Optimal Management of CTEPH

Optimal treatment strategies for patients with chronic thromboembolic pulmonary hypertension.


Charles D. Burger, MD: In patients who have a confirmed diagnosis of Group 4 CTEPH [chronic thromboembolic pulmonary hypertension], which generally involves imaging studies such as a VQ [ventilation—perfusion] scan and right heart catheterization to document the pulmonary hypertension, a review of whether the patient is eligible for curative surgery with pulmonary thromboendarterectomy is a paramount, first-order priority. We decide that in a multidisciplinary conference involving cardiology, pulmonology, anesthesiology, critical care, chest radiology, and cardiothoracic surgery to review all the tests the patient had. If they’re eligible for surgery and they undergo a pulmonary thromboendarterectomy, 85% of the time it’s curative. About 10% of the time, pulmonary hypertension may be persistent but is much improved. There can be some significant complications in 5% or less of the cases. But it’s very important to determine whether a patient might be eligible for that surgery and offer the surgery if they are eligible.

If they are not eligible for that surgery, either because of the anatomy of the blockage or if they have multiple other illnesses that wouldn’t make them a good candidate for major surgery, then consideration for treatment with riociguat, which is the only FDA-approved medication for inoperable CTEPH, is the next order of business. That medication is generally started at a low dose and titrated upward to its maximal dose over several weeks, and then a reassessment of the patient as to how well they’re doing is performed.

They almost always—I can’t really think of an exception, quite frankly—require lifelong anticoagulation. The only exception would be if they just cannot tolerate it because they have bleeding episodes on the anticoagulation. But we’ve generally been able to manage even those patients in different ways, with different anticoagulants or different methods of protecting the patient. Lastly, because they have some stress on the heart, they may need medications to help manage fluid retention. Those are usually diuretics, for example, and then oxygen therapy if that’s an issue.

Hilary M. DuBrock, MD: The OPUS [OPsumit Users] registry was a long-term, prospective, multicenter, United States—based registry of patients who were newly started on macitentan. It’s a real-world registry describing real-world use of macitentan in the United States. They recently published their outcomes of patients treated with macitentan for chronic thromboembolic pulmonary hypertension. What they found is about 28% of patients were treated with macitentan as monotherapy in chronic thromboembolic pulmonary hypertension, and the majority—about 70% of patients—were treated with macitentan in dual combination therapy, most commonly in addition to riociguat. This provided some real-world data regarding macitentan and described it as being relatively well tolerated in patients with chronic thromboembolic pulmonary hypertension and in patients who were treated with riociguat in addition to macitentan for CTEPH.

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