Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

Chronic thromboembolic pulmonary hypertension (CTEPH) is high blood pressure in the small blood vessels of your lungs. It is caused by blood clots and scarring. CTEPH can happen to anyone, but it's often curable.

Survival was significantly better than for the cohort of 173 inoperable CTEPH patients (median age of 73 years) treated with medical therapy; the KM-estimated 3-, 5- and 10-year survival rates were 84% (95% CI: 78–89%), 72% (95% CI: 63–79%) and 39% (95% CI: 29–49%), respectively.

Optimal medical treatment for CTEPH consists of anticoagulants, plus diuretics and oxygen in cases of heart failure or hypoxaemia [1]. The aim of anticoagulation in CTEPH is to prevent in situ pulmonary artery thrombosis and recurrent venous thromboembolism [14].

CTEPH is a rare form of pulmonary hypertension. It occurs when there’s abnormally high pressure in your lung’s small blood vessels. Abnormally high pressures are a result of prior blood clots in your lungs.

Who gets chronic thromboembolic pulmonary hypertension?

CTEPH only happens to people with a history of blood clots in the lungs(pulmonary embolism). If you have high pressures without pulmonary embolisms, then you don’t have CTEPH. You have another condition.

How common is CTEPH?

There are approximately 5,000 new CTEPH cases per year in the U.S. This might be underestimated since the condition isn't always correctly diagnosed.

How can chronic thromboembolic pulmonary hypertension affect my health?

With pulmonary hypertension and CTEPH, pressure is too high on the right side of your heart. This leads to a backup of oxygen-poor blood. Your heart then works harder to push it out to your lungs. Blood also takes longer to travel through your lungs, causing a drop in oxygen levels.

SYMPTOMS AND CAUSES

What causes CTEPH?

The condition occurs when tiny blood clots in lung blood vessels don’t dissolve. This causes scar-like tissue to develop, leading to blood vessel narrowing. The more severe the narrowing, the higher the pressure.

CTEPH risk factors

Chronic thromboembolic pulmonary hypertension risk factors include:

Blood type other than Type O.
Bone infection (osteomyelitis).
Certain cancers.
Chronic gastrointestinal diseases, such as inflammatory bowel disease.
Being assigned female at birth.
Infected heart tissue due to a pacemaker.
Personal or family history of blood clots or blood clotting disorders. These include antiphospholipid syndrome.
Prior spleen removal (splenectomy).
Thyroid hormone replacement therapy.

What are CTEPH symptoms?

Chronic thromboembolic pulmonary hypertension symptoms include:

Chest pain or tightness.
Coughing up blood, which is rare.
Cyanosis, fingers and toes with a bluish tint.
Dizziness.
Fainting (syncope).
Fatigue.
Pounding in your chest, also known as heart palpitations.
Shortness of breath, especially during physical activity.
Swollen legs due to fluid retention (edema).

DIAGNOSIS AND TESTS

How is CTEPH diagnosed?

Your care may start with tests to check for signs of CTEPH. These include:

Lung ventilation-perfusion scan (V/Q scan).
Pulmonary angiogram, which shows whether there are blood clots or narrow blood vessels.
Pulmonary function tests.
Transthoracic echocardiogram (TTE).

Additional studies are typically necessary. They help confirm a chronic thromboembolic pulmonary hypertension diagnosis and its severity. You may need:

Right heart catheterization.
CT pulmonary or digital subtraction angiography. These tests use advanced imaging techniques to check your pulmonary arteries.
MRI.
Exercise stress test.

MANAGEMENT AND TREATMENT

How is chronic thromboembolic pulmonary hypertension treated?

A procedure is typically necessary. There are two options:

Pulmonary endarterectomy: This open surgical procedure removes blood clots from your lung arteries.
Pulmonary balloon angioplasty: This procedure uses tiny balloons at the tip of long tubes (catheters). They push aside scar tissue to open up narrowed arteries.

Do I have additional CTEPH treatment options?

Riociguat tablets, a pulmonary hypertension medication, can bring some symptom relief. This drug is for people who can't have a procedure. It’s also for patients whose pulmonary hypertension continues after surgery.

PREVENTION

Is chronic thromboembolic pulmonary hypertension preventable?

CTEPH is a complication of many health conditions, some of which are out of your control. You can lower your risk by taking good care of your lungs. This includes avoiding tobacco or quitting smoking.

OUTLOOK / PROGNOSIS

What is the outlook for patients who have CTEPH?

Pulmonary endarterectomy and pulmonary balloon angioplasty can potentially cure CTEPH. People who have one of these procedures typically have an excellent prognosis. Despite this positive outlook, many patients struggle with anxiety and depression. This is especially true in people who aren't eligible for curative treatment. If you experience these issues, let your healthcare provider know. They can address your concerns and recommend therapies to help you feel better.

LIVING WITH

What’s important to know about living with chronic thromboembolic pulmonary hypertension?

You'll be on blood thinners for the rest of your life. If you take warfarin, you might have to avoid certain foods. These include foods high in vitamin K, like soy and broccoli. Light exercise, like walking, helps many people gain strength and feel their best. Your healthcare provider may recommend a medically supervised exercise program(pulmonary rehabilitation). Key Takeaways Chronic thromboembolic hypertension is a form of pulmonary hypertension. It's a rare condition that causes high blood pressure in the small vessels of the lungs. CTEPH is potentially curable if you undergo a procedure. Many people who receive treatment for this condition have an excellent prognosis.