Title:
Stenting of Ductus Venosus as a Palliation for Severe Portal Hypertension due to Congenital Hepatic Fibrosis Introduction:
Portal hypertension due to liver cirrhosis is a significant cause of morbidity and mortality. Treatment options in these patients include symptomatic treatment of esophageal varices via endoscopic treatment, symptomatic management of ascites, and liver transplant. Portosystemic shunt creation can be challenging in newborns and infants. We present a newborn with severe portal hypertension, esophageal varices, upper GI bleeding, and severe ascites refractory to medical management who was palliated via stenting the ductus venosus. To the best of our knowledge, stenting of the patent ductus venosus to create a portosystemic shunt to relieve portal hypertension has not been reported in English literature.
Clinical Case:
A one-month-old, late preterm male weighing 3.1 kg with a complex medical history including complete trisomy 21, atrial septal defect, duodenal atresia (status-post surgical repair), congenital hypothyroidism, congenital hepatic fibrosis, and severe portal hypertension with ascites and esophageal varices causing recurrent upper gastrointestinal bleeding was being managed for worsening ascites with multiple paracenteses. An echocardiogram revealed an atrial septal defect with patent but diminutive ductus venosus with a 9-mmHg gradient across the ductus venosus. He was discussed with the liver transplant team who recommended waiting until at least his body weight is 10 kg to be considered a candidate for liver transplant. He was therefore considered for stent implantation in the ductus venosus as a palliation for portal hypertension while waiting for liver transplant. Vascular access was obtained in the right internal jugular vein using a 5 Fr Prelude Ideal
TM sheath (Merit Medical, Utah, USA). An 0.014” choice wire was advanced across the ductus venosus and a 4 Fr angle glide catheter was advanced inside the ductus venosus and an angiogram was obtained which demonstrated a small ductus venosus (~1.3mm in diameter). It also demonstrated a large-caliber portal venous with a mean portal venous pressure of 14 mmHg against mean IVC pressure of 4 mmHg. Pulmonary vascular resistance was normal (~1.1 iWU). He underwent angioplasty of the ductus venosus using a 3mm NC Trek
ã balloon (Abbott, Chicago, IL). The ductus venosus was then stented using a 5x18 mm Resolute Onyx
ã (Medtronic, Minneapolis, MN) zotarolimus-eluting stent. Echocardiogram demonstrated good positioning of the stent with complete coverage of the ductus venosus and laminar flow from the portal vein to the IVC. He did well post-procedure with mild elevation in serum ammonia. His last follow up at the age of 5 months demonstrated resolution of ascites and no further episodes of upper GI bleed.
Discussion:
Ductus venosus generally closes within 2 weeks after birth. Management of liver cirrhosis, severe portal hypertension, and resulting ascites and variceal upper GI bleeding can be difficult especially in newborns and infants. Stent implantation in the ductus venosus can create a portosystemic shunt to relieve portal hypertension as a palliative strategy for children with severe portal hypertension.