Cardiogenic Shock, ECMO and a Rare Heart-Lung Transplant

Corey Saul started his 20s as a healthy young man. Little did he know, he was kicking off a tumultuous decade. By the time he turned 28, Corey had gotten a surprise genetic diagnosis, survived cardiogenic shock and received a combined heart-lung transplant. Thanks to Inova’s groundbreaking Cardiogenic Shock Team and the multidisciplinary experts at the Inova Heart and Vascular Institute, Corey has a second chance at life.

Corey had asthma as a child, but eventually seemed to outgrow it . In his mid-20s, his breathing troubles returned. His doctor referred him to a lung specialist, who diagnosed pulmonary hypertension (PH). Corey began taking medication for PH, but his symptoms worsened. He was short of breath and retaining so much fluid that his weight shot up from 170 pounds to 230 pounds. “At that point I basically couldn’t do anything, because I’d immediately get out of breath,” he recalls.

As his health worsened, he was hospitalized near his home in Radford, Va. Suspecting he was in severe heart failure, physicians there placed a call to Inova’s Cardiogenic Shock Program. It was a call that ultimately saved Corey’s life.

The Cardiogenic Shock Team

Even with advances in treatment, cardiogenic shock is fatal about half the time. In 2016, Inova convened a task force of experts to develop a standard assessment and treatment for cardiogenic shock and form a team to implement it to improve survival. This initiative launched Inova’s Standardized Team-based Approach to Cardiogenic Shock Care, which has been published and replicated nationally and internationally:

If any physician in the region suspects a patient may be in cardiogenic shock, they can place a single call to Inova’s transfer center, which activates the cardiogenic shock team. That team includes a heart failure cardiologist, interventional cardiologist, cardiac surgeon, critical care physician and cardiac critical care physician. Those on-call team members join a group call with the referring physician to assess the case and determine a care plan based on a standardized protocol.

“Inova was one of the first centers to implement this approach and demonstrate its benefits, and we continue to be one of the few centers that is able to do it consistently, even as our volume grows,” says Carolyn Rosner, NP, IHVI’s Director of Innovative Programs and an advanced practice provider who oversees Inova’s cardiogenic shock and ECMO programs.

In 2016, 30-day survival for cardiogenic shock patients at Inova was 47%, right around the national average. After implementing the team-based approach in early 2017, 30-day survival rose to 57.9% in 2017 and 76.6% in 2018. Those findings were published in the Journal of the American College of Cardiology in 2019. The shock team has maintained those outcomes: In 2021, the national survival rate was still around 50% and att IHVI, 73% of patients survived cardiogenic shock.

When Corey’s doctor called Inova’s shock team, they put the wheels of his care into motion before he even arrived at IHVI. “We have all these eyes focused on the patient, with different disciplines to ensure that whatever a patient needs, the right specialists will know about it from the earliest possible point,” Rosner says. “And members of this multidisciplinary team continue to follow the patient throughout the course of their hospitalization.”

Multidisciplinary Care for a Critically Ill Patient

For Corey, the multidisciplinary care was critical. He was transferred to IHVI on June 24, 2022, where he was evaluated by the cardiogenic shock team. “The information provided from Corey’s doctor indicated he had pulmonary hypertension. But we quickly realized that wasn’t the whole story,” says shock team member Christopher A. Thomas, MD, a critical care physician and lung transplant specialist.  

He and his advanced heart failure colleagues jumped into action to perform a comprehensive exam. “We work very closely together to understand a patient’s pathology and provide a full spectrum of care,” Thomas says. They determined that Corey had restrictive cardiomyopathy, which had likely been lurking undetected for years, Thomas says. Eventually that undiagnosed cardiomyopathy led to pre- and post-capillary PH and his worsening health.

Corey was admitted to the cardiac intensive care unit and began medical therapy while being evaluated for transplant. His physicians ordered various genetic tests, hoping to learn more about the underlying source of his heart disease. But they didn’t have time to wait for the results. Corey went into refractory cardiogenic shock and was placed on ECMO.

“We determined that he would need a heart transplant. Soon it became clear that his lung disease was so significant, he would need a lung transplant as well,” says Jamie L. Kennedy, MD, Medical Director of the Heart Transplant Team.

A Combined Heart-Lung Transplant — and a Diagnosis  

Thousands of people receive heart transplants or lung transplants each year in the U.S., but combined heart-lung transplants are relatively rare. In 2022, just 51 such procedures were performed in the U.S. The transplant experts at Inova are routinely among those who provide heart-lung transplants, including three in 2022 alone. 

One of those was Corey’s. He was listed for a combined heart and bilateral lung transplant on July 28, and received the donor organs on August 4. The transplant went smoothly, and he was discharged home on September 1. “I’ve been to a lot of hospitals, but Inova is by far the best,” Corey says. “What makes them stand out is the people: the doctors and nurses and other people who cared for me. They make a good team.”

Shortly after his transplant, the results of Corey’s genetic tests came back. They showed he has Emery-Dreifuss muscular dystrophy (EDMD). Most people with Emery-Dreifuss MD develop heart problems by the time they’re adults. For some, like Corey, heart problems are the first sign of the disease, Kennedy says. Unfortunately, by the time Corey’s doctors discovered his illness, it had already caused significant damage to his heart. That damage, in turn, caused the problems in his lungs.

The good news is that Corey’s EDMD won’t affect his donor heart. Back home in western Virginia, he continues to receive cardiac rehabilitation, and is gaining strength each day. He admits he’s still recovering from the shock of his near-death experience. But he’s grateful for the care he received and is taking time to appreciate the small things. “It’s nice just to be able to walk without being out of breath,” he says. “And I’m so happy to be home where I can be around my family. You take that for granted until you’re so far away.”

While Corey was lucky to survive the combined transplant, his case underscores the importance of referring patients early for suspected heart or lung disease. “If we can identify patients before they develop severe PH, they have better outcomes,” Thomas says. “For all the referrals I get [for pulmonary disease], probably 9 in 10 of them could have come to me sooner. If a physician suspects something isn’t right — especially with young patients — I’m happy to evaluate them. You really can’t refer too early.”

Fortunately, Corey’s medical journey delivered him to Inova. “We’re unique at Inova in that we have all of these specialties represented on the cardiogenic shock team, and also have the full spectrum of options for patients, including temporary mechanical support devices and transplant services,” Rosner says. “We’re lucky to have a full complement of services and specialties, as well as an operational structure that makes us nimble in implementing change and fosters the types of innovations that allow us to stay on the frontlines of delivering patient care.”