What Are the Symptoms of CTEPH?

The symptoms of CTEPH are frequently nonspecific and similar to many other cardiopulmonary diseases such as asthma, chronic obstructive pulmonary disease, or heart failure. The most common symptoms may include increased shortness of breath with exertion such as climbing stairs and an increased intolerance to exercise. Other symptoms may include:

  • Swelling in your abdomen
  • Swelling in your legs
  • Dizziness or feeling lightheaded
  • Heart pounding (palpitations)
  • Fatigue
  • Tightness in the chest or stomach
  • Coughing up blood, also called hemoptysis.

When To See Your Healthcare Provider

CTEPH can be hard to diagnose due to presenting with symptoms similar to other diseases. It is important to see your healthcare provider when noticing unexplained shortness of breath and fatigue. Alert your healthcare provider if you have a history of blood clots. Prompt treatment for CTEPH is important in order to have the most positive health outcomes.

How CTEPH Is Diagnosed

Your healthcare provider will need to order several tests to confirm that you have CTEPH. In order to be diagnosed with CTEPH, you first need to have a diagnosis of pulmonary hypertension. 

  • Ventilation-perfusion (V/Q) Scan: Measures how well air and blood flow into your lungs. Considered the best screening test for diagnosing CTEPH.
  • Right Heart Catheterization: This test is invasive, so it is not usually performed unless other tests cannot produce a firm diagnosis. It involves inserting a catheter (small tube) into a large vein in either the neck, arm, or groin, and threading it through the right side of the heart and into the pulmonary artery. This allows measurement of the blood pressure in the lungs.
  • Lung function tests (Breathing tests): Checks for diseases like asthma or COPD.
  • Echocardiogram: Ultrasound of the heart to check the size and condition of the chambers of the heart. It can also be used to estimate the blood pressure in the pulmonary arteries.
  • Pulmonary Angiogram: A dynamic X-ray of the blood vessels in your lungs using contrast injection.
  • Computed Tomography (CT or CAT) Scan Pulmonary Angiograph: Diagnostic imaging that shows cross-section images of your lungs using contrast to outline your blood vessels.
  • Magnetic Resonance Imaging (MRI): Non-invasive detailed imaging of your lungs that uses magnets and radio waves.
  • 6-Minute Walk Test: Objectively measures how far you can walk and to see if your oxygen levels drop when you are physically active.

Page last updated: September 25, 2023

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