Department of Medicine, School of Medicine and Public Health, University of Wisconsin-Madison, Madison, WI.
School of Pharmacy, University of Wisconsin-Madison, Madison, WI.
Transplantation. 2021 Feb 1;105(2):346-353. doi: 10.1097/TP.0000000000003248.
Portopulmonary hypertension is present in an estimated 5.3% to 8.5% of liver transplant candidates. Untreated, 5-year survival is estimated between 14% and 28%. Moderate-severe disease is a contraindication to liver transplant due to the high perioperative mortality, but patients optimized with pulmonary vasodilator therapy can become eligible for transplant. There is minimal data regarding posttransplant outcomes and ability to discontinue pulmonary vasodilator therapy posttransplant.
We performed a single-center retrospective analysis to evaluate long-term outcomes of patients with moderate-severe portopulmonary hypertension who were optimized with pulmonary vasodilator therapy, became eligible for liver transplant, and subsequently underwent transplant. We identified 24 patients optimized with pulmonary vasodilator therapy who underwent subsequent liver transplantation and 25 patients who were treated with pulmonary vasodilator therapy alone.
In the transplanted cohort, 1-year survival from portopulmonary hypertension diagnosis date: 95.8%, 3-year survival: 90.9%, and 5-year survival: 90.9%. Posttransplant; 1-, 3-, and 5-year survival was 86.9%. Among transplanted patients, 41.6% (10/24) were optimized with nonparenteral therapy. Following transplantation, 100% (14/14) of the surviving patients were able to discontinue parenteral therapy; median time: 7.2 months (interquartile range: 5.1-8.9 mo), while 61.9% (13/21) were able to discontinue pulmonary vasodilator therapy altogether; median time: 13.9 months (interquartile range: 5.1-17.6 mo).
Patients who are optimized with pulmonary vasodilator therapy before liver transplant can have excellent long-term outcomes posttransplant. Oral pulmonary vasodilator therapy can be effective treatment to qualify a patient for transplant, and the majority are able to wean from pulmonary vasodilator therapy entirely posttransplant.
据估计，在肝移植候选者中，有 5.3%至 8.5%存在门脉肺高压。未经治疗，5 年生存率估计在 14%至 28%之间。中度至重度疾病是肝移植的禁忌症，因为围手术期死亡率很高，但接受肺动脉扩张剂治疗的患者可以有资格接受移植。关于移植后的结果和移植后停止肺动脉扩张剂治疗的能力的数据很少。
我们进行了一项单中心回顾性分析，以评估接受肺动脉扩张剂治疗后病情得到优化、有资格接受肝移植并随后接受移植的中度至重度门脉肺高压患者的长期结果。我们确定了 24 例接受肺动脉扩张剂治疗并随后接受肝移植的患者和 25 例仅接受肺动脉扩张剂治疗的患者。
在移植组中，从门脉肺高压诊断日期算起的 1 年生存率：95.8%，3 年生存率：90.9%，5 年生存率：90.9%。移植后，1、3 和 5 年的生存率分别为 86.9%。在移植患者中，41.6%（10/24）接受了非肠外治疗的优化。移植后，100%（14/14）幸存患者能够停止肠外治疗；中位时间：7.2 个月（四分位距：5.1-8.9 个月），而 61.9%（13/21）能够完全停止肺动脉扩张剂治疗；中位时间：13.9 个月（四分位距：5.1-17.6 个月）。
在肝移植前接受肺动脉扩张剂治疗的患者在移植后可以获得极好的长期结果。口服肺动脉扩张剂治疗是使患者有资格接受移植的有效治疗方法，大多数患者在移植后可以完全停止肺动脉扩张剂治疗。
