DuBrock Hilary M, Goldberg David S, Sussman Norman L, Bartolome Sonja D, Kadry Zakiyah, Salgia Reena J, Mulligan David C, Kremers Walter K, Kawut Steven M, Krowka Michael J, Channick Richard N
Department of Medicine, Massachusetts General Hospital Boston, MA USA.
Department of Medicine, Perelman School of Medicine at the University of Pennsylvania Philadelphia, PA USA.
Transplantation. 2017 Jul;101(7):1609-1615. doi: 10.1097/TP.0000000000001666.
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.
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).
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.
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.
当前器官获取与移植网络（Organ Procurement Transplantation Network）政策给予终末期肝病模型（Model for End-Stage Liver Disease，MELD）例外积分给患有门肺高压（portopulmonary hypertension，POPH）的患者，但一些潜在的重要因素，如肝病严重程度和肺动脉高压，并未纳入例外积分中，而这些因素可能会影响生存率。本研究的目的是确定POPH患者等待名单上死亡的显著预测因素。
我们对器官获取与移植网络数据库中2006年至2014年间被批准获得POPH MELD例外的患者进行了一项回顾性队列研究，这些患者的血流动力学符合POPH（定义为平均肺动脉压>25 mmHg且肺血管阻力[PVR]≥240 dynes·s·cm）。使用Cox比例风险模型，我们确定了等待名单上死亡（或因临床病情恶化而被移除）的预测因素。
共纳入190名成年人。年龄（风险比[HR]，1.04；95%置信区间[CI]，1.00 - 1.08；P = 0.0499）、初始原生MELD评分（HR，1.11；95% CI，1.05 - 1.17；P < 0.001）和初始PVR（每100 dynes·s·cm的HR，1.12；95% CI，1.02 - 1.23；P = 0.02）是等待名单上死亡的仅有的显著单变量预测因素，并且在多变量模型中仍然是显著预测因素，该多变量模型的c统计量为0.71。PVR和平均肺动脉压不是移植后死亡的显著预测因素。
肝病严重程度和POPH（分别通过MELD和PVR评估）均与获得POPH MELD例外批准的患者等待名单上的死亡显著相关，但与移植后死亡无关。这两个因素都应潜在地纳入POPH MELD例外积分中，以更准确地反映等待名单上的死亡风险。
