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.

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