CTEPH may have a long silent period in which there are no symptoms. As the condition progresses, you may experience:

• Chest pain 
• Fatigue
• Irregular or pounding heartbeat (heart palpitations)
• Lightheadedness
• Shortness of breath, especially with physical activity
• Swelling in the legs (edema)

In many cases, CTEPH develops after a pulmonary embolism—a sudden blockage in a lung artery. A pulmonary embolism usually happens when a blood clot from somewhere else in the body breaks off and travels to the lungs.

CTEPH is not always linked to pulmonary embolisms. About one in four people with CTEPH have no history of a pulmonary embolism.

The exact risk of CTEPH after a pulmonary embolism is not known. Researchers estimate about 3% of patients with pulmonary embolism will develop CTEPH.

Other factors that may increase your risk of CTEPH include:

• Cancer
• Chronic inflammatory diseases, such as inflammatory bowel disease
• Autoimmune conditions that make you more likely to develop blood clots—for example, antiphospholipid antibody syndrome
• Chronic indwelling devices, especially infection from cardiac devices, such as a pacemaker or implantable cardiac defibrillator
• Hypothyroidism
• Spleen removal (splenectomy)
• Prior large pulmonary embolism
• History of recurrent pulmonary embolism

CTEPH is associated with many health conditions; some you can control, and others you cannot. You can reduce your risk by taking steps to keep your lungs and body healthy:

• Eat healthy foods.
• Exercise routinely.
• Maintain a healthy weight.
• Move throughout the day to break up long periods of sitting.
• Quit smoking.

Diagnosing CTEPH can be challenging because:

• It may be difficult to distinguish from other forms of PH.
• Patients may not have a history of pulmonary embolism.
• Symptoms may not show up right away.

Seeking care at a specialized center like ours can help ensure you get the right diagnosis. Our multidisciplinary team provides a comprehensive evaluation that typically includes several tests:

• CT pulmonary angiography: This CT imaging test detects clots in the pulmonary arteries. 
• Transthoracic echocardiogram: An echocardiogram uses sound waves to create a moving picture of your heart. A transthoracic echocardiogram applies the sound waves to the surface of your chest. This test helps us diagnose PH.
• Ventilation perfusion (V/Q) scan: A V/Q scan measures air movement (ventilation) and blood circulation (perfusion) in the lungs. We use this test to screen for CTEPH.