Skip to main content
Top
Published in: BMC Pulmonary Medicine 1/2019

Open Access 01-12-2019 | Idiopathic Pulmonary Fibrosis | Research article

Borderline pulmonary hypertension is associated with exercise intolerance and increased risk for acute exacerbation in patients with interstitial lung disease

Authors: Kenji Nemoto, Shuji Oh-ishi, Tatsuya Akiyama, Yuki Yabuuchi, Hitomi Goto, Mizu Nonaka, Yuika Sasatani, Hiroaki Tachi, Naoki Arai, Hiroaki Ishikawa, Kentaro Hyodo, Isano Hase, Yukiko Miura, Takio Takaku, Kenji Hayashihara, Takefumi Saito

Published in: BMC Pulmonary Medicine | Issue 1/2019

Login to get access

Abstract

Background

Pulmonary hypertension (PH) is traditionally defined as a resting mean pulmonary artery pressure (mPAP) of ≥25 mmHg, while mPAP in the range of 21 to 24 mmHg is recognized as “borderline PH.” Interstitial lung disease (ILD) is complicated by the development of PH, which is known to be linked with exercise intolerance and a poor prognosis. Even though it has recently been recommended that PH is redefined as a mPAP of > 20 mmHg, little is known about the clinical significance of borderline PH in ILD. We evaluated whether borderline PH has an impact on the exercise capacity, risk of acute exacerbation (AE), and mortality in patients with ILD.

Methods

A total of 80 patients with ILD who underwent right heart catheterization (RHC) between November 2013 and October 2016 were included. The patients were divided into 3 groups according to the mPAP values: mPAP ≤20 mmHg (No-PH group; n = 56), 20 < mPAP < 25 mmHg (Bo-PH group; n = 18), and mPAP ≥25 mmHg (PH group; n = 6). The demographic, hemodynamic, spirometric, and 6-min walk test (6MWT) data of the patients were collected. In addition, the 1-year incidence of AEs and 1-year survival of the patients after the initial RHC were also evaluated.

Results

There were no significant differences among the 3 groups in the mean age, pulmonary function parameters or the PaO2, however, 6-min walk distance was significantly lower in both the Bo-PH and PH groups (p < 0.001 for both) as compared to the No-PH group. The results of the Kaplan-Meier analysis revealed that while there was no significant difference in the 1-year survival rate among the three groups, the 1-year incidence of AEs was significantly higher in both the Bo-PH and PH groups (p < 0.001, p = 0.023, respectively) as compared to the No-PH group.

Conclusions

The current study suggested that borderline PH may be associated with poorer exercise tolerance and an increased risk of AEs in patients with ILD. Therefore, the physicians should pay close attention to the presence of even mild elevation of the mPAP at the initial evaluation in patients with ILD.
Literature
1.
go back to reference Hoeper MM, Bogaard HJ, Condliffe R, Frantz R, Khanna D, Kurzyna M, et al. Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol. 2013;62:D42–50.CrossRef Hoeper MM, Bogaard HJ, Condliffe R, Frantz R, Khanna D, Kurzyna M, et al. Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol. 2013;62:D42–50.CrossRef
2.
go back to reference Simonneau G, Montani D, Celermajer DS, Denton CP, Gatzoulis MA, Krowka M, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 2019;53:1801913.CrossRef Simonneau G, Montani D, Celermajer DS, Denton CP, Gatzoulis MA, Krowka M, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 2019;53:1801913.CrossRef
3.
go back to reference Lettieri CJ, Nathan SD, Barnett SD, Ahmad S, Shorr AF. Prevalence and outcomes of pulmonary arterial hypertension in advanced idiopathic pulmonary fibrosis. Chest. 2006;129:746–52.CrossRef Lettieri CJ, Nathan SD, Barnett SD, Ahmad S, Shorr AF. Prevalence and outcomes of pulmonary arterial hypertension in advanced idiopathic pulmonary fibrosis. Chest. 2006;129:746–52.CrossRef
4.
go back to reference Patel NM, Lederer DJ, Borczuk AC, Kawut SM. Pulmonary hypertension in idiopathic pulmonary fibrosis. Chest. 2007;132:998–1006.CrossRef Patel NM, Lederer DJ, Borczuk AC, Kawut SM. Pulmonary hypertension in idiopathic pulmonary fibrosis. Chest. 2007;132:998–1006.CrossRef
5.
go back to reference Nathan SD, Noble PW, Tuder RM. Idiopathic pulmonary fibrosis and pulmonary hypertension. Am J Respire Crit Care Med. 2007;175:875–80.CrossRef Nathan SD, Noble PW, Tuder RM. Idiopathic pulmonary fibrosis and pulmonary hypertension. Am J Respire Crit Care Med. 2007;175:875–80.CrossRef
6.
go back to reference Cottin V, Nunes H, Brillet PY, Delaval P, Devouassoux G, Tillie-Leblond I, et al. Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity. Eur Respir J. 2005;26:586–93.CrossRef Cottin V, Nunes H, Brillet PY, Delaval P, Devouassoux G, Tillie-Leblond I, et al. Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity. Eur Respir J. 2005;26:586–93.CrossRef
7.
go back to reference Cottin V, Pavec JL, Prévot G, Mal H, Humbert M, Simonneau G, Cordier JF, GERM“O”P. Pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema syndrome. Eur Respir J. 2010;35:105–11.CrossRef Cottin V, Pavec JL, Prévot G, Mal H, Humbert M, Simonneau G, Cordier JF, GERM“O”P. Pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema syndrome. Eur Respir J. 2010;35:105–11.CrossRef
8.
go back to reference Takahashi K, Taniguchi H, Ando M, Sakamoto K, Kondoh Y, Watanabe N, et al. Mean pulmonary arterial pressure as a prognostic indicator in connective tissue disease associated with interstitial lung disease: a retrospective cohort study. BMC Pulm Med. 2016;16:55.CrossRef Takahashi K, Taniguchi H, Ando M, Sakamoto K, Kondoh Y, Watanabe N, et al. Mean pulmonary arterial pressure as a prognostic indicator in connective tissue disease associated with interstitial lung disease: a retrospective cohort study. BMC Pulm Med. 2016;16:55.CrossRef
9.
go back to reference Seeger W, Adir Y, Barberà JA, Champion H, Coghlan JG, Cottin V, et al. Pulmonary hypertension in chronic lung disease. J Am Coll Cardiol. 2013;62:D109–16.CrossRef Seeger W, Adir Y, Barberà JA, Champion H, Coghlan JG, Cottin V, et al. Pulmonary hypertension in chronic lung disease. J Am Coll Cardiol. 2013;62:D109–16.CrossRef
10.
go back to reference Judge EP, Fabre A, Adamali HI, Egan JJ. Acute exacerbations and pulmonary hypertension in advanced idiopathic pulmonary fibrosis. Eur Respir J. 2012;40:93–100.CrossRef Judge EP, Fabre A, Adamali HI, Egan JJ. Acute exacerbations and pulmonary hypertension in advanced idiopathic pulmonary fibrosis. Eur Respir J. 2012;40:93–100.CrossRef
11.
go back to reference Minai OA, Santacruz JF, Alster JM, Budev MM, McCarthy K. Impact of pulmonary hemodynamics on 6-min walk test in idiopathic pulmonary fibrosis. Respir Med. 2012;106:1613–21.CrossRef Minai OA, Santacruz JF, Alster JM, Budev MM, McCarthy K. Impact of pulmonary hemodynamics on 6-min walk test in idiopathic pulmonary fibrosis. Respir Med. 2012;106:1613–21.CrossRef
12.
go back to reference Hamada K, Nagai S, Tanaka S, Handa T, Shigematsu M, Nagao T, et al. Significance of pulmonary arterial pressure and diffusion capacity of the lung as prognosticator in patients with idiopathic pulmonary fibrosis. Chest. 2007;131:650–6.CrossRef Hamada K, Nagai S, Tanaka S, Handa T, Shigematsu M, Nagao T, et al. Significance of pulmonary arterial pressure and diffusion capacity of the lung as prognosticator in patients with idiopathic pulmonary fibrosis. Chest. 2007;131:650–6.CrossRef
13.
go back to reference Kimura M, Taniguchi H, Kondoh Y, Kimura T, Kataoka K, Nishiyama O, et al. Pulmonary hypertension as a prognostic indicator at the initial evaluation in idiopathic pulmonary fibrosis. Respiration. 2013;85:456–63.CrossRef Kimura M, Taniguchi H, Kondoh Y, Kimura T, Kataoka K, Nishiyama O, et al. Pulmonary hypertension as a prognostic indicator at the initial evaluation in idiopathic pulmonary fibrosis. Respiration. 2013;85:456–63.CrossRef
14.
go back to reference Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183:788–824.CrossRef Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183:788–824.CrossRef
15.
go back to reference American Thoracic Society. Standardization of spirometry, 1994 update. Am J Respir Crit Care Med. 1995;152:1107–36.CrossRef American Thoracic Society. Standardization of spirometry, 1994 update. Am J Respir Crit Care Med. 1995;152:1107–36.CrossRef
16.
go back to reference ATS Committee on Proficiency Standards for Clinical Pulmonary Function Laboratories. ATS statement: guidelines for the six-minute walk test. Am J Respire Crit Care Med. 2002;166:111–7.CrossRef ATS Committee on Proficiency Standards for Clinical Pulmonary Function Laboratories. ATS statement: guidelines for the six-minute walk test. Am J Respire Crit Care Med. 2002;166:111–7.CrossRef
17.
go back to reference Collard HR, Ryerson CJ, Corte TJ, Jenkins G, Kondoh Y, Lederer DJ, et al. Acute exacerbation of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2016;194:265–75.CrossRef Collard HR, Ryerson CJ, Corte TJ, Jenkins G, Kondoh Y, Lederer DJ, et al. Acute exacerbation of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2016;194:265–75.CrossRef
18.
go back to reference Park IN, Kim DS, Shim TS, Kin CM, Lee SD, Koh Y, et al. Acute exacerbation of interstitial pneumonia other than idiopathic pulmonary fibrosis. Chest. 2007;132:214–20.CrossRef Park IN, Kim DS, Shim TS, Kin CM, Lee SD, Koh Y, et al. Acute exacerbation of interstitial pneumonia other than idiopathic pulmonary fibrosis. Chest. 2007;132:214–20.CrossRef
19.
go back to reference Suzuki A, Taniguchi H, Watanabe N, Kondoh Y, Kimura T, Kataoka K, et al. Significance of pulmonary arterial pressure as a prognostic indicator in lung-dominant connective tissue disease. PLoS One. 2014;9:e108339.CrossRef Suzuki A, Taniguchi H, Watanabe N, Kondoh Y, Kimura T, Kataoka K, et al. Significance of pulmonary arterial pressure as a prognostic indicator in lung-dominant connective tissue disease. PLoS One. 2014;9:e108339.CrossRef
20.
go back to reference Song JW, Hong SB, Lim CM, Koh Y, Kim DS. Acute exacerbation of idiopathic pulmonary fibrosis: incidence, risk factors and outcome. Eur Respir J. 2011;37:356–63.CrossRef Song JW, Hong SB, Lim CM, Koh Y, Kim DS. Acute exacerbation of idiopathic pulmonary fibrosis: incidence, risk factors and outcome. Eur Respir J. 2011;37:356–63.CrossRef
21.
go back to reference Farkas L, Gauldie J, Voelkel NF, Kolb M. Pulmonary hypertension and idiopathic pulmonary fibrosis. A tale of angiogenesis, apoptosis, and growth factors. Am J Respir Cell Mol Biol. 2011;45:1–15.CrossRef Farkas L, Gauldie J, Voelkel NF, Kolb M. Pulmonary hypertension and idiopathic pulmonary fibrosis. A tale of angiogenesis, apoptosis, and growth factors. Am J Respir Cell Mol Biol. 2011;45:1–15.CrossRef
Metadata
Title
Borderline pulmonary hypertension is associated with exercise intolerance and increased risk for acute exacerbation in patients with interstitial lung disease
Authors
Kenji Nemoto
Shuji Oh-ishi
Tatsuya Akiyama
Yuki Yabuuchi
Hitomi Goto
Mizu Nonaka
Yuika Sasatani
Hiroaki Tachi
Naoki Arai
Hiroaki Ishikawa
Kentaro Hyodo
Isano Hase
Yukiko Miura
Takio Takaku
Kenji Hayashihara
Takefumi Saito
Publication date
01-12-2019
Publisher
BioMed Central
Published in
BMC Pulmonary Medicine / Issue 1/2019
Electronic ISSN: 1471-2466
DOI
https://doi.org/10.1186/s12890-019-0932-5

Other articles of this Issue 1/2019

BMC Pulmonary Medicine 1/2019 Go to the issue
Obesity Clinical Trial Summary

At a glance: The STEP trials

A round-up of the STEP phase 3 clinical trials evaluating semaglutide for weight loss in people with overweight or obesity.

Developed by: Springer Medicine

Highlights from the ACC 2024 Congress

Year in Review: Pediatric cardiology

Watch Dr. Anne Marie Valente present the last year's highlights in pediatric and congenital heart disease in the official ACC.24 Year in Review session.

Year in Review: Pulmonary vascular disease

The last year's highlights in pulmonary vascular disease are presented by Dr. Jane Leopold in this official video from ACC.24.

Year in Review: Valvular heart disease

Watch Prof. William Zoghbi present the last year's highlights in valvular heart disease from the official ACC.24 Year in Review session.

Year in Review: Heart failure and cardiomyopathies

Watch this official video from ACC.24. Dr. Biykem Bozkurt discuss last year's major advances in heart failure and cardiomyopathies.