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Review
. 2017 Mar 29;26(143):160121.
doi: 10.1183/16000617.0121-2016. Print 2017 Mar 31.

An epidemiological analysis of the burden of chronic thromboembolic pulmonary hypertension in the USA, Europe and Japan

Henning Gall  1 , Marius M Hoeper  2 , Manuel J Richter  3 , William Cacheris  4 , Barbara Hinzmann  5 , Eckhard Mayer  6
Affiliations
Review

An epidemiological analysis of the burden of chronic thromboembolic pulmonary hypertension in the USA, Europe and Japan

Henning Gall et al. Eur Respir Rev. .

Abstract

Epidemiological data for chronic thromboembolic pulmonary hypertension (CTEPH) are limited and there are conflicting reports regarding its pathogenesis.A literature review was conducted to identify CTEPH epidemiological data up to June 2014. Data were analysed to provide estimates of the incidence of CTEPH in the USA, Europe and Japan. An epidemiological projection model derived country-specific estimates of future incidence and diagnosis rates of CTEPH.Overall, 25 publications and 14 databases provided quantitative epidemiological data. In the USA and Europe, the crude annual incidence of diagnosed pulmonary embolism and crude annual full (i.e. diagnosed and undiagnosed) incidence of CTEPH were 66-104 and 3-5 cases per 100 000 population, respectively, while in Japan these rates were lower at 6.7 and 1.9 per 100 000 population, respectively. In 2013, 7-29% of CTEPH cases in Europe and the USA were diagnosed, and the majority of patients were in New York Heart Association functional class III/IV at diagnosis. The projection model indicated that incidence of CTEPH will continue to increase over the next decade.These data suggest that CTEPH is underdiagnosed and undertreated, and there is an urgent need to increase awareness of CTEPH. High-quality epidemiological studies are required to increase understanding of CTEPH.

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Conflict of interest statement

Conflict of interest: Disclosures can be found alongside this article at err.ersjournals.com

Figures

FIGURE 1
FIGURE 1
Proportion of patients who develop chronic thromboembolic pulmonary hypertension (CTEPH) following pulmonary embolism (PE). #: studies followed-up individual patients after PE.
FIGURE 2
FIGURE 2
a) Annual diagnosed incidence of pulmonary embolism (PE), and b) annual full incidence of chronic thromboembolic pulmonary hypertension (CTEPH) per 100 000 population in Europe, the USA and Japan (crude rates for the year 2015).
FIGURE 3
FIGURE 3
Distribution of patients with chronic thromboembolic pulmonary hypertension by New York Heart Association functional class (NYHA FC) at time of diagnosis by region. EU: European Union.
FIGURE 4
FIGURE 4
Proportions of patients with chronic thromboembolic pulmonary hypertension (CTEPH) who were diagnosed in Europe, the USA and Japan in 2013.
See this image and copyright information in PMC

References

    1. Galiè N, Humbert M, Vachiery JL, et al. . 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). Eur Respir J 2015; 46: 903–975. - PubMed
    1. Fedullo P, Kerr KM, Kim NH, et al. . Chronic thromboembolic pulmonary hypertension. Am J Respir Crit Care Med 2011; 183: 1605–1613. - PubMed
    1. Hoeper MM, Madani MM, Nakanishi N, et al. . Chronic thromboembolic pulmonary hypertension. Lancet Respir Med 2014; 2: 573–582. - PubMed
    1. Peacock A, Simonneau G, Rubin L. Controversies, uncertainties and future research on the treatment of chronic thromboembolic pulmonary hypertension. Proc Am Thorac Soc 2006; 3: 608–614. - PubMed
    1. Lang IM, Madani M. Update on chronic thromboembolic pulmonary hypertension. Circulation 2014; 130: 508–518. - PubMed