Chronic Thromboembolic Pulmonary Hypertension

What is CTEPH?

Chronic thromboembolic pulmonary hypertension (CTEPH) is a potentially life-threatening condition where the heart’s pulmonary artery is blocked by acute or recurrent blood clots. This can lead to progressive pulmonary hypertension and right heart failure. CTEPH is considered one of the leading causes of severe pulmonary hypertension. Several aspects of CTEPH’s origins are not fully known, which complicates its treatment. Recent studies suggest that damage to the central pulmonary arteries due to organized blood clots may not be the only reason for progressive pulmonary hypertension and right heart failure.

What causes CTEPH?

CTEPH used to be difficult to diagnose but advances in diagnostics and pulmonary surgical techniques have made this disease treatable and even potentially curable. The prevalence of CTEPH is still unknown. CTEPH is rare in patients surviving an acute pulmonary embolism—recent reports suggest one to four percent. However, the disease is sometimes misdiagnosed because of nonspecific symptoms and variations in disease development.

The true number of patients with of CTEPH is limited since two-thirds of these patients have no known history of pulmonary embolism. CTEPH is often identified in patients with unexplained pulmonary hypertension and no previous history of venous thrombosis or pulmonary embolisms. The European Society of Cardiology in association with the European Respiratory Society published guidelines for the diagnostic and therapeutic approach to patients with CTEPH, which were endorsed by the International Society of Heart and Lung Transplant.

How is CTEPH diagnosed?

Patients with unexplained pulmonary hypertension should be evaluated for CTEPH. This is particularly true when for patients have a previous history of venous thromboembolism.

Techniques to diagnose CTEPH is include:

Echocardiography
Chest radiograph
Ventilation/perfusion (V/Q) lung scan
Computed tomographic angiography (CTA)
Right heart catheterization
Pulmonary angiography

How is CTEPH treated?

In an effort to prevent further blood clots, lifelong anticoagulation is one of the most important therapies in the treatment of CTEPH. Expert medical and surgical teams can also determine if surgery is an option. Pulmonary thromboendarterectomy, or PTE, is the surgical removal of all thromboembolic material and obstructive lesions within the pulmonary arteries. PTE is a well-established procedure pioneered and developed at the University of San Diego, California (UCSD) for the treatment of CTEPH. Experienced centers and teams, including the Virginia Mason team, now report highly successful PTE outcomes, with one-year survival rates of greater than 95 percent.

PTE has become the gold standard for treatment of CTEPH, resulting in a significant reduction of pulmonary artery pressure and improvement in right ventricular function, quality of life and survival.

Unfortunately, some patients are not candidates for PTE because the obstructions in the lungs are in small blood vessels that are too difficulty to reach. As an alternative, medications, like the drug Adempas (riociquat), can be used to lower the pressures in the lungs. In addition, percutaneous balloon pulmonary angioplasty can be performed to open up the small vessels in the lungs with a catheter.

Why choose Virginia Mason Franciscan Health for your CTEPH care?

Our surgical and medical team has brought nearly a decade of experience in the multidisciplinary care of chronic thromboembolic pulmonary hypertension (CTEPH) and pulmonary thromboendarterectomy (PTE). We provide an experienced multidisciplinary approach with our pulmonary, cardiology, radiology, anesthesiology, critical care and cardiac surgical specialists to ensure the highest quality patient centered approach. Read about the experiences of Robert Moraca, MD, as a cardiac surgeon who specializes in PTE.

For more information about CTEPH or to make an appointment, please call 206-223-6198.