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Predictors of Waitlist Mortality in Portopulmonary Hypertension.

ABSTRACT: 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.

SUBMITTER: DuBrock HM

PROVIDER: S-EPMC5481480 | biostudies-literature | 2017 Jul

REPOSITORIES: biostudies-literature

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Predictors of Waitlist Mortality in Portopulmonary Hypertension.

DuBrock Hilary M HM Goldberg David S DS Sussman Norman L NL Bartolome Sonja D SD Kadry Zakiyah Z Salgia Reena J RJ Mulligan David C DC Kremers Walter K WK Kawut Steven M SM Krowka Michael J MJ Channick Richard N RN

Transplantation 20170701 7

<h4>Background</h4>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.<h4>Methods</h4>We performed a ...[more]

PMID: 28207639

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Project description:BackgroundPortopulmonary hypertension (POPH), pulmonary arterial hypertension that develops in the setting of portal hypertension, can lead to right-sided heart failure and death. Being female is a known risk factor for POPH, but little is known about the effect of sex on clinical manifestations, hemodynamics, treatment response, and survival.Research questionWe sought to characterize sex differences in clinical characteristics, pulmonary hemodynamics, treatment response, and survival in patients with POPH.Study design and methodsWe performed a retrospective cohort study of adult candidates for liver transplant (LT) who had POPH within the Organ Procurement and Transplantation Network database. Females and males were compared. Multivariate regression was performed to assess the association between sex and pulmonary vascular resistance (PVR) and survival. Patients were also stratified by age (50 years) to determine how age modifies the relationship between sex and hemodynamics and survival.ResultsWe included 190 adults (103 male, 87 female). Compared with men, women had a lower model for end-stage liver disease (MELD) score (12.1± 4.2 vs 13.8 ± 4.9; P = .01) and were more likely to have autoimmune liver disease. Women had a higher baseline PVR (610.6 ± 366.6 vs 461.0 ± 185.3 dynes-s-cm-5; P < .001) and posttreatment PVR (244.6 ± 119.5 vs 202.0 ± 87.7 dynes-s-cm-5; P = .008) and a greater treatment response (ΔPVR) (-359.3 ± 381.9 vs -260.2 ± 177.3 dynes-s-cm-5; P = .03). In multivariate analysis, female sex (or gender) remained associated with a higher baseline PVR (P = .008). Women and men had overall similar survival (P > .05). When patients were stratified by age, being female was independently associated with worse waiting list survival after adjusting for MELD and PVR in younger patients (HR, 6.61; 95% CI, 1.25-35.08; P = .03) but not in older patients.InterpretationCompared with male candidates, female candidates for LT who had POPH had a higher PVR and lower MELD score and were more likely to have autoimmune liver disease. Women and men had similar overall survival, but female sex (or gender) was associated with worse survival in younger patients.

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Predictors of Waitlist Mortality in Portopulmonary Hypertension.

Publications

Predictors of Waitlist Mortality in Portopulmonary Hypertension.

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