Echocardiographic estimate of pulmonary artery pressure in sarcoidosis patients - real world data from a multi-national study

Marloes P Huitema*, Marco C Post, Jan C Grutters, Athol U Wells, Vasilis Kouranos, Oksana A Shlobin, Steven D Nathan, Daniel A Culver, Joseph Barney, Rohit Gupta, Eva Carmona, Esam H Alhamad, Mary B Scholand, Marlies Wijsenbeek, Sivagini Ganesh, Elyse E Lower, Peter J Engel, Robert P Baughman

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

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Abstract

INTRODUCTION: Echocardiographic measurement of the right ventricular systolic pressure (RVSP) is commonly used for estimating systolic pulmonary artery pressure (PASP) measured during right heart catheterization (RHC) in patients suspected for pulmonary hypertension (PH). Generally, there seems to be a strong correlation. However, this has been reported as less robust in sarcoidosis. We aim to investigate the correlation between RVSP and RHC measurements using real world data and analyzed factors influencing the relationship between RVSP and PASP in sarcoidosis.

METHODS & RESULTS: Data of patients with and without sarcoidosis associated PH who had both a measurable echocardiographic RVSP and invasive PASP were collected from the RESAPH registry, PULSAR study and Cincinnati Sarcoidosis Clinic database (n=173, 60.1% female, mean age 56.0±9.5 years). Among them, 124 had PH confirmed by RHC. There was a strong correlation between RVSP and PASP (r=0.640). This correlation was significant in both male and female, white or non-white, forced vital capacity (FVC) >60%, and presence of fibrosis (p<0.001). However, it was less robust in patients with FVC of 50% or less. RVSP was considered inaccurate if the difference with PASP was > 10mmHg. Inaccurate echocardiographic estimation of the invasive PASP occurred in 50.8%, with overestimation mostly in patients without PH, and underestimation in patients with severe PH. An RVSP>50mmHg was associated with worse survival.

CONCLUSIONS: In this real world multicenter cohort of sarcoidosis patients, we found a significant correlation between RVSP as determined by echocardiography and invasive PASP. Over- or underestimation of PASP occurred frequently. Therefore, echocardiographic RVSP measurement alone to screen for PH in sarcoidosis should be used with caution.

Original languageEnglish
Article numbere2021032
JournalSarcoidosis Vasculitis and Diffuse Lung Diseases
Volume38
Issue number4
DOIs
Publication statusPublished - 2021

Bibliographical note

Funding Information:
The statistical analysis was performed in collaboration with Hans Kelder (epidemiologist and statistician). This work was supported by Gilead Pharmaceuticals (NIH 1UL1TR001425-01) and ZonMW topzorg (project number 842001006)

Funding Information:
Funding sources: This work was supported by Gilead Pharmaceu-

Publisher Copyright:
© Mattioli 1885.

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