Search

Background: Portopulmonary hypertension (POPH) is the presence of pulmonary arterial (PA) hypertension in patients with portal hypertension and is associated with significant morbidity and mortality. In a cohort of POPH patients, we describe the clinical outcomes of POPH patients who underwent liver transplantation (LT)., Methods: Retrospectively collected data from a prospectively assembled cohort of all consecutive POPH adults evaluated in 3 transplant centers from 1996 to 2019., Results: From a cohort of 228 POPH patients, 50 patients underwent LT. Significant hemodynamic improvement after PA-targeted therapy was observed, with 58% receiving only monotherapy pretransplant. After LT, 21 (42%) patients were able to discontinue and remained off PA-targeted therapy. The 1-, 3-, and 5-y unadjusted survival rates after LT were 72%, 63%, and 60%, respectively. An elevated pulmonary vascular resistance (PVR) before LT was associated with worse survival rate (HR, 1.91; 95% CI, 1.07-3.74, P = 0.04). No survival difference was observed in those granted MELD exception or transplants performed before or after the year 2010., Conclusions: Significant number of POPH patients discontinued PA-targeted therapy after LT. Higher PVR before LT was associated with worse survival, as was monotherapy use. Despite effective PA-targeted therapies, POPH survival outcomes after LT in our cohort were modest and may reflect the need for more aggressive therapy., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Liver arteriovenous malformations (AVM) in hereditary hemorrhagic telangiectasia (HHT) can necessitate liver transplantation. There is limited data on HHT patients undergoing liver transplantation (LT) in the United States., Methods: Two sources of data were used: (1) Scientific Registry of Transplant Recipients (SRTR) database (1998-2016) (2) Single center liver transplant database (Mayo Clinic Rochester, MN). The aims of this study were (1) to determine trends in LT for HHT-related liver involvement in the United States using the SRTR database; (2) to identify clinical characteristics, indications, and outcomes for LT in HHT., Results: Thirty-nine HHT patients were listed for LT in the SRTR database from 1998-2016 to 1998-2001 (n = 1); 2002-2005 (n = 4); 2006-2010 (n = 10), and 2011-2016 (n = 24). Twenty-four underwent LT at a median age of 47.5 years (interquartile range, 37.0-58.5 years). Median calculated MELD score at time of LT was 8.0 (interquartile range, 7.0-9.5), and 75% received an exception MELD score. Two status-1 patients died during transplant surgery. Nineteen (86%) patients were alive after a median post-LT follow-up of 48 months, whereas 2 patients were lost to follow-up. Five of the aforementioned HHT patients underwent LT at Mayo Clinic, 4 with high output cardiac failure, and 1 with biliary ischemia. All 5 were alive at the time of last follow-up with good graft function and resolution of heart failure., Conclusions: Outcomes after LT for HHT patients are excellent with 86% survival after a median follow-up of 48 months and resolution of heart failure. LT listing for HHT has increased in substantially in more recent eras.

Background: The current Organ Procurement Transplantation Network policy grants Model for End-Stage Liver Disease (MELD) exception points to patients with portopulmonary hypertension (POPH), but potentially important factors, such as severity of liver disease and pulmonary hypertension, are not included in the exception score, and may affect survival. The purpose of this study was to identify significant predictors of waitlist mortality in patients with POPH., Methods: We performed a retrospective cohort study of patients in the Organ Procurement and Transplantation Network database with hemodynamics consistent with POPH (defined as mean pulmonary arterial pressure >25 mm Hg and pulmonary vascular resistance [PVR] ≥240 dynes·s·cm) who were approved for a POPH MELD exception between 2006 and 2014. Using a Cox proportional hazards model, we identified predictors of waitlist mortality (or removal for clinical deterioration)., Results: One hundred ninety adults were included. Age (hazard ratio [HR], 1.04; 95% confidence interval [CI], 1.00-1.08; P = 0.0499), initial native MELD score (HR, 1.11; 95% CI, 1.05-1.17; P < 0.001), and initial PVR (HR, 1.12 per 100 dynes·s·cm; 95% CI, 1.02-1.23; P = 0.02) were the only significant univariate predictors of waitlist mortality and remained significant predictors in a multivariate model, which had a c-statistic of 0.71. PVR and mean pulmonary arterial pressure were not significant predictors of posttransplant mortality., Conclusions: Both the severity of liver disease and POPH (as assessed by MELD and PVR, respectively) were significantly associated with waitlist, but not posttransplant, mortality in patients with approved MELD exceptions for POPH. Both factors should potentially be included in the POPH MELD exception score to more accurately reflect waitlist mortality risk.

Background: Pulmonary hypertension (PH) exists when mean pulmonary artery pressure (mPAP) is 25 mm Hg or greater. mPAP of 35 mm Hg or greater, with increased pulmonary vascular resistance from portopulmonary hypertension, has been associated with increased post-liver transplant (LT) mortality. However, mPAP of 35 mm Hg or greater can also occur in the setting of normal pulmonary vascular resistance from a high flow state and/or increased pulmonary venous volume. We describe the frequency of patients with mPAP of 35 mm Hg or greater at LT and correlate their clinical outcomes with hemodynamic characteristics., Methods: We reviewed hemodynamics of 300 consecutive adult patients undergoing LT. Primary and secondary outcomes were transplant hospitalization mortality and 1-year mortality., Results: Overall, PH was documented in 117 (39%) of 300 transplant recipients. Five patients were receiving ongoing treatment for portopulmonary hypertension at the time of transplant. mPAP of 35 mm Hg or greater was seen in 31 of 300 patients (10.3%; median, 38 mm Hg; range, 35-46 mm Hg). No intraoperative deaths occurred. Transplant hospitalization mortality was 0% for those with mPAP of 35 mm Hg or greater (vs 2.2% if mPAP < 35 mm Hg, P = 1.0). One-year mortality was similar for patients with and without mPAP of 35 mm Hg or greater (3.2% vs 6.0%, P = 1.0). No deaths were associated with right heart failure., Conclusions: At the time of LT, 39% of recipients had PH (mPAP ≥ 25 mm Hg) and 10.3% had mPAP ≥ 35 mm Hg. When associated with a high flow state and/or increased volume, transplant hospitalization and 1-year posttransplant outcomes were not adversely affected.

Two distinct pulmonary vascular disorders, hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH) may occur as a consequence of hepatic parenchymal or vascular abnormalities. HPS and POPH have major clinical implications for liver transplantation. A European Respiratory Society Task Force on Pulmonary-Hepatic Disorders convened in 2002 to standardize the diagnosis and guide management of these disorders. These International Liver Transplant Society diagnostic and management guidelines are based on that task force consensus and should continue to evolve as clinical experience dictates. Based on a review of over 1000 published HPS and POPH articles identified via a MEDLINE search (1985-2015), clinical guidelines were based on, selected single care reports, small series, registries, databases, and expert opinion. The paucity of randomized, controlled trials in either of these disorders was noted. Guidelines are presented in 5 parts; I. Definitions/Diagnostic criteria; II. Hepatopulmonary syndrome; III. Portopulmonary hypertension; IV. Implications for liver transplantation; and V. Suggestions for future clinical research.

Introduction: Pulmonary hypertension (PH) is common in patients on dialysis where it is associated with reduced survival. The possible association between PH and kidney transplant recipient survival has not been previously evaluated., Methods: In this retrospective study, we screened for PH pretransplant in 215 transplant candidates using cardiac echocardiography and measurements of right ventricular systolic pressure (RVSP)., Results: Sixty-eight percent of patients had normal RVSP (<35 mm Hg), 47 (22%) had mild to moderately elevated RVSP (36-50), and 22 (10%) had markedly elevated RVSP more than 50 suggestive of severe PH. Time on dialysis was the strongest correlate of an elevated RVSP (r=0.253, P<0.001) and this relationship was independent of other variables. Elevated RVSP was observed in 25%, 25%, 38%, and 58% of patients not on dialysis, on dialysis for less than 1 year, more than 1 to 2 years, or more than 2 years, respectively. An RVSP more than 50 was associated with significantly reduced posttransplant survival (hazard ratio =3.75 [1.17-11.97], P=0.016). This relationship seemed to be independent of other variables including older age, reduced left ventricular ejection fraction, low serum albumin, and delayed graft function., Conclusion: These analyses suggest for the first time that pretransplant PH correlates with patient survival after kidney transplantation.