Download PDF
Pneumologie 2023; 77(11): 937-946
DOI: 10.1055/a-2145-4807
Übersicht

Chronisch thromboembolische pulmonale Hypertonie (Gruppe 4)

Chronic thromboembolic pulmonary hypertension
Stefan Guth
1   Abteilung für Thoraxchirurgie, Kerckhoff-Klinik GmbH, Bad Nauheim, Deutschland
,
Heinrike Wilkens
2   Klinik für Innere Medizin 5, Universitätsklinikum des Saarlandes, Homburg, Deutschland
,
Michael Halank
3   Medizinische Klinik und Poliklinik I, Universitätsklinikum Carl Gustav Carus an der Technischen Universität Dresden, Dresden, Deutschland
,
Matthias Held
4   Medizinische Klinik mit Schwerpunkt Pneumologie & Beatmungsmedizin, Missionsärztliche Klinik Würzburg, Würzburg, Deutschland
,
Lukas Hobohm
5   Centrum für Thrombose und Hämostase (CTH), Universitätsmedizin der Johannes Gutenberg-Universität Mainz, Mainz, Deutschland
,
Stavros Konstantinides
5   Centrum für Thrombose und Hämostase (CTH), Universitätsmedizin der Johannes Gutenberg-Universität Mainz, Mainz, Deutschland
,
Albert Omlor
2   Klinik für Innere Medizin 5, Universitätsklinikum des Saarlandes, Homburg, Deutschland
,
Hans-Jürgen Seyfarth
6   Bereich Pneumologie, Klinik für Onkologie, Gastroenterologie, Hepatologie und Pneumologie, Universitätsklinikum Leipzig, Leipzig, Deutschland
,
Hans-Joachim Schäfers
7   Klinik für Thorax-Herz-Gefäßchirurgie, Universitätsklinikum des Saarlandes, Homburg, Deutschland
,
Eckhard Mayer
1   Abteilung für Thoraxchirurgie, Kerckhoff-Klinik GmbH, Bad Nauheim, Deutschland
,
Christoph B. Wiedenroth
1   Abteilung für Thoraxchirurgie, Kerckhoff-Klinik GmbH, Bad Nauheim, Deutschland
› Author Affiliations
› Further Information
Also available at
   Permissions and Reprints

Zusammenfassung

Die chronisch thromboembolische Lungengefäßerkrankung (CTEPD) ist eine wichtige Spätfolge der akuten Lungenembolie, bei der die Thromben in fibröses Gewebe transformiert werden, mit der Gefäßwand verwachsen und zu chronischen Obstruktionen führen. Man unterscheidet die CTEPD ohne pulmonale Hypertonie (PH) mit einem pulmonalarteriellen Mitteldruck bis 20 mmHg von der Form mit PH. Hierbei spricht man weiterhin von chronisch thromboembolischer pulmonaler Hypertonie (CTEPH).

Liegt der Verdacht auf eine CTEPH vor, sollten initial eine Echokardiografie und eine Ventilations-/Perfusions-Szintigrafie erfolgen, um Perfusionsdefekte nachzuweisen. Danach empfiehlt sich die Zuweisung zu einem CTEPH-Zentrum, wo weitere bildgebende Diagnostik und eine Rechtsherzkatheteruntersuchung erfolgen, um die Diagnose zu sichern und das Therapiekonzept festzulegen.

Drei Therapieoptionen stehen aktuell zur Verfügung. Therapie der Wahl ist die pulmonale Endarteriektomie (PEA). Für nichtoperable Patient*innen oder Patient*innen nach PEA und residueller PH, gibt es eine gezielte medikamentöse PH-Therapie sowie das interventionelle Verfahren der pulmonalen Ballonangioplastie (BPA). Zunehmend werden PEA, BPA und die medikamentöse Therapie im Rahmen multimodaler Konzepte kombiniert.

Alle Patient*innen müssen nachgesorgt werden, was vorzugsweise in (CTE)PH-Zentren erfolgen sollte. Für CTEPH-Zentren werden eine Mindestanzahl von PEA-Operationen (50/Jahr) und BPA-Interventionen (100/Jahr) gefordert.

Abstract

Chronic thromboembolic pulmonary disease (CTEPD) is an important late complication of acute pulmonary embolism, in which the thrombi transform into fibrous tissue, become integrated into the vessel wall, and lead to chronic obstructions. CTEPD is differentiated into cases without pulmonary hypertension (PH), characterized by a mean pulmonary arterial pressure up to 20 mmHg and a form with PH. Then, it is still referred to as chronic thromboembolic pulmonary hypertension (CTEPH).

When there is suspicion of CTEPH, initial diagnostic tests should include echocardiography and ventilation/perfusion scan to detect perfusion defects. Subsequently, referral to a CTEPH center is recommended, where further imaging diagnostics and right heart catheterization are performed to determine the appropriate treatment.

Currently, three treatment modalities are available. The treatment of choice is pulmonary endarterectomy (PEA). For non-operable patients or patients with residual PH after PEA, PH-targeted medical therapy, and the interventional procedure of balloon pulmonary angioplasty (BPA) are available. Increasingly, PEA, BPA, and pharmacological therapy are combined in multimodal concepts.

Patients require post-treatment follow-up, preferably at (CTE)PH centers. These centers are required to perform a minimum number of PEA surgeries (50/year) and BPA interventions (100/year).

Schlüsselwörter

chronisch thromboembolische pulmonale Hypertonie - CTEPH - chronisch thromboembolische pulmonale Erkrankung - CTEPD - pulmonale Endarteriektomie - PEA - pulmonale Ballonangioplastie - BPA

Keywords

chronic thromboembolic pulmonary hypertension - CTEPH - chronic thromboembolic disease - CTED - pulmonary endarterectomy - PEA - balloon pulmonary angioplasty - BPA


Publication History

Article published online:
14 November 2023

© 2023. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 

  • Literatur

  • 1 Delcroix M. et al. ERS statement on chronic thromboembolic pulmonary hypertension. Eur Respir J 2021; 57: 2002828
    CrossrefPubMedGoogle Scholar
  • 2 Held M. et al. Frequency and characterization of CTEPH and CTEPD according to the mPAP threshold > 20 mm Hg: Retrospective analysis from data of a prospective PE aftercare program. Respir Med 2023; 210: 107177
    CrossrefPubMedGoogle Scholar
  • 3 Guerin L. et al. Prevalence of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism. Prevalence of CTEPH after pulmonary embolism. Thromb Haemost 2014; 112: 598-605
    Article in Thieme ConnectPubMedGoogle Scholar
  • 4 Klok FA, Delcroix M, Bogaard HJ. Chronic thromboembolic pulmonary hypertension from the perspective of patients with pulmonary embolism. J Thromb Haemost 2018; 16: 1040-1051
    CrossrefPubMedGoogle Scholar
  • 5 Klok FA. et al. Derivation of a clinical prediction score for chronic thromboembolic pulmonary hypertension after acute pulmonary embolism. J Thromb Haemost 2016; 14: 121-128
    CrossrefPubMedGoogle Scholar
  • 6 Konstantinides SV. et al. 2019 ESC Guidelines for the diagnosis and management of acute pulmonary embolism developed in collaboration with the European Respiratory Society (ERS). Eur Heart J 2020; 41: 543-603
    CrossrefPubMedGoogle Scholar
  • 7 Bonderman D. et al. Risk factors for chronic thromboembolic pulmonary hypertension. Eur Respir J 2009; 33: 325-331
    CrossrefPubMedGoogle Scholar
  • 8 Narechania S, Renapurkar R, Heresi GA. Mimickers of chronic thromboembolic pulmonary hypertension on imaging tests: a review. Pulm Circ 2020; 10: 2045894019882620
    CrossrefPubMedGoogle Scholar
  • 9 Xi XY. et al. Value of (18)F-FDG PET/CT in differentiating malignancy of pulmonary artery from pulmonary thromboembolism: a cohort study and literature review. Int J Cardiovasc Imaging 2019; 35: 1395-1403
    CrossrefPubMedGoogle Scholar
  • 10 Helmersen D, Provencher S, Hirsch AM. et al. Diagnosis of chronic thromboembolic pulmonary hypertension: A Canadian Thoracic Society clinical practice guideline update. Canadian Journal of Respiratory, Critical Care, and Sleep Medicine 2019; 3: 1-22
    CrossrefPubMedGoogle Scholar
  • 11 Lasch F. et al. Comparison of MRI and VQ-SPECT as a Screening Test for Patients With Suspected CTEPH: CHANGE-MRI Study Design and Rationale. Front Cardiovasc Med 2020; 7: 51
    CrossrefPubMedGoogle Scholar
  • 12 Nagel C. et al. Exercise training improves exercise capacity and quality of life in patients with inoperable or residual chronic thromboembolic pulmonary hypertension. PLoS One 2012; 7: e41603
    CrossrefPubMedGoogle Scholar
  • 13 Nagel C. et al. Supervised Exercise Training in Patients with Chronic Thromboembolic Pulmonary Hypertension as Early Follow-Up Treatment after Pulmonary Endarterectomy: A Prospective Cohort Study. Respiration 2020; 99: 577-588
    CrossrefPubMedGoogle Scholar
  • 14 Bunclark K. et al. A multicenter study of anticoagulation in operable chronic thromboembolic pulmonary hypertension. J Thromb Haemost 2020; 18: 114-122
    CrossrefPubMedGoogle Scholar
  • 15 Humbert M. et al. Oral anticoagulants (NOAC and VKA) in chronic thromboembolic pulmonary hypertension. J Heart Lung Transplant 2022; 41: 716-721
    CrossrefPubMedGoogle Scholar
  • 16 Burmeister C. et al. Head-to-head Comparison Between Direct Oral Anticoagulants and Vitamin K Antagonists for Chronic Thromboembolic Pulmonary Hypertension: A Systematic Review and Meta-Analysis. Curr Probl Cardiol 2023; 48: 101232
    CrossrefPubMedGoogle Scholar
  • 17 Ordi-Ros J. et al. Rivaroxaban Versus Vitamin K Antagonist in Antiphospholipid Syndrome: A Randomized Noninferiority Trial. Ann Intern Med 2019; 171: 685-694
    CrossrefPubMedGoogle Scholar
  • 18 Pengo V. et al. Rivaroxaban vs warfarin in high-risk patients with antiphospholipid syndrome. Blood 2018; 132: 1365-1371
    CrossrefPubMedGoogle Scholar
  • 19 Hsieh WC. et al. Residual pulmonary hypertension after pulmonary endarterectomy: A meta-analysis. J Thorac Cardiovasc Surg 2018; 156: 1275-1287
    CrossrefPubMedGoogle Scholar
  • 20 Kim NH. et al. Chronic thromboembolic pulmonary hypertension. Eur Respir J 2019; 53
    PubMedGoogle Scholar
  • 21 Madani MM. et al. Pulmonary endarterectomy: recent changes in a single institution's experience of more than 2,700 patients. Ann Thorac Surg 2012; 94: 97-103 discussion 103
    CrossrefPubMedGoogle Scholar
  • 22 Lankeit M. et al. Pulmonary endarterectomy in chronic thromboembolic pulmonary hypertension. J Heart Lung Transplant 2017; DOI: 10.1016/j.healun.2017.06.011.
    CrossrefPubMedGoogle Scholar
  • 23 Delcroix M. et al. Long-Term Outcome of Patients With Chronic Thromboembolic Pulmonary Hypertension: Results From an International Prospective Registry. Circulation 2016; 133: 859-871
    CrossrefPubMedGoogle Scholar
  • 24 Newnham M. et al. CAMPHOR score: patient-reported outcomes are improved by pulmonary endarterectomy in chronic thromboembolic pulmonary hypertension. Eur Respir J 2020; 56: 1902096
    CrossrefPubMedGoogle Scholar
  • 25 Vuylsteke A. et al. Circulatory arrest versus cerebral perfusion during pulmonary endarterectomy surgery (PEACOG): a randomised controlled trial. Lancet 2011; 378: 1379-1387
    CrossrefPubMedGoogle Scholar
  • 26 D'Armini AM. et al. Pulmonary endarterectomy for distal chronic thromboembolic pulmonary hypertension. J Thorac Cardiovasc Surg 2014; 148: 1005-1011 1012 e1-2, discussion 1011-2
    CrossrefPubMedGoogle Scholar
  • 27 Quadery SR. et al. The impact of patient choice on survival in chronic thromboembolic pulmonary hypertension. Eur Respir J 2018; 52: 1800589
    CrossrefPubMedGoogle Scholar
  • 28 Guth S. et al. Exercise right heart catheterisation before and after pulmonary endarterectomy in patients with chronic thromboembolic disease. Eur Respir J 2018; 52: 1800458
    CrossrefPubMedGoogle Scholar
  • 29 Taboada D. et al. Outcome of pulmonary endarterectomy in symptomatic chronic thromboembolic disease. Eur Respir J 2014; 44: 1635-1645
    CrossrefPubMedGoogle Scholar
  • 30 Madani M. et al. Pulmonary Endarterectomy. Patient Selection, Technical Challenges, and Outcomes. Ann Am Thorac Soc 2016; 13 (Suppl. 03) S240-S247
    CrossrefPubMedGoogle Scholar
  • 31 Ghofrani HA. et al. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension. N Engl J Med 2013; 369: 319-329
    CrossrefPubMedGoogle Scholar
  • 32 Jais X. et al. Bosentan for treatment of inoperable chronic thromboembolic pulmonary hypertension: BENEFiT (Bosentan Effects in iNopErable Forms of chronIc Thromboembolic pulmonary hypertension), a randomized, placebo-controlled trial. J Am Coll Cardiol 2008; 52: 2127-2134
    CrossrefPubMedGoogle Scholar
  • 33 Reichenberger F. et al. Long-term treatment with sildenafil in chronic thromboembolic pulmonary hypertension. Eur Respir J 2007; 30: 922-927
    CrossrefPubMedGoogle Scholar
  • 34 Guth S. et al. Current strategies for managing chronic thromboembolic pulmonary hypertension: results of the worldwide prospective CTEPH Registry. ERJ Open Res 2021; 7: 00850-2020
    CrossrefPubMedGoogle Scholar
  • 35 Brenot P. et al. French experience of balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension. Eur Respir J 2019; 53: 1802095
    CrossrefPubMedGoogle Scholar
  • 36 Fukui S. et al. Right ventricular reverse remodelling after balloon pulmonary angioplasty. Eur Respir J 2014; 43: 1394-1402
    CrossrefPubMedGoogle Scholar
  • 37 Kataoka M. et al. Percutaneous transluminal pulmonary angioplasty for the treatment of chronic thromboembolic pulmonary hypertension. Circ Cardiovasc Interv 2012; 5: 756-762
    CrossrefPubMedGoogle Scholar
  • 38 Kriechbaum SD. et al. Galectin-3, GDF-15, and sST2 for the assessment of disease severity and therapy response in patients suffering from inoperable chronic thromboembolic pulmonary hypertension. Biomarkers 2020; 25: 578-586
    CrossrefPubMedGoogle Scholar
  • 39 Kriechbaum SD. et al. Mid-regional pro-atrial natriuretic peptide and copeptin as indicators of disease severity and therapy response in CTEPH. ERJ Open Res 2020; 6: 00356-2020
    CrossrefPubMedGoogle Scholar
  • 40 Lang I. et al. Balloon pulmonary angioplasty in chronic thromboembolic pulmonary hypertension. Eur Respir Rev 2017; 26: 160119
    CrossrefPubMedGoogle Scholar
  • 41 Mahmud E. et al. Balloon Pulmonary Angioplasty for Chronic Thromboembolic Pulmonary Hypertension. Interv Cardiol Clin 2018; 7: 103-117
    CrossrefPubMedGoogle Scholar
  • 42 Mizoguchi H. et al. Refined balloon pulmonary angioplasty for inoperable patients with chronic thromboembolic pulmonary hypertension. Circ Cardiovasc Interv 2012; 5: 748-755
    CrossrefPubMedGoogle Scholar
  • 43 Ogawa A. et al. Balloon Pulmonary Angioplasty for Chronic Thromboembolic Pulmonary Hypertension: Results of a Multicenter Registry. Circ Cardiovasc Qual Outcomes 2017; 10: e004029
    CrossrefPubMedGoogle Scholar
  • 44 Olsson KM. et al. Balloon pulmonary angioplasty for inoperable patients with chronic thromboembolic pulmonary hypertension: the initial German experience. Eur Respir J 2017; 49: 1602409
    CrossrefPubMedGoogle Scholar
  • 45 Roller FC. et al. Correlation of native T1 mapping with right ventricular function and pulmonary haemodynamics in patients with chronic thromboembolic pulmonary hypertension before and after balloon pulmonary angioplasty. Eur Radiol 2019; 29: 1565-1573
    CrossrefPubMedGoogle Scholar
  • 46 Sugimura K. et al. Percutaneous transluminal pulmonary angioplasty markedly improves pulmonary hemodynamics and long-term prognosis in patients with chronic thromboembolic pulmonary hypertension. Circ J 2012; 76: 485-488
    CrossrefPubMedGoogle Scholar
  • 47 Inami T. et al. Long-Term Outcomes After Percutaneous Transluminal Pulmonary Angioplasty for Chronic Thromboembolic Pulmonary Hypertension. Circulation 2016; 134: 2030-2032
    CrossrefPubMedGoogle Scholar
  • 48 Ejiri K. et al. Vascular Injury Is a Major Cause of Lung Injury After Balloon Pulmonary Angioplasty in Patients With Chronic Thromboembolic Pulmonary Hypertension. Circ Cardiovasc Interv 2018; 11: e005884
    CrossrefPubMedGoogle Scholar
  • 49 Shimokawahara H. et al. Vessel Stretching Is a Cause of Lumen Enlargement Immediately After Balloon Pulmonary Angioplasty: Intravascular Ultrasound Analysis in Patients With Chronic Thromboembolic Pulmonary Hypertension. Circ Cardiovasc Interv 2018; 11: e006010
    CrossrefPubMedGoogle Scholar
  • 50 Jais X. et al. Balloon pulmonary angioplasty versus riociguat for the treatment of inoperable chronic thromboembolic pulmonary hypertension (RACE): a multicentre, phase 3, open-label, randomised controlled trial and ancillary follow-up study. Lancet Respir Med 2022; 10: 961-971
    CrossrefPubMedGoogle Scholar
  • 51 Wiedenroth CB. et al. Balloon pulmonary angioplasty for inoperable patients with chronic thromboembolic disease. Pulm Circ 2018; 8: 2045893217753122
    CrossrefPubMedGoogle Scholar
  • 52 Romanov A. et al. Pulmonary Artery Denervation for Patients With Residual Pulmonary Hypertension After Pulmonary Endarterectomy. J Am Coll Cardiol 2020; 76: 916-926
    CrossrefPubMedGoogle Scholar
  • 53 Bresser P. et al. Continuous intravenous epoprostenol for chronic thromboembolic pulmonary hypertension. Eur Respir J 2004; 23: 595-600
    CrossrefPubMedGoogle Scholar
  • 54 Nagaya N. et al. Prostacyclin therapy before pulmonary thromboendarterectomy in patients with chronic thromboembolic pulmonary hypertension. Chest 2003; 123: 338-343
    CrossrefPubMedGoogle Scholar
  • 55 Reesink HJ. et al. Bosentan as a bridge to pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension. J Thorac Cardiovasc Surg 2010; 139: 85-91
    CrossrefPubMedGoogle Scholar
  • 56 Araszkiewicz A. et al. Balloon pulmonary angioplasty for the treatment of residual or recurrent pulmonary hypertension after pulmonary endarterectomy. Int J Cardiol 2019; 278: 232-237
    CrossrefPubMedGoogle Scholar
  • 57 Shimura N. et al. Additional percutaneous transluminal pulmonary angioplasty for residual or recurrent pulmonary hypertension after pulmonary endarterectomy. Int J Cardiol 2015; 183: 138-142
    CrossrefPubMedGoogle Scholar
  • 58 Cannon JE. 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
    CrossrefPubMedGoogle Scholar
  • 59 Wiedenroth CB. et al. Combined pulmonary endarterectomy and balloon pulmonary angioplasty in patients with chronic thromboembolic pulmonary hypertension. J Heart Lung Transplant 2016; 35: 591-596
    CrossrefPubMedGoogle Scholar
  • 60 Humbert M. et al. Risk assessment in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. Eur Respir J 2019; 53: 1802004
    CrossrefPubMedGoogle Scholar
  • 61 Delcroix M. et al. Risk assessment in medically treated chronic thromboembolic pulmonary hypertension patients. Eur Respir J 2018; 52: 1800248
    CrossrefPubMedGoogle Scholar
  • 62 Benza RL. et al. REVEAL risk score in patients with chronic thromboembolic pulmonary hypertension receiving riociguat. J Heart Lung Transplant 2018; 37: 836-843
    CrossrefPubMedGoogle Scholar
  • 63 Andreassen AK. et al. Balloon pulmonary angioplasty in patients with inoperable chronic thromboembolic pulmonary hypertension. Heart 2013; 99: 1415-1420
    CrossrefPubMedGoogle Scholar
  • 64 Wiedenroth CB. et al. Sequential treatment with riociguat and balloon pulmonary angioplasty for patients with inoperable chronic thromboembolic pulmonary hypertension. Pulm Circ 2018; 8: 2045894018783996
    CrossrefPubMedGoogle Scholar
  • 65 Humbert M. et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J 2023; 61: 2200879
    CrossrefPubMedGoogle Scholar