Thakral, Nimish

AbstractBackgroundNodular regenerative hyperplasia (NRH) is a rare condition characterized clinically by the development of non‐cirrhotic portal hypertension. NRH is the histopathological result in the liver of various systemic disease processes including autoimmune disorders, haematological malignancies and medications. However, natural history of this condition has been limited to small case series while patient outcomes pertaining to different aetiologies of NRH are largely unknown.MethodsA retrospective cohort of consecutive patients diagnosed with pathology‐confirmed NRH at Mayo Clinic between 2002 and 2017 was identified. The histological diagnosis of NRH was determined by expert liver pathologists. Patients with metastatic liver disease, history of liver transplantation or younger than 18 were excluded. Potential aetiologies of NRH were classified as haematological, rheumatological, drug‐associated, miscellaneous or idiopathic. Long‐term mortality was analysed using Kaplan‐Meier estimation and Cox regression models.ResultsOne hundred and sixty‐seven consecutive patients with pathology‐confirmed NRH were analysed over a 15‐year period and followed for a median time of 50 months (1–306 months). The mean age at diagnosis was 53 years. No aetiology or risk factor for NRH was identified in the majority of patients (94, 56.3%), whereas an associated, possibly causal, condition was found in 73 patients (secondary NRH). The most common presenting feature was elevated liver tests (80%), but no significant differences in laboratory tests were seen based on aetiology of NRH. Compared to idiopathic NRH, those with an identified cause had a higher rate of splenomegaly at presentation (54% vs. 27%, p = 0.002). Portal hypertension‐related complications at diagnosis were common, with ascites present in one‐third of patients. Overall transplant‐free survival was 63% at 5 years. Median survival in idiopathic NRH was 9.4 years compared to 7.3 years in secondary NRH. Age, renal function and volume status at presentation were significantly associated with survival; however, MELD score was not.ConclusionsThe rates of liver‐related complications and mortality in NRH are low, and only a small number of patients ultimately require liver transplantation. Most patients do not have an identified risk factor or aetiology for NRH, and liver‐related outcomes do not appear to differ based on associated, possibly causal, conditions.

Background and Aims Hepatologists often determine whether transcatheter aortic valve replacement (TAVR) or surgical aortic valve replacement (SAVR) is preferred for patients with cirrhosis and severe aortic stenosis. The goal of this cohort study is to compare outcomes following TAVR and SAVR in patients with cirrhosis to inform the preferred intervention. Approach and Results Prospectively collected data on 105 consecutive patients with cirrhosis and aortic stenosis who underwent TAVR (n = 55) or SAVR (n = 50) between 2008 and 2016 were reviewed retrospectively. Two control groups were included: 2,680 patients without cirrhosis undergoing TAVR and SAVR and 17 patients with cirrhosis who received medical therapy alone. Among the 105 patients with cirrhosis, the median Society of Thoracic Surgeons score was 3.8% (1.5, 6.9), and the median Model for End‐Stage Liver Disease (MELD) score was 11.6 (9.4, 14.0). The TAVR group had similar in‐hospital (1.8% vs. 2.0%) and 30‐day mortality (3.6% vs. 4.2%) as the SAVR group. During the median follow‐up of 3.8 years (95% confidence interval, 3.0‐6.9), there were 63 (60%) deaths. MELD score (adjusted hazard ratio, 1.13; 95% confidence interval, 1.05‐1.21; P = 0.002) was an independent predictor of long‐term survival. In the subgroup of patients with MELD score <12, the TAVR group had reduced survival compared with the SAVR group (median survival of 2.8 vs. 4.4 years; P = 0.047). However, in those with MELD score ≥12, survival after TAVR, SAVR, and medical therapy was similar (1.3 vs. 2.1 vs. 1.6 years, respectively; P = 0.53). Conclusion In select patients with cirrhosis, both TAVR and SAVR have acceptable and comparable short‐term outcomes. MELD score, but not Society of Thoracic Surgeons score, independently predicts long‐term survival after TAVR and SAVR. For patients with MELD score <12, SAVR is a preferred procedure; however, neither procedure appears superior to medical therapy in patients with MELD score ≥12.