What Is CTEPH?
CTEPH — or chronic thromboembolic pulmonary hypertension — is a rare, life-threatening medical condition typically caused by old blood clots in the lungs (pulmonary emboli).
Most people who have suffered a blood clot, can be treated with blood thinners to restore blood flow to the lungs, which helps improve their breathing and exercise tolerance. However in others, blood clots might not dissolve and can lead to progressive right-sided heart failure and/or development of pulmonary hypertension (increased blood pressure in the lung).
The presence of pulmonary hypertension in the setting of old blood clots is referred as CTEPH. CTEPH is considered the only form of pulmonary hypertension that is potentially curable.
CTEPH causes and risk factors
A blood clot in the lungs is known as a pulmonary embolism (PE), which can become lodged there andulate other clots. PE can block blood flow into the lungs and can be life threatening. These blood clots usually form in the legs that break loose and then travel to the lung.
Lifestyle and health issues that can increase the risk of getting blood clots include:
- Inactivity for long periods of time (such as after surgery or hospitalization, or a long flight or car trip)
If a PE goes untreated, or if it does not get better with blood thinning therapy, the clot can become chronic and scar-like over time. When this happens, blood backs up in the lungs and causes increased pressures, and a rare but often treatable type of severe pulmonary hypertension known as CTEPH can develop.
It's also possible for CTEPH to develop from multiple small clots over a long period of time, as opposed to one or two large blood clots.
People without evidence of prior pulmonary clot can still develop CTEPH.
CTEPH risk factors include:
- Ventricular shunts (heart shunts)
- Indwelling catheters
- Spleen removal
- Thyroid replacement therapy
- Inflammatory bowel disease
- A history of cancer
CTEPH is more common in women and in those with a family history of blood clots.
Refer a patient to UPMC's CTEPH Program
Call 1-877-PH4-UPMC (1-877-744-8762), toll free or email us at PHprogram@upmc.edu.