Cor triatriatum dexter masquerading as Ebstein's anomaly

Abstract

We report the case of a child with cor triatriatum dexter masquerading as Ebstein's anomaly on transthoracic echocardiography. This was attributed to a floppy membrane arising from the right atrium, protruding into the tricuspid valve and pushing the leaflets downwards, giving an impression of Ebstein's anomaly. The importance of recognising this pitfall will prevent misdiagnosis of Ebstein's anomaly.

Rare combination of bilateral divided atrial chambers and pulmonary vein stenosis with review of the literature

“…[9][10][11] One case presented with recurrent syncope and sudden death because of a sail-like structure that obstruct the right ventricle inflow, 11 although others might be misdiagnosed because of misleading echocardiographic images confusing it with more common lesions such as Ebstein's anomaly. 12 The majority of cases with prominent Chiari network with no obstruction or minor obstruction will pass undetected. 8,13 Echocardiography is usually diagnostic and will suffice in most cases 14 to plan and proceed with the surgical management.…”

“…The patient may present at a later age with cyanosis because of streaming of the blood across an atrial communication 9 11 One case presented with recurrent syncope and sudden death because of a sail-like structure that obstruct the right ventricle inflow, 11 although others might be misdiagnosed because of misleading echocardiographic images confusing it with more common lesions such as Ebstein’s anomaly 12 . The majority of cases with prominent Chiari network with no obstruction or minor obstruction will pass undetected 8 …”

Persistent embryonic right venous valve giving a cor triatriatum dexter appearance in a cyanotic neonate

“…Aberrations of the tricuspid valve, including Ebstein anomaly, may mislead the diagnosis. 4 Surgical resection is warranted when persistent desaturation is observed.…”

Cor triatriatum dexter: unusual features in utero and after birth

“…In contrast, in two reports regarding paediatric cases, the cardiac features were initially considered as Ebstein's anomaly with the Chiari's network [1,2], due to the displacement of the tricuspid valve by a moving membrane; their cases also had hypoplastic right ventricle, associated in the second one to the tricuspid regurgitation. On the contrary, in our case, the tricuspid valve was normally inserted, not dysplastic and with only a tiny flow into the right ventricular cavity.…”

“…The membrane sometimes mimicks Ebstein's anomaly [1,2]. Frequently, the anomalies of the right-sided sections are associated.…”