Child Health and Development, Tokyo, Japan.
Congenital complete atrioventricular block (CCAVB) in infants can lead to congenital chylothorax. This rare condition, potentially caused by maternal autoantibodies and bradycardia, requires careful consideration of chylothorax in atypical pleural effusions.
Area of Science:
- Neonatal Cardiology
- Pediatric Immunology
- Thoracic Surgery
Background:
- Congenital complete atrioventricular block (CCAVB) is a rare cardiac anomaly.
- Congenital chylothorax is a significant cause of neonatal respiratory distress.
- The co-occurrence of CCAVB and congenital chylothorax is exceptionally rare.
Observation:
- A male neonate presented with fetal bradycardia and right pleural effusion.
- Maternal serum revealed high levels of anti-Sjögren's-syndrome-related antigen A autoantibodies.
- Postnatal diagnosis confirmed CCAVB requiring temporary pacing and congenital chylothorax.
Findings:
- Pleural effusion, characterized by lymphocytosis and high triglycerides, indicated chylothorax.
- Treatment with prednisolone, octreotide, and total parenteral nutrition resolved the pleural effusion.
- The infant's cardiac output improved with temporary external pacing.
Implications:
- Maternal autoantibodies and bradycardia-induced venous congestion may link CCAVB and congenital chylothorax.
- Congenital chylothorax should be suspected in neonates with CCAVB and atypical pleural effusions.
- Early diagnosis and multidisciplinary management are crucial for improving outcomes in these complex cases.
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