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

Henning Gall, Marius M Hoeper, Manuel J Richter, William Cacheris, Barbara Hinzmann, Eckhard Mayer, Henning Gall, Marius M Hoeper, Manuel J Richter, William Cacheris, Barbara Hinzmann, Eckhard Mayer

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.

Conflict of interest statement

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

Copyright ©ERS 2017.

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.

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.
    1. Fedullo P, Kerr KM, Kim NH, et al. . Chronic thromboembolic pulmonary hypertension. Am J Respir Crit Care Med 2011; 183: 1605–1613.
    1. Hoeper MM, Madani MM, Nakanishi N, et al. . Chronic thromboembolic pulmonary hypertension. Lancet Respir Med 2014; 2: 573–582.
    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.
    1. Lang IM, Madani M. Update on chronic thromboembolic pulmonary hypertension. Circulation 2014; 130: 508–518.
    1. Simonneau G, Torbicki A, Dorfmüller P, et al. . The pathophysiology of chronic thromboembolic pulmonary hypertension. Eur Respir Rev 2017; 26: 160112.
    1. Lang IM, Pesavento R, Bonderman D, et al. . Risk factors and basic mechanisms of chronic thromboembolic pulmonary hypertension: a current understanding. Eur Respir J 2013; 41: 462–468.
    1. Egermayer P, Peacock AJ. Is pulmonary embolism a common cause of chronic pulmonary hypertension? Limitations of the embolic hypothesis. Eur Respir J 2000; 15: 440–448.
    1. Hoeper MM, Mayer E, Simonneau G, et al. . Chronic thromboembolic pulmonary hypertension. Circulation 2006; 113: 2011–2020.
    1. Pepke-Zaba J, Delcroix M, Lang I, et al. . Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry. Circulation 2011; 124: 1973–1981.
    1. Bonderman D, Skoro-Sajer N, Jakowitsch J, et al. . Predictors of outcome in chronic thromboembolic pulmonary hypertension. Circulation 2007; 115: 2153–2158.
    1. Bonderman D, Wilkens H, Wakounig S, et al. . Risk factors for chronic thromboembolic pulmonary hypertension. Eur Respir J 2009; 33: 325–331.
    1. Kim NH, Delcroix M, Jenkins DP, et al. . Chronic thromboembolic pulmonary hypertension. J Am Coll Cardiol 2013; 62: Suppl. 25, D92–D99.
    1. Hurdman J, Condliffe R, Elliot CA, et al. . ASPIRE registry: assessing the Spectrum of Pulmonary hypertension Identified at a REferral centre. Eur Respir J 2012; 39: 945–955.
    1. Condliffe R, Kiely DG, Gibbs JS, et al. . Improved outcomes in medically and surgically treated chronic thromboembolic pulmonary hypertension. Am J Respir Crit Care Med 2008; 177: 1122–1127.
    1. Freed DH, Thomson BM, Berman M, et al. . Survival after pulmonary thromboendarterectomy: effect of residual pulmonary hypertension. J Thorac Cardiovasc Surg 2011; 141: 383–387.
    1. Korkmaz A, Ozlu T, Ozsu S, et al. . Long-term outcomes in acute pulmonary thromboembolism: the incidence of chronic thromboembolic pulmonary hypertension and associated risk factors. Clin Appl Thromb Hemost 2012; 18: 281–288.
    1. Mayer E, Jenkins D, Lindner J, et al. . Surgical management and outcome of patients with chronic thromboembolic pulmonary hypertension: results from an international prospective registry. J Thorac Cardiovasc Surg 2011; 141: 702–710.
    1. Rahnavardi M, Yan TD, Cao C, et al. . Pulmonary thromboendarterectomy for chronic thromboembolic pulmonary hypertension: a systematic review. Ann Thorac Cardiovasc Surg 2011; 17: 435–445.
    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.
    1. Bresser P, Pepke-Zaba J, Jaïs X, et al. . Medical therapies for chronic thromboembolic pulmonary hypertension: an evolving treatment paradigm. Proc Am Thorac Soc 2006; 3: 594–600.
    1. Dartevelle P, Fadel E, Mussot S, et al. . Chronic thromboembolic pulmonary hypertension. Eur Respir J 2004; 23: 637–648.
    1. Delcroix M. Chronic post-embolic pulmonary hypertension: a new target for medical therapies? Eur Respir Rev 2013; 22: 258–264.
    1. Keogh AM, Mayer E, Benza RL, et al. . Interventional and surgical modalities of treatment in pulmonary hypertension. J Am Coll Cardiol 2009; 54: Suppl. 1, S67–S77.
    1. Ghofrani HA, D'Armini AM, Grimminger F, et al. . Riociguat for the treatment of chronic thromboembolic pulmonary hypertension. N Engl J Med 2013; 369: 319–329.
    1. Simonneau G, D'Armini AM, Ghofrani HA, et al. . Riociguat for the treatment of chronic thromboembolic pulmonary hypertension: a long-term extension study (CHEST-2). Eur Respir J 2015; 45: 1293–1302.
    1. Simonneau G, D'Armini AM, Ghofrani HA, et al. . Predictors of long-term outcomes in patients treated with riociguat for chronic thromboembolic pulmonary hypertension: data from the CHEST-2 open-label, randomised, long-term extension trial. Lancet Respir Med 2016; 4: 372–380.
    1. Pepke-Zaba J, Ghofrani HA, Hoeper MM. Medical management of chronic thromboembolic pulmonary hypertension. Eur Respir Rev 2017; 26: 160107.
    1. Wiedenroth CB, Liebetrau C, Breithecker A, et al. . Combined pulmonary endarterectomy and balloon pulmonary angioplasty in patients with chronic thromboembolic pulmonary hypertension. J Heart Lung Transplant 2016; 35: 591–596.
    1. Kataoka M, Inami T, Hayashida K, et al. . Percutaneous transluminal pulmonary angioplasty for the treatment of chronic thromboembolic pulmonary hypertension. Circ Cardiovasc Interv 2012; 5: 756–762.
    1. Kim NH. Chronic thromboembolic pulmonary hypertension. Medscape Pulmon Med 2007. Date last accessed: March 2, 2017.
    1. Mizoguchi H, Ogawa A, Munemasa M, et al. . Refined balloon pulmonary angioplasty for inoperable patients with chronic thromboembolic pulmonary hypertension. Circ Cardiovasc Interv 2012; 5: 748–755.
    1. Lang I, Meyer BC, Ogo T, et al. . Balloon pulmonary angioplasty in chronic thromboembolic pulmonary hypertension. Eur Respir Rev 2017; 26: 160119.
    1. Lang IM. Chronic thromboembolic pulmonary hypertension – not so rare after all. N Engl J Med 2004; 350: 2236–2238.
    1. Tapson VF, Humbert M. Incidence and prevalence of chronic thromboembolic pulmonary hypertension: from acute to chronic pulmonary embolism. Proc Am Thorac Soc 2006; 3: 564–567.
    1. Healthcare Cost and Utilization Project (HCUP). The 2010 Nationwide Inpatient Sample (NIS). Date last accessed: August 31, 2014. Date last updated: December 14, 2016.
    1. Instituto Nacional de Estadística (INE). 2009. Base de datos INEbase. Encuesta de morbilidad hospitalaria [INEbase Database. Hospital Morbidity Survey]. Date last accessed: March 2, 2017. Date last updated: 2015.
    1. French Institute for Public Health Surveillance (PMSI). 2011. French National Database. Date last accessed: March 2, 2017. Date last updated: 2016.
    1. Japan Intractable Diseases Research Foundation and Japanese Intractable Diseases Information Center. The Japanese Intractable Disease Survey 2010 (CTEPH). 2010.
    1. The NHS Information Centre for Health and Social Care. UK Hospital Episode Statistics 2011. Date last accessed: March 15, 2017. Date last updated: 2014.
    1. Gall H, Preston IR, Hinzmann B, et al. . An international physician survey of chronic thromboembolic pulmonary hypertension management. Pulm Circ 2016; 6: 472–482.
    1. Preston IR, Hinzmann B, Heinz S, et al. . An international physician survey of pulmonary arterial hypertension management. Pulm Circ 2016; 6: 338–346.
    1. World Bank. World Bank DataBank HealthStats: Population Estimates and Projections Database. Date last accessed: January 25, 2017. Date last updated: October 4, 2016.
    1. World Bank. 2017. World Bank DataBank Health Nutrition and Population Statistics. Date last accessed: January 25, 2017. Date last updated: 2017.
    1. Otero R, Oribe M, Ballaz A, et al. . Echocardiographic assessment of pulmonary arterial pressure in the follow-up of patients with pulmonary embolism. Thromb Res 2011; 127: 303–308.
    1. Poli D, Grifoni E, Antonucci E, et al. . Incidence of recurrent venous thromboembolism and of chronic thromboembolic pulmonary hypertension in patients after a first episode of pulmonary embolism. J Thromb Thrombolysis 2010; 30: 294–299.
    1. Surie S, Gibson NS, Gerdes VE, et al. . Active search for chronic thromboembolic pulmonary hypertension does not appear indicated after acute pulmonary embolism. Thromb Res 2010; 125: e202–e205.
    1. Dentali F, Donadini M, Gianni M, et al. . Incidence of chronic pulmonary hypertension in patients with previous pulmonary embolism. Thromb Res 2009; 124: 256–258.
    1. Martí D, Gómez V, Escobar C, et al. . Incidencia de hipertensión pulmonar tromboembólica crónica sintomática y asintomática. [Incidence of symptomatic and asymptomatic chronic thromboembolic pulmonary hypertension]. Arch Bronconeumol 2010; 46: 628–633.
    1. Klok FA, van Kralingen KW, van Dijk AP, et al. . Prospective cardiopulmonary screening program to detect chronic thromboembolic pulmonary hypertension in patients after acute pulmonary embolism. Haematologica 2010; 95: 970–975.
    1. Noble S, Pasi J. Epidemiology and pathophysiology of cancer-associated thrombosis. Br J Cancer 2010; 102: Suppl. 1, S2–S9.
    1. Sakuma M, Nakamura M, Yamada N, et al. . Venous thromboembolism: deep vein thrombosis with pulmonary embolism, deep vein thrombosis alone, and pulmonary embolism alone. Circ J 2009; 73: 305–309.
    1. Tanabe N. Analysis of chronic thromboembolic pulmonary hypertension. Ministry of Health, Wealth and Labor (Japan) Intractable Disease Database, 2008.
    1. Kröger K, Moerchel C, Moysidis T, et al. . Incidence rate of pulmonary embolism in Germany: data from the federal statistical office. J Thromb Thrombolysis 2010; 29: 349–353.
    1. Jenkins DP, Madani M, Mayer E, et al. . Surgical treatment of chronic thromboembolic pulmonary hypertension. Eur Respir J 2013; 41: 735–742.
    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.
    1. Tapson VF, Platt DM, Xia F, et al. . Monitoring for pulmonary hypertension following pulmonary embolism: the INFORM study. Am J Med 2016; 129: 978–985.
    1. Kim NH. Group 4 pulmonary hypertension: chronic thromboembolic pulmonary hypertension: epidemiology, pathophysiology, and treatment. Cardiol Clin 2016; 34: 435–441.
    1. Hoeper MM, Humbert M, Souza R, et al. . A global view of pulmonary hypertension. Lancet Respir Med 2016; 4: 306–322.
    1. Konstantinides S 2016. Follow-Up after Acute Pulmonary Embolism – a Prospective Observational Multicenter Cohort Study – FOCUS. (accessed on 27 Aug 2016).
    1. Kitamukai O, Sakuma M, Takahashi T, et al. . Incidence and characteristics of pulmonary thromboembolism in Japan 2000. Intern Med 2003; 42: 1090–1094.
    1. Kobayashi T, Nakamura M, Sakuma M, et al. . Incidence of pulmonary thromboembolism (PTE) and new guidelines for PTE prophylaxis in Japan. Clin Hemorheol Microcirc 2006; 35: 257–259.
    1. Kominami S, Tanabe N, Ota M, et al. . HLA-DPB1 and NFKBIL1 may confer the susceptibility to chronic thromboembolic pulmonary hypertension in the absence of deep vein thrombosis. J Hum Genet 2009; 54: 108–114.
    1. Kumasaka N, Sakuma M, Shirato K. Incidence of pulmonary thromboembolism in Japan. Jpn Circ J 1999; 63: 439–441.
    1. Tanabe N, Kimura A, Amano S, et al. . Association of clinical features with HLA in chronic pulmonary thromboembolism. Eur Respir J 2005; 25: 131–138.
    1. Guérin L, Couturaud F, Parent F, et al. . Prevalence of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism Prevalence of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism. Prevalence of CTEPH after pulmonary embolism. Thromb Haemost 2014; 112: 598–605.
    1. Gopalan D, Delcroix M, Held M. Diagnosis of chronic thromboembolic pulmonary hypertension. Eur Respir Rev 2017; 26: 160108.
    1. Held M, Grün M, Holl R, et al. . Chronisch thromboembolische pulmonale Hypertonie: Latenz bis zur Diagnosesicherung und klinischer Zustand bei Diagnosestellung. [Chronic thromboembolic pulmonary hypertension: time delay from onset of symptoms to diagnosis and clinical condition at diagnosis]. Dtsch Med Wochenschr 2014; 139: 1647–1652.
    1. Strange G, Gabbay E, Kermeen F, et al. . Time from symptoms to definitive diagnosis of idiopathic pulmonary arterial hypertension: the delay study. Pulm Circ 2013; 3: 89–94.
    1. Mathai SC, Ghofrani HA, Mayer E, et al. . Quality of life in patients with chronic thromboembolic pulmonary hypertension. Eur Respir J 2016; 48: 526–537.
    1. Delcroix M, Lang I, Pepke-Zaba J, et al. . Long-term outcome of patients with chronic thromboembolic pulmonary hypertension: results from an international prospective registry. Circulation 2016; 133: 859–871.
    1. Ende-Verhaar C, Cannegieter S, Vonk-Noordegraaf A, et al. . Varying incidences of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism: the final thread that links them all. Eur Heart J 2016; 37: 406–407.

Source: PubMed

赞助者和合作者

医疗条件

药物干预

3
订阅