Chronic thromboembolic pulmonary hypertension (CTEPH) is a condition caused by persistent blockages in the pulmonary arteries. It’s most commonly treated with surgery. Several surgical procedures may be recommended to dramatically improve — and, in many cases, cure — CTEPH.

CTEPH begins as a type of pulmonary arterial hypertension (PAH) that develops due to chronic blood clots in the lungs’ arteries and the right ventricle of the heart. These blood clots restrict blood flow in the lungs.

In some people, CTEPH develops when a blood clot fails to dissolve after an acute pulmonary embolism — a clot that forms somewhere else in the body and ends up in a lung. Other people with CTEPH have no history of acute pulmonary embolism. PAH is often treated with blood thinners to stop blood clots from forming. However, these chronic blood clots can develop slowly over time — even in people who take blood thinners.

Pulmonary Endarterectomy

Pulmonary endarterectomy (PEA), sometimes called “pulmonary thromboendarterectomy,” is the primary surgical treatment suggested for CTEPH. PEA restores blood flow by removing blood clots and scar tissue in the lungs’ arteries.

During PEA, a heart-lung machine (also called a cardiopulmonary bypass machine) keeps a person alive while their heart is stopped. As its name suggests, the heart-lung machine takes over for the heart and lungs during surgery. It keeps blood pumping and oxygenated throughout the body. This allows the surgeon to safely open the pulmonary arteries and remove the blockages.

When Is Pulmonary Endarterectomy Performed?

PEA is the premier medical treatment for people with CTEPH who are eligible for surgery. Because the procedure is so intensive, eligibility must be carefully assessed. Before PEA can be performed, specialists use medical imaging to evaluate the extent of a person’s disease. A person is considered to have operable CTEPH if a surgeon can reach the blood clots blocking their arteries. A person’s blood clots must also severely impact blood flow to the lungs and heart.

Pulmonary angiography is the most common medical imaging technique used to determine the location of a blood clot. This test uses X-rays and a special contrast dye to show how blood flows through the lungs.

Doctors will also test a person’s hemodynamic impairment, or how severely blood flow is blocked in the heart. Hemodynamic impairment is commonly measured using right heart catheterization. This procedure assesses a person’s mean (average) pulmonary artery pressure, cardiac output, and pulmonary vascular resistance.

Doctors use the New York Heart Association (NYHA) Functional Classification system for heart failure to determine if a person’s thromboembolism is impacting their quality of life. A doctor may use the NYHA guide to classify the level of heart failure experienced by a person with CTEPH, based on their symptoms and medical testing results. People with CTEPH who are eligible for PEA are classified as having class 2 heart failure or greater. Many people undergoing PEA fall into class 4, the most severe class.

Other considerations for PEA candidates include their comorbidities, or other medical conditions they have along with CTEPH. Some comorbidities can increase the risk for serious complications either before or after surgery.

Outcomes of Pulmonary Endarterectomy

PEA is a major open-heart surgery that comes with serious risks, including death. That said, PEA has a 95 percent survival rate.

Although there are risks, PEA is the only treatment for CTEPH that has the potential to cure the disease. Following PEA, people often experience normal blood flow. Some move toward an improved NYHA functional class or experience improved exercise capacity. After undergoing a pulmonary endarterectomy, people usually take a blood thinner like Coumadin (warfarin) for the rest of their lives to help prevent additional blockages.

Balloon Pulmonary Angioplasty

Balloon pulmonary angioplasty (BPA) is a minimally invasive procedure used to treat the symptoms of CTEPH. During BPA, a person is placed under light sedation. A catheter is used to insert a deflated balloon into their blocked arteries. The balloon is then inflated, which pushes the blood clot to the walls of the blood vessels and opens a pathway to restore blood flow. The balloon is then deflated and removed.

When Do Doctors Recommend Balloon Pulmonary Angioplasty?

BPA may be an option if doctors determine a person’s CTEPH is inoperable. BPA is sometimes also indicated for people who have undergone PEA but have recurrent pulmonary hypertension.

Outcomes of Balloon Pulmonary Angioplasty

BPA can improve symptoms of CTEPH, but people with CTEPH benefit the most from multiple follow-up BPA procedures. BPA is a newer treatment option for CTEPH, and the effects of BPA on survival are not fully understood. Research into the long-term outcomes of BPA is ongoing.

Lung Transplantation

A lung transplant is another surgical option for people with CTEPH. This procedure involves replacing both of a person’s damaged lungs with those of an organ donor. It is often difficult to obtain a lung transplant, as the donor lungs must be a genetic match to the person receiving treatment.

When Is Lung Transplantation Recommended?

For people with CTEPH who are ineligible for surgeries like PEA or BPA, lung transplantation may still be an option. Additionally, a lung transplant may be recommended for people who have received PEA but continue to experience symptoms of CTEPH.

Generally, medications are the first course of treatment for people who can’t have surgery. Medications aim to treat the symptoms associated with CTEPH and can include:

• Phosphodiesterase inhibitors, like sildenafil
• Endothelin receptor antagonists, like Tracleer (bosentan)
• Intravenous prostacyclins, like Flolan (epoprostenol) or Orenitram (treprostinil)
• Soluble guanylate cyclase stimulators, like Adempas (riociguat)

Lung transplantation is generally not considered unless other therapies have been tried with little success.

Outcomes of Lung Transplantation

Lung transplantation has the potential to improve the health and quality of life for people living with CTEPH. Having a lung transplant is a major surgical procedure. It carries the risk of infection and other complications. People who have received a lung transplant must take immunosuppressants for the rest of their lives to prevent rejection of the donor lung. Additionally, they must see their doctor frequently to monitor any symptoms of lung rejection, disease, or infection.

Talk With Others Who Understand

On myPHteam, the social network for people with pulmonary hypertension and their loved ones, more than 42,800 members come together to ask questions, give advice, and share their stories with others who understand life with pulmonary hypertension.

Have you had any surgical procedures to relieve your CTEPH symptoms? Share your experience in the comments below, or start a conversation by posting on your Activities page.