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

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.

Fetal Heart Size

“…Comparisons between the point-to-point trace and electronic ellipse methods for measurements of the area and circumference were performed by using within-subject repeated measures analysis of variance (NCSS 10; NCSS, Ebstein's malformation 7,14,48,57,61,69 Tricuspid valve dysplasia 7,44,56 Tricuspid valve atresia 44…”

Surgical management of symptomatic neonates with Ebstein’s anomaly: choice of operation

“…In 2000, we reported our initial results with complete biventricular repair in eight neonates with 87% survival. 6 All the patients underwent repair of their tricuspid valve using a modification of the Danielson technique, [23][24][25] fenestrated closures of the atrial septal defect, reduction atrioplasty, and repair of all associated cardiac defects. [24][25][26] Subsequently, this experience expanded to surgically treated 26 neonates and 9 young (<4 months old) infants.…”

Management of congenital diaphragmatic hernia with transposition of the great arteries

“…For example, patients with Ebstein anomaly accompanied by severe tricuspid regurgitation, or with hypoplastic left heart syndrome accompanied by restrictive foramen ovale, are intensively evaluated during prenatal period, and successfully treated with surgical or catheter intervention after birth. 5,6 In the present case, prenatal assessment facilitated prediction of the clinical course after birth and development of the treatment strategy. Thus, comprehensive evaluation of the fetus and precise preparation for the postnatal management were crucial for intact survival.…”

“…Prenatal diagnosis of severe and multiple congenital anomalies is essential to determine postnatal care and introduction of intensive treatment immediately after birth. For example, patients with Ebstein anomaly accompanied by severe tricuspid regurgitation, or with hypoplastic left heart syndrome accompanied by restrictive foramen ovale, are intensively evaluated during prenatal period, and successfully treated with surgical or catheter intervention after birth . In the present case, prenatal assessment facilitated prediction of the clinical course after birth and development of the treatment strategy.…”

“…4 An outbreak of MRSA colonization is occasionally difficult to control, however, despite following standard recommendations. 2,[5][6][7] We report herein an outbreak of MRSA colonization in an NICU that was difficult to control, in which airborne transmission from a patient with inherited ichthyosis was suspected to be the major mode of transmission.…”