Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare form of high blood pressure affecting the lungs. It is caused by blood clots in the lung vessels that get stuck, clog the arteries and eventually form scar tissue. This scar tissue narrows the lung’s blood vessels, making it difficult for blood to pass through. CTEPH is challenging to diagnose, often leading to delayed or missed diagnoses.

Inova’s Pulmonary Hypertension Program is the only Pulmonary Hypertension Association-accredited Comprehensive Center of Excellence in the Washington, DC region. We have built a multidisciplinary program equipped to provide patients with state-of-the-art medical care to manage different types of pulmonary hypertension, including CTEPH.

Signs and Symptoms of CTEPH

  • Shortness of breath, especially if it occurs three months to a year after having an acute blood clot in a lung artery or leg vein
  • Leg swelling
  • Fatigue
  • Dizziness
  • Fainting
  • Heart palpitations (feeling the heart pounding in the chest)

Risk Factors for CTEPH

Risk factors for CTEPH include:

  • Younger or older age
  • Recurrent, unprovoked or large clot in a lung artery
  • Elevated lung pressure in the lungs at the time of the acute blood clot in the lungs
  • Cancer
  • Chronic inflammatory diseases
  • Absence of spleen
  • Female gender
  • Genetic markers showing a high risk of blood clotting disorders
  • Family history of blood clots

CTEPH Diagnosis

The following tests help us make a proper diagnosis of CTEPH:

  • Ventilation/perfusion scan (V/Q scan) learn more
  • Chest CT scan
  • Pulmonary angiogram
  • Right heart catheterization
  • Echocardiogram
  • Bloodwork

CTEPH Treatments

Inova offers a multidisciplinary, multimodality approach to the diagnosis and treatment of patients with CTEPH. Our goal is to relieve the patient’s symptoms, reduce the patient’s reliance on medicines and improve survival. Our multimodality treatment plans are customized for each patient and may include:

  • Surgery (which cures CTEPH for many patients)
  • Balloon Pulmonary Angioplasty (BPA)
  • Medication
  • A combination of one or more of these treatment options

Whether these treatments are used separately or in combination depends on each patient. Our team evaluates patients based on their medical records and a series of tests performed at Inova to determine the best course of action.

Pulmonary thromboendarterectomy (PTE) surgery, also referred to as a pulmonary endarterectomy, is the best treatment for CTEPH. PTE removes chronic blood clots from the lung’s arteries and is effective for most patients with large or proximal clots. While most patients feel better, can breathe easier and have lower blood pressure in the lung’s arteries, some may still have residual elevated pressure in the lungs after surgery. We have other treatments available for these patients.

About the Procedure
PTE surgery takes place under general anesthesia. You will be “asleep” during the procedure, and a special piece of equipment called a cardiopulmonary bypass machine will take over the work of your heart and lungs during the surgery.

During the procedure, the surgeon removes the clots from your pulmonary arteries. Once the clots are removed, the team takes you off of the cardiopulmonary bypass. Surgery typically takes 4-6 hours. After surgery, you will be transported to the Intensive Care Unit (ICU), usually by mid to late afternoon.

After Surgery
Your doctor will assess you periodically while you are in the ICU to determine when it is safe for you to begin breathing on your own. When you demonstrate that you can successfully breathe without the ventilator, we’ll remove your breathing tube and place you on oxygen with a mask or tube that delivers oxygen through your nostrils. Patients typically stay on a breathing machine for approximately 24 hours.

Most patients get out of bed and begin walking (with assistance) after coming off of the ventilator. You will also be provided pain medicine as needed and restart blood thinners as soon as bleeding appears to be a minimal risk. Your chest will drain fluid for several days after surgery, and we will remove the drains once drainage slows.

Once you have all tubes removed, are walking the halls well and need less oxygen, we will perform several tests to determine your new baseline. We'll assess your ongoing supplemental oxygen needs, postsurgery heart function, changes to the previously-blocked arteries and fluid levels. Most patients are well enough to travel home on discharge, but depending on how far away you live, we may ask you to stay close to the hospital for a brief time for monitoring and follow-up care.

We will provide you and your primary care doctor with follow-up information about your surgery as well as the instructions you need as you heal at home, including precautions, medications and exercise restrictions. Most patients do not need oxygen after surgery, but those who do are often able to wean from it as their lungs improve.

Please remember that this is major surgery and it is normal not to feel fully recovered for several months.

Inova Fairfax Hospital is the only hospital in Virginia, Maryland, and Washington, DC, to offer BPA, an emerging treatment for patients with chronic clots that are inoperable or do not improve after surgery.

The procedure improves blood flow to the lungs, reduces shortness of breath and increases exercise tolerance by opening blood vessels that have been chronically narrowed or blocked.

CTEPH patients typically require four to six BPA procedures, with the first two performed within two weeks of each other and subsequent procedures spaced out between one and three months. Most patients spend one night in the hospital for observations and are discharged the next day.

About the Procedure
BPA procedures take two to four hours. During the procedure, a small tube (catheter) with a balloon at the end is inserted into the blocked blood vessels. The balloon is then inflated, pushing the lungs’ scar tissue aside and opening up the blood vessel to help the blood flow better.

Although surgery is the preferred treatment for CTEPH, patients who are not a candidate for surgery or who have persistent pulmonary hypertension after surgery will need medication, either alone or in combination with BPA. Riociguat is currently the only FDA-approved medication specifically for CTEPH.

All CTEPH patients should continue to take blood thinners (anticoagulants), as prescribed by their doctors, for the rest of their lives to keep new blood clots from forming. Your doctor will talk with you about the best medication to take as well as risks and potential interactions with other medicines.