Jenny Yang, MD - phaware® interview 574

From “Untreatable” to Life-Changing: The New Era of CTEPH Care

What happens when patients are told everything looks “normal” but they still can’t breathe? Dr. Jenny Yang explains how new testing and team-based care are solving one of pulmonary medicine’s most overlooked mysteries and changing what’s possible for CTEPH patients.

This Special Edition episode is sponsored by Liquidia.

My name is Dr. Jenny Yang. I'm an Associate Professor of Medicine at the University of California, San Diego. I did all my training at UCSD. I did my residency and fellowship and graduated in 2021 and then joined the faculty at UCSD with a focus on pulmonary vascular disease. So, I do both PAH, CTEPH, and acute PE. I was lucky to have a lot of really great mentors who introduced me to people within CTS, ATS, CHEST. I was able to find these opportunities to do these speaking engagements and give presentations. So through those, I was able to meet other people and sort of create this network. It's been really nice to be more involved in these organizations and get to meet other people and sort of bring the collaborations and things I learned here back to UCSD, also.

Sometimes, I'll attend a fair bit of the sessions and do some of my own learning. I think there's a lot of new research and sort of case series, case reports of interesting findings. I'll go to learn more myself and see if that applies to some of our patients. Then, some of them are sort of myself giving presentations and sort of passing on what I know to others and hopefully doing the same. At the recent CHEST Conference in Chicago, I was part of a panel talking about chronic thromboembolic disease. My focus was also speaking about the diagnosis and the utility of exercise testing after an acute PE.

So after an acute PE, there's sort of this wide spectrum of outcomes that can occur and we all sort of have gained a better understanding of CTEPH, but there's also patients who can have these chronic PEs or chronic thromboembolic disease without pulmonary hypertension. Their resting tests might look normal. Their echoes and right heart catheterization might all be normal, but they are still quite symptomatic.

At UCSD, we've started doing more invasive cardiopulmonary exercise tests. What that is we end up combining a right heart catheterization with a cardiopulmonary exercise test. These cardiopulmonary exercise tests, it's tests that you might see some like athletes do to sort of really see how great their endurance is. But for our test, these patients will have nose clips and sort of a mouthpiece on, and that's really to measure their gas exchange during exercise. They'll have all sort of little stickers on their chest to measure heart rate and things like that. But we also add in the right heart catheterization part of it. We'll do these exercise tests in the cath lab, where they'll get a regular right heart catheterization. So they'll have the catheter in the neck and then we actually get them up off the table onto an upright bike in the cath lab and have them exercise with the Swan-Ganz catheter in place so that we get a mix of their hemodynamics while they're exercising and also all of their gas exchange parameters while they're exercising also. We get a wealth of information on sort of what's limiting them with exercise.

Normally, with our regular right heart catheterization when we're sort of diagnosing PH, we sort of put it in, get the numbers and then immediately take it out. But for these, we'll leave it in for the duration of exercise. The treatment strategy for CTEPH has really evolved into this multi-modality approach, where for many years surgery was the only answer. If patients weren't surgical candidates, there was really nothing else that could be offered. Over the last 10, 20 years, there's been a lot of advancements in both medical therapy and also balloon pulmonary angioplasty. There's a lot more options for patients now. Having this team of providers who are experts in surgery, the PTE surgery, balloon pulmonary angioplasty, pulmonary hypertension in general, and finding ways that each individualized patient can be approached is sort of the key takeaway.

Beyond the physicians on the team, the really important aspect are also our ancillary staff, our nursing, our respiratory therapists, everyone involved in the team, our pharmacists. They play a huge role in taking care of these patients. Our nurses do a lot of hard work to get these medications approved for our patients, contacting them weekly to make sure that they're titrating these medications up appropriately if they're having side effects, getting through all the insurance approval processes of obtaining these medications. Then, on the inpatient side, after a PTE surgery, for example, having the respiratory therapist and the nursing staff who know how to take care of these patients are also very important in the success of how these patients do.

I think it's really unique in CTEPH that there's a lot online that you can see. A lot of time patients will come to us with sort of preexisting ideas of what they want or don't want for this disease state. We only know who comes to us. I would guess that there are a lot of people who never make it to us, whether that's because they physically can't make it because of financial reasons or sort of other social barriers or because they're just not interested in the idea of a surgery or some sort of intervention. So the referring provider then never sends them because they're just not interested. We sometimes do get patients who are willing to come and meet with us who come with no interest in getting surgery and they sort of say that very upfront during their clinic visit. They don't want surgery even though they clearly have operable disease. Depending on the patient, we will lay out all the risks and say the benefits of surgery. Sometimes, patients will realize that maybe they do want surgery. I think it's always worthwhile if they can. We can at least meet them and discuss it with them. If they still choose no surgery, then at least we've been able to discuss all the risks and benefits with them.

One of the big concerns that some of our patients have is with age, patients who are sort of in their 70s, even early 80s, who we recommend to undergo this big open heart surgery, they have a lot of concerns about their quality of life afterwards and that they're doing sort of okay right now and is it really worth this big surgery? We've had some patients in their late 70s, early 80s that after meeting with us, discussing everything, they decide to undergo surgery. It's been life changing for them. We sometimes hear back from them. They'll send us pictures that they're now able to sort of pick up their grandkids, can go out and actually play with them and spend a whole day out with them as opposed to before they were maybe using the wheelchair more or not wanting to go out just because it was so exhausting to spend a day out.

We've done these PT surgeries on a wide range of patients. Average age is probably somewhere in like 40s to 50s or so, but we've done as young as children, so like nine, 10 year olds, and then as old as in their mid to even slightly later 80s. There's no strict contraindication to age. It's sort of we take the whole thing all together and discuss as a group whether or not we think this patient would benefit from surgery. When they're sort of younger than 14 years old, we usually don't actually take care of them at the adult hospital. They go to the children's hospital. We don't take care of those ones as much, but the 14, 15, 16 year olds, they can get their surgery at the UCSD campus and the discussion, everything is obviously with the parents. Their journey to getting to us is oftentimes a little bit different because you don't necessarily always think about CTEPH right away in a 14-year-old. Most of them get asthma diagnoses and sort of have a little bit of a longer journey to getting to UCSD.

Our surgeon actually will go to the children's hospital and operate with the pediatric cardiac surgeon. Our surgeon's the one who's mainly doing the endarterectomy set portion of it, but then the care of these patients afterwards is at our children's hospital. I think a lot of it is the equipment that we have at our hospital just is not feasible in an eight-year-old. But overall, this is very uncommon to have this in pediatrics this young.

Every three years, the International CTEPH Association does a big CTEPH conference. It's in Warsaw, Poland this year, but it'll be in June. That's a pretty big conference that the International CTEPH Association sets up. It's a couple of days focused purely on CTEPH and sort of any updates and changes over the last three years since the last conference.

Hopefully, at this conference, there'll be a lot of sort of new and exciting things that we'll talk about in CTEPH. I think there's still a lot to be learned. We've come a long ways in terms of how we manage these patients and our approach to these patients and gaining further understanding and awareness. But I think there’s still a lot of work to be done in the field. Hopefully, at this conference, we'll be able to learn a lot more about these CTEPH patients and how best to care for them.

Thanks for listening. My name is Dr. Jenny Yang, and I'm aware that my patients are rare.

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