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. This is caused by a pro-inflammatory state secondary to unresolved clot that results in pulmonary vessel remodeling. In time this leads to abnormally high pressure in your lungs and the development of right heart failure.
How does CTEPH develop?
CTEPH develops over time in patients with a history of blood clots (pulmonary embolism). The body was not able to resolve the clot, which leads to an inflammatory response and remodeling of the vessels in the lung. This remodeling leads to narrowing of the pulmonary vessels causing a backup of fluid into the right side of the heart.
Why is CTEPH bad?
As blood vessels narrow, the pressure in the lungs begins to rise leading to pulmonary hypertension (high blood pressure of the lungs). As the disease progresses, the lungs no longer receive adequate blood flow, which can lead to impairment in gas exchange – the body taking in oxygen and releasing carbon dioxide. 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. Often they experience weight gain, leg swelling, and bloating, which is indicative of volume overload because of progressive right heart failure.
How is CTEPH treated?
Once CTEPH develops, the most effectiveintervention is a surgical procedure known as pulmonary endarterectomy that reestablishes normal blood flow to the lungs. This procedure is potentially curative.
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 balloon pulmonary angioplasty (BPA). BPA has comparable outcomes to PTE surgery, and it requires several attempts to achieve desired improvement in right heart functioning. It is typically selected for patients who are not surgical candidates or for patients who prefer non-surgical treatment modalities.
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
- Fainting
- Fatigue
- Pounding in your chest
- Dizziness
- Swelling of legs, back or abdomen
- Distended neck veins
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 CTEPH. So any patient with a history of acute pulmonary embolism should have follow up to ensure healthy 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 and cardiac MRI allows for detailed assessment of cardiac function
- Ventilation-perfusion scan (VQ scan) or SPECT CT imaging which assesses 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.