Ebstein anomaly in an elderly patient

Case contributed by Dr Vincent Tatco

Presentation

Patient presented with shortness of breath and underwent CT angiography of the pulmonary arteries to rule out pulmonary thromboembolism.

Patient Data

Age: 60
Gender: Female

The main pulmonary trunk arises from the right ventricle. The pulmonary arteries are well opacified without intraluminal defects to suggest pulmonary thromboembolism. The main pulmonary trunk, right and left pulmonary arteries are within normal calibers. There is no demonstrable peripheral stenosis, aneurysm or definite arteriovenous malformation.

The bilateral superior and inferior pulmonary veins drain into the left atrium.

The heart is enlarged with a markedly dilated right atrium and leftward bowing of the atrial septum. The tricuspid valve is displaced towards the cardiac apex resulting to an atrialized proximal portion of the right ventricle and a small functional right ventricle. The atrialized portion of the right ventricle is dilated with associated leftward bowing of the ventricular septum. The right ventricular outflow tract is also dilated. The left atrium and left ventricle are within normal size.  These findings are compatible with Ebstein anomaly.

There is reflux of contrast into the inferior vena cava and hepatic veins as well as into the dilated coronary sinus which is suggestive of tricuspid regurgitation.

Minimal calcifications are seen along the aortic and mitral valve annuli. 

The right-sided superior and inferior vena cavae drain into the right atrium. 

The aorta is normal in caliber and arises from the left ventricle. The arch and descending segment of the aorta are left-sided. 

The aorta, coronary arteries and aortic arch branches are atherosclerotic.

Case Discussion

This case shows Ebstein anomaly with tricuspid regurgitation in an elderly patient.  The anomaly was also demonstrated on echocardiography. Ebstein anomaly occurs in about 1 of every 200,000 live births and initial presentation by older patients is rare, as only 5% survive beyond the fifth decade. Patients often have symptoms associated with heart failure due to the dilated and atrialized right ventricle compressing the left ventricle and, additionally, reducing preload for the left heart.

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Case information

rID: 42794
Case created: 8th Feb 2016
Last edited: 16th Apr 2017
Systems: Cardiac, Chest
Inclusion in quiz mode: Included

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