Thrombose chronique embolique pulmonaire hypertension (TCEPH)

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DESCRIPTION

Chronic thromboembolic pulmonary hypertension (CTEPH) is a type of chronic thromboembolism. It can also be called CTEPH.

SYMPTOMS

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.

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).

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