PT  - JOURNAL ARTICLE
AU  - Nick H. Kim
AU  - Marion Delcroix
AU  - Xavier Jais
AU  - Michael M. Madani
AU  - Hiromi Matsubara
AU  - Eckhard Mayer
AU  - Takeshi Ogo
AU  - Victor F. Tapson
AU  - Hossein-Ardeschir Ghofrani
AU  - David P. Jenkins
TI  - Chronic thromboembolic pulmonary hypertension
AID  - 10.1183/13993003.01915-2018
DP  - 2018 Jan 01
TA  - European Respiratory Journal
PG  - 1801915
4099  - http://erj.ersjournals.com/content/early/2018/10/11/13993003.01915-2018.short
4100  - http://erj.ersjournals.com/content/early/2018/10/11/13993003.01915-2018.full
AB  - Chronic thromboembolic pulmonary hypertension (CTEPH) is a complication of pulmonary embolism and a major cause of chronic PH leading to right heart failure and death. Lung ventilation/perfusion scintigraphy is the screening test of choice; a normal scan rules out CTEPH. In the case of an abnormal perfusion scan, a high-quality pulmonary angiogram is necessary to confirm and define the pulmonary vascular involvement and prior to making a treatment decision. PH is confirmed with right heart catheterisation, which is also necessary for treatment determination. In addition to chronic anticoagulation therapy, each patient with CTEPH should receive treatment assessment starting with evaluation for pulmonary endarterectomy, which is the guideline recommended treatment. For technically inoperable cases, PH-targeted medical therapy is recommended (currently riociguat based on the CHEST studies), and balloon pulmonary angioplasty should be considered at a centre experienced with this challenging but potentially effective and complementary intervention.State of the art and research perspectives in chronic thromboembolic pulmonary hypertension, including treatment algorithm http://ow.ly/C3Iy30mfUly