ORIGINAL ARTICLE
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
Yusa Asari
1
&
Yoshioki Yamasaki
1
&
Kosei Tsuchida
1
&
Kengo Suzuki
2
&
Yoshihiro J. Akashi
2
&
Takahiro Okazaki
1
&
Shoichi Ozaki
1
&
Hidehiro Yamada
3
&
Kimito Kawahata
1
Received: 20 February 2018 /Revised: 2 May 2018 /Accepted: 8 May 2018
#
International League of Associations for Rheumatology (ILAR) 2018
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.
Keywords Borderline mean pulmonary artery pressure
.
Connective tissue diseases
.
Left heart disease
.
Pulmonary arterial
hypertension
Introduction
Pulmonary hypertension (PH) is defined as a mean pulmonary
artery pressure (PAP) ≥ 25 mmHg, measured by right heart
catheterization (RHC) at rest [1]. PH is a severe and fatal
complication of connective tissue diseases (CTDs), such as
systemic sclerosis (SSc) [2]. Early identification of PH is as-
sociated with improved long-term survival in patients with
CTD-associated PH (CTD-PH) [3, 4].
Borderline mean PAP (mPAP), defined by values of 21–
24 mmHg, may comprise a transition phase from a normal
pulmonary hemodynamic condition to pulmonary arterial hy-
pertension (PAH), with this range of pressures possibly being
representative of the early stage of PAH [5, 6]. Though early
* Yosh io ki Yamasa ki
yams@marianna-u.ac.jp
1
Division of Rheumatology and Allergology, Department of Internal
Medicine, St. Marianna University School of Medicine, 2-16-1,
Sugao, Miyamae, Kawasaki City, Kanagawa 216-8511, Japan
2
Division of Cardiology, Department of Internal Medicine, St.
Marianna University School of Medicine, 2-16-1, Sugao, Miyamae,
Kawasaki City, Kanagawa 216-8511, Japan
3
Medical Center for Rheumatic Diseases, Seirei Yokohama Hospital,
215, Iwaicho, Hodogaya, Yokohama City, Kanagawa 240-8521,
Japan
Clinical Rheumatology
https://doi.org/10.1007/s10067-018-4142-y
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