Chronic Thromboembolic Pulmonary Hypertension, or CTEPH, is a rare, life-threatening medical condition typically caused by old blood clots in the lungs (pulmonary emboli).
Most patients 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 other patients 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.
A blood clot in the lungs is known as a pulmonary embolism (PE), which can become lodged there and accumulate 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. Smoking, inactivity for long periods of time (such as following surgery or hospitalization, long flight or car trip), obesity and cancer can increase the risk of developing blood clots. 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 chronic thromboembolic pulmonary hypertension (CTEPH) can develop. It is 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.
Patients without evidence of prior pulmonary clot can still develop CTEPH. These risk factors for development of CTEPH include ventricular shunts (heart shunts), indwelling catheters, spleen removal, thyroid replacement therapy, inflammatory bowel disease and a history of malignancy (cancer).
CTEPH is more common in women and in those with a family history of blood clots.
The symptoms of CTEPH are non-specific, and similar to other types of pulmonary hypertension. Symptoms can often be similar to those of other cardiopulmonary conditions like pneumonia, heart failure or chronic obstructive pulmonary disease (COPD), so it often goes undiagnosed or is detected late in the advancement of the disease.
The most common symptoms of CTEPH include:
Less frequent symptoms include:
As the disease progresses, symptoms of light-headedness or exercise-related dizziness may occur. Abdominal (stomach) and lower extremity swelling may develop as your lung pressures increase, and the right ventricle, which pumps blood into the lungs, begins to weaken.
Pulmonary emboli are quite common with approximately 600,000 diagnosed each year. Among people with PE, CTEPH affects approximately 2-4% of those individuals. About 5,000 Americans are diagnosed with CTEPH each year.
Additionally, almost one third of people diagnosed with CTEPH have no known history of PE.
To determine the best route for treatment, a UPMC specialist will:
Testing is essential to the diagnosis and evaluation of CTEPH. Common tests include:
UPMC's Comprehensive Pulmonary Hypertension Program is one of only a few programs nationwide specializing in treatment of CTEPH. Because this disease is so rare and has similar symptoms to other cardiopulmonary conditions, diagnosing and treating this disease requires the kind of multi-disciplinary expertise that is the hallmark of our physicians.
This disease is potentially curable, and UPMC's experts are well-skilled in providing treatment options that are best suited for each person, even at advanced stages of disease. When properly treated, the survival rates of people with CTEPH are comparable to those without any history of pulmonary hypertension.
Treatment options include:
Belinda Rivera-Lebron, MD, MSAssistant Professor of Medicine Division of Pulmonary, Allergy & Critical Care Medicineriveralebronbn@upmc.edu
Other members of the multidisciplinary team:
Call 1-877-PH4-UPMC (1-877-744-8762), toll free or email us at PHprogram@upmc.edu.
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