Catheter approach to redirect hepatic venous return for treatment of unilateral pulmonary arteriovenous malformations after fontan.

OBJECTIVES

The goal of this report is to describe a percutaneous approach to rerouting hepatic venous return in patients who developed progressive cyanosis due to unilateral pulmonary arteriovenous malformations (PAVM) after the total cavopulmonary connection (TCPC) operation.

BACKGROUND

Unilateral PAVM can develop in patients after TCPC operation when there is unequal distribution of hepatic venous return between the two lungs. This often results in progressive cyanosis and the need for surgical re-intervention. A percutaneous based approach for rerouting hepatic venous return has never been described.

METHODS

We retrospectively reviewed the clinical data on four patients who underwent percutaneous rerouting procedures. One patient with a misaligned TCPC underwent realignment of the circuit with a bare metal stent. In three patients a combination of bare metal and covered stents were needed to achieve the desired results.

RESULTS

The rerouting procedures were successful in all patients with significant improvement in oxygen saturation from a median of 75% (range 55-80%) to a median of 90% (range 84-92%) (P = 0.02). There were no recorded short term or intermediate term complications with maximum follow-up time of 43 months.

CONCLUSIONS

Percutaneous rerouting of hepatic venous flow is feasible and should be considered when a surgical approach is not possible; this strategy may serve as a viable alternative to complex operative approaches in select cases. Furthermore studies are needed to determine the long-term efficacy of this procedure.