Treating CTEPH: Surgery, Medication or Balloon Angioplasty? (Podcast)

For patients with chronic thromboembolic pulmonary hypertension (CTEPH), surgery —pulmonary endarterectomy — is almost always the preferred treatment option because of the associated positive short- and long-term benefits. However, not all patients qualify for surgery.

“When I'm teaching my fellows about [CTEPH], I always tell them that this is really where we earn our paychecks in the CTEPH realm with making this decision, because it's a very complex and big one in terms of who's a good candidate and who may not benefit potentially,” says Alice Goyanes ,MD, a pulmonary hypertension specialist at Cleveland Clinic, on a recent episode of the Respiratory Exchange podcast.

For patients who are not candidates for surgery, Dr. Goyanes explains that there are typically two other treatment options. Most of the time, patients are first started on vasodilator therapy, which is titrated to the highest tolerated dose. In the United States, the only FDA-approved medication for CTEPH is Riociguat.

Once they’re started on the medication, the patient is then referred for balloon angioplasty, if it makes sense.

“It's not appropriate for very large blood vessels, and it's not appropriate for complete occlusions of the blood vessels because of the way the procedure physically works,” says Dr. Goyanes.

Over the past half-decade, more studies and data about outcomes for balloon angioplasty have come out.

“[Previous studies have shown patients] get an initial drop and then have ongoing stability of improved mean pulmonary pressure afterward,” says Dr. Goyandes. “So, it's a really nice thing to be able to offer patients who may not be able to take on a huge open-heart surgery.”

“ With the availability of FDA-approved medication in the form of Riociguat, I think things improved significantly for a lot of people, and now, and more recently in the last probably 10 years or so, the availability of balloon pulmonary angioplasty has really lifted expectations dramatically and outcomes,” says Gustavo Heresi, MD, the Director of the Pulmonary Vascular and Chronic Thromboembolic Pulmonary Hypertension Program at Cleveland Clinic and the host of this Respiratory Exchange episode.

In addition to covering the various treatment options for CTEPH, the episode also covers:

• How CTEPH is diagnosed
• Diagnostic evaluation and the significance of accurate diagnoses
• Imaging modalities and their usage
• Understanding how and when to combine treatments
• Benefits of a multidisciplinary approach to care

Click the podcast player above to listen to the episode now, or read on for a short, edited excerpt. Check out more Respiratory Exchange episodes at clevelandclinic.org/podcasts/respiratory-exchange or wherever you get your podcasts.

Excerpt from the podcast

Gustavo Heresi, MD: So, we have this conversation, and the team concludes that surgery's not a good option. So, tell us a little bit about what treatment options are out there for patients who are not good surgical candidates.

Alice Goyanes, MD: Absolutely. So, under those circumstances, typically patients receive two other kinds of treatment options if they're not a surgical candidate. Most of the time, we start with getting the patient on vasodilator therapy.

So, in the United States the only FDA-approved medication that we have for CTEPH is Riociguat, which is a soluble guanylate cyclase stimulator that helps promote nitric oxide availability in the pulmonary vasculature and promote vasodilation. In the clinical trial that supported this medication getting started, patients had improvement in pulmonary vascular resistance (PVR) as well as improvement in exercise capacity. And it received FDA approval just under a decade ago for this particular disease.

So, our practice generally is, once we know somebody's not a surgical candidate, if their PVR is above three Wood units, which used to be the definition of precapillary pulmonary hypertension, until the most recent guidelines came out a couple of years ago, which moved the PVR definition a little bit lower. All of the clinical trials were done for patients with a PVR greater than three. So, for that patient population, we'll get them started on Riociguat and titrate up to the highest tolerated dose.

Most of the time, we don't necessarily have to add any other vasodilator therapies before we then refer the patient for this treatment regimen called balloon angioplasty.

Under select circumstances, we will sometimes consider other vasodilators which have been demonstrated to help improve hemodynamics, but when they've done large clinical trials, it's been harder to see the improvements of exercise in the past few years and the quality of life. So, regardless, typically, Riociguat is sort of the fundamental vasodilator that we work to get all our patients on because it has the highest quality evidence of benefit.

Once they're titrated and tolerating Riociguat, then we refer them for something called balloon angioplasty. Balloon angioplasty is an interventional radiology procedure that can target some of the medium- and smaller-sized blood vessels that have clots in them.

It's not appropriate for very large blood vessels, and it's not appropriate for complete occlusions of the blood vessels because of the way the procedure physically works. The interventional radiologist basically performs a right heart catheterization, then goes into the pulmonary vasculature, threads a wire into the blood vessel where there's chronic thromboembolic disease and then inflates a small balloon over that wire to push the chronic thromboembolic disease to the sides of the blood vessel. If you can picture what tree roots going through a water pipe look like, that's sort of the best type of morphology to be treated with balloon angioplasty. So, again, it’s generally pushing the blood clots to the sides of the blood vessel, and then that improves distal flow from where the obstruction was previously.

When patients undergo balloon angioplasty, we can typically treat a couple of segments of the lung at a time. You can't do the entire lung at once because there's an amount of intravenous contrast that gets used, and then also exposure to fluoroscopy, because they use that for isolating the blood vessel that they're going to focus on. And so, the patient comes back for repeated treatment sessions that are typically spaced out over the course of, you know, months to a year.

So, when a patient's undergoing balloon angioplasty, I always counsel them that, you know, "This is going to be a little bit of a longer-term relationship, compared to surgery, which is you get everything fixed at once, and then you have a much bigger recovery at that period of time."

The outcomes for balloon angioplasty, we've been getting more data about this over the course of the past five or six years, as people have been publishing some of their experiences. The most robust data and registries for this procedure and condition are from Okinawa, Japan. Their five-year outcome data suggest that the sustained improvement in pulmonary pressures that you get with balloon angioplasty is sustained. So, they get an initial drop, and then they report that they have ongoing stability of the improvement in the mean pulmonary pressure afterward. So, it's a really nice thing to be able to offer patients who may not be able to take on a huge open-heart surgery.