Surgeons at The Johns Hopkins Hospital have performed a technically challenging heart transplant on a 19-year-old patient with congenital heart disease who presented in shock, restoring health to the young adult.

In a 12-hour procedure in April, a surgical team led by cardiothoracic surgeon Chetan Pasrija and pediatric cardiothoracic surgeon Marcelo Cardarelli, removed the patient’s diseased heart and replaced it with a healthy donor heart.

Because of the patient’s anatomy, the procedure involved multiple steps.

The patient had been born with double outlet right ventricle, a rare disease in which his left ventricle — the part of the heart responsible for pumping blood to the body — wasn’t fully formed.

“Instead of the right side of the heart pumping blood to the pulmonary arteries and to the lungs, and the left side of the heart pumping blood to the aorta and to the body, his right ventricle was pumping blood both to the pulmonary arteries and to the aorta,” Pasrija explains. “It falls in the spectrum of a larger disease process known as hypoplastic left heart syndrome.”

The patient had undergone a series of four palliative surgeries at Johns Hopkins in his infancy and as a young child, including a Fontan procedure, an operation done so the single ventricle only pumps oxygenated blood to the body. The Fontan palliation involves connecting the inferior vena cava (a vein carrying blood from the abdomen and lower body back to the heart) and the superior vena cava (a vein carrying blood from the head, neck and arms back to the heart) to the pulmonary arteries.

“A transplant after a Fontan palliation is considered one of the most advanced and complicated procedures,” notes cardiologist Ari Cedars, director of the Johns Hopkins adult congenital heart disease program. “There are not that many institutions in the United States that do it, and there are even fewer who do it well.”

Some Serious Reconstruction Work

Leading up to the heart transplant in April, the patient experienced a rapid deterioration in his condition. He required inpatient care beginning about a month prior to the transplant when he came to the hospital in shock, says Cedars, who had seen the patient once before this episode as he transitioned from the pediatric to the adult congenital heart disease program

Cedars and colleagues with Johns Hopkins’ heart failure team took some innovative measures to stabilize the patient, including administering two types of medications to support the heart muscle and implanting a temporary, partial mechanical heart to help his weakened heart continue to pump blood. Even with these efforts, the patient had marginal heart function and required almost daily titrations of his medications to maintain good perfusion, Pasrija says. 

So the team put him on the heart transplant waiting list and, due to insurance limitations, shared his information with other transplant centers in the region, all of which turned him down.

By April, when a new heart became available, the patient’s native heart was so diseased that when surgeons started the transplant operation, it stopped beating spontaneously after they opening his chest, requiring emergent connection to the heart-lung machine, Pasrija says.

During the transplant, the Johns Hopkins surgeons had to do some serious reconstruction work. First, they disconnected the inferior and superior vena cavae from the pulmonary artery, closed the holes in the pulmonary artery and created a new hole to connect the donor heart pulmonary artery to the recipient. In this case, the patient had two superior vena cavae, a right and a left. The surgical team did an additional reconstruction to take the left-sided superior vena cava, bring it to the right side, and create a new superior vena cava structure. Both heart donor tissue and bovine pericardium were used during these parts of the case.

“That’s one reason why a lot of institutions don’t offer this surgery, because it is quite complex and is known to have significantly increased likelihood of perioperative morbidity and mortality,” says Pasrija.

Comfort with Complexity

The patient did extremely well with the transplant, Pasrija says. Doctors were able to close the chest during the operation and remove his breathing tube the next day. After about a month, he was discharged home, where he continues to recover.

“He really had an uneventful postoperative course, which is not that common for patients who have the surgery, given how complex it is,” Pasrija says. “That speaks to our team and our comfort level with this operation.”

The heart transplant was an example of “an opportunity for us to help these patients,” Pasrija says. “Many of them, when they are really young, go through a lot of procedures and then live a pretty normal life. But once they develop heart failure, they get into a situation where there aren’t a lot of folks who are interested in taking on the complexity of their situation.”

Only a handful of transplant centers in the Northeast are equipped to perform these procedures, which often involve the patient and their family relocating for a year to be near the hospital during surgery and recovery, Pasrija says.

Noting the very positive outcome in this case, Cardarelli observes, “It must be an incredible feeling transitioning from being bed ridden and connected to an assist device pumping the blood your heart can’t pump anymore, to suddenly — out of nowhere — a precious gift: the gift of a normal heart and a pretty normal life when you are just becoming an adult. All was made possible by a selfless donor, his family and a large and mostly anonymous team of great professionals.”