CTICasesPulmonary AVMs from Hepatic Venous Exclusion in Heterotaxy
Imaging findings
A patient with heterotaxy (polysplenia, bilateral left-sidedness, bilateral hyparterial bronchi, dextrocardia, double-outlet right ventricle) with dual SVCs and azygos continuation of the IVC underwent a surgical repair in which the hepatic venous outflow (extracardiac Fontan) was directed into the left-sided SVC to the left lung, while systemic venous blood via the azygos continuation was routed to the right lung. On CT the left lung appears normal, but the right lung, which receives no hepatic venous blood, contains numerous macroscopic pulmonary arteriovenous malformations, including large ones.
Key takeaways
Pulmonary arteriovenous malformations develop in lung not receiving hepatic venous return, because a hepatic factor is thought to prevent AVM formation, the same mechanism underlying hepatopulmonary syndrome. In this surgically palliated heterotaxy patient, hepatic venous blood was directed entirely to the left lung, so florid macroscopic AVMs formed only in the right lung, a striking demonstration of the protective role of hepatic venous drainage.
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