Candice Lee, MD: Challenges in CTEPH Diagnosis and Treatment

Candice Lee, MD

In an interview with HCPLive, Candice Lee, MD, McGinnis Thoracic & Cardiovascular Associates, Allegheny General Hospital, discusses the challenges in chronic thromboembolic pulmonary hypertension (CTEPH) care and the treatment options for patients who are diagnosed with the rare condition.

Lee, a cardiac surgeon, is an expert in Pulmonary Thromboendarterectomy (PTE) surgery, potentially the only treatment that can cure CTEPH.

Speaking specifically to CTEPH, could you describe the general manifestations of the conditions and how it is diagnosed?

I think the problem with CTEPH is that it can be present with really nonspecific symptoms. Oftentimes, it just presents with shortness of breath or feeling more tired than normal or not being able to do the same activity that you were able to do beforehand. And oftentimes, it gets attributed to more common diseases like asthma or COPD or heart failure. So that's the one thing why we can sometimes miss a diagnosis of CTEPH.

If it is diagnosed, who generally makes up the population?

It tends to occur most often in patients who have had blood clots, because that's what it really is. It's these blood clots that have been completely dissolved, or maybe somebody has had repetitive episodes of blood clots in the lungs. And that causes changes in the blood vessels in the lungs. Scarring and also narrowing of these blood vessels over time can cause high blood pressure in the lungs.

The patients that often develop it are people that have risk factors for developing these blood clots, like coagulation problems, history of cancer, or history of a prior splenectomy. It can tend to occur in females on birth control pills. Being overweight and not doing a lot of activity, and getting blood clots that travel up to lungs or having indwelling catheters or pacemakers can also cause it. But, about 50% of patients so half of the CTEPH patient population have no prior history of blood clots.

What are the treatment options for CTEPH?

When CTEPH is diagnosed, the first thing we want to do is just determine whether or not they're a candidate for surgery. There's a specific treatment called PTE surgery, which is open heart surgery where we clean out the blood clots, and it's the one treatment that can actually cure CTEPH. In patients that have CTEPH, we always evaluate if they're a candidate for that.

If they're not a candidate for open heart surgery, maybe they have significant other health issues that make them not a candidate, or the nature of their disease, like the blood clots, and the changes in the lungs are just too far out for us to get adequately through surgery, than the other options are medical therapy. And then also, we do have a less invasive procedure where we use balloons and catheters to open up some of the smaller blood vessels in the lungs. So, those are two options as well.

Specifically with PTE, how often is it performed versus not?

PTE is still pretty rare. We estimate and again, this is probably an underestimation just because of how difficult it is to diagnose CTEPH. But, we estimate there's probably about 10,000 to 15,000 CTEPH cases a year in the United States, but there's only about 250 to 300 PTE cases being done in the US, and that's at less than 20 centers across the country. So, a lot of patients aren't getting a procedure that they could potentially get.

What does the timeline look like for a patient?

Like I said, the big thing is the diagnosis. Oftentimes patients that have CTEPH, it can take up to two years sometimes for them to actually come to a diagnosis. Once they have a diagnosis of CTEPH, they should be referred to a center that deals with CTEPH that can offer surgery and other options. Once they come to our center for evaluation, we try to get our patients through pretty quickly. Come up with the diagnosis, do any further additional testing that needs to be done in order to determine if they're candidates for surgery and that can transpire in a few weeks to months.

Is there anything else you think is important to mention?

CTEPH disease is a rare disorder, but it can be more common than we really appreciate. And it does have a significant mortality that's associated, or risk of death if we don't treat it appropriately. We just want to get the message out that this is a disease process that can be treated. Patients can go on to live good lives and good quality of life. The thing that we're offering here at Allegheny General and Allegheny Health Network is access to heart failure and pulmonary specialists, some of these more advanced treatment options and then just an overall comprehensive care for the patient.