Omphalocele associated with coronary sinus atrial septal defect: report of two cases
C H Gumbiner1, P J Hofschire, J P Cheatham
1Section of Pediatric Cardiology, University of Nebraska Medical Center.
Insights
Two children with omphalocele, a birth defect, also had a coronary sinus atrial septal defect. This heart condition may be missed if not specifically examined in infants with omphalocele.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Developmental Biology
Background:
- Omphalocele is a congenital abdominal wall defect.
- Congenital heart disease (CHD) is common in infants with omphalocele.
- Atrial septal defects (ASDs) are a type of CHD.
Observation:
- Two pediatric patients with omphalocele were identified.
- These patients presented with an unusual form of atrial communication: a coronary sinus atrial septal defect.
- This specific defect was part of a complex congenital heart disease presentation.
Findings:
- The coronary sinus atrial septal defect was associated with omphalocele in both cases.
- This type of ASD may be overlooked during routine preoperative cardiac evaluations.
- The co-occurrence suggests a potential link beyond chance.
Implications:
- Highlights the importance of targeted echocardiographic assessment for coronary sinus ASDs in neonates with omphalocele.
- Suggests a possible shared developmental pathway or syndrome involving omphalocele and specific cardiac anomalies.
- Further research is warranted to elucidate the underlying mechanisms and clinical significance of this association.
Abstract:
Two children with omphalocele were found to have coronary sinus atrial septal defect as part of their congenital heart disease complex. This unusual atrial communication may elude preoperative detection unless specifically sought. Its occurrence in conjunction with omphalocele may represent more than a chance association.
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