Subscribe to RSS
DOI: 10.1055/a-2012-0899
Chronisch thromboembolische pulmonale Hypertonie
Chronic thromboembolic pulmonary hypertension
Die chronisch thromboembolische pulmonale Hypertonie (CTEPH) ist eine seltene Erkrankung, aber gut behandelbare Spätfolge nach einer akuten Lungenembolie. Daher wird eine Nachuntersuchung nach einer ausreichenden Antikoagulation von mindestens 3 Monaten empfohlen. Es stehen 3 Behandlungsmethoden zur Verfügung: die pulmonale Endarteriektomie, die pulmonale Ballonangioplastie und Medikamente, die auf die pulmonale Hypertonie wirken. Die Entscheidung über die Behandlung sollte von einem erfahrenen multidisziplinären Team in spezialisierten CTEPH-Zentren getroffen werden.
Abstract
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare disease, but an important late sequela after acute pulmonary embolism. Therefore, follow-up after at least three months of sufficient anticoagulation is recommended. Patients with suspected CTEPH should be referred to specialized CTEPH centers for further evaluation and treatment.
Three treatment modalities are available: pulmonary endarterectomy (PEA), balloon pulmonary angioplasty (BPA) and pulmonary hypertension-targeted drugs. The indication for surgery depends mainly on the localization of the pulmonary arterial obstructions. Severe comorbidities as well as advanced age need individual evaluation, but do not present strict exclusion criteria. Multimodal treatments are common practice in inoperable CTEPH. However, treatment decision making in an experienced multidisciplinary team is mandatory.
-
CTEPH ist eine wichtige Komplikation nach überstandener Lungenembolie.
-
Diagnostik und Therapie der CTEPH sollten in entsprechend spezialisierten Zentren erfolgen.
-
Es gibt 3 etablierte Therapiemodalitäten: die pulmonale Endarteriektomie, die medikamentöse Therapie und die pulmonale Ballonangioplastie.
-
Das therapeutische Konzept hängt maßgeblich von der Lokalisation der pulmonalarteriellen Obstruktionen und der pulmonalen Hämodynamik ab.
-
Patienten mit CTEPH sollen langfristig (mindestens jährlich) verlaufskontrolliert werden.
Publication History
Article published online:
10 November 2023
© 2023. Thieme. All rights reserved.
Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany
-
Literatur
- 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
- 2 Simonneau G, Torbicki A, Dorfmüller P. et al. The pathophysiology of chronic thromboembolic pulmonary hypertension. Eur Respir Rev 2017; 26: 160112
- 3
Humbert M,
Kovacs G,
Hoeper MM.
et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension.
Eur Respir J 2023; 61: 2200879
MissingFormLabel
- 4 Quadery SR, Swift AJ, Billings CG. et al. The impact of patient choice on survival in chronic thromboembolic pulmonary hypertension. Eur Respir J 2018; 52: 1800589
- 5 Delcroix M, Torbicki A, Gopalan D. et al. ERS statement on chronic thromboembolic pulmonary hypertension. Eur Respir J 2021; 57: 2002828
- 6 Ende-Verhaar YM, Cannegieter SC, Noordegraaf AV. et al. Incidence of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism: A contemporary view of the published literature. Eur Respir J 2017; 49: 1601792
- 7 Kramm T, Wilkens H, Fuge J. et al. Incidence and characteristics of chronic thromboembolic pulmonary hypertension in Germany. Clin Res Cardiol 2018; 107: 548-553
- 8 Held M, Pfeuffer-Jovic E, Wilkens H. 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
- 9 Boon GJAM, Ende-Verhaar YM, Beenen LFM. et al. Prediction of chronic thromboembolic pulmonary hypertension with standardised evaluation of initial computed tomography pulmonary angiography performed for suspected acute pulmonary embolism. Eur Radiol 2022; 32: 2178-2187
- 10 Valerio L, Mavromanoli AC, Barco S. et al. Chronic thromboembolic pulmonary hypertension and impairment after pulmonary embolism: the FOCUS study. Eur Heart J 2022; 43: 3387-3398
- 11 Bonderman D, Wilkens H, Wakounig S. et al. Risk factors for chronic thromboembolic pulmonary hypertension. Eur Respir J 2009; 33: 325-331
- 12 Simonneau G, Dorfmüller P, Guignabert C. et al. Chronic thromboembolic pulmonary hypertension: the magic of pathophysiology. Ann Cardiothorac Surg 2022; 11: 106-119
- 13 Viswanathan G, Kirshner HF, Nazo N. et al. Single-Cell Analysis Reveals Distinct Immune and Smooth Muscle Cell Populations that Contribute to Chronic Thromboembolic Pulmonary Hypertension. Am J Respir Crit Care Med 2023; 207: 1358-1375
- 14
Konstantinides SV,
Meyer G,
Becattini C.
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
MissingFormLabel
- 15 Masy M, Giordano J, Petyt G. et al. Dual-energy CT (DECT) lung perfusion in pulmonary hypertension: concordance rate with V/Q scintigraphy in diagnosing chronic thromboembolic pulmonary hypertension (CTEPH). Eur Radiol 2018; 28: 5100-5110
- 16 de Perrot M, Gopalan D, Jenkins D. et al. Evaluation and management of patients with chronic thromboembolic pulmonary hypertension – consensus statement from the ISHLT. J Heart Lung Transplant 2021; 40: 1301-1326
- 17 Wiedenroth CB, Bandorski D, Ariobi K. et al. Does Age Matter? Pulmonary Endarterectomy in the Elderly Patient with CTEPH. Thorac Cardiovasc Surg 2022; 70: 663-670
- 18 Humbert M, Simonneau G, Pittrow D. et al. Oral anticoagulants (NOAC and VKA) in chronic thromboembolic pulmonary hypertension. J Heart Lung Transplant 2022; 41: 716-721
- 19 Pengo V, Denas G, Zoppellaro G. et al. Rivaroxaban vs warfarin in high-risk patients with antiphospholipid syndrome. Blood 2018; 132: 1365-1371
- 20 Nagel C, Prange F, Guth S. 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
- 21 Kim NH, Delcroix M, Jaïs X. et al. Chronic thromboembolic pulmonary hypertension. Eur Respir J 2019; 53: 1801915
- 22 Lankeit M, Krieg V, Hobohm L. et al. Pulmonary endarterectomy in chronic thromboembolic pulmonary hypertension. J Heart Lung Transplant 2018; 37: 250-258
- 23 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
- 24 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
- 25 Guth S, D’Armini AM, Delcroix M. et al. Current strategies for managing chronic thromboembolic pulmonary hypertension: results of the worldwide prospective CTEPH Registry. ERJ Open Res 2021; 7: 00850-02020
- 26 Brenot P, Jaïs X, Taniguchi Y. et al. French experience of balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension. Eur Respir J 2019; 53: 1802095
- 27 Olsson KM, Wiedenroth CB, Kamp JC. et al. Balloon pulmonary angioplasty for inoperable patients with chronic thromboembolic pulmonary hypertension: the initial German experience. Eur Respir J 2017; 49: 1602409
- 28 Darocha S, Araszkiewicz A, Kurzyna M. et al. Balloon Pulmonary Angioplasty in Technically Operable and Technically Inoperable Chronic Thromboembolic Pulmonary Hypertension. J Clin Med 2021; 10: 1038
- 29 Wiedenroth CB, Rolf A, Steinhaus K. et al. Riociguat and balloon pulmonary angioplasty improve prognosis in patients with inoperable chronic thromboembolic pulmonary Hypertension. J Heart Lung Transplant 2023; 42: 134-139
- 30 Jaïs X, Brenot P, Bouvaist H. 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
- 31 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
- 32 Araszkiewicz A, Darocha S, Pietrasik 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
- 33 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 Transplantat 2016; 35: 591-596
- 34 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