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Hemodynamic Response to Oral Vasodilator Therapy in Systemic Sclerosis-Related Pulmonary Hypertension

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A Correction to this article was published on 20 January 2023

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Abstract

Purpose

Although classified as group 1 pulmonary arterial hypertension (PAH), patients with systemic sclerosis-related pulmonary hypertension (SSc-PH) experience poorer clinical response to PAH therapy and increased mortality compared to those with idiopathic PAH. Due to heterogeneity in phenotypes, identifying patients likely to respond to therapy is challenging. The goal of this study was to determine clinical factors associated with hemodynamic response, defined by a > 20% reduction in pulmonary vascular resistance on repeat right heart catheterization.

Methods

We applied a time-to-event model using a retrospective cohort of 39 patients with precapillary SSc-PH, defined by a mean pulmonary artery pressure of ≥ 25 mmHg and pulmonary arterial wedge pressure (PAWP) ≤ 15 mmHg on right heart catheterization.

Results

Patients with PAWP ≤ 8 mmHg were nearly fourfold more likely to achieve a hemodynamic response compared to those with PAWP > 8 mmHg (HR 3.88; 95% CI: 1.20, 12.57); each 1 mmHg increase in PAWP was associated with a decreased hazard for hemodynamic response (HR 0.84; 95% CI: 0.70, 1.00).

Conclusion

In patients with precapillary SSc-PH, PAWP was associated with time to hemodynamic response, suggesting the importance of subclinical cardiac disease in determining hemodynamic response to oral vasodilator therapy.

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Fig. 1

Data Availability

Data will be made available upon request on an individual basis. To protect the rights and privacy of subjects in the study, in addition to removing direct identifiers, indirect identifiers and other information that could lead to “deductive disclosure” of participants’ identities or be considered as identifiers under HIPAA (such as facility names, exact dates of birth, and examination dates) will be removed, ensuring HIPAA compliance. Even though the final dataset will be stripped of identifiers prior to release for sharing, there remains the possibility of deductive disclosure of subjects with unusual characteristics. Thus, the data and associated documentation will be made available to users only under a data sharing agreement that provides for (1) a commitment to using the data only for research purposes and not to identify any individual participant, (2) a commitment to securing the data using appropriate computer technology, and (3) a commitment to destroying or returning the data after analyses are completed. Investigators requesting the data will be required to go through the Boston University Medical Campus and Boston Medical Center IRB and to comply with HIPAA exemption requirements. Disclosures to outside sources will be documented, in compliance with HIPAA requirements.

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Funding

J. K. L. was supported by NIH/NHLBI 1F32HL156614-01 and received research support from United Therapeutics. K. R. G. was supported by a Parker B. Francis Fellowship Award. A. M. B. was supported by NIH/NHLBI 1R01HL155955-01A1. D. M. G. was supported by the American Heart Association: FTF17FTF33670369. R. S. W. was supported in part by resources from the VA Boston Healthcare system. M. P. L. was supported by NIAMS CCCR P30 AR072571. E. S. K. was supported by NIH/NHLBI 1UG3 HL143192-01A1, NCATS 2UL1TR001430-05A1, and HRSA U1EMC27864-08–00 and received research support from Bayer, Forma Therapeutics, Novartis, and United Therapeutics.

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Authors and Affiliations

  1. The Pulmonary Center, Boston University Chobanian & Avedisian School of Medicine, Boston, MA, USA

    Justin K. Lui, Ruchika A. Sangani, Kari R. Gillmeyer, Renda Soylemez Wiener & Elizabeth S. Klings

  2. Department of Medicine, Boston University Chobanian & Avedisian School of Medicine, Boston, MA, USA

    Justin K. Lui, Ruchika A. Sangani, Kari R. Gillmeyer, Jasmine Vakhshoorzadeh, Marcin A. Trojanowski, Andreea M. Bujor, Deepa M. Gopal, Renda Soylemez Wiener & Elizabeth S. Klings

  3. Section of Rheumatology, Boston University Chobanian & Avedisian School of Medicine, Boston, MA, USA

    Marcin A. Trojanowski, Andreea M. Bujor & Michael P. LaValley

  4. Section of Cardiovascular Medicine, Boston University Chobanian & Avedisian School of Medicine, Boston, MA, USA

    Deepa M. Gopal

  5. Center for Healthcare Organization & Implementation Research, VA Boston Healthcare System, Boston, MA, USA

    Renda Soylemez Wiener

  6. Department of Biostatistics, Boston University School of Public Health, Boston, MA, USA

    Michael P. LaValley

Authors
  1. Justin K. Lui
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  2. Ruchika A. Sangani
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  3. Kari R. Gillmeyer
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  4. Jasmine Vakhshoorzadeh
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  5. Marcin A. Trojanowski
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  6. Andreea M. Bujor
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  7. Deepa M. Gopal
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  8. Renda Soylemez Wiener
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  9. Michael P. LaValley
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  10. Elizabeth S. Klings
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Contributions

J. K. L.: conceptualization, data curation, formal analysis, investigation, methodology, roles/writing—original draft, writing—review and editing. R. A. S.: data curation, methodology, writing—review and editing. K. R. G.: formal analysis, methodology, writing—review and editing. J. V.: data curation, methodology, writing—review and editing. M. A. T.: data curation, methodology, writing—review and editing. A M. B.: data curation, methodology, writing—review and editing. D. M. G.: methodology, writing—review and editing. R. S. W.: methodology, writing—review and editing. M. P. L.: formal analysis, investigation, methodology, writing—review and editing. E. S. K.: conceptualization, formal analysis, investigation, methodology, writing—review and editing.

Corresponding author

Correspondence to Justin K. Lui.

Ethics declarations

Ethics Approval and Consent to Participate

This was retrospective, single-center, observational study of patients with systemic sclerosis enrolled into the Scleroderma Biorepository at Boston University Chobanian & Avedisian School of Medicine. At the time of enrollment, patients were consented for inclusion in this longitudinal clinical registry. The study was approved by the Boston University Medical Campus and Boston Medical Center Institutional Review Board.

Consent for Publication

The authors affirm that the study participants provided informed consent for publication of results in a medical book or journal.

Competing Interests

J. K. L. received research support from United Therapeutics. E. S. K. received royalties for 3 topic cards in UpToDate. She is a consultant/advisory board member for Bluebird Bio, FORMA Therapeutics, Vertex, and CSL Behring for sickle cell disease-related clinical trials (no conflict with the present work). All other authors declare no financial interests.

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Lui, J.K., Sangani, R.A., Gillmeyer, K.R. et al. Hemodynamic Response to Oral Vasodilator Therapy in Systemic Sclerosis-Related Pulmonary Hypertension. Cardiovasc Drugs Ther (2023). https://doi.org/10.1007/s10557-022-07420-1

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