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Complete atrioventricular septal defect and Ebstein's anomaly.

T Fukuda1, T Suzuki, M Sato

  • 1Department of Cardiovascular Surgery, Tokyo Metropolitan Children's Hospital, 1-3-1 Umezono, Kiyose, Tokyo 204-8567, Japan.

Pediatric Cardiology
|March 25, 1999
PubMed
Summary

This case report details a rare congenital heart defect combining complete atrioventricular septal defect (AVSD) and Ebstein's anomaly. Surgical repair was attempted in an infant, but sadly, the patient did not survive.

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Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Disease
  • Cardiac Surgery

Background:

  • Complete atrioventricular septal defect (AVSD) and Ebstein's anomaly are rare congenital heart conditions.
  • The simultaneous occurrence of both complete AVSD and Ebstein's anomaly is exceptionally rare.
  • Surgical repair for this combined anomaly has not been previously reported.

Observation:

  • A 3-month-old infant presented with a rare combination of complete AVSD and Ebstein's anomaly.
  • The infant underwent surgical treatment for this complex cardiac defect.
  • The patient experienced a fatal outcome on the third postoperative day.

Findings:

  • Postmortem examination confirmed complete AVSD and Ebstein's anomaly with downward displacement of the tricuspid valve.

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  • Additional findings included left ventricular outflow tract obstruction.
  • Hypertensive pulmonary vascular disease was also noted during the autopsy.

    • Implications:

    • This case highlights the extreme rarity and complexity of combined complete AVSD and Ebstein's anomaly.
    • The findings underscore the significant surgical and anatomical challenges associated with this dual cardiac malformation.
    • Further investigation into the morphological features and potential surgical strategies for this rare condition is warranted.