Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

Chronic thromboembolic pulmonary hypertension (CTEPH) is a form of pulmonary hypertension (PH) that can develop following a pulmonary embolism (PE). CTEPH occurs when the acute PE fails to completely dissolve and the patient is left with chronic, occlusive thrombi which form scars inside the vessel, often leading to pulmonary hypertension, abnormal function of the right side of the heart, and, if untreated, death.

Although it is generally believed that CTEPH originates with acute blood clots in the , legs and lungs (VTE),  interestingly, up to 40% of CTEPH patients will have no clear history suggestive of acute PE and may simply develop gradually worsening shortness of breath.  In fact, the symptoms of CTEPH mimic many more common cardiac and pulmonary diseases and diagnosis is often delayed, with a median time between symptom onset and diagnosis of 2 years

Unlike other forms of pulmonary hypertension, CTEPH is potentially curable by surgery, namely pulmonary thromboendarterectomy (PTE).  In addition, other treatment options include balloon pulmonary angioplasty (BPA) and medication.   As the management of CTEPH differs significantly from that in other forms of pulmonary hypertension, appropriate diagnosis is essential to patient management.  

Properly diagnosing CTEPH requires considering it in patients who have persistent symptoms after an acute PE, as well as patients with unexplained shortness of breath, chest pain, dizziness, or palpitations.  

Several tests may be done to confirm a diagnosis of CTEPH and determine optimal treatment, including:

• Echocardiogram: An ultrasound of the heart looking at the size and function of the heart and estimating the presence and severity of pulmonary hypertension
• V/Q scan: A nuclear scan of the lungs, looking for areas that are not receiving blood flow
• CT scan:  A detailed xray of the lungs, heart and blood vessels
• Heart catheterization and pulmonary angiogram:  A procedure in which a small catheter is placed in the veins of and guided into the pulmonary arteries.  The pressure in the arteries is measured and dye is injected into these arteries to determine where the chronic blood clots are located

The team at UCLA, one of only a few in the country, is extremely experienced at diagnosing and treating CTEPH.  Dr. Channick has evaluated and treated patients with CTEPH, referred to him from around the world, for more than 30 years.