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      Incidence and characteristics of chronic thromboembolic pulmonary hypertension in Germany

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          Abstract

          Background

          The incidence of chronic thromboembolic pulmonary hypertension (CTEPH) is unknown. Previous studies from the United Kingdom and Spain have reported incidence rates of 1.75 and 0.9 per million, respectively. These figures, however, may underestimate the true incidence of CTEPH.

          Methods

          We prospectively enrolled patients newly diagnosed with CTEPH within 2016 in Germany. Data were obtained from the three German referral centers and from the German branch of COMPERA, a European pulmonary hypertension registry. The CTEPH incidence was calculated based on German population data, and patient characteristics and treatment patterns were described.

          Results

          A total of 392 patients were newly diagnosed with CTEPH within 2016 in Germany, yielding an incidence of 5.7 new cases per million adults. The (mean ± standard deviation) age was 63.5 ± 15.0 years; males and females were equally affected; 76.3% of the patients had a history of venous thromboembolism. A total of 197 (50.3%) patients underwent pulmonary endarterectomy. Almost all non-operated patients received targeted drug therapy, and 49 patients (25.1% of the non-operated patients) were treated with balloon pulmonary angioplasty.

          Conclusion

          The incidence of CTEPH in Germany 2016 was 5.7 per million adults and thus higher than previously reported from other countries. Half of the patients were operated while the remaining patients received medical or interventional therapies.

          Clinical trials registration

          http://www.clinicaltrials.gov NCT02660463 and NCT01347216.

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          Thrombosis: a major contributor to global disease burden.

          G.E. Raskob, P Angchaisuksiri, A.N. Blanco … (2014)
          Thrombosis is the common pathology underlying ischemic heart disease, ischemic stroke, and venous thromboembolism (VTE). The Global Burden of Disease Study 2010 (GBD 2010) documented that ischemic heart disease and stroke collectively caused 1 in 4 deaths worldwide. GBD 2010 did not report data for VTE as a cause of death and disability.
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            Surgical management and outcome of patients with chronic thromboembolic pulmonary hypertension: results from an international prospective registry.

            Eckhard Mayer, David Jenkins, Jaroslav Lindner … (2011)
            Pulmonary endarterectomy is a curative surgical treatment option for the majority of patients with chronic thromboembolic pulmonary hypertension. The current surgical management and postoperative outcome of patients enrolled in an international registry on chronic thromboembolic pulmonary hypertension were investigated. The registry included newly diagnosed (≤6 months) consecutive patients with chronic thromboembolic pulmonary hypertension from February 2007 to January 2009. A total of 679 patients were registered from 1 Canadian and 26 European centers, of whom 386 (56.8%) underwent surgery. The median age of patients undergoing surgery was 60 years, and 54.1% were male. Previous pulmonary embolism was confirmed for 79.8% of patients. Perioperative complications occurred in 189 patients (49.2%): infection (18.8%), persistent pulmonary hypertension (16.7%), neurologic (11.2%) or bleeding (10.2%) complications, pulmonary reperfusion edema (9.6%), pericardial effusion (8.3%), need for extracorporeal membrane oxygenation (3.1%), and in-hospital mortality due to perioperative complications (4.7%). Documented 1-year mortality was 7%. Preoperative exercise capacity was predictive of 1-year mortality. Postoperative pulmonary vascular resistance predicted in-hospital and 1-year mortality. In patients evaluated within 1 year after surgery, the median pulmonary vascular resistance had decreased from 698 to 235 dyn x s x cm(-5) (95% confidence limit, 640-874 and 211-255, respectively, n = 70) and the median 6-minute walk distance had increased from 362 to 459 m (95% confidence limit, 340-399 and 440-473, respectively, n = 168). New York Heart Association functional class improved with most patients progressing from class III/IV to class I/II. Pulmonary endarterectomy is associated with a low in-hospital mortality rate and improvements in hemodynamics and exercise capacity. Copyright © 2011 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.
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              Survival in pulmonary hypertension in Spain: insights from the Spanish registry.

              M Palma, Pilar Gutiérrez-Escribano, J Segovia … (2012)
              A pulmonary hypertension (PH) registry (Spanish Registry of Pulmonary Arterial Hypertension) was undertaken to analyse prevalence, incidence and survival of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) in Spain, and to assess the applicability of recent survival prediction equations. Voluntary reporting of previously diagnosed and incident PAH or CTEPH cases (July 2007-June 2008) was performed. Demographic, functional and haemodynamic variables were evaluated. 866 patients with PAH and 162 with CTEPH were included. PAH associated with toxic oil syndrome and pulmonary veno-occlusive disease were reported for the first time in a PAH registry. Estimated prevalences were as follows: PAH, 16 and CTEPH, 3.2 cases per million adult inhabitants (MAI). Estimated incidences were as follows: PAH, 3.7 and CTEPH, 0.9 cases per MAI per yr. 1-, 3- and 5-yr survival was 87%, 75% and 65%, respectively, with no differences between PAH and CTEPH. Male sex, right atrial pressure and cardiac index were independent predictors of death. Matching between observed survival and that predicted by different equations was closer when the characteristics of the cohorts were similar. Epidemiology and survival of PAH patients in the Spanish registry are similar to recent registries. Characteristics of the population from which survival prediction equations are derived influence their applicability to a different cohort. CTEPH is much less prevalent than PAH, although has a similar survival rate.
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                Author and article information

                Contributors
                Marius M. Hoeper:
                ORCID: http://orcid.org/0000-0001-9086-2293
                +49 511-532-3530 , hoeper.marius@mh-hannover.de
                Journal
                Journal ID (nlm-ta): Clin Res Cardiol
                Journal ID (iso-abbrev): Clin Res Cardiol
                Title: Clinical Research in Cardiology
                Publisher: Springer Berlin Heidelberg (Berlin/Heidelberg )
                ISSN (Print): 1861-0684
                ISSN (Electronic): 1861-0692
                Publication date (Electronic): 15 February 2018
                Publication date PMC-release: 15 February 2018
                Publication date (Print): 2018
                Volume: 107
                Issue: 7
                Pages: 548-553
                Affiliations
                [1 ]Kerckhoff Heart and Lung Center, Bad Nauheim, Germany
                [2 ]GRID grid.411937.9, Clinic for Internal Medicine V-Pneumology, Allergology and Critical Care Medicine, , Saarland University Hospital, ; Homburg, Germany
                [3 ]ISNI 0000 0000 9529 9877, GRID grid.10423.34, Department of Respiratory Medicine and German Center of Lung Research (DZL/BREATH), , Hannover Medical School, ; 30623 Hanover, Germany
                [4 ]GRID grid.411937.9, Department of Thoracic and Cardiovascular Surgery, , Saarland University Hospital, ; Homburg, Germany
                [5 ]ISNI 0000 0000 9323 8675, GRID grid.418217.9, Epidemiology Unit, , German Rheumatism Research Centre, ; Berlin, Germany
                [6 ]ISNI 0000 0001 2111 7257, GRID grid.4488.0, Institute for Clinical Pharmacology, Medical Faculty, , Technical University, ; Dresden, Germany
                [7 ]ISNI 0000 0000 9529 9877, GRID grid.10423.34, Clinic for Cardiothoracic, Vascular and Transplantation Surgery, , Hannover Medical School, ; Hanover, Germany
                Author information
                http://orcid.org/0000-0001-9086-2293
                Article
                Publisher ID: 1215
                DOI: 10.1007/s00392-018-1215-5
                PMC ID: 6002445
                PubMed ID: 29450722
                SO-VID: e9d05f45-d5c5-4d08-805a-907fd4c36db3
                Copyright © © The Author(s) 2018
                License:

                Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.

                History
                Date received : 28 November 2017
                Date accepted : 8 February 2018
                Categories
                Subject: Original Paper
                Custom metadata
                issue-copyright-statement © Springer-Verlag GmbH Germany, part of Springer Nature 2018

                ScienceOpen disciplines: Cardiovascular Medicine
                Keywords: chronic thromboembolic pulmonary hypertension,incidence,epidemiology,pulmonary endarterectomy,balloon pulmonary angioplasty
                Data availability:
                ScienceOpen disciplines: Cardiovascular Medicine
                Keywords: chronic thromboembolic pulmonary hypertension, incidence, epidemiology, pulmonary endarterectomy, balloon pulmonary angioplasty

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