Features of polysplenia syndrome (a.k.a. left sidedness or left isomerism):
- interruption of the hepatic segment of the IVC with azygos continuation (present in 65% of cases); the azygos vein is typically also enlarged
- multiple spleens - usually at least one almost normal sized spleen with numerous additional spleens of variable size
- dextrocardia is present in 35-40% of cases with the prevalence of congenital heart disease even higher at nearly 1 in 2 patients
- hepatic veins drain directly into the right atrium