Some patients may have repeated episodes of blood clots travelling to the lungs and developing severe lung artery abnormalities. These results in elevated lung pressures. We call this chronic thromboembolic pulmonary hypertension or CTEPH. Patients have to undergo a right heart catheterization study, a pulmonary angiogram (contrast injection of the arteries) as well as a ventilation perfusion scan ( radioisotope scan) for the diagnosis to be confirmed. Some patients may benefit from surgery. In those who have smaller, distal vessel disease, balloon pulmonary angioplasty may be considered.

A catheter is introduced in the lung arteries and contrast is administered to highlight the diseased areas (webs, slits and obstruction). These lesions are crossed with a very fine wire. A small size balloon is inflated to break up the webs and obstruction. No stents are required. As most patients have many obstructed lesions, repeat dilation of the blocked vessels are needed. Medical treatment may also be offered for some selected patients to improve their symptoms.

Irene Lang, Bernhard C. Meyer, Takeshi Ogo, Hiromi Matsubara, Marcin Kurzyna, Hossein-Ardeschir Ghofrani, Eckhard Mayer, Philippe Brenot

European Respiratory Review 2017 26: 160119; DOI: 10.1183/16000617.0119-2016