Inclusion of Hepatic Venous Drainage in Patients with Pulmonary Arteriovenous Fistulas

Background: It is well known that hepatic vein (HV) inclusion can ameliorate cyanosis in patients with pulmonary arteriovenous fistulas (PAVFs) during the sequence of Fontan type repair. Previously, we reported that most patients with bidirectional cavopulmonary shunt (BCPS) have clinical or subclinical evidence of a right to left shunt through PAVFs. Methods: We studied 33 patients who already had clinical and subclinical PAVFs after BCPS. All patients have taken Fontan completion with HV inclusion. The state of PAVFs was reevaluated by pulmonary angiogram, contrast echocardiography, and lung scintigraphy 7.7 ± 2.4 years after HV inclusion. Results: After Fontan completion, the mean oxygen saturation increased from 80.2 ± 7.4% to 91.5 ± 9.8% in the entire cohort. Moreover, the amount of right-to-left shunting through the PAVFs, measured by lung scintigraphy, was decreased from a mean of 23.8 ± 15.1 to 13.0 ± 8.2%. The degree of severity, for most patients, was decreased as demonstrated by contrast echocardiography. However, 5 patients (16.7%) showed persistent PAVFs, even after the HV inclusion. They all had left isomerism with azygous continuation of the IVC and the conduit was positioned on the contralateral side to the SVC with azygous drainage. Conclusions: Most PAVFs regressed after Fontan completion. Left isomerism with azygous continuation of the IVC had risk for persistent PAVFs when the HV conduit was positioned at the contralateral side to the SVC receiving the azygous drainage. Therefore, appropriate design avoiding unilateral streaming of HV flow should be considered for HV inclusion surgery.