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

If the clots are accessible, and the patient is able to withstand surgery, the treatment of choice is surgical pulmonary thromboendarterectomy (PTE), also called pulmonary endarterectomy (PEA). During this surgery, the breastbone is split, the patient is put on a heart-lung machine, and the heart is stopped. Surgeons use special tools to remove the clots. 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 patient is not able to have the surgery, treatment might include:

  • Percutaneous balloon pulmonary angioplasty: This procedure calls for a small incision to place small balloons to destroy the clots in the arteries. It is usually done more than once. It might also be done in a patient who has already had PTE. It has been shown to improve blood flow and breathing.
  • Double lung transplant: This procedure replaces a patient’s lungs with donor lungs.
  • Riociguat (Adempas®): This is the only drug approved to treat patients with CTEPH who cannot have surgery, or for patients 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 2 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 as you are able. 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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