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Clinical Trial
. 2017 Dec;21(6):1088-1096.
doi: 10.1007/s10157-016-1344-y. Epub 2016 Oct 19.

Idiopathic pre-capillary pulmonary hypertension in patients with end-stage kidney disease: effect of endothelin receptor antagonists

Masato Nishimura  1 , Toshiko Tokoro  2 , Satoru Yamazaki  3 , Tetsuya Hashimoto  3 , Hiroyuki Kobayashi  3 , Toshihiko Ono  3
Affiliations
Clinical Trial

Idiopathic pre-capillary pulmonary hypertension in patients with end-stage kidney disease: effect of endothelin receptor antagonists

Masato Nishimura et al. Clin Exp Nephrol. 2017 Dec.

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.

Conclusion: 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.

Keywords: End-stage kidney disease; Endothelin receptor antagonist; Pre-capillary; Pulmonary hypertension; Right heart catheterization.

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Conflict of interest statement

Conflict of interest

All the authors have declared no competing interest.

Research involving human participants

All procedures performed in studies involving human participants were in accordance with the ethical standards of The Ethics Committee for Human Research of Toujinkai Hospital (Approved No. 1512), and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

Written informed consent was obtained from the patients with idiopathic pre-capillary PH, or the family of the patients when the patients were dead.

Figures

Fig. 1
Fig. 1
Flow chart of patient selection. The levels of dyspnea were World Health Organization functional class ≥ II, and hypotension was in a state of systolic blood pressure consistently below 100 mmHg. LVEF left ventricular ejection fraction, AVF arteriovenous fistula, AS aortic valvular stenosis, MS mitral valvular stenosis, PH pulmonary hypertension
Fig. 2
Fig. 2
Changes in systolic pressure gradients in tricuspid valve at onset of pulmonary hypertension and at 3 months after the onset. Mean systolic pressure gradients in tricuspid valve tended to increase in 4 patients without endothelin receptor antagonists (a) (77.5 ± 11.1 versus 92.5 ± 18.7 mmHg, P = 0.097), whereas those decreased in patients administered with endothelin receptor antagonists (b) (78.8 ± 15.7 versus 46.8 ± 32.7 mmHg, P = 0.015)
Fig. 3
Fig. 3
Heart failure death-free survival rates in patients with or without endothelin receptor antagonists. Mean survival time in patients without endothelin receptor antagonists was 7.5 ± 2.1 months. Heart failure death-free survival rates at 1 and 3 years were 100 and 71 % in patients with endothelin receptor antagonists, whereas they were 25 and 0 % in patients without
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