Department of Cardiovascular Medicine, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Science.
Division of Cardiology, Okayama Medical Center.
Circ J. 2020 Jan 24;84(2):245-251. doi: 10.1253/circj.CJ-19-0784. Epub 2019 Dec 21.
The waiting period for lung transplantation (LT) is approximately 3 years in Japan. The prognosis of patients with pulmonary arterial hypertension (PAH) awaiting LT is poor without LT. Patients at the present center often survive in the long term after registration for LT. The aim of this study was to elucidate why some patients survive in the long term by investigating changes in pulmonary artery pressure (PAP) after registration, and medication used.
This study involved 57 patients with PAH who were enrolled in a registry for LT at Okayama University Hospital. We divided patients into 3 groups according to outcome: LT (n=27); death without LT (n=21); and survival without LT (n=9). The median interval from PAH diagnosis to epoprostenol treatment was shorter in the survival group (58 days) than in the LT group (378 days) and death group (545 days). Eight patients in the survival group, 13 in the LT group, and 13 in the death group underwent right heart catheterization after registration. Percent change in mean PAP after registration was significantly greater in the survival group (-32%) than in the LT group (-13%) and death group (1%; P<0.01).
Even after LT registration, patients who received epoprostenol infusion soon after diagnosis of PAH often had marked reduction in PAP and long-term survival without LT.
在日本，肺移植（LT）的等待期约为 3 年。未经 LT 治疗，肺动脉高压（PAH）患者的预后较差。目前中心的患者在 LT 登记后往往能长期存活。本研究旨在通过研究 LT 登记后肺动脉压（PAP）的变化和用药情况，阐明为何一些患者能长期存活。
本研究纳入了在冈山大学医院登记 LT 的 57 例 PAH 患者。我们根据结局将患者分为 3 组：LT（n=27）；未 LT 死亡（n=21）；未 LT 存活（n=9）。存活组从 PAH 诊断到依前列醇治疗的中位间隔时间（58 天）短于 LT 组（378 天）和死亡组（545 天）。登记后有 8 例存活组、13 例 LT 组和 13 例死亡组接受了右心导管检查。登记后平均 PAP 的百分比变化在存活组（-32%）显著大于 LT 组（-13%）和死亡组（1%；P<0.01）。
即使在 LT 登记后，PAH 诊断后很快接受依前列醇输注的患者，PAP 往往明显降低，且无需 LT 即可长期存活。
