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Research ArticleSystemic Sclerosis

Exercise Echocardiography Predicts Future Development of Pulmonary Hypertension in a High-risk Cohort of Patients with Systemic Sclerosis

Kaitlin A. Quinn, Stephanie R. Wappel, Tunay Kuru and Virginia D. Steen
The Journal of Rheumatology May 2020, 47 (5) 708-713; DOI: https://doi.org/10.3899/jrheum.190226
Kaitlin A. Quinn
From the Division of Rheumatology, Division of Pulmonary, and Department of Medicine, MedStar Georgetown University Hospital, Washington, DC, USA.
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  • For correspondence: KAQ01@gunet.georgetown.edu
Stephanie R. Wappel
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Tunay Kuru
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Virginia D. Steen
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Abstract

Objective. To evaluate whether a positive exercise echocardiogram (EE) predicts future development of pulmonary arterial hypertension (PAH) in a high-risk cohort of patients with systemic sclerosis (SSc).

Methods. Patients with SSc with features associated with an increased risk for PAH were recruited into a prospective, observational cohort. All patients underwent clinical assessment and EE. A positive EE was defined as an increase of ≥ 20 mmHg in the right ventricular systolic pressure with exercise. All patients with positive EE underwent right heart catheterization (RHC).

Results. The study included 85 patients. In the positive EE cohort, 10 of 43 patients (23%) developed resting pulmonary hypertension (PH) on RHC over a mean 4-year followup period [4 with PAH, 5 with pulmonary venous hypertension (PVH), and 1 with PH associated with interstitial lung disease]. In the persistently negative EE cohort, only 3 of 42 patients (7%) developed resting PH (1 PAH, 2 PVH; p = 0.04). Of the remaining 33 patients in the positive EE group who did not develop resting PH, 22 (67%) had a persistently positive EE over an average 5-year followup period.

Conclusion. In this high-risk cohort of patients with SSc, a positive EE may predict the future development of resting PH. In addition, a majority of patients may have a persistently positive EE for years without progression to resting PH. Finally, a consistently negative EE may identify patients at low risk for future PH.

Key Indexing Terms:
  • SYSTEMIC SCLEROSIS
  • PULMONARY HYPERTENSION
  • EXERCISE ECHOCARDIOGRAPHY
  • Accepted for publication October 25, 2019.
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The Journal of Rheumatology
Vol. 47, Issue 5
1 May 2020
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Exercise Echocardiography Predicts Future Development of Pulmonary Hypertension in a High-risk Cohort of Patients with Systemic Sclerosis
Kaitlin A. Quinn, Stephanie R. Wappel, Tunay Kuru, Virginia D. Steen
The Journal of Rheumatology May 2020, 47 (5) 708-713; DOI: 10.3899/jrheum.190226

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Exercise Echocardiography Predicts Future Development of Pulmonary Hypertension in a High-risk Cohort of Patients with Systemic Sclerosis
Kaitlin A. Quinn, Stephanie R. Wappel, Tunay Kuru, Virginia D. Steen
The Journal of Rheumatology May 2020, 47 (5) 708-713; DOI: 10.3899/jrheum.190226
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Keywords

SYSTEMIC SCLEROSIS
PULMONARY HYPERTENSION
EXERCISE ECHOCARDIOGRAPHY

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