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Semin Respir Crit Care Med 2023; 44(06): 840-850
DOI: 10.1055/s-0043-1770122
Review Article

Monographic Issue on Pulmonary Hypertension: Medical and Interventional Treatment for Chronic Thromboembolic Pulmonary Hypertension

Marion Delcroix
1   Clinical Department of Respiratory Diseases, Department of Chronic Diseases and Metabolism (CHROMETA), Laboratory of Respiratory Diseases and Thoracic Surgery (BREATHE), KU Leuven-University of Leuven, University Hospitals of Leuven, Leuven, Belgium
2   European Reference Network on Rare Pulmonary Diseases (ERN-LUNG), Belgium
,
Catharina Belge
1   Clinical Department of Respiratory Diseases, Department of Chronic Diseases and Metabolism (CHROMETA), Laboratory of Respiratory Diseases and Thoracic Surgery (BREATHE), KU Leuven-University of Leuven, University Hospitals of Leuven, Leuven, Belgium
2   European Reference Network on Rare Pulmonary Diseases (ERN-LUNG), Belgium
,
Geert Maleux
3   Clinical Department of Radiology, University Hospitals of Leuven, Leuven, Belgium
,
Laurent Godinas
1   Clinical Department of Respiratory Diseases, Department of Chronic Diseases and Metabolism (CHROMETA), Laboratory of Respiratory Diseases and Thoracic Surgery (BREATHE), KU Leuven-University of Leuven, University Hospitals of Leuven, Leuven, Belgium
2   European Reference Network on Rare Pulmonary Diseases (ERN-LUNG), Belgium
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Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of acute pulmonary embolism. The reasons why clots do not resorb are incompletely understood, but the result is partial or complete fibrothrombotic obstruction of pulmonary arteries. A secondary microvasculopathy aggravates the pulmonary hypertension (PH) as a consequence of high flow and shear stress in the nonoccluded arteries. The treatment of CTEPH has long been purely surgical, but many patients were inoperable because of inaccessible lesions or severe comorbidities. Alternatives were developed, including medical therapy and more recently balloon pulmonary angioplasty (BPA). Depending on the generation of the obstructed vessels, the treatment will be surgical, up to the (sub)segmental level, or by BPA for more distal vessels. PH drugs are used to treat the microvasculopathy. The current paper describes the therapeutic management of inoperable patients: the medical approach with PH drugs used in mono- or combination therapy; the proper use of anticoagulants in CTEPH; the technique, indications, and results at short- and long-term of BPA; the multimodal approach for inoperable patients combining PH drugs and BPA; and the effects of rehabilitation. It shows the importance of a multidisciplinary approach to the disease.

Keywords

CTEPH - balloon pulmonary angioplasty - medical treatment - anticoagulation

Note

The University Hospitals of Leuven are part of the European Reference Network for rare lung diseases (ERN-Lung).




Publication History

Article published online:
11 August 2023

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