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Review
. 2021 Jan;37(Suppl 1):17-25.
doi: 10.1007/s12055-020-00942-z. Epub 2020 Mar 21.

Ebstein's anomaly in the neonate

T K Susheel Kumar  1
Affiliations
Review

Ebstein's anomaly in the neonate

T K Susheel Kumar. Indian J Thorac Cardiovasc Surg. 2021 Jan.

Abstract

Ebstein's anomaly is a rare congenital heart defect characterized by apical displacement of the tricuspid valve and reduced volume of the true functional right ventricle. In its most severe form, the disease presents as right heart failure and cyanosis in the neonate. Although many of these neonates can be managed conservatively, surgical intervention may become necessary in the sickest of them. Surgical intervention in this subgroup can be challenging and requires meticulous appreciation of the anatomy and physiology on a case to case basis. Multicenter studies are required to define a uniform algorithm and approach towards these patients.

Keywords: Complications; Ebstein; Neonate; Operation.

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Conflict of interest statement

Conflict of interestThe author declares that there is no conflict of interest.

Figures

Fig. 1
Fig. 1
GOSE score. RA, right atrium; aRV, atrialized right ventricle; LA, left atrium; LV, left ventricle
Fig. 2
Fig. 2
(A) Approximation of annuloplasty stitch creates two openings, the “caudal” orifice containing the entrance to the atrialized right ventricle. (B) Closure of the caudal opening plicates the atrialized right ventricle and creates a competent monocuspid valve
Fig. 3
Fig. 3
Detachment of anterior leaflet from tricuspid annulus. Subvalvar attachments are mobilized
Fig. 4
Fig. 4
Annuloplasty stitch with reattachment of anterior leaflet
Fig. 5
Fig. 5
Algorithm for initial management of Ebstein’s anomaly in neonates. O2, oxygen; PVR, pulmonary vascular resistance; PGE1, prostaglandin; iNO, inhaled nitric oxide
Fig. 6
Fig. 6
Algorithm for management of Ebstein’s anomaly with anatomic pulmonary atresia. RV, right ventricle; TR, tricuspid regurgitation; BTS, Blalock–Taussig shunt; RV-PA, right ventricle to pulmonary artery
Fig. 7
Fig. 7
Algorithm for management of Ebstein’s anomaly with functional pulmonary atresia. LV, left ventricle; TR, tricuspid regurgitation; BTS, Blalock–Taussig shunt; iNO, inhaled nitric oxide; MPA, main pulmonary artery
See this image and copyright information in PMC

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