What Is Chronic Thromboembolic Pulmonary Hypertension (CTEPH)?

CTEPH is a form of pulmonary hypertension — or high blood pressure in the lungs — that can occur when an embolism (blood clot) gets stuck in a blood vessel in one of these organs. This can block blood flow within the lung, leading to symptoms such as:

In this relatively rare condition, the blood pressure in your lungs can rise, often to dangerous levels. If you’ve been diagnosed with chronic thromboembolic pulmonary hypertension (CTEPH), you’ll want to know about the best CTEPH treatment.

CTEPH Causes and Risk Factors

Having one or more pulmonary emboli during your lifetime is a major risk factor for developing CTEPH. If you have been treated for a pulmonary embolism and have continued shortness of breath or develop shortness of breath months or even years after completing your treatment, you should talk to your doctor about CTEPH.

CTEPH Treatment Options

Although CTEPH can be life threatening, it is treatable. In fact, this condition is the only type of pulmonary hypertension that can be cured with surgery in some people. This treatment, called pulmonary thromboendarterectomy (PTE), has been found to cure about 90 percent of patients with CTEPH, depending on how advanced the disease is. Here’s what you should know about CTEPH treatment:

Pulmonary thromboendarterectomy: Surgery for CTEPH

Pulmonary thromboendarterectomy (PTE) is a complex procedure that requires a surgical team that is very experienced in this approach. During this type of surgery, the surgeon makes an incision in your chest and then uses special tools to clear blockages in the pulmonary arteries in the lungs. Because your heart will be stopped during PTE, you will need to be attached to a heart-lung machine, which will temporarily take over the function of your heart and lungs. About 10 percent of patients still experience symptoms after this CTEPH treatment, but the others have a complete resolution of the problem. Your doctor can tell you about the risks and benefits of PTE.

Your doctor will also determine whether or not PTE is right for you by weighing several different factors. These include:

  • The severity of your symptoms
  • How high your pulmonary blood pressure is
  • Whether the clots can be reached through surgery
  • Whether you have any other medical conditions

If you are not a candidate for surgery, or if you have persistent pulmonary hypertension after the procedure, there are medical treatments available to help manage your disease. However, patients should not delay an evaluation at a CTEPH center first to determine if they are candidates for potentially curative PTE.

If you would like to learn more about CTEPH and PTE as a treatment option or to schedule an appointment with a CTEPH specialist, visit the UPMC Comprehensive Pulmonary Hypertension Program website or call  412-648-6161, or toll-free at 1-877-PH4-UPMC, or email PHprogram@upmc.edu.