Liver Transplantation for Porto-sinusoidal Vascular Liver Disorder: Long-term Outcome

Abstract

Background. Porto-sinusoidal vascular liver disorder (PSVD) is a rare disease that occasionally requires liver transplantation (LT), despite usually presenting preserved liver function. There remains a paucity of data pertaining to LT in PSVD. The aim was to identify features associated with post-LT outcomes in PSVD. Methods. Retrospective multicentre study of 79 patients who received LT for PSVD. Results. Median post-LT follow-up was 37 (range 1-261) mo. Refractory ascites 24 (30%), hepatic encephalopathy 16 (20%), and hepatopulmonary syndrome 13 (16.3%) were the most frequent indications for LT. Hepatocellular carcinoma was the indication in only 2 patients. Twenty-four patients died, 7 due to liver and 17 to non-liver related causes. Post-LT survival was 82.2%, 80.7%, and 68.6% at 1, 2, and 5 y, respectively. Post-LT survival was significantly better in patients without (n = 58) than in those with a persistent severe PSVD-associated condition (n = 21). Pre-LT hyperbilirubinemia levels and creatinine >100 µmol/L were also independently associated with poor survival. Six patients (7.6%) required a second LT. Recurrence of PSVD was confirmed by liver biopsy in only 1 patient and in 3 further patients it was likely. Conclusions. LT in PSVD is associated with an acceptable outcome in the absence of associated severe conditions. However, persistence of a severe associated condition, pre-LT high bilirubin levels, or creatinine >100 µmol/L impact outcome, and these are features that should be considered when evaluating PSVD patients for LT. PSVD recurrence is possible after LT and needs to be explored, at least, in cases of posttransplant portal hypertension.

Updates in noncirrhotic portal hypertension

“…Liver transplantation has also been performed in such younger patients with excellent outcomes for refractory ascites, HE, hepatopulmonary syndrome, and refractory bleeding from ectopic varices although recurrence of disease is well known. [11,12] Prognosis INCPH has traditionally been considered a nonprogressive disease although quality studies on natural history are limited. Patients with INCPH have a higher risk of VH and PVT than those with compensated cirrhosis, thus mandating a relook on the benign nature of the disease and justifying PVT screening.…”

“…Splenectomy with devascularization can be performed for patients with refractory variceal bleeds and/or symptomatic hypersplenism as the lifespan of transjugular intrahepatic portosystemic shunt is shorter (for a disease of the young patient), and a splenic abscess is not uncommon with splenic artery embolization. Liver transplantation has also been performed in such younger patients with excellent outcomes for refractory ascites, HE, hepatopulmonary syndrome, and refractory bleeding from ectopic varices although recurrence of disease is well known 11,12 …”

Management of liver disease and portal hypertension in common variable immunodeficiency (CVID)

“…Taking into account the limited data and poor prognosis associated with liver disease in CVID, a careful benefit-risk balance may help in clinical decision making: Benefits include the life-saving nature of LT, particularly in young patients. 100 , 101 On the other hand, risks include the persistence of the immune disturbance and a high risk of infectious complications. Therefore, careful consideration should be given to LT in CVID.…”

Evidence for Alloimmune Sinusoidal Injury in De Novo Nodular Regenerative Hyperplasia After Liver Transplantation

“…Of the etiologies for NRH in native liver, azathioprine and vascular issues are higher up on the list of differential causes, however the number of unexplained cases remains high [ 11 ]. Porto-sinusoidal vascular disease (PSVD), which produces NRH is reported as an uncommon cause of recurrent disease after LT [ 14 ]. Chronic antibody-mediated rejection (cAMR), less well defined than acute antibody-mediated rejection (aAMR), is suspected by the Banff group to be one of the likely causes of NRH [ 15 , 16 ].…”