The Chronic Thromboembolic Pulmonary Hypertension (CTEPH) program at Baylor St. Luke’s Medical Center is one of a handful of specialized programs in the country offering expert multidisciplinary care for patients with CTEPH.
What is CTEPH?
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare form of pulmonary hypertension where there is consistently abnormally high pressure in your lung’s small blood vessels.
How does CTEPH develop?
CTEPH develops over time in patients with a history of blood clots (pulmonary embolism).
Why is CTEPH bad?
As blood vessels narrow, the pressure in the lungs begins to rise leading to pulmonary hypertension. With time, the lungs don’t receive adequate blood flow, which can damage the lungs. As the patient receives less oxygen, they become short of breath. This makes it difficult to enjoy everyday activities.
As a result of CTEPH, the right side of the heart, which pumps blood through the lungs, becomes tired and weak from the heavy workload. This can cause irreversible damage that may also impact the liver and kidneys. Some patients may develop kidney and liver failure.
How is CTEPH treated?
Once CTEPH develops, the most effective cure is a surgical procedure known as pulmonary endarterectomy that reestablishes normal blood flow to the lungs.
This surgery can reverse pulmonary hypertension and reverse damage to the heart and other organs. With surgery, patients can expect excellent long-term survival approaching 90% at five years. Without surgery, patients have substantially reduced long-term survival and quality of life.
Are there non-surgical options for CTEPH?
Medications may be an option for patients with CTEPH to prevent further clot buildup and to relax the wall of the blood vessels.
In addition, patients may be candidates for a minimally invasive procedure called pulmonary balloon angioplasty.
How do you know if you have CTEPH?
CTEPH is a vastly underdiagnosed disease. But some of the symptoms include:
- Chest pain
- Shortness of breath
- Pounding in your chest
- Swelling of legs, neck or abdomen
Any patient with a history of blood clot formations should consider seeing a specialist if they are having trouble performing daily activities due to less energy or shortness of breath.
Survivors of acute pulmonary embolism can develop long-term pulmonary hypertension. So any patient with a history of acute pulmonary embolism should undergo an annual echocardiogram to check the function of the heart.
What tests are performed to diagnose and treat CTEPH?
Your physician may perform a few tests to check for CTEPH:
- Right heart catheterization allows the cardiologist to determine the pressures in the lung and heart
- Pulmonary angiography provides direct visualization of the pulmonary arteries by injecting dye through the blood vessels
- Echocardiogram allows an assessment of the function of the heart
- Ventilation-perfusion scan (VQ scan) allows an assessment of the distribution of blood flow and ventilation throughout the lungs
- Chest computed tomography angiogram (CTA) allows a three-dimensional view of the lungs and their blood vessels
- Pulmonary function tests allow the physician to determine the health of the lungs.
- Exercise testing allows an assessment of the body’s response to increased oxygen demand during exercise
In addition, patients will have blood tests drawn and will visit with members of the pulmonary, cardiology and cardiothoracic surgical teams.