Asplenia syndrome

Case contributed by Dr Mostafa El-Feky

Presentation

Congenital cardiac disease.

Patient Data

Age: 1 year
Gender: Male
CT

Chest

Visceroatrial situs: Solitus                                         

Cardiac position: Levocardia

Atrioventricular and ventriculoarterial connections: discordant.

This is a non-ECG-gated angiographic study not suited for assessment of intracardiac anatomy. 

The anatomical right ventricle is located on the left side, with right ventricular dilatation and hypertrophy. The anatomical left ventricle is hypoplastic and located on the right.

Atrioventricular septal defect noted.

The aorta is seen arising from the anatomical right ventricle.

Aplasia of the main pulmonary artery is noted.

Double SVC noted, the right SVC drains into the right atrium and the left SVC drains into the left atrium.

Supra-cardiac TAPVR, the pulmonary veins drains into the right SVC.

PDA stent noted ending in the left and right pulmonary arterial confluence, showing internal hypodensity through its upper portion, suggestive of partial thrombosis.

The hemi-azygous vein drains into the left SVC, showing non-opacified lumen, mostly delayed filling.

Right-sided aortic arch, with mirror image branching pattern.

Non-visualization of the spleen in the upper abdominal scan (asplenia).

Bilateral trilobed lung.

Case Discussion

Features are suggestive of right isomerism (asplenia syndrome). It is associated with congenital heart anomalies in 50% of cases.

PlayAdd to Share

Case information

rID: 73083
Published: 17th Jan 2020
Last edited: 17th Jan 2020
Inclusion in quiz mode: Included
Institution: Dar El Ashaa Center

Updating… Please wait.

 Unable to process the form. Check for errors and try again.

 Thank you for updating your details.