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Observational Study
. 2025 Jul 14;66(1):2402268.
doi: 10.1183/13993003.02268-2024. Print 2025 Jul.

Survival trends in patients with chronic thromboembolic pulmonary hypertension: an observational study

Takatoyo Kiko  1 , Ryotaro Asano  1   2 , Yusuke Yoshikawa  3 , Hiroyuki Endo  1 , Shinya Fujisaki  1 , Ryo Takano  1 , Mitsumasa Akao  1 , Naruhiro Nishi  1 , Hiroya Hayashi  1 , Soshiro Ogata  4 , Akiyuki Kotoku  5 , Hiroki Horinouchi  5 , Yosuke Inoue  6 , Jin Ueda  1 , Yoshimasa Seike  6 , Akihiro Tsuji  1 , Tetsuya Fukuda  5 , Kunihiro Nishimura  4 , Hitoshi Matsuda  6 , Takeshi Ogo  7
Affiliations
Observational Study

Survival trends in patients with chronic thromboembolic pulmonary hypertension: an observational study

Takatoyo Kiko et al. Eur Respir J. .

Abstract

Background: Treatment options for patients with chronic thromboembolic hypertension (CTEPH) have increased over the past decade. However, it is unknown whether the outcomes of patients with CTEPH have changed as well.

Methods: This retrospective study analysed the data of 834 patients with CTEPH, categorised into early (April 1980-December 1999), middle (January 2000-September 2010) and current (October 2010-December 2023) eras. The primary end-point was all-cause mortality. Multivariable Cox proportional hazards models were used to assess changes in all-cause mortality after adjusting for confounding variables.

Results: 95, 210 and 529 patients were from the early, middle and current eras, respectively. The proportion of patients who did not receive CTEPH therapy decreased from 65% in the early era to 36% and 3% in the middle and current eras, respectively. Meanwhile, the adoption of multimodal treatment increased from 0% to 58% over time. The Kaplan-Meier analysis revealed significant improvements in overall survival (log-rank p<0.001). The 5-year survival rates improved from 68% in the early era to 85% and 93% in the middle and current eras, respectively. The adjusted hazard ratios for mortality were 0.291 (95% CI 0.154-0.550; p<0.001) for the early versus middle era, 0.447 (95% CI 0.249-0.804; p=0.007) for the middle versus current era and 0.130 (95% CI 0.067-0.254; p<0.001) for the early versus current era.

Conclusion: The long-term prognosis of patients with CTEPH has significantly improved in recent decades.

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Conflict of interest statement

Conflict of interest: T. Ogo reports personal fees from Nippon Shinyaku Co., Janssen Pharmaceutical K.K., Bayer Yakuhin, Ltd, GlaxoSmithKline K.K., Pfizer Japan Inc., and Mochida Pharmaceutical Co, outside the submitted work. The remaining authors have no potential conflicts of interest to disclose.

Figures

None
Overview of the study. CTEPH: chronic thromboembolic pulmonary hypertension; BPA: balloon pulmonary angioplasty; PEA: pulmonary endarterectomy.
FIGURE 1
FIGURE 1
Study protocol. CTEPH: chronic thromboembolic pulmonary hypertension; PH: pulmonary hypertension.
FIGURE 2
FIGURE 2
Changes in treatment over time. BPA: balloon pulmonary angioplasty; PEA: pulmonary endarterectomy.
FIGURE 3
FIGURE 3
Kaplan–Meier analysis for all-cause mortality.
See this image and copyright information in PMC

Comment in

References

    1. Kim NH, Delcroix M, Jenkins DP, et al. Chronic thromboembolic pulmonary hypertension. J Am Coll Cardiol 2013; 62: Suppl., D92–D99. doi: 10.1016/j.jacc.2013.10.024 - DOI - PubMed
    1. Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J 2022; 43: 3618–3731. doi: 10.1093/eurheartj/ehac237 - DOI - PubMed
    1. Riedel M, Stanek V, Widimsky J, et al. Longterm follow-up of patients with pulmonary thromboembolism. Late prognosis and evolution of hemodynamic and respiratory data. Chest 1982; 81: 151–158. doi: 10.1378/chest.81.2.151 - DOI - PubMed
    1. Kunieda T, Nakanishi N, Satoh T, et al. Prognoses of primary pulmonary hypertension and chronic majorvessel thromboembolic pulmonary hypertension determined from cumulative survival curves. Intern Med 1999; 38: 543–546. doi: 10.2169/internalmedicine.38.543 - DOI - PubMed
    1. Cannon JE, Su L, Kiely DG, et al. Dynamic risk stratification of patient long-term outcome after pulmonary endarterectomy: results from the United Kingdom national cohort. Circulation 2016; 133: 1761–1771. doi: 10.1161/CIRCULATIONAHA.115.019470 - DOI - PMC - PubMed

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