Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, and AP-HP, Service de Pneumologie, Hôpital Bicêtre, Le Kremlin Bicêtre; INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France.
AP-HP Hôpital Paul-Brousse, Centre Hépato-Biliaire, Villejuif, F-94800, France; Univ Paris-Sud, UMR-S 1193, Université Paris-Saclay, Villejuif, F-94800, France; Inserm, Unité 1193, Université Paris-Saclay, Villejuif, F-94800, France; DHU Hepatinov, Villejuif, F-94800, France.
Hepatology. 2017 May;65(5):1683-1692. doi: 10.1002/hep.28990. Epub 2017 Mar 14.
Portopulmonary hypertension (PoPH) is diagnosed in 2-6% of liver transplantation (LT) candidates. We studied outcomes of candidates for LT suffering from PoPH. Data were collected retrospectively from a prospective registry. Pulmonary hemodynamic variables were collected at the time of PoPH diagnosis, at last evaluation before LT, and within 6 months and beyond 6 months after LT. Forty-nine patients (35 males, 48 ± 8 years) were analyzed (median Model for End-Stage Liver Disease score 20). At baseline, mean pulmonary artery pressure (mPAP) was 44 ± 10 mm Hg (range 26-73 mm Hg), cardiac index was 3.5 ± 0.9 L/min/m , and pulmonary vascular resistance was 5.6 ± 2.8 Wood units. Hemodynamic reassessment performed in 35 patients who were treated with pulmonary arterial hypertension-targeted therapies before LT resulted in significant decreases in both mPAP (36 ± 7 versus 47 ± 10 mm Hg, P < 0.0001) and pulmonary vascular resistance (3.0 ± 1.4 versus 6.1 ± 3.1 Wood units, P < 0.0001). Fourteen patients (29%) died without having had access to LT. Thirty-five patients underwent LT and were followed up for a median of 38 months. Eight patients (23%) died after LT including 5 due to PoPH (after 1 day to 6 months). Among survivors (n = 27), all patients treated with intravenous epoprostenol were weaned off post-LT, and endothelin receptor antagonist or phosphodiesterase type 5 inhibitors were continued in 15/27 patients (55%). At last evaluation, 20/27 patients (74%) had mPAP <35 mm Hg and 8 of them (30%) had mPAP <25 mm Hg. Overall survival estimates after LT were 80%, 77%, and 77% at 6 months, 1 year, and 3 years, respectively.
Stabilization or reversibility of PoPH seems to be an attainable goal using the combination of pulmonary arterial hypertension-targeted therapies and LT in patients who are transplantation candidates. (Hepatology 2017;65:1683-1692).
本研究旨在探讨合并 PoPH 的 LT 候选者的结局。
我们回顾性地从一个前瞻性登记处收集了数据。在 PoPH 诊断时、LT 前的最后一次评估时、LT 后 6 个月内和 6 个月后收集了肺血流动力学变量。
49 例（35 例男性，48±8 岁）患者进行了分析（中位终末期肝病模型评分 20）。基线时，平均肺动脉压（mPAP）为 44±10mmHg（范围 26-73mmHg），心指数为 3.5±0.9L/min/m ，肺血管阻力为 5.6±2.8Wood 单位。在 35 例接受肺动脉高压靶向治疗的患者中进行了血液动力学再评估，结果 mPAP（36±7 与 47±10mmHg，P＜0.0001）和肺血管阻力（3.0±1.4 与 6.1±3.1Wood 单位，P＜0.0001）均显著降低。14 例（29%）患者在未接受 LT 治疗的情况下死亡。35 例患者接受 LT 治疗，并随访中位数为 38 个月。LT 后 8 例（23%）患者死亡，其中 5 例死于 PoPH（1 天后至 6 个月）。在幸存者（n=27）中，所有接受静脉依前列醇治疗的患者均在 LT 后停药，15/27 例（55%）患者继续使用内皮素受体拮抗剂或磷酸二酯酶 5 抑制剂。最后评估时，27 例患者中有 20 例（74%）mPAP＜35mmHg，其中 8 例（30%）mPAP＜25mmHg。LT 后 6 个月、1 年和 3 年的总体生存率估计分别为 80%、77%和 77%。
使用肺动脉高压靶向治疗联合 LT，合并 PoPH 的 LT 候选者的 PoPH 稳定或逆转似乎是可以实现的目标。
