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Impact of cancer on the prevalence, management, and outcome of patients with chronic thromboembolic pulmonary hypertension

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Abstract

Introduction

Chronic thromboembolic pulmonary hypertension (CTEPH) results from unresolved thrombotic obstruction of the pulmonary vasculature. Cancer is a known risk factor for CTEPH. This study aimed to determine the impact of cancer on the prevalence, management, and outcomes of patients with CTEPH.

Materials and methods

In this retrospective study involving 99 patients sequentially diagnosed with CTEPH in our hospital, the prevalence of 10 comorbid conditions including a past history of cancer at the time of CTEPH diagnosis were calculated.

Results

Among the 99 patients, 17 (17%) had a history of cancer. Breast cancer (n = 6) was the most common cancer type, followed by gastrointestinal cancer (n = 3), uterine cancer (n = 2), and malignant lymphoma (n = 2). Between patients with and without cancer, there were no differences in the demographics, severity of CTEPH, and management; however, the 5-year survival rate was lower for patients with cancer (65%) than for those without (89%). In addition, patients with cancer had significantly worse survival than those without (p = 0.03 by log-rank test). During follow-up, nine patients developed cancer after the diagnosis of CTEPH. Among the 99 patients, 13 died during follow-up, 6 (46%) of whom died of cancer.

Conclusions

17% of our patients with CETPH were diagnosed with cancer, with breast and gastrointestinal tract cancers being the most common. Cancer comorbidity was associated with a poor prognosis and contributed to death in 46% of deceased patients. The impact of cancer on CTEPH should be further evaluated in the future.

Key points

  • In the CTEPH cohort, 17% had a history of cancer, with breast and gastrointestinal tract cancers being the most common.

  • Of the 99 patients, 9 (9%) developed cancer after CTEPH diagnosis, indicating the need for careful cancer surveillance in patients with CTEPH during follow-up.

  • Cancer comorbidity was associated with a poor prognosis and contributed to death in 43% of deceased patients.

  • Future studies should evaluate the impact of cancer on CTEPH.

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References

  1. Humbert M, Kovacs G, Hoeper MM et al (2023) 2022 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J 61. https://doi.org/10.1183/13993003.00879-2022

  2. Riedel M, Stanek V, Widimsky J, Prerovsky I (1982) Longterm follow-up of patients with pulmonary thromboembolism. Late prognosis and evolution of hemodynamic and respiratory data. Chest 81:151–158. https://doi.org/10.1378/chest.81.2.151

    Article  CAS  PubMed  Google Scholar 

  3. Taniguchi Y, Jaïs X, Jevnikar M et al (2019) Predictors of survival in patients with not-operated chronic thromboembolic pulmonary hypertension. J Heart Lung Transplant 38:833–842. https://doi.org/10.1016/j.healun.2019.04.006

    Article  PubMed  Google Scholar 

  4. Kim NH, Lang IM (2012) Risk factors for chronic thromboembolic pulmonary hypertension. Eur Respir Rev 21:27–31. https://doi.org/10.1183/09059180.00009111

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  5. Bonderman D, Wilkens H, Wakounig S et al (2009) Risk factors for chronic thromboembolic pulmonary hypertension. Eur Respir J 33:325–331. https://doi.org/10.1183/09031936.00087608

    Article  CAS  PubMed  Google Scholar 

  6. Noble S, Pasi J (2010) Epidemiology and pathophysiology of cancer-associated thrombosis. Br J Cancer 102 Suppl 1(Suppl 1):S2-9:S2–S9. https://doi.org/10.1038/sj.bjc.6605599

  7. Kerr KM, Elliott CG, Chin K et al (2021) Results from the United States chronic thromboembolic pulmonary hypertension registry: enrollment characteristics and 1-year follow-up. Chest 160:1822–1831. https://doi.org/10.1016/j.chest.2021.05.052

    Article  PubMed  PubMed Central  Google Scholar 

  8. Pepke-Zaba J, Delcroix M, Lang I et al (2011) Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry. Circulation 124:1973–1981. https://doi.org/10.1161/CIRCULATIONAHA.110.015008

    Article  PubMed  Google Scholar 

  9. Tosun H, Kırkıl G, Deveci F et al (2016) The incidence and related risk factors of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism. Turk Thorac J 17:53–58. https://doi.org/10.5578/ttj.17.2.011

    Article  PubMed  PubMed Central  Google Scholar 

  10. Delcroix M, Lang I, Pepke-Zaba J et al (2016) Long-term outcome of patients with chronic thromboembolic pulmonary hypertension: results from an international prospective registry. Circulation 133:859–871. https://doi.org/10.1161/CIRCULATIONAHA.115.016522

    Article  CAS  PubMed  Google Scholar 

  11. Gall H, Felix JF, Schneck FK et al (2017) The Giessen Pulmonary hypertension Registry: Survival in pulmonary hypertension subgroups. J Heart Lung Transplant 36:957–967. https://doi.org/10.1016/j.healun.2017.02.016

    Article  PubMed  Google Scholar 

  12. Barco S, Klok FA, Konstantinides SV et al (2020) Sex-specific differences in chronic thromboembolic pulmonary hypertension. Results from the european CTEPH registry. J Thromb Haemost 18:151–161. https://doi.org/10.1111/jth.14629

    Article  PubMed  Google Scholar 

  13. Nakamura J, Tsujino I, Ohira H et al (2022) Various factors contribute to death in patients with different types of pulmonary hypertension: a retrospective pilot study from a single tertiary center. Respir Investig 60:647–657. https://doi.org/10.1016/j.resinv.2022.05.001

    Article  CAS  PubMed  Google Scholar 

  14. Fukuda K, Date H, Doi S et al (2019) Guidelines for the treatment of pulmonary hypertension (JCS 2017/JPCPHS 2017). Circ J 83:842–945. https://doi.org/10.1253/circj.CJ-66-0158

    Article  PubMed  Google Scholar 

  15. Galiè N, Humbert M, Vachiery JL et al (2015) 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: the Joint Task Force for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology (ESC) and the european respiratory society (ERS): endorsed by: Association for European Paediatric and congenital cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Respir J 46:903–975. https://doi.org/10.1183/13993003.01032-2015

    Article  CAS  PubMed  Google Scholar 

  16. Hosokawa K, Abe K, Funakoshi K et al (2023) Long-term outcome of chronic thromboembolic pulmonary hypertension using direct oral anticoagulants and warfarin: a japanese prospective cohort study. J Thromb Haemost S 1538–7836. https://doi.org/10.1016/j.jtha.2023.03.036. 23)00270-2

  17. Tonelli AR, Arelli V, Minai OA et al (2013) Causes and circumstances of death in pulmonary arterial hypertension. Am J Respir Crit Care Med 188:365–369. https://doi.org/10.1164/rccm.201209-1640OC

    Article  PubMed  PubMed Central  Google Scholar 

  18. Cancer statistics. https://gdb.ganjoho.jp/graph_db/gdb1?showData=&dataType=30&graphId=103&totalTarget=40&year=2015&years=2003&years=2004&years=2005&years=2006&years=2007&years=2008&years=2009&years=2010&years=2011&years=2012&years=2013&years=2014&avgStep=&survivalAgeKbn=AAA&ageSybt=0&ageSt=009&ageEd=A85&currentAge=0&smTypes=6&smType=1&sexType=0&marumeAgeKbn=A85&stage=0&elapsedYears=5, Accessed 30 Mar 2023. Cancer Information Service, National Cancer Center, Japan (National Cancer Registry, Ministry of Health, Labour and Welfare

  19. Kozu K, Sugimura K, Ito M et al (2020) Current status of long-term prognosis among all subtypes of pulmonary hypertension in Japan. Int J Cardiol 300:228–235. https://doi.org/10.1016/j.ijcard.2019.11.139

    Article  PubMed  Google Scholar 

  20. Bravos E, Cottin V, Dauphin C et al (2019) Cancer incidence in patients with pre-capillary pulmonary hypertension. J Heart Lung Transplant 38:778–780. https://doi.org/10.1016/j.healun.2019.03.007

    Article  PubMed  Google Scholar 

  21. Sørensen HT, Skajaa N, Klok FA, Laugesen K, Farkas DK (2022) Cancer risk in pulmonary hypertension patients. Clin Epidemiol 14:173–177. https://doi.org/10.2147/CLEP.S345054

    Article  PubMed  PubMed Central  Google Scholar 

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Acknowledgements

We would like to thank Editage (www.editage.com) for English language editing.

Funding

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

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Authors and Affiliations

Authors

Contributions

Conceptualization: JN, IT, TS; Methodology: JN, IT, TS; Formal analysis and investigation: JN, HS, TN, AS, TW, HO, IY; Writing - original draft preparation: JN; Writing - review and editing: IT, MS, SK; Funding acquisition: NA; Resources: NA; Supervision: SK.

Corresponding author

Correspondence to Ichizo Tsujino.

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Conflict of interest

IT, TS, and SK received research funding from Nihon Shinyaku Co. Ltd., Nippon Boehringer Ingelheim Co. Ltd., and Mochida Pharmaceutical Co. Ltd. JN, HS, TN, AS, TW, HO, MS, IY declare no conflicts of interest.

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Nakamura, J., Tsujino, I., Shima, H. et al. Impact of cancer on the prevalence, management, and outcome of patients with chronic thromboembolic pulmonary hypertension. J Thromb Thrombolysis 56, 588–593 (2023). https://doi.org/10.1007/s11239-023-02868-w

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  • DOI: https://doi.org/10.1007/s11239-023-02868-w

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