Critical ebstein anomaly in a fetus successfully managed by elective preterm delivery and surgical intervention without delay after birth

Kiyomi Tsukimori, Eiji Morihana, Naoki Fusazaki, Yasushi Takahata, Shinichiro Oda, Hideaki Kado

Research output: Contribution to journalArticle

7 Citations (Scopus)

Abstract

This report describes a case of Ebstein anomaly in a fetus with cardiomegaly, severe tricuspid regurgitation, pulmonary regurgitation, and retrograde ductal flow that showed a marked increase in the size of the right atrium with advancing gestational age. Elective preterm delivery was performed at 35 weeks gestation. The prostaglandin E1 infusion resulted in more pronounced systemic hypotension and acidosis secondary to circular shunt across the patent ductus arteriosus as well as pulmonary regurgitation and tricuspid regurgitation. Emergency surgical intervention consisting of main pulmonary artery ligation, ductus arteriosus ligation, central shunt creation, and plication of the right atrium without cardiopulmonary bypass was performed 4 h after birth. At the age of 16 days, the Starnes procedure was performed. The infant's postoperative course was uneventful. A fetus that has Ebstein anomaly associated with pulmonary regurgitation is at risk for circular shunt across the patent ductus arteriosus after delivery. Planned delivery and surgical intervention without delay after birth are useful for the treatment of such cases.

Original languageEnglish
Pages (from-to)343-346
Number of pages4
JournalPediatric Cardiology
Volume33
Issue number2
DOIs
Publication statusPublished - Feb 1 2012

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Cardiology and Cardiovascular Medicine

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