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.
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:
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:
CTEPH is more common in women and in those with a family history of blood clots.
Call 1-877-PH4-UPMC (1-877-744-8762), toll free or email us at PHprogram@upmc.edu.
CTEPH symptoms are non-specific, and similar to other types of pulmonary hypertension and cardiopulmonary conditions like:
It often goes undiagnosed or is detected late in the advancement of the disease.
The most common symptoms of CTEPH include:
Less common symptoms include:
As the disease progresses, symptoms of light-headedness or exercise-related dizziness may occur.
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 (PE) are quite common with approximately 600,000 diagnosed each year. Among people with PE, CTEPH affects approximately 2 to 4 percent 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:
To make a referral to UPMC's CTEPH program, call 1-877-PH4-UPMC (1-877-744-8762), toll free or email us at PHprogram@upmc.edu.
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.
Also called Pulmonary endarterectomy (PEA). In this complex surgical procedure performed with cardiopulmonary bypass, the patient’s body temperature is cooled and cessation of blood flow is sustained for a short period of time to allow for complete visualization and removal of the chronic pulmonary emboli. The goal is to restore normal blood flow to the lungs and prevent heart failure and death. This surgery can potentially cure CTEPH, leaving the patient without emboli and without the need for pulmonary vasodilator therapy. The success of the surgery depends on the combination of proper patient selection as well as surgical expertise and technique. PTE is considered safe with an experienced team.
For patients with inoperable CTEPH or those with residual pulmonary hypertension after surgery, this minimallyinvasive procedure might be an option. UPMC is one of very few centers in the country offering this treatment. The technique for BPA is similar to a right-heart catheterization, where a catheter is introduced into the pulmonary artery with a balloon at its tip. The balloon is inflated in the area where the chronic blood clots are located, pushing the clots against the wall of the artery and restoring blood flow. BPA can complement surgery and/or medical therapy to improve the quality of life of CTEPH patients.
For patients who do not achieve a cure from PTE or are not surgical candidates, medications are a treatment option.
Some patients may be candidates for a lung transplant. Our physicians are leading experts in lung transplantation as part of the UPMC Thomas E. Starzl Transplantation Institute, a nationally recognized center of excellence in transplantation.
To make a referral to UPMC's CTEPH program, call 1-877-PH4-UPMC (1-877-744-8762), toll free or email us at PHprogram@upmc.edu.
Belinda Rivera-Lebron, MD, MS
Assistant Professor of Medicine
Division of Pulmonary, Allergy & Critical Care Medicine
riveralebronbn@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.