Creation of an Endovascular Bypass for Hepatopathy in Inferior Vena Cava Atresia

Purpose: To delineate a subset of patients with declining hepatopathy secondary to congenital obstruction of the inferior vena cava. We outline a multidisciplinary approach to evaluation and a procedure for early intervention.

Materials and Methods: A 30-year-old man underwent several months of repeat emergency department visits for abdominal pain and nausea. An outpatient hepatology workup was undertaken during which a liver biopsy was prompted, revealing severe venous congestion without cirrhosis. During evaluation for surgical portocaval shunt creation, venography confirmed abrupt occlusion of the intrahepatic inferior vena cava (IVC) with retrograde flow and collateralization. The hepatic veins were identified draining into the IVC below the level of the occlusion. The patient continued to decline, including rising transaminitis, worsening lower extremity edema, and abdominal varicosities. A multidisciplinary liver team along with a review of the literature determined that given the patient’s largely patent hepatic venous drainage, transvenous recanalization of the right hepatic vein to the nonatretic IVC segment just inferior to the right atrial ostia would provide a minimally invasive and durable treatment option for this patient.

Results: Right internal jugular access was obtained and a target snare was passed to the level of obstruction. Via transhepatic access of the right hepatic vein, a venogram demonstrated stasis of contrast at the expected junction with the IVC. Blunt puncture of the RHV and IVC was performed with the back end of a stiff wire for through-and-through access. A tract was formed and reinforced with coaxial 10-mm ´ 4-cm and 12-mm ´ 6-cm stents. Technical success was achieved as brisk flow via the tract with cessation of collateral flow was established. After overnight monitoring and an unremarkable hepatic sonogram, the patient was discharged. Early clinical success was noted at follow-up, and functional status continued to improve along with resolution of abdominal varices. Follow-up sonography and liver biopsy are pending.

Conclusions: When managed expeditiously by an interdisciplinary team, a subset of patients progressing to cirrhosis and transplant may be offered a minimally invasive alternative. Several factors are crucial for the success of the procedure, including pathology findings of venous congestion without cirrhosis as well as a patent portion of hepatic vein with viable trajectory to a patent caval outflow.