What Are the Symptoms of CTEPH?
CTEPH's early symptoms can be easily confused with many other diseases. Many people with CTEPH do not have any symptoms for a long time.
Early Stage Symptoms
In the early stages of CTEPH, symptoms can easily be confused for other common problems. Symptoms include:
- fatigue,
- chest pain, and
- shortness of breath (especially during exercise).
These common symptoms make it hard for both patients and doctors to suspect CTEPH.
Late Stage Symptoms
If a patient has CTEPH for many years, symptoms are more severe and life-threatening. Symptoms can include signs of heart failure, including:
- fainting
- edema (when your body swells and retains fluids), and
- a blue color in the fingers and toes.
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Treatment for CTEPH
Surgery is the best treatment option for CTEPH. Patients who have surgery have better health outcomes than patients who do not. Over 89 percent of patients who have surgery are still alive three years after being diagnosed.
For patients who do not have surgery, just over 70 percent are alive three years after being diagnosed. Patients with CTEPH have the best chance of becoming healthy again if they have surgery.
Our cardiology specialists will perform many tests, including imaging and evaluation, to determine if you are a good candidate for surgery.
If you are not a strong candidate for surgery, our doctors will discuss non-surgical medical treatments including drugs and lab procedures.
Treating CTEPH With Surgery
To treat CTEPH, cardiovascular surgeons perform a type of surgery called pulmonary endarterectomy or PEA. The goals of PEA surgery are to:
- decrease heart failure symptoms like edema,
- decrease shortness of breath, and
- increase survival rates.
PEA surgery decreases pulmonary vascular resistance. Decreasing this resistance helps push and circulate blood through your lungs. Surgery also improves ventilation and perfusion matching, which help regulate the amount of air and blood delivered to your lungs.
PEA is a complex, demanding surgery. Surgeons who perform PEA must have specialist training and sophisticated care management skills.
Even though CTEPH surgery is complex, it is very successful. The survival rate for PEA is over 95 percent.
What Happens During PEA Surgery?
During a PEA procedure, surgeons clear all thromboembolic material that is blocking blood flow in your pulmonary arteries. Surgeons open your chest through your sternum. The surgery lasts eight to 10 hours.
Surgeons first make small incisions in the left and right pulmonary arteries one at a time. Surgeons then carefully remove the old blood clots that are compressed (or squeezed) against the blood vessel wall. These blood clots often have more fibrous connective tissue than normal. Surgeons carefully dissect these old blood clots.
Cooling Your Body
The blood vessels in your lungs deliver blood to your brain. Your surgical team must make sure they can safely operate on these important blood vessels. To do this, surgeons use a technique called deep hypothermic circulatory arrest so they can safely operate on the large blood vessels inside the lungs that deliver blood to your brain.
During hypothermic circulatory arrest, your body will be cooled to 20 degrees Celsius. The surgical team will place you on a heart-lung bypass machine to protect your body's organs and support your brain. Your heart will also stop temporarily, but your surgical team will carefully and safely monitor and control your heart's activity.
After Surgery
After surgery, patients have a risk of developing the same complications as traditional heart surgery. Patients also have additional risks like neurological problems and reperfusion lung injury. This injury causes tissue damage in your lungs when blood returns to your lungs after they have not received oxygen for a while.
You will need to stay in the intensive care unit so hospital staff can monitor for signs of these unique complications.
How Many People Have PEA Surgery?
Because CTEPH is underdiagnosed, medical centers in the US perform fewer PEA surgeries than medical centers in Europe. Cardiologists perform almost twice as many PEA procedures in Europe each year.
The US has few comprehensive CTEPH treatment centers. Many patients with CTEPH do not receive the referrals they need.
Treating CTEPH Without Surgery
Surgery is the most successful way to treat CTEPH disease. But patients have a few different options for treating CTEPH without surgery.
Riociguat is an oral drug that makes it easier for you to exercise, improving your overall quality of life. Riociguat is best for patients who cannot have PEA surgery, or for patients who have reoccurring pulmonary hypertension after PEA surgery.
Balloon Pulmonary Angioplasty
Balloon pulmonary angioplasty is a non-surgical alternative to PEA surgery. Patients have this procedure in outpatient cardiology labs. Cardiologists use small balloons to dilate (or open) blocked blood vessels.
Patients will need many sessions to open blocked blood vessels. Most patients need an average of five sessions.
Balloon pulmonary angioplasty may be best for frail and elderly patients with CTEPH.
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