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Pulmonary vascular disease
Original research
Pulmonary artery compliance in different forms of pulmonary hypertension
  1. Amber McCormick1,
  2. Amita Krishnan1,
  3. David Badesch2,
  4. Raymond L Benza3,
  5. Todd M Bull2,
  6. Teresa De Marco4,
  7. Jeremy Feldman5,
  8. Anna R Hemnes6,
  9. Russel Hirsch7,
  10. Evelyn Horn8,
  11. Jaime Kennedy9,
  12. Stephen C Mathai10,
  13. Wesley McConnell11,
  14. Steven C Pugliese12,
  15. Jeffrey S Sager13,
  16. Oksana A Shlobin14,
  17. Marc A Simon4,
  18. Matthew R Lammi1
  1. 1 Comprehensive Pulmonary Hypertension Center, Louisiana State University Health Sciences Center, New Orleans, Louisiana, USA
  2. 2 Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado Anschutz Medical Campus, Denver, Colorado, USA
  3. 3 Department of Internal Medicine, Division of Cardiovascular Medicine, The Ohio State University Wexner Medical Center, Columbus, Ohio, USA
  4. 4 Division of Cardiology, University of California San Francisco, San Francisco, California, USA
  5. 5 Arizona Pulmonary Specialists, Phoenix, Arizona, USA
  6. 6 Allergy, Pulmonary, and Critical Care Medicine, Vanderbilt University, Nashville, Tennessee, USA
  7. 7 The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA
  8. 8 Division of Cardiology, Weill Cornell Medical College, New York, New York, USA
  9. 9 Inova Heart and Vascular Institute, Falls Church, Virginia, USA
  10. 10 Pulmonary and Critical Care Medicine, Johns Hopkins University, Baltimore, Maryland, USA
  11. 11 Kentuckiana Pulmonary Associates, Louisville, Kentucky, USA
  12. 12 Pulmonary, Allergy, and Critical Care, University of Pennsylvania, Philadelphia, Pennsylvania, USA
  13. 13 Cottage Pulmonary Hypertension Center, Santa Barbara, California, USA
  14. 14 Inova Fairfax Hospital, Advanced Lung Disesae and Transplant Program, Falls Church, Virginia, USA
  1. Correspondence to Dr Matthew R Lammi, Comprehensive Pulmonary Hypertension Center, Louisiana State University Health Sciences Center, New Orleans, Louisiana, USA; mlammi{at}lsuhsc.edu

Abstract

Objective Pulmonary artery compliance (PAC), estimated as stroke volume (SV) divided by pulmonary artery pulse pressure (PP), may be a predictor of survival in pulmonary arterial hypertension (PAH). Resistance–compliance (RC) time, the product of PAC and pulmonary vascular resistance, is reported to be a physiological constant. We investigated if differences in PAC and RC time exist between pulmonary hypertension (PH) subgroups and examined whether PAC is an independent predictor of transplant-free survival in PAH.

Methods This was a retrospective analysis of adult PAH (n=532) and chronic thromboembolic PH (CTEPH, n=84) patients enrolled in the US Pulmonary Hypertension Association Registry from 2015 to 2019. PAC and RC time were compared between PH subgroups (connective tissue disease-PAH (CTD-PAH), idiopathic/heritable-PAH (i/h-PAH), drug/toxin-PAH (d/t-PAH)). Cox proportional hazards models were constructed for transplant-free survival, adjusting for REVEAL 2.0 risk score.

Results There were no differences in estimated PAC between PAH subgroups, nor between PAH and CTEPH. RC time was shorter in CTEPH compared with PAH (median 0.55 (IQR 0.45–0.64) vs 0.62 (0.52–0.73) s, p<0.0001). RC time was shortest in CTD-PAH when compared with i/h-PAH and d/t-PAH ((0.59±0.18) vs (0.65±0.20) vs (0.73±0.25) s, p=0.0001). PAC was associated with transplant-free survival (HR 0.72, 95% CI 0.55 to 0.94, p=0.02) but was not an independent predictor of outcome after adjustment for REVEAL 2.0 score.

Conclusion PAC was similar between PH groups and was not an independent predictor of transplant-free survival in PAH. RC time was different between PH subgroups, challenging RC time constancy.

Trial registration number NCT04071327

  • Pulmonary Arterial Hypertension
  • Pulmonary Embolism

Data availability statement

Data may be obtained from a third party and are not publicly available. At this point, data are only available to the PHAR investigators.

https://doi.org/10.1136/heartjnl-2022-321760

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    Data availability statement

    Data may be obtained from a third party and are not publicly available. At this point, data are only available to the PHAR investigators.

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    Footnotes

    • Contributors Concept/design: AM, AK and MRL; data acquisition: AM, AK and MRL; data analysis/interpretation: AM, AK, MRL, DB, RLB, TMB, TDM, JF, ARH, RH, EH, JK, SCM, WM, SCP, JSS, OAS and MAS; drafting of the manuscript: AM, AK and MRL; critical revision of the manuscript: DB, RLB, TMB, TD, JF, ARH, RH, EH, JK, SCM, WM, SCP, JSS, OAS and MAS. MRL is the guarantor of this manuscript and accepts full responsibility for the work and conduct of the study, had access to the data and controlled the decision to publish.

    • Funding The Pulmonary Hypertension Association provides funding (no award number available) for the PHAR, which was used for this manuscript.

    • Disclaimer The Pulmonary Hypertension Association did not have any input regarding the design, conduct or interpretation of study results.

    • Competing interests ARH: consultant for Bayer, Janssen, United Therapeutics, GossamerBio and Complexa. Holds stock in Tenax Therapeutics. MAS: research support from Novartis. Haemodyanamic core lab for Aadi. Consulting/scientific advisory board for Janssen, Acceleron, Liquidia, Bial, Impulse Dynamics. OAS: consultant and speaker for United Therapeutics, Johnson and Johnson, and Bayer. JSS: research grants from Janssen, PhaseBio, Reata, Bayer, United Therapeutics and Covance. Consultant for Janssen, Bayer and United Therapeutics. TDM: consultant for Actelion/Johnson and Johnson, United Therapeutic, SCIOS-Bial and TrioHealth. Advisory Boards for Altevant and Liquidia; MRL: dlinical trial participation with Gilead, Actelion/Janssen, Bayer, United Therapeutics, Altavant and Acceleron (all funds to the institution).

    • Patient and public involvement Patients and/or the public were involved in the design, or conduct, or reporting, or dissemination plans of this research. Refer to the Methods section for further details.

    • Provenance and peer review Not commissioned; externally peer reviewed.

    • Author note Prior abstract: McCormick A, Krishnan A, Badesch DB, et al. Pulmonary Artery Compliance in Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension: The Pulmonary Hypertension Association Registry. American Journal of Respiratory and Critical Care Medicine. 2020;201:A6061

    • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.

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