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 highly-skilled surgical procedure, UPMC surgeons open the pulmonary arteries and remove the blood clot and, restore proper blood flow to the lungs. PTE is a cardiothoracic surgical procedure, which lasts 4-6 hours, with the goal of improving blood flow from the right side of the heart through the pulmonary arteries, relieving stress on the right side of the heart (preventing heart failure), allowing you to breathe easier and improve your quality of life. The operation is done utilizing cardiopulmonary bypass. Patient’s body temperature is cooled and cessation (stoppage) of blood flow is sustained for a short period of time to allow for complete visualization and removal of the chronic pulmonary thromboemboli.
Depending on how advanced the disease is, this surgery can cure up to 90 percent of all patients.
There are a lot of factors which go into the decision of proper candidacy for PTE surgery. Our multidisciplinary team will determine if you will benefit from PTE surgery and if the benefits will outweigh the associated risks. Much has been learned over the past decade and has improved the safety of the operation. This surgery is generally considered a safe surgery with an experienced team. The success of the surgery is the combination of proper patient selection as well as surgical expertise and technique.
In the 10 percent of individuals who do not achieve a cure from PTE and in those deemed not to be optimal surgical candidates, medications can be used to provide treatment for pulmonary hypertension.
When surgery or medications fail to cure CTEPH, some people may become candidates for a lung transplant.
Our doctors are leading experts in lung transplantation as part of the UPMC Thomas E. Starzl Transplantation Institute, a nationally regarded center of excellence in transplantation.
Belinda Rivera-Lebron, MD, MS
Assistant Professor of Medicine Division of Pulmonary, Allergy & Critical Care Medicineriveralebronbn@upmc.edu
Other members of the multidisciplinary team:
Affiliated with the University of Pittsburgh Schools of the Health Sciences
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