Hemodynamic heterogeneity of connective tissue disease patients with borderline mean pulmonary artery pressure and its distinctive characters from those with normal pulmonary artery pressure: a retrospective study

Hemodynamic heterogeneity of connective tissue disease patients with borderline mean pulmonary... To clarify whether patients with connective tissue disease (CTD)-associated borderline mean pulmonary artery pressure (mPAP) have distinctive hemodynamic characteristics from those with normal mPAP and whether pathogenesis is as heterogeneous as manifest pulmonary hypertension (PH). Seventy-five CTD patients who underwent right heart catheterization (RHC) from 2008 through 2016 were retrospectively analyzed. We compared between-group differences in clinical and hemodynamic findings: normal mPAP (n = 35), borderline mPAP (n = 15), and PH (n = 25). A therapeutic intervention trial based on RHC results was performed in nine patients. The values of tricuspid regurgitation pressure gradient (TRPG) in patients with borderline mPAP were comparable at rest but became higher after exercise compared to those with a normal mPAP (P = 0.01). Pulmonary artery wedge pressure in patients with borderline mPAP was higher than in those with normal mPAP (P < 0.0001) and comparable to those with PH. Each of the three patients was treated for pre-capillary and post-capillary disease and two for interstitial lung disease (ILD). During the mean follow-up period of 40 months, mPAP or TRPG normalized in all patients treated for pre-capillary and post-capillary disease. One patient with severe ILD developed to PH and died from it. CTD patients with borderline mPAP, the underlining pathogenesis of which is heterogeneous as PH, have distinctive hemodynamic characteristics from those with normal mPAP. Whether a specific treatment targeting the inflammatory process or local hemodynamics may alter the clinical course to PH is a topic for future research. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Clinical Rheumatology Springer Journals

Hemodynamic heterogeneity of connective tissue disease patients with borderline mean pulmonary artery pressure and its distinctive characters from those with normal pulmonary artery pressure: a retrospective study

Download PDF
8 pages
Read Article

Loading next page...
 
/lp/springer_journal/hemodynamic-heterogeneity-of-connective-tissue-disease-patients-with-rWkxYuvtUY
Publisher
Springer Journals
Copyright
Copyright © 2018 by International League of Associations for Rheumatology (ILAR)
Subject
Medicine & Public Health; Rheumatology
ISSN
0770-3198
eISSN
1434-9949
DOI
10.1007/s10067-018-4142-y
Publisher site
See Article on Publisher Site

Abstract

To clarify whether patients with connective tissue disease (CTD)-associated borderline mean pulmonary artery pressure (mPAP) have distinctive hemodynamic characteristics from those with normal mPAP and whether pathogenesis is as heterogeneous as manifest pulmonary hypertension (PH). Seventy-five CTD patients who underwent right heart catheterization (RHC) from 2008 through 2016 were retrospectively analyzed. We compared between-group differences in clinical and hemodynamic findings: normal mPAP (n = 35), borderline mPAP (n = 15), and PH (n = 25). A therapeutic intervention trial based on RHC results was performed in nine patients. The values of tricuspid regurgitation pressure gradient (TRPG) in patients with borderline mPAP were comparable at rest but became higher after exercise compared to those with a normal mPAP (P = 0.01). Pulmonary artery wedge pressure in patients with borderline mPAP was higher than in those with normal mPAP (P < 0.0001) and comparable to those with PH. Each of the three patients was treated for pre-capillary and post-capillary disease and two for interstitial lung disease (ILD). During the mean follow-up period of 40 months, mPAP or TRPG normalized in all patients treated for pre-capillary and post-capillary disease. One patient with severe ILD developed to PH and died from it. CTD patients with borderline mPAP, the underlining pathogenesis of which is heterogeneous as PH, have distinctive hemodynamic characteristics from those with normal mPAP. Whether a specific treatment targeting the inflammatory process or local hemodynamics may alter the clinical course to PH is a topic for future research.

Journal

Clinical Rheumatology – Springer Journals

Published: May 18, 2018

References

  • Updated clinical classification of pulmonary hypertension
    Simonneau, G; Gatzoulis, MA; Adatia, I; Celermajer, D; Denton, C; Ghofrani, A; Gomez Sanchez, MA; Krishna Kumar, R; Landzberg, M; Machado, RF; Olschewski, H; Robbins, IM; Souza, R
  • Clinical characteristics and survival of Japanese patients with connective tissue disease and pulmonary arterial hypertension: a single-center cohort
    Shirai, Y; Yasuoka, H; Okano, Y; Takeuchi, T; Satoh, T; Kuwana, M
  • Outcome in 91 consecutive patients with pulmonary arterial hypertension receiving epoprostenol
    Kuhn, KP; Byrne, DW; Arbogast, PG; Doyle, TP; Loyd, JE; Robbins, IM
  • Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era
    Condliffe, R; Kiely, DG; Peacock, AJ; Corris, PA; Gibbs, JSR; Vrapi, F; Das, C; Elliot, CA; Johnson, M; DeSoyza, J; Torpy, C; Goldsmith, K; Hodgkins, D; Hughes, RJ; Pepke-Zaba, J; Coghlan, JG
  • Borderline pulmonary pressures in scleroderma—a “pre-pulmonary arterial hypertension” condition?
    Kovacs, G; Olschewski, H
  • Borderline mean pulmonary artery pressure in patients with systemic sclerosis: transpulmonary gradient predicts risk of developing pulmonary hypertension
    Valerio, CJ; Schreiber, BE; Handler, CE; Denton, CP; Coghlan, JG
  • Characterization of patients with borderline pulmonary arterial pressure
    Kovacs, G; Avian, A; Tscherner, M; Foris, V; Bachmaier, G; Olschewski, A; Olschewski, H
  • Simple exercise echocardiography using a Master’s two-step test for early detection of pulmonary arterial hypertension
    Suzuki, K; Akashi, YJ; Manabe, M; Mizukoshi, K; Kamijima, R; Kou, S; Takai, M; Izumo, M; Kida, K; Yoneyama, K; Omiya, K; Yamasaki, Y; Yamada, H; Nobuoka, S; Miyake, F
  • The 1982 revised criteria for the classification of systemic lupus erythematosus
    Tan, EM; Cohen, AS; Fries, JF; Masi, AT; Mcshane, DJ; Rothfield, NF; Schaller, JG; Talal, N; Winchester, RJ
  • Mixed connective tissue disease and antinuclear antibodies
    Kasukawa, R; Tojo, T; Miyawaki, S
  • Polymyositis and dermatomyositis (first of two parts)
    Bohan, A; Peter, JB
  • The Master two-step test
    Master, AM
  • Early detection of pulmonary vascular disease in pulmonary arterial hypertension: time to move forward
    Lau, EM; Manes, A; Celermajer, DS; Galie, N
  • Pulmonary arterial hypertension therapy may be safe and effective in patients with systemic sclerosis and borderline pulmonary artery pressure
    Kovacs, G; Maier, R; Aberer, E; Brodmann, M; Graninger, W; Kqiku, X; Scheidl, S; Tröster, N; Hesse, C; Rubin, L; Olschewski, H
  • Prevalence of pulmonary hypertension in systemic sclerosis in European Caucasians and metaanalysis of 5 studies
    Avouac, J; Airo, P; Meune, C
  • High prevalence of occult left heart disease in scleroderma-pulmonary hypertension
    Fox, BD; Shimony, A; Langleben, D; Hirsch, A; Rudski, L; Schlesinger, R; Eisenberg, MJ; Joyal, D; Hudson, M; Boutet, K; Serban, A; Masetto, A; Baron, M
  • Left heart abnormalities in connective tissue disease patients with pre-capillary pulmonary hypertension as well as borderline mean pulmonary arterial pressure
    Tsuchida, K; Yamada, H; Yamasaki, Y; Suzuki, K; Kongoji, K; Akashi, Y; Ozaki, S
  • Long-term effects of darusentan on left-ventricular remodelling and clinical outcomes in the endothelin a receptor antagonist trial in heart failure (EARTH): randomised, double-blind, placebo-controlled trial
    Anand, I; McMurray, J; Cohn, JN; Konstam, MA; Notter, T; Quitzau, K; Ruschitzka, F; Lüscher, TF
  • A randomized controlled trial of epoprostenol therapy for severe congestive heart failure: the Flolan international randomized survival trial (FIRST)
    Califf, RM; Adams, KF; McKenna, WJ
  • Survival in pulmonary hypertension associated with the scleroderma spectrum of diseases: impact of interstitial lung disease
    Mathai, SC; Hummers, LK; Champion, HC; Wigley, FM; Zaiman, A; Hassoun, PM; Girgis, RE
  • Pulmonary veno-occlusive disease and scleroderma associated pulmonary hypertension
    Johnson, SR; Patsios, D; Hwang, DM; Granton, JT

You’re reading a free preview. Subscribe to read the entire article.

Try 2 weeks free now

DeepDyve is your
personal research library

It’s your single place to instantly
discover and read the research
that matters to you.

Enjoy affordable access to
over 18 million articles from more than
15,000 peer-reviewed journals.

All for just $49/month

Explore the DeepDyve Library

or browse the journals available

Search

Query the DeepDyve database, plus search all of PubMed and Google Scholar seamlessly

Organize

Save any article or search result from DeepDyve, PubMed, and Google Scholar... all in one place.

Access

Get unlimited, online access to over 18 million full-text articles from more than 15,000 scientific journals.

Your journals are on DeepDyve

Read from thousands of the leading scholarly journals from SpringerNature, Wiley-Blackwell, Oxford University Press and more.

All the latest content is available, no embargo periods.

See the journals in your area

DeepDyve

Freelancer

DeepDyve

Pro

Price

FREE

$49/month
$360/year

Save searches from
Google Scholar,
PubMed

Create folders to
organize your research

Export folders, citations

Read DeepDyve articles

Abstract access only

Unlimited access to over
18 million full-text articles

Print

20 pages / month

PDF Discount

20% off

Sign up
for free
Start 14 day
Free Trial