Obstet Gynecol Sci.  2014 Nov;57(6):530-533. 10.5468/ogs.2014.57.6.530.

An unusual ultrasonographic manifestation of a fetal Ebstein anomaly

• Cha MY
• Won HS
• Lee MY
• Woo KH
• Shim JY

• KMID: 2314022
• DOI: http://doi.org/10.5468/ogs.2014.57.6.530

Abstract

An Ebstein anomaly is a rare congenital heart defect defined by an inferior displacement of the septal and posterior leaflets of the tricuspid valve from the tricuspid annulus. This anomaly shows various ultrasonographic manifestations, thus making the prenatal diagnosis sometimes difficult. We here report a rare case of an Ebstein anomaly which was prenatally suspected as the absence of the tricuspid valve with functional pulmonary atresia because of non-visible tricuspid leaflets on an echocardiograph at 24 weeks of gestation. An emergency cesarean section was performed at 35 weeks of gestation as fetal hydrops were seen on a follow-up scan. Postnatal surgery confirmed Ebstein anomaly type-D which demonstrates an almost complete atrialization of the right ventricle with the exception of a small and infundibular component. Because of its rarity, prenatal findings of a type-D Ebstein anomaly have not been reported previously. We suggest from this first such case report that this anomaly should be considered as a possible diagnosis when the tricuspid leaflets are not well visualized.

Keyword

Ebstein anomaly; Echocardiography; Fetal heart; Prenatal diagnosis

MeSH Terms

Cesarean Section
Diagnosis
Ebstein Anomaly*
Echocardiography
Emergencies
Female
Fetal Heart
Follow-Up Studies
Heart Defects, Congenital
Heart Ventricles
Hydrops Fetalis
Pregnancy
Prenatal Diagnosis
Pulmonary Atresia
Tricuspid Valve

Figure

• 

  Fig. 1 Four-chamber view of the heart in the study patient showing the dilatation of the right ventricle, with no obvious dilatation of the right atrium (A). Three-dimensional ultrasound demonstrating no discernible tricuspid leaflets (arrows) (B). Color Doppler imaging showing a to-andfro from the right atrium to the right ventricle (C,D). Rt, right; Lt, left; RA, right atrium; LA, left atrium; RV, right ventricle; LV, left ventricle.

• 

  Fig. 2 Postnatal echocardiogram showing an almost completely atrialized right ventricle (aRV) with only a small outlet portion operating as a functional RV (*) and a severe downward displacement of the tricuspid valve leaflet insertion site (+) (A). Right ventricular outlet view also showing a very small functional RV (B), and severe tricuspid regurgitation on color Doppler (arrow) (C). RA, right atrium; LA, left atrium; LV, left ventricle; aRV, atrialized right ventricle; PA, pulmonary artery; fRV, functional right ventricle; TV, tricuspid valve.

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