Chronic thromboembolic pulmonary hypertension (CTEPH)

Blood clots can form in the arteries of the lungs (pulmonary embolisms), but the body usually breaks them down on its own or with medication. In some people, the clots don’t go away completely. This raises the pressure in the lung arteries (pulmonary hypertension) and makes the heart work harder. Chronic thromboembolic pulmonary hypertension (CTEPH), a rare and serious condition, happens when the old blood clots don’t dissolve and instead turn into scar tissue that blocks normal blood flow.

CTEPH is usually treated by a team of pulmonary hypertension specialists, who are often lung doctors (pulmonologists) or heart doctors (cardiologists), along with radiologists and other clinicians. Our pulmonary vascular disease providers offer the most effective care available to treat pulmonary hypertension and CTEPH. As part of this care, the Harron Lung Center provides more advanced treatment options than any other lung program in the southeastern Pennsylvania and New Jersey regions.

CTEPH doesn’t always cause noticeable symptoms at first, and they can be mistaken for asthma, COPD, or heart failure. The most common sign is shortness of breath during activities like climbing stairs and exercising.

Other symptoms can include:

• Swelling in the legs (edema)
• Feeling dizzy or fainting
• A fast or pounding heartbeat (heart palpitations)
• Feeling very tired
• Discomfort in your chest
• Fingers and toes with a bluish tint (cyanosis)
• Coughing up blood (hemoptysis)

A history of pulmonary embolisms is the most common cause of CTEPH, but it’s still unclear why only some people develop the condition.

Other factors that may increase the risk of CTEPH include:

• Certain underlying conditions like chronic inflammatory diseases, some cancers, or having had a splenectomy
• Blood clotting disorders like antiphospholipid syndrome that make blood clots more likely
• Infections related to implanted medical devices like pacemakers or shunts
• Thyroid hormone replacement therapy, in certain cases

Because CTEPH is a rare condition with symptoms that mimic other disorders, it’s often misdiagnosed and can progress slowly for months or longer. If left untreated, chronic blood clots in the lungs can lead to serious complications. That’s why it’s important to see a care team that understands this condition.

At Penn Medicine, our team has years of experience diagnosing and managing CTEPH. We’re home to one of the largest pulmonary vascular disease programs in the country and are recognized as a Pulmonary Hypertension Association-accredited Center of Comprehensive Care.