What are the treatments for chronic thromboembolic pulmonary hypertension (CTEPH)?

What are the treatments for chronic thromboembolic pulmonary hypertension (CTEPH)?

If the clots are accessible, and you are able to withstand surgery, the treatment of choice is surgical pulmonary thromboendarterectomy (PTE), also called pulmonary endarterectomy (PEA). During this surgery, done through an incision (cut) in the breastbone, you are put on a heart-lung machine and cooled from 37 degrees Celsius to 18 degrees Celsius. After cooling happens, the circulation is stopped. This lets the surgeon look into the arteries of the lungs. Surgeons use special tools to carefully separate the clots from the normal wall of the artery. This delicate surgery should be done by a specialist team with experience. In these circumstances, the surgery is safe and can cure this disease.

If the you are not able to or do not want to have open surgery, treatment might include:

• Percutaneous balloon pulmonary angioplasty: This procedure calls for a small puncture. It uses small balloons and catheters (tubes) to break the scars in the arteries. It is usually done more than once. It might also be done in someone who has already had PTE. It has been shown to improve blood flow and breathing.
• Double lung transplant: This procedure replaces your lungs with donor lungs. If you are not a candidate for PTE surgery or balloon angioplasty, lung transplantation may be an option.
• Riociguat (Adempas®): This is the only drug approved to treat people with CTEPH who cannot have surgery, or for people with pulmonary hypertension that continues after surgery.