Version 1
: Received: 27 August 2024 / Approved: 28 August 2024 / Online: 28 August 2024 (14:47:33 CEST)
How to cite:
Shayan, S.; Ghoshouni, H.; Kavoosi, H.; Almasi, S.; Javadinezhad, Z.; Taghavi, S.; Omidvar, R.; Mirtajaddini, M.; Naderi, N. How Should Pulmonary Hypertension in Systemic Sclerosis Be Better Investigated?. Preprints2024, 2024082087. https://doi.org/10.20944/preprints202408.2087.v1
Shayan, S.; Ghoshouni, H.; Kavoosi, H.; Almasi, S.; Javadinezhad, Z.; Taghavi, S.; Omidvar, R.; Mirtajaddini, M.; Naderi, N. How Should Pulmonary Hypertension in Systemic Sclerosis Be Better Investigated?. Preprints 2024, 2024082087. https://doi.org/10.20944/preprints202408.2087.v1
Shayan, S.; Ghoshouni, H.; Kavoosi, H.; Almasi, S.; Javadinezhad, Z.; Taghavi, S.; Omidvar, R.; Mirtajaddini, M.; Naderi, N. How Should Pulmonary Hypertension in Systemic Sclerosis Be Better Investigated?. Preprints2024, 2024082087. https://doi.org/10.20944/preprints202408.2087.v1
APA Style
Shayan, S., Ghoshouni, H., Kavoosi, H., Almasi, S., Javadinezhad, Z., Taghavi, S., Omidvar, R., Mirtajaddini, M., & Naderi, N. (2024). How Should Pulmonary Hypertension in Systemic Sclerosis Be Better Investigated?. Preprints. https://doi.org/10.20944/preprints202408.2087.v1
Chicago/Turabian Style
Shayan, S., Marzieh Mirtajaddini and Nasim Naderi. 2024 "How Should Pulmonary Hypertension in Systemic Sclerosis Be Better Investigated?" Preprints. https://doi.org/10.20944/preprints202408.2087.v1
Abstract
Introduction:
Pulmonary hypertension (PH) in systemic sclerosis (SSc) can be caused by different pathophysiological mechanisms, including precapillary PH (pulmonary arterial hypertension), post-capillary PH (secondary to left heart diastolic dysfunction), or combined pre- and post-capillary PH. To unmask occult post-capillary PH, fluid loading with saline during right heart catheterization (RHC) is recommended. In the present study, we investigated the response to fluid challenge test (FCT) in patients with SSc who were referred for RHC due to their high likelihood of PH on screening echocardiography.
Methods: Demographic data, clinical characteristics, echocardiographic and RHC data, and cardiac magnetic resonance imaging (CMR) results were obtained from the patients' hospital records or by contacting their rheumatologist. A positive FCT was defined as an increase in pulmonary capillary wedge pressure (PCWP) to more than18 mmHg with rapid saline infusion. Patients were followed for a median (IQR) of 15 (7-20) months.
Results:
Of the total 98 scleroderma patients 34 patients (29 female) were included. The mean age (SD) was 50.6(8.2) years. Baseline RHC data showed that 20 patients had no PH and 14 patients had mild precapillary PH (mPAH). Ten patients (50%) of the patients with no PH and 10 patients (71.4%) with mPAH had a significant increase in PCWP after a fluid loading. There was no statistically significant difference between the patients with positive and negative FCT in terms of the presence of LGE/inflammation on CMR examination. During follow-up, 3 patients died. Mortality was due to SSc-related complications in two patients.
Conclusion:
This study provides preliminary evidence that FCT may be a useful adjunct to baseline RHC testing to identify SSc patients with latent postcapillary PH. Further studies are needed to confirm these results and determine the optimal role of the fluid challenge test in the clinical management of SSc patients with suspected PH.
Keywords
Systemic sclerosis; pulmonary hypertension; fluid challenge test
Subject
Medicine and Pharmacology, Cardiac and Cardiovascular Systems
Copyright:
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
