Background: Assessment of prognosis is of major importance when deciding on a therapeutic strategy in patients with pulmonary arterial hypertension (PAH). Objectives: The aim of this study was to investigate the prognostic value of pulmonary hemodynamics during exercise and changes during treatment in patients with PAH. Methods: Consecutive incident patients (n = 49) with PAH undergoing right heart catheterization at rest and during a constant workload cycle exercise in supine position were included. Predictors of survival were identified at baseline using Cox proportional hazard regression models in a univariate analysis unadjusted and adjusted for age and gender. Results: During a median follow-up period of 42 months, 13 (27%) of the 49 patients studied died. Two predictors of death were found: rest-to-exercise changes in heart rate and systolic pulmonary artery pressure. Adjusted hazard ratios were 0.92 (95% CI 0.86–0.99) and 0.93 (95% CI 0.88–0.99), respectively. These 2 variables were correlated with each other (r = 0.55, p < 0.001). Conclusions: Rest-to-exercise changes in heart rate and systolic pulmonary artery pressure measured at diagnosis are predictors of survival in patients with PAH. These measurements taken from an exercise test reflect right ventricular function.

1.
Humbert
M
,
Sitbon
O
,
Chaouat
A
,
Bertocchi
M
,
Habib
G
,
Gressin
V
, et al
Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era
.
Circulation
.
2010
Jul
;
122
(
2
):
156
63
.
[PubMed]
0009-7322
2.
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
Jan
;
53
(
1
):
1801913
.
[PubMed]
0903-1936
3.
Galiè
N
,
Humbert
M
,
Vachiery
JL
,
Gibbs
S
,
Lang
I
,
Torbicki
A
, et al
2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT)
.
Eur Respir J
.
2015
Oct
;
46
(
4
):
903
75
.
[PubMed]
0903-1936
4.
Hassoun
PM
.
Drumming up prognostic significance in a heartbeat in pulmonary arterial hypertension
.
Am J Respir Crit Care Med
.
2012
Feb
;
185
(
4
):
354
5
.
[PubMed]
1073-449X
5.
Holverda
S
,
Gan
CT
,
Marcus
JT
,
Postmus
PE
,
Boonstra
A
,
Vonk-Noordegraaf
A
.
Impaired stroke volume response to exercise in pulmonary arterial hypertension
.
J Am Coll Cardiol
.
2006
Apr
;
47
(
8
):
1732
3
.
[PubMed]
0735-1097
6.
Vonk-Noordegraaf
A
,
Haddad
F
,
Chin
KM
,
Forfia
PR
,
Kawut
SM
,
Lumens
J
, et al
Right heart adaptation to pulmonary arterial hypertension: physiology and pathobiology
.
J Am Coll Cardiol
.
2013
Dec
;
62
(
25
Suppl
):
D22
33
.
[PubMed]
0735-1097
7.
Castelain
V
,
Chemla
D
,
Humbert
M
,
Sitbon
O
,
Simonneau
G
,
Lecarpentier
Y
, et al
Pulmonary artery pressure-flow relations after prostacyclin in primary pulmonary hypertension
.
Am J Respir Crit Care Med
.
2002
Feb
;
165
(
3
):
338
40
.
[PubMed]
1073-449X
8.
Provencher
S
,
Hervé
P
,
Sitbon
O
,
Humbert
M
,
Simonneau
G
,
Chemla
D
.
Changes in exercise haemodynamics during treatment in pulmonary arterial hypertension
.
Eur Respir J
.
2008
Aug
;
32
(
2
):
393
8
.
[PubMed]
0903-1936
9.
Chaouat
A
,
Sitbon
O
,
Mercy
M
,
Ponçot-Mongars
R
,
Provencher
S
,
Guillaumot
A
, et al
Prognostic value of exercise pulmonary haemodynamics in pulmonary arterial hypertension
.
Eur Respir J
.
2014
Sep
;
44
(
3
):
704
13
.
[PubMed]
0903-1936
10.
Blumberg
FC
,
Arzt
M
,
Lange
T
,
Schroll
S
,
Pfeifer
M
,
Wensel
R
.
Impact of right ventricular reserve on exercise capacity and survival in patients with pulmonary hypertension
.
Eur J Heart Fail
.
2013
Jul
;
15
(
7
):
771
5
.
[PubMed]
1388-9842
11.
Sanz
J
,
Sánchez-Quintana
D
,
Bossone
E
,
Bogaard
HJ
,
Naeije
R
.
Anatomy, Function, and Dysfunction of the Right Ventricle: JACC State-of-the-Art Review
.
J Am Coll Cardiol
.
2019
Apr
;
73
(
12
):
1463
82
.
[PubMed]
0735-1097
12.
Faure
M
,
Chaouat
A
,
Gomez
E
,
Mercy
M
,
Guillaumot
A
,
Chabot
F
.
Prognostic value of systolic pulmonary artery pressure change at exercise in pulmonary arterial hypertension
.
Eur Respir J
.
2015
;
46
: 0903-1936
13.
ATS Committee on Proficiency Standards for Clinical Pulmonary Function Laboratories
.
ATS statement: guidelines for the six-minute walk test
.
Am J Respir Crit Care Med
.
2002
Jul
;
166
(
1
):
111
7
.
[PubMed]
1073-449X
14.
Rhee
RL
,
Gabler
NB
,
Sangani
S
,
Praestgaard
A
,
Merkel
PA
,
Kawut
SM
.
Comparison of Treatment Response in Idiopathic and Connective Tissue Disease-associated Pulmonary Arterial Hypertension
.
Am J Respir Crit Care Med
.
2015
Nov
;
192
(
9
):
1111
7
.
[PubMed]
1073-449X
15.
Weatherald
J
,
Boucly
A
,
Chemla
D
,
Savale
L
,
Peng
M
,
Jevnikar
M
, et al
Prognostic Value of Follow-Up Hemodynamic Variables After Initial Management in Pulmonary Arterial Hypertension
.
Circulation
.
2018
Feb
;
137
(
7
):
693
704
.
[PubMed]
0009-7322
16.
Humbert
M
,
Sitbon
O
,
Chaouat
A
,
Bertocchi
M
,
Habib
G
,
Gressin
V
, et al
Pulmonary arterial hypertension in France: results from a national registry
.
Am J Respir Crit Care Med
.
2006
May
;
173
(
9
):
1023
30
.
[PubMed]
1073-449X
17.
Spruijt
OA
,
de Man
FS
,
Groepenhoff
H
,
Oosterveer
F
,
Westerhof
N
,
Vonk-Noordegraaf
A
, et al
The effects of exercise on right ventricular contractility and right ventricular-arterial coupling in pulmonary hypertension
.
Am J Respir Crit Care Med
.
2015
May
;
191
(
9
):
1050
7
.
[PubMed]
1073-449X
18.
Wensel
R
,
Opitz
CF
,
Anker
SD
,
Winkler
J
,
Höffken
G
,
Kleber
FX
, et al
Assessment of survival in patients with primary pulmonary hypertension: importance of cardiopulmonary exercise testing
.
Circulation
.
2002
Jul
;
106
(
3
):
319
24
.
[PubMed]
0009-7322
19.
Hasler
ED
,
Müller-Mottet
S
,
Furian
M
,
Saxer
S
,
Huber
LC
,
Maggiorini
M
, et al
Pressure-Flow During Exercise Catheterization Predicts Survival in Pulmonary Hypertension
.
Chest
.
2016
Jul
;
150
(
1
):
57
67
.
[PubMed]
0012-3692
20.
De Cort
SC
,
Innes
JA
,
Barstow
TJ
,
Guz
A
.
Cardiac output, oxygen consumption and arteriovenous oxygen difference following a sudden rise in exercise level in humans
.
J Physiol
.
1991
Sep
;
441
(
1
):
501
12
.
[PubMed]
0022-3751
21.
Grünig
E
,
Tiede
H
,
Enyimayew
EO
,
Ehlken
N
,
Seyfarth
HJ
,
Bossone
E
, et al
Assessment and prognostic relevance of right ventricular contractile reserve in patients with severe pulmonary hypertension
.
Circulation
.
2013
Oct
;
128
(
18
):
2005
15
.
[PubMed]
0009-7322
22.
Brubaker
PH
,
Kitzman
DW
.
Chronotropic incompetence: causes, consequences, and management
.
Circulation
.
2011
Mar
;
123
(
9
):
1010
20
.
[PubMed]
0009-7322
23.
Kovacs
G
,
Herve
P
,
Barbera
JA
,
Chaouat
A
,
Chemla
D
,
Condliffe
R
, et al
An official European Respiratory Society statement: pulmonary haemodynamics during exercise
.
Eur Respir J
.
2017
Nov
;
50
(
5
):
1700578
.
[PubMed]
0903-1936
24.
Naeije
R
,
D’Alto
M
.
Sex matters in pulmonary arterial hypertension
.
Eur Respir J
.
2014
Aug
;
44
(
2
):
553
4
.
[PubMed]
0903-1936
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.