CTEPH: Chronic Thromboembolic Pulmonary Hypertension Treatment
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Chronic thromboembolic pulmonary hypertension (CTEPH) turns your life upside down. You may have been a healthy, active person just months or years ago. But now, you have heart failure. Even small tasks might wear you out and make you short of breath. Doctors may not have known exactly what’s wrong and you’ve been looking for answers for a long time.
CTEPH Treatment at UVA Health
At UVA Health, we can help you get your life back. We’re one of the few centers in the U.S. with the knowledge and experience needed to help patients with CTEPH. In fact, our heart failure services received a score of "high performing" from U.S. News & World Report. We've also been named a certified Center of Comprehensive Care for Pulmonary Hypertension by the Pulmonary Hypertension Association (PHA).
CTEPH makes it harder for your heart to pump blood. It's the only type of pulmonary hypertension that can be fixed with surgery. The surgery, called pulmonary thromboendarterectomy, takes a skilled team of many different experts.
We start by opening your chest (median sternotomy). We’ll use a heart-lung machine to keep oxygen flowing to your body (cardiopulmonary bypass). Then we’ll open your lung arteries to remove blockages (clots and scars) causing CTEPH.
This lowers blood pressure in your heart, so there’s less stress on it.
Unblocking Lung Arteries
CTEPH makes it harder for the heart to pump blood and leads to symptoms of heart failure. Christopher Scott, MD, explains its causes and treatments.
Chronic thromboembolic pulmonary hypertension is a very unique disease process. Essentially, it is the formation of clots and inflammatory blockages in the pulmonary arteries. This occurs typically months to years of time. It puts an immense strain on the heart to be able to pump against, essentially, a restriction. This leads to the development of heart failure and heart failure-like symptoms, and ultimately, left untreated, it's a fatal condition.
The surgery that is recommended to treat this disease process is a surgery called pulmonary thromboendarterectomy. This is a complex surgery that is performed through a median sternotomy, meaning we split the breast bone down the middle. We then go on cardiopulmonary bypass, or the heart lung machine. We are then able to open both of the pulmonary arteries and remove all of the clot and inflammatory material within the pulmonary arteries that's causing the blockage and remove it completely on both sides, relieving the obstruction.
What this operation achieves is the heart is then not beating against such a high pressure and there's less stress on the heart. With time, the heart will recover and often function will go back to normal. They can go on living normal lives and it's quite a game-changing procedure for them.
The medical treatment option is, essentially, a palliative option in the sense that it provides relief to patients. It is certainly not curative of the disease process, nor is it a good, long-term treatment option.
The treatment of chronic thromboembolic pulmonary hypertension is a unique disease process that requires a really skilled, multidisciplinary team. It's a very unique program to be able to offer here, and something that we're excited to grow upon, to build upon, to attract more patients so that they can be appropriately diagnosed and treated and don't have to suffer with this for the rest of their life.
Getting the Right CTEPH Diagnosis
Diagnosing CTEPH is difficult. It takes a specialized team. We’ll perform these tests:
- Ventilation-perfusion scan (VQ scan) to measure airflow and blood flow in the lungs
- Echocardiogram or ultrasound to see how well the heart is pumping
- CT with contrast to see blockages in the lungs
- Pulmonary angiogram to look at blood vessels in lungs
- Heart catheterization to measure pulmonary system pressure and function of the heart
After Surgery & After CTEPH
You’ll spend 7-10 days in the hospital, starting in the intensive care unit (ICU). You'll move to another unit as you’re doing better.
For many patients, their heart function goes back to normal. They’re able to have the same quality of life they had before CTEPH.
You’ll need to keep seeing a lung or heart specialist to watch for signs of heart failure. You’ll also need to take medicine to keep clots from forming in your lungs again.
When CTEPH Surgery Isn’t an Option
Sometimes patients are too weak for surgery. In these cases, we offer medicines that reduce symptoms and help you feel as comfortable as possible.
What Is CTEPH?
CTEPH happens when blood clots stick to the walls of blood vessels in your lungs (pulmonary embolism). They block blood from flowing by making the blood vessels smaller. This makes blood pressure in your lungs go up. Your heart has a harder time pumping blood through your lungs and getting oxygen out to your body.
Chronic thromboembolic pulmonary hypertension symptoms include:
- Chest pain or tightness
- Bluish color in fingers and toes
- Dizziness
- Fainting
- Fatigue
- Pounding in your chest
- Trouble breathing