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.

What are the complications/side effects of the treatments of chronic thromboembolic pulmonary hypertension (CTEPH)?

PTE surgery is delicate. If you have the surgery, you should expect to be in the hospital for about 10 days after surgery. The breastbone takes about two months to heal. You should be able to resume almost all activities by three months after PTE.

If you take Adempas® to treat CTEPH, you might have the following side effects:

  • Stomach issues, including nausea.
  • Dizziness.
  • Headache.
  • Swollen feet and legs.

Untreated, CTEPH gets worse and can be fatal.

What can you do to help relieve symptoms of chronic thromboembolic pulmonary hypertension (CTEPH)?

  • Pay attention to your body, and do not overdo it. Low-intensity exercise (like walking) is recommended. Stop if you get dizzy or short of breath.
  • Follow a low-salt diet as recommended by your healthcare provider.
  • Follow any dietary needs that might go along with your blood-thinner medication.
  • You should continue to take blood-thinners for the rest of your life.

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