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Year : 2006  |  Volume : 16  |  Issue : 4  |  Page : 827-829
Antenatal diagnosis of Ebstein's anomaly by ultrasonography- a case report

Dept Of Radiodiagnosis, Rural Medical College , Pravara Institue Of Medical Sciences , Loni, BK Pin : 413736, Ahmednagar, India

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Date of Submission27-Jan-2006
Date of Acceptance10-Oct-2006

Keywords: Ebstein′s anomaly, antenatal, four chamber view, fetal echocardiography

How to cite this article:
Divekar S A, Kawade R D, Aironi V D, Sachdev Y P, Kashyape R S. Antenatal diagnosis of Ebstein's anomaly by ultrasonography- a case report. Indian J Radiol Imaging 2006;16:827-9

How to cite this URL:
Divekar S A, Kawade R D, Aironi V D, Sachdev Y P, Kashyape R S. Antenatal diagnosis of Ebstein's anomaly by ultrasonography- a case report. Indian J Radiol Imaging [serial online] 2006 [cited 2020 Dec 5];16:827-9. Available from:

   Introduction Top

Ebstein's anomaly is a rare congenital cardiac malformation characterized by inferior displacement of the tricuspid valve leaflets into the cavity of the right ventricle [1],[2]. In the fetus, an ultrasound and echocardiography has been proven to be reliable in differentiating the variants of Ebstein's anomaly [3],[4]. We report a case of Ebstein's anomaly diagnosed on the routine obstetric ultrasound, antenatally at 27 weeks of gestation. Additionally colpocephaly was also present.

   Case report Top

A 26 year primigravida, having no positive history including any drug intake, visited for routine antenatal foetal ultrasonography at 27th weeks menstrual age.

The past history (including the drug history) was non contributory. The general, systemic and obstetric examination was unremarkable. On ultrasonography, there was a single live intrauterine foetus in vertex lie. The growth parameters were corresponding to the menstrual age and the amniotic fluid was adequate.

On examining the four chamber view, the right atrium was found to be severely dilated. There was downward displacement of septal and posterior leaflets of the tricuspid valve, which were tethered to the muscular part of ventricular septum (fig 1). The valve was also thickened, deformed with abnormal movement on dynamic scanning. The effective right ventricular cavity was severely hypoplastic.The findings were consistent with Ebstein's anomaly. The echocardiogram also showed moderate to severe tricuspid valve regurgitation confirmed on colour Doppler (Fig 7), and spectral waveform (Fig 8), a small to moderate peri-membranous ventricular septal defect, with a left-to-right shunt, and a secundum atrial septal defect. There was cardiomegaly with pulmonary hypoplasia (fig 6). However, there was no evidence of hydrops fetalis.

In addition, there was associated colpocephaly (dilatation of occipital horns of both lateral ventricles) (fig 5).

Considering the prognosis, termination of the pregnancy was suggested to the mother. An informed choice was made and the mother chose to abort. Findings were confirmed on autopsy of aborted foetus.

   Discussion Top

Ebstein's anomaly is an uncommon congenital cardiac anomaly with reported incidence of 0.2 - 0.5 % of all cardiac anomalies. In this anomaly, the tricuspid valve leaflets do not attach to their normal location at the annular ring, but displaced downwards, reducing the effective right ventricular cavity. The right ventricle is divided into proximal 'atrialized' right ventricle, having a thin wall and distal small trabecular functioning portion of right ventricle having a thick wall. It is associated with atrial septal defects (up to 90%), anatomic or functional pulmonary atresia (in a small percentage), and rare ventricular septal defect [4]. Ebstein's anomaly can lead to severe tricuspid regurgitation and cardiac dysfunction in utero, resulting in fetal cardiomegaly, heart failure, pulmonary hypoplasia, hydrops, and tachyarrhythmias [2],[3],[4].

Fetal echocardiography is a very sensitive (91.6%) and specific (99.9%) tool for antenatal diagnosis of congenital heart disease in high risk pregnancies [5] Systemic assessment of the four-chamber view can pick up more than half of the intra cardiac abnormalities such as Ebstein's anomaly and tricuspid valve dysplasia .

Ebstein's anomaly is known to have a very poor prognosis during the perinatal period, with an overall mortality rate as high as 85%.Spontaneous intrauterine death is reported (48%). 35% of those who were live-born died of a combination of hypoxia and severe congestive heart failure[8].

Early detection of tricuspid valve disease makes it possible to consider to the option of termination of pregnancy in view of the poor postnatal course of the anomaly. Early presentation is associated with other cardiac lesions, usually pulmonary stenosis or atresia.

Thus, in general, the earlier the patient presents with the malformation, the poorer the prognosis. The Ebstein's anomaly has to be differentiated from the much more common tricuspid dysplasia which has a far better prognosis. This differentiation is primarily based on the downward displacement of the tricuspid valve leading to the large right atrium and small hypoplastic right ventricle. Most of the fetuses with Ebstein's anomaly have additionally severe hydrops, heart failure or complete heart block with a high incidence of fetal loss or neonatal deaths. Its association with other extra cardiac anomalies is very rare and has been reported in only a few cases.

The echocardiographic appearance, marked cardiomegaly, and the presence of associated lesions are all risk factors for mortality.

   References Top

1.Frescura C, Angelini A, Daliento L, Thiene G. Morphological aspects of Ebstein's anomaly in adults. Thorac Cardiovasc Surg 2000; 48:203-208.  Back to cited text no. 1  [PUBMED]  
2.Anderson KR, Zuberbuhler JR, Anderson RH, Becker AE, Lie JT. Morphologic spectrum of Ebstein's anomaly of the heart. Mayo Clin Proc 1979; 54: 174-180  Back to cited text no. 2  [PUBMED]  
3.Celermajer DS, Bull C, Till JA, et al. Ebstein's anomaly: presentation and outcome from fetus to adult. J Am Coll Cardiol 1994; 23:170-176.  Back to cited text no. 3  [PUBMED]  
4.Roberson DA, Silverman NH. Ebstein's anomaly: echocardiographic and clinical features in the fetus and neonate. J Am Coll Cardiol 1989; 14:1300-1307   Back to cited text no. 4  [PUBMED]  
5.Saxena A, Shrivastava S, Kothari SS. Value of antenatal echocardiography in high risk patients to diagnose congenital cardiac defects in fetus. Indian J Pediatr 1995;62:575-582.   Back to cited text no. 5    
6.Hornberger LK, Sahn DJ, Kleinman CS, Copel JA, Reed KL.Tricuspid valve disease with significant tricuspid insufficiency in the fetus: diagnosis and outcome. J Am Coll Cardiol 1991;17:167-173.  Back to cited text no. 6  [PUBMED]  
7.Ebstein's anomaly: presentation and outcome from fetus to adult. Celermajer DS, Bull C, Till JA, Cullen S, Vassillikos VP, Sullivan ID, Allan L, Nihoyannopoulos P,Somerville J, Deanfield JE. J Am Coll Cardiol 1994;23:170-6.  Back to cited text no. 7  [PUBMED]  
8.Paladini D, Chita SK, Allan LD. Prenatal measurement of cardiothoracic ratio in evaluation of heart disease. Arch Dis Child 1990;65:20-3  Back to cited text no. 8  [PUBMED]  

Correspondence Address:
R D Kawade
Dept Of Radiodiagnosis, Rural Medical College , Pravara Institue Of Medical Sciences , Loni, BK Pin : 413736, Ahmednagar
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0971-3026.32360

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  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7], [Figure - 8], [Figure - 9]


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