Background Polycystic liver disease (PCLD) is characterized by cystic replacement of the hepatic parenchyma, leading to hepatic dysfunction, portal hypertension, and hepatomegaly. Patients with liver dysfunction and/or symptomatic disease are eligible for liver transplantation. However, little is known about these patients' waitlist outcomes relative to others with chronic liver disease. Methods We used Organ Procurement and Transplantation Network/United Network for Organ Sharing data from February 27, 2002 to December 31, 2015 to compare waitlist outcomes of adult patients with PCLD to those with chronic liver failure (CLF) and hepatocellular carcinoma. Results The study cohort included 620 patients with PCLD, 18 240 patients with hepatocellular carcinoma, and 98 567 patients with CLF. Compared with CLF patients, PCLD patients had significantly lower bilirubin and international normalized ratio at waitlisting, and less ascites and encephalopathy. However, they were significantly more likely to have severe chronic kidney disease. Moreover, patients with PCLD were more than 70% more likely to be transplanted compared with patients with CLF (odds ratio, 1.72; 95% confidence interval, 1.46-2.02) and had significantly longer posttransplant survival (P < 0.001). PCLD patients with exceptions were 5.7 times more likely to be transplanted than those without (odds ratio, 5.67; 95% confidence interval, 3.95-8.15) and measures of hepatic/renal dysfunction were inversely associated with the receipt of exceptions. Conclusions Despite having more preserved liver synthetic function than patients with CLF on the waitlist, patients with PCLD are preferentially transplanted because they frequently receive exception points in an unstandardized fashion.
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