Stratta C, Lavezzo B, Ballaris M A, Panio A, Crucitti M, Andruetto P, Fanelli V, Grosso Marra W, Ranieri M V, Salizzoni M
Anestesia e Rianimazione 9, Centro Trapianto di Fegato, Torino, Italy.
Transplant Proc. 2013 Sep;45(7):2774-5. doi: 10.1016/j.transproceed.2013.07.001.
Portopulmonary hypertension has been reported in 2% to 9% of candidates for liver transplantation (OLT). If it is moderate to severe, it represents a contraindication to the procedure until pulmonary vasodilatative therapy has been optimized. We report the case of a 43-year-old man, scheduled for OLT due to alcoholic cirrhosis with hemosiderosis. His Model for End-Stage Liver Disease was 25 at that time. The preoperative evaluation showed a severe alteration of diffusion (pO2 68 mm Hg), without hepatopulmonary syndrome or portopulmonary hypertension (PPH) upon basal and dobutamine stress echocardiography. At the beginning of the OLT the hemodynamic profile showed mean pulmonary artery pressure (mPAP) 38 mm Hg, wedge pressure (WP) 19 mm Hg, cardiac output (CO) 9.1 L/min, pulmonary vascular resistance (PVR) 166 dyne s/cm(5), transpulmonary gradient (TPG) 19 mm Hg, which lead us to promptly initiate inhaled nitric oxide (iNO) and intravenous epoprostenol 2 to 5 ng/kg/min. Upon graft reperfusion the hemodynamic profile was: mPAP 47 mm Hg, WP 23 mm Hg, CO 14.2 L/min, PVR 135 dyne s/cm(5), TPG 24 mm Hg, and at the end of surgery, mPAP 39 mm Hg, WP 20 mm Hg, CO 10.6 L/min, PVR 123 dyne s/cm(5), TPG 19 mm Hg. On postoperative day (POD) 3, we observed severe worsening of PPH: mPAP 60 mm Hg, WP 10 mm Hg, CO 9.8 L/min, PVR 395 dyne s/cm(5), TPG 50 mm Hg even with maximal pulmonary vasodilatatory therapy (ambrisentan 5 mg, intravenous sildenafil 20 mg × 3 and epoprostenol 22 ng/kg/min, iNO). Severe acute respiratory distress syndrome (ARDS) was presents. Therefore we decided to begin veno-venous extracorporeal membrane oxygenation (v-v ECMO) to correct the hypoxic vasoconstriction. Subsequent weaning from inotropic support with iNO and epoprostenol was possible on POD 7 due to mPAP 42 mm Hg, WP 15 mm Hg, CO 7.9 L/min, PVR 273 dyne s/cm(5), and TPG 27 mm Hg. On POD 11 he was weaned from ECMO due to: mPAP 40 mm Hg, WP 16 mm Hg, CO 6.5 L/min, PVR 295 dyne s/cm(5) and TPG 24 mm Hg. The patient was extubated on POD 17. The cardiac catheterization 1 month after OLT showed: mPAP 28 mm Hg, WP 13 mm Hg, CO 5.4 L/min, PVR 220 dyne s/cm(5) and TPG 15 mm Hg. ECMO rescue therapy in this "extreme" case allowed us to correct hypoxemia responsible for worsening of pulmonary hypertension allowing time to reach the goal of vasodilatatory therapy.
据报道，肝移植（OLT）候选者中2%至9%的人患有门肺高压。如果是中度至重度，在优化肺血管扩张治疗之前，这是该手术的禁忌症。我们报告了一例43岁男性患者，因酒精性肝硬化伴含铁血黄素沉着症而计划进行OLT。当时他的终末期肝病模型评分为25分。术前评估显示弥散严重改变（动脉血氧分压68 mmHg），基础和多巴酚丁胺负荷超声心动图检查未发现肝肺综合征或门肺高压（PPH）。OLT开始时，血流动力学参数显示平均肺动脉压（mPAP）38 mmHg，楔压（WP）19 mmHg，心输出量（CO）9.1 L/min，肺血管阻力（PVR）166达因·秒/厘米⁵，跨肺压梯度（TPG）19 mmHg，这使我们立即开始吸入一氧化氮（iNO）并静脉输注依前列醇2至5 ng/kg/min。移植肝再灌注时的血流动力学参数为：mPAP 47 mmHg，WP 23 mmHg，CO 14.2 L/min，PVR 135达因·秒/厘米⁵，TPG 24 mmHg，手术结束时，mPAP 39 mmHg，WP 20 mmHg，CO 10.6 L/min，PVR 123达因·秒/厘米⁵，TPG 19 mmHg。术后第3天（POD 3），我们观察到PPH严重恶化：mPAP 60 mmHg，WP 10 mmHg，CO 9.8 L/min，PVR 395达因·秒/厘米⁵，TPG 50 mmHg，即使采用最大剂量的肺血管扩张治疗（安立生坦5 mg、静脉注射西地那非20 mg×3次以及依前列醇22 ng/kg/min、iNO）。出现了严重的急性呼吸窘迫综合征（ARDS）。因此，我们决定开始进行静脉-静脉体外膜肺氧合（v-v ECMO）以纠正低氧性血管收缩。随后，由于mPAP 42 mmHg，WP 15 mmHg，CO 7.9 L/min，PVR 273达因·秒/厘米⁵，TPG 27 mmHg，在POD 第7天停用了iNO和依前列醇的血管活性药物支持。在POD 11天，由于mPAP 40 mmHg，WP 16 mmHg，CO 6.5 L/min，PVR 295达因·秒/厘米⁵，TPG 24 mmHg，他脱离了ECMO。患者于POD 17天拔管。OLT术后1个月的心脏导管检查显示：mPAP 28 mmHg，WP 13 mmHg，CO 5.4 L/min，PVR 220达因·秒/厘米⁵，TPG 15 mmHg。在这个“极端”病例中，ECMO挽救治疗使我们能够纠正导致肺动脉高压恶化的低氧血症，从而有时间实现血管扩张治疗的目标。
