Critical Ebstein Anomaly in a Fetus Successfully Managed by Elective Preterm Delivery and Surgical Intervention Without Delay After Birth

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 3...

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Published inPediatric cardiology Vol. 33; no. 2; pp. 343 - 346
Main Authors Tsukimori, Kiyomi, Morihana, Eiji, Fusazaki, Naoki, Takahata, Yasushi, Oda, Shinichiro, Kado, Hideaki
Format Journal Article
LanguageEnglish
Published New York Springer-Verlag 01.02.2012
Springer
Subjects
Adult
Cardiac Surgery
Cardiology
Case Report
Congenital heart disease
Delivery, Obstetric
Ebstein Anomaly - diagnostic imaging
Ebstein Anomaly - surgery
Echocardiography
Female
Humans
Infant, Newborn
Infant, Premature
Infant, Premature, Diseases - surgery
Medicine
Medicine & Public Health
Neonatology
Pregnancy
Premature Birth
Prostaglandins E
Ultrasonography, Prenatal
Vascular Surgery
Japan
Ebstein anomaly
Prenatal diagnosis
Circular shunt
Pulmonary regurgitation
Surgical intervention
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Summary: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.
Bibliography:ObjectType-Case Study-2
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ISSN:0172-0643
1432-1971
DOI:10.1007/s00246-011-0124-4