title: Idiopathic pre-capillary pulmonary hypertension in patients with end-stage kidney disease: effect of endothelin receptor antagonists.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Cardiovascular Division, Toujinkai Hospital, 83-1, Iga, Momoyama-cho, Fushimi-ku, Kyoto, 612-8026, Japan. mnishimura@tea.ocn.ne.jp.
authors.forename: Masato
authors.initials: M
authors.lastname: Nishimura
authors.affiliation: Department of Nephrology, Toujinkai Hospital, Kyoto, Japan.
authors.forename: Toshiko
authors.initials: T
authors.lastname: Tokoro
authors.affiliation: Department of Urology, Toujinkai Hospital, Kyoto, Japan.
authors.forename: Satoru
authors.initials: S
authors.lastname: Yamazaki
authors.affiliation: Department of Urology, Toujinkai Hospital, Kyoto, Japan.
authors.forename: Tetsuya
authors.initials: T
authors.lastname: Hashimoto
authors.affiliation: Department of Urology, Toujinkai Hospital, Kyoto, Japan.
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Kobayashi
authors.affiliation: Department of Urology, Toujinkai Hospital, Kyoto, Japan.
authors.forename: Toshihiko
authors.initials: T
authors.lastname: Ono
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D065128:Endothelin Receptor Antagonists / Q000627:therapeutic use*; D005260:Female; D006333:Heart Failure / Q000209:etiology / Q000401:mortality / Q000517:prevention & control*; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000188:drug therapy / Q000401:mortality / Q000503:physiopathology; D007564:Japan / Q000453:epidemiology; D007676:Kidney Failure, Chronic / Q000150:complications*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies
chemical_list: D065128:Endothelin Receptor Antagonists
keywords: End-stage kidney disease; Endothelin receptor antagonist; Pre-capillary; Pulmonary hypertension; Right heart catheterization