There are several treatments that have been developed to help people with chronic thromboembolic pulmonary hypertension (CTEPH). Depending on the patient’s disease, comorbidities (other health problems, age, etc), and personal preferences the CTEPH team might recommend a combination of different treatment approaches.
The treatment of choice for qualifying CTEPH patients is a surgery called a pulmonary thromboendarterectomy, or PTE. This surgery is a highly specialized procedure that removes the large, old blood clots contributing to PH from the arteries in the lungs. Since PTE could potentially cure CTEPH for some patients, it is the first recommended treatment. Every patient diagnosed with CTEPH should be evaluated by an expert CTEPH team, including CTEPH physicians and PTE surgeons, to assess their candidacy for PTE surgery. Learn more about PTE surgery process.
Balloon pulmonary angioplasty (BPA) is a newer treatment for some CTEPH patients. During a BPA, a doctor will guide a small tube (called a catheter) through a large vein usually starting in the patient’s groin or neck. Doctors will then guide the catheter through the right side of the heart and into vessels of the lungs that contain old clot or scar tissue. The doctor will inflate a balloon at the end of the catheter, which pushes the old clot/scar tissue to the sides of the blood vessel. This allows blood to flow through the vessel more freely.
There are different doctors who perform BPA procedures whom might be different than a regular PH doctor. These doctors typically include cardiologists, pulmonologists and interventional radiologists. All the experts involved should work closely together during treatment.
BPA is frequently not a one-time treatment for CTEPH patients. Many patients require multiple procedures over time.
If a patient’s clots are considered inoperable, PH-specific medications have been shown to have some benefit in reducing blood pressure in the lungs and improving functional status. An FDA-approved PH-specific drug called riociguat is available for these CTEPH patients. Medications should not be used in lieu of a careful evaluation for PTE by an expert or multidisciplinary center and is only indicated for patients deemed not to be good candidates for PTE or for patients who still have some PH after PTE. Learn more.