Nishimura Masato, Tokoro Toshiko, Yamazaki Satoru, Hashimoto Tetsuya, Kobayashi Hiroyuki, Ono Toshihiko
Cardiovascular Division, Toujinkai Hospital, 83-1, Iga, Momoyama-cho, Fushimi-ku, Kyoto, 612-8026, Japan.
Department of Nephrology, Toujinkai Hospital, Kyoto, Japan.
Clin Exp Nephrol. 2017 Dec;21(6):1088-1096. doi: 10.1007/s10157-016-1344-y. Epub 2016 Oct 19.
We examined the prevalence, prognosis, and effect of endothelin receptor antagonists on survival in end-stage kidney disease patients with idiopathic pre-capillary pulmonary hypertension.
We investigated 1988 end-stage kidney disease patients in Toujinkai Hospital from January 1, 2001 to December 31, 2014. Pulmonary hypertension was screened by symptoms (dyspnea, hypotension, or near syncope) and echocardiography, and diagnosed by computed tomography with enhancement, pulmonary flow scintigraphy, and right heart catheterization.
Fifteen patients (67 ± 11 years; 12 women and 3 men) were diagnosed as idiopathic pre-capillary pulmonary hypertension; mean pulmonary arterial pressure, pulmonary vascular resistance, or pulmonary artery wedge pressure were 55 ± 11 mmHg, 7.5 ± 2.9 Woods units, or 12 ± 2 mmHg, respectively. Of the 15 patients, 14 received hemodialysis, and 1 was in a pre-dialysis stage. Patients were followed through December 31, 2015, and 11 died of heart failure; their mean survival time was 26.4 ± 21.0 months. Endothelin receptor antagonists were used for 11 patients, and mean survival times were 57.3 ± 12.1 months in patients with endothelin receptor antagonists and 7.5 ± 2.1 months in those without. In the Kaplan-Meier analysis, heart failure death-free survival rates were higher in patients with endothelin receptor antagonists than in those without (P < 0.001); 100 versus 25 % at one year and 71 versus 0 % at 3 years.
The prognosis of idiopathic pre-capillary pulmonary hypertension seems to be poor in end-stage kidney disease patients. Administration of endothelin receptor antagonists might improve the survival by inhibiting heart failure death. Registration of clinical trials This study was registered to the ClinicalTrials.gov ( https://clinicaltrials.gov/ ): protocol identifier, NCT02743091.
我们研究了内皮素受体拮抗剂对特发性毛细血管前肺动脉高压的终末期肾病患者的患病率、预后及生存的影响。
我们调查了2001年1月1日至2014年12月31日期间东京开医院的1988例终末期肾病患者。通过症状（呼吸困难、低血压或接近晕厥）和超声心动图筛查肺动脉高压，并通过增强计算机断层扫描、肺血流闪烁显像和右心导管检查进行诊断。
15例患者（67±11岁；12名女性和3名男性）被诊断为特发性毛细血管前肺动脉高压；平均肺动脉压、肺血管阻力或肺动脉楔压分别为55±11mmHg、7.5±2.9伍兹单位或12±2mmHg。15例患者中，14例接受血液透析，1例处于透析前阶段。对患者随访至2015年12月31日，11例死于心力衰竭；他们的平均生存时间为26.4±21.0个月。11例患者使用了内皮素受体拮抗剂，使用内皮素受体拮抗剂的患者平均生存时间为57.3±12.1个月，未使用的患者为7.5±2.1个月。在Kaplan-Meier分析中，使用内皮素受体拮抗剂的患者无心力衰竭死亡生存率高于未使用的患者（P<0.001）；1年时分别为100%对25%，3年时为71%对0%。
特发性毛细血管前肺动脉高压在终末期肾病患者中的预后似乎较差。给予内皮素受体拮抗剂可能通过抑制心力衰竭死亡来提高生存率。临床试验注册 本研究已在ClinicalTrials.gov（https://clinicaltrials.gov/）注册：方案标识符，NCT02743091。
