A Surgical First: Multidisciplinary Team Brings Advanced Treatment and Comprehensive Care to CTEPH Patients

November 8, 2022 by

Nicholas Teman, MD; Christopher Scott, MD; Andrew Mihalek, MD; Venkat Manguta, MD; and Allison Kirkner, ACNP

Nicholas Teman, MD; Christopher Scott, MD; Andrew Mihalek, MD; Venkat Manguta, MD; and Allison Kirkner, ACNP (left-right; photo credit Kay Taylor)

If you’re a welder and you learn a new skill, you fire up your torch and give it a try. If you’re a teacher whose class is getting restless, you might change up your lesson plan on the fly. But in the healthcare field, the stakes are too high to improvise. Changes in practice take time. They take planning. And they take teamwork.

This comes as no surprise to the robust UVA Health team that worked to launch a new, comprehensive program for patients with chronic thromboembolic pulmonary hypertension (CTEPH). Never heard of it? You’re not alone.

This complex type of pulmonary hypertension is pretty rare, occurring in around 5,000 of the 600,000 people diagnosed with pulmonary emboli (PE) or blood clots. Most PEs resolve in time, but in these patients, the clots cause inflammation and scarring that narrows the pulmonary arteries. Left untreated, CTEPH leads to heart failure.

“This is a condition that aggressively affects a patient’s lifestyle,” says pulmonologist Andrew Mihalek, MD. Patients may suffer from debilitating symptoms like shortness of breath, fatigue, and chest pain. Unfortunately, because CTEPH is so rare and because the symptoms may be attributed to other conditions, patients living with the condition can go undiagnosed for years.

Embracing Opportunity

UVA Health is a Pulmonary Hypertension Association Clinical Center of Excellence. We’ve long had the expertise and resources in place to provide the advanced, comprehensive care CTEPH patients need. Yet, this team saw an opportunity to do more. They saw an opportunity to offer these patients a potential cure and a better quality of life.

Unlike other causes of pulmonary hypertension, CTEPH can be fixed. “It is the only correctable variant of pulmonary hypertension,” says Mihalek. That fix is done by performing a pulmonary thromboendarterectomy (PTE), a procedure in which surgeons remove the scar tissue from the pulmonary arteries.

This surgery is challenging and high risk. Yet, according to cardiothoracic surgeon Nicholas Teman, MD, it is the best option for patients who have exhausted medical therapy. “Compared to having chronic pulmonary hypertension and the symptoms that go along with that, I think that surgery is lower risk than leaving the disease process alone,” he says.

If it could be done, this team was determined that it would be done at UVA Health. But first … they needed a plan.

Advancing Care, Together

So began a year-and-a-half-long deep dive. A multidisciplinary gathering of minds with the sole purpose of creating a comprehensive CTEPH program that included both medical therapy and PTE. In the room were experts from: pulmonology, cardiology, cardiothoracic surgery, nuclear medicine, radiology, vascular science, hematology, anesthesiology, critical care, and more.

According to Allison Kirkner, ACNP, each person had something unique to offer, and all had one big thing in common. “There has been a 7 a.m. meeting on the books once a month for the past year-and-a-half to two years. That’s a lot to ask of someone,” she says. “We ended up with a team willing to do whatever was necessary to bring the most advanced therapies possible to our patients.”

Kirkner and thoracic surgeon Christopher Scott, MD, had prior experience caring for patients with CTEPH. Scott was recruited by UVA Health, in part, because of his experience performing PTE surgeries and his involvement in building a high-volume CTEPH program in Colorado.

Andrew Mihalek and I had a mutual interest in this procedure, so we teamed up and assembled this diverse team of like-minded individuals. Every person on the team is very important and has a different area of expertise that they bring to the table that allows us to be able to deliver high-level care.” ~Dr. Scott

Kirkner was the team member charged with putting the pieces together. “Allison grounded us,” says Mihalek. “She had a logical understanding of what was needed to care for these patients pre- and post-operatively, and she had an energy and drive that helped us see things through.”

There were many others key to getting this new program up and running, of course. Mihalek credits interventional radiologist John Fritz Angle, MD, for continuously showing up and enthusiastically supporting the launch of the program. And he lauded nuclear medicine radiologist Prem Batchala, MD, for researching and mastering a nuclear medicine study that makes confirming a CTEPH diagnosis possible. “This kind of multidisciplinary collaboration, in the long run, will benefit the care I can provide my patients, those with CTEPH and without,” he says.

Plan of Action

Over the course of many months, throughout the pandemic and Kirkner’s maternity leave, this team evaluated CTEPH patients’ complex care needs from every possible angle. They reviewed cases, played different scenarios and asked tough questions. From these ongoing team discussions came best practices and protocols that would shape the current CTEPH program at UVA Health, one of the few of its kind in the U.S.

“Patients with CTEPH can expect to be evaluated thoroughly and, if appropriate, offered medical and surgical treatment options. We have the ability to provide all of this care efficiently at UVA Health,” says Scott. “Previously, patients would have to travel long distances — often across the country — to be evaluated and surgically treated for CTEPH. We have opened up access regionally for patients and hope to expand our reach even further.”

With guidelines in place and after months of training for all team members on the key aspects of managing CTEPH patients medically and intra-operatively, it was time to put all of the planning into practice. This meant preparing for the team’s first PTE surgery, screening existing patients to determine who might be the best candidates.

The patient selected to go first: a 69-year-old breast cancer survivor with CTEPH who has received care at UVA Health for many years. “She had been dealing with symptoms of CTEPH for over a decade, and her lifestyle had really come to a complete halt. Even though she was on maximum medical therapy, she was still having symptoms and she relied on continuous oxygen therapy,” says Scott. “She was very happy to hear that there was a surgical option available in her own backyard.”

A Bellwether for Success

PTE is one of the most technically challenging cardiothoracic procedures we do, according to Scott. It can take up to 8 hours or more and requires:

  • Median sternotomy
  • Cardiopulmonary bypass
  • Deep hypothermic circulatory arrest
  • Removal of the chronic blockages within the pulmonary artery, extending down into the lungs

When it came time to operate on their first patient, Scott and the dozens of team members required to make this surgery possible were prepped and ready. “There’s always a little bit of nerves involved before you get going with a new program, especially when there are so many moving pieces. But we all felt very prepared,” says Scott. “We were ready to go.”

The outcome couldn’t have been better. “We had a very good surgical outcome and we were able to remove all of the critical blockages the patient had,” says Scott

The patient also “sailed through” the very acute phase in the ICU after surgery, he adds. The team followed her progress closely, implementing daily multidisciplinary rounds. She was out of the hospital in 7 days compared to the standard 14 days.

“These patients have the potential to be really sick in pretty predictable ways, and our team was prepared for any issues she may have encountered,” says Kirkner. “By that third day, there was a sense of relief and a sense of ‘mission accomplished.’ It’s a startling thing to hear patients who have had this major surgery, who have been on a ventilator and in bed for multiple days, say: ‘I already feel better.’ That is always very validating. It’s really good to hear that all of that work that went into making sure that this patient had a good outcome paid off and she’s better going out of the hospital than when she came in.”

“It didn’t feel like it was the first patient,” adds Teman. “It feels like we’ve been doing this for years because of all of our planning. We had all of the pieces in place to handle the infrastructure, and at no point did we ever feel like we were unprepared for any surprises.”

The UVA Heath team sees this patient’s positive outcome as a bellwether for the program’s future. “Launching this program has been a great example of a multidisciplinary approach to a complex problem,” says Scott. “A lot of people came together to build this and make this something that we can grow to benefit our own patients, but also allow us to be a referral base for other patients throughout the region. There aren’t many centers in our area who offer this surgery and the comprehensive management of this rare condition.”

Article written by Holly Ford.

Filed Under: Clinical, Faculty