MedStar Washington Hospital Center Washington, District of Columbia
Nuval Cherian, MD MedStar Washington Hospital Center, Washington, DC
Introduction: Non-cirrhotic portal hypertension (NCPH) is a heterogeneous group of disease characterized by elevated portal pressures in the absence of cirrhosis. While common in other parts of the world, such as parts of Asia, it is more rare in the United States. Complications of the disease include esophageal varices, ascites, and jaundice from portal hypertensive gastropathy. In rare circumstances, disease progresses to the point where liver transplant is required. There are few studies on patients listed for transplant with this disease process.
Methods: We requested patient records from UNOS for patients who underwent initial liver transplantation who were initially listed from January 2003 through December 2022 with a diagnosis of NCPH. Since this diagnosis is not specifically tracked by UNOS, we queried patient diagnoses for the text "noncirrhotic portal hypertension", "non-cirrhotic portal hypertension” or "NCPH". In total, 13 patients were found over the 20-year period. Here we describe the patient characteristics, disease characteristics, co-morbid conditions, and outcomes.
Results: Of the 13 patients who underwent transplant 1 died, 1 was lost to follow-up and 11 are still living at time of last follow up. Median follow up time after transplant was 3.90 years (1.95-4.92). Further patient data is described in table 1.
Discussion: Liver transplantation in NCPH is very poorly understood with only a few small studies describing it. Despite a very small sample, here we see that portal vein thromboses, TIPS procedures and prior abdominal surgeries seem to be prominent in these patients. Future studies should look at how these disease characteristics, along with other factors, affect outcomes so that we can better serve these patients. Given the rarity of transplantation for this disease, further study will require collaboration among multiple sites and systems. These studies would ideally also include data on variceal bleeding, a source of significant mortality in this disease that is not tracked by UNOS data
A limitation of this study is reliance on pre-transplant diagnosis. NCPH is often misdiagnosed in the pre-transplant period given lab and imaging findings that often mimic cirrhosis. A prior study of the disease showed that many of the transplant patients without evidence of cirrhosis on explant, did previously carry a diagnosis of cirrhosis. As such, the number of patient's with NCPH could potentially be underrepresented due to our methodology.
Disclosures:
Nuval Cherian indicated no relevant financial relationships.
Nuval Cherian, MD. P2391 - Patients Undergoing Liver Transplant for Non-Cirrhotic Portal Hypertension in the UNOS Database, ACG 2023 Annual Scientific Meeting Abstracts. Vancouver, BC, Canada: American College of Gastroenterology.
