Tween Misses Old Heart but Grateful for New One After Transplant

A multispecialty Stanford heart team takes heroic measures to ensure a good outcome for a complex heart transplant

Last fall, Zoë started feeling faint, dizzy, and sweaty during school golf tournaments. She and her family knew she had a heart issue, but up until then it was controlled and didn’t cause her problems.

“We came in for a routine checkup and were told her heart function was declining rapidly. Instead of going home that day as we anticipated, Zoë was sent straight to the Cardiovascular Intensive Care Unit (CVICU),” says Melissa, Zoë’s mom and a longtime preop and recovery nurse at Stanford Medicine Children’s Health.

Zoë, age 12, was born with a rare, fast heart rhythm called junctional tachycardia, which was diagnosed by the neonatology and electrophysiology teams at Stanford Children’s. Medicines controlled it when she was young, but as Zoë grew, she developed another heart issue, cardiomyopathy—a disorder of the heart muscle.

When she went to the CVICU, the family knew something major was up. That fateful day was the start of a long journey. Zoë wouldn’t leave the hospital again for several months.

Placing a VAD as a bridge to transplant

Within a week of her admission, Zoë’s heart failure advanced to the point where she needed a ventricular assist device (VAD). VADs are pumps that support failing hearts to ensure that there’s proper blood flow to the body. Fortunately, Stanford Children’s is a national volume leader and innovator in the use of VADs.

“We have very long and historic experience supporting kids with VADs while they await transplant. We have advanced protocols to decrease infection rates, avoid strokes, and help them stay as healthy as possible,” says John Dykes, MD, Zoë’s primary pediatric cardiologist with the Pediatric Advanced Cardiac Therapies (PACT) program and medical director of the VAD program at Stanford Children’s.

Elisabeth Martin, MD, pediatric heart surgeon and the surgical director of the Lung and Heart-Lung Transplant Program at Stanford Children’s, used advanced imaging to implant the device. “Zoë is the coolest kid. She was scared and sick, but she stayed very calm through the VAD surgery discussion and process,” says Dr. Martin.

Often, children who have VADs are able to go home while they await transplant, but that wasn’t the case for Zoë. The VAD supported the left side of her heart, but her right side was still vulnerable to rhythm issues, which worsened. She needed a heart transplant, and fast. The Betty Irene Moore Children’s Heart Center is working hard to expand the donor pool to get kids to transplant more quickly. Zoë’s wait was just 20 days.

A heart transplant with an adult-sized heart

The heart failure team is passionate about expanding donors for kids on the heart transplant waiting list. In this way, Stanford Children’s has been able to reduce wait time to transplantation and help more patients at our hospital survive. The team is leading national efforts to impact results at other institutions as well.

“By being aggressive yet safe with donor acceptance, we not only benefit our patients, but we benefit all patients on the waiting list to help them move up faster,” says Seth Hollander, MD, medical director of Heart Transplantation on the PACT team.

Dr. Dykes’s innovative donor heart matching technique is one way to find a match sooner. It uses CT scans to measure the volume of the donor and recipient hearts to see if they are a similar size, rather than traditional and less-precise height and weight measurements.

“We are one of a few centers in the world that offer fast 24/7 total cardiac volume size-matching capabilities, in large part because we have such a wonderful 3D and Quantitative Imaging Lab (3DQ Lab),” Dr. Dykes says.

Dr. Dykes credits the 3DQ Lab team for likely being the first in the nation to pioneer a standardized method for size-matching that gives transplant doctors the information they need quickly.

In fact, members of the 3DQ Lab, Robert Wilkerson and Chris LeCastillo in particular, jumped in to provide Zoë with a special keepsake, a 3-D model of her original heart. 3-D heart models help heart surgeons visualize highly complex heart surgeries. “When Zoë received her 3-D model, she was beaming and all smiles,” says Andrea Fasbinder, MSN, RN, PHN, ventricular assist device (VAD) nurse coordinator on the PACT team at Stanford Children’s.

Because Zoë’s heart was enlarged due to her dilated cardiomyopathy, she was able to accept an adult donor heart.

“Facing the prospect of heart transplant was hard for Zoë; it was very difficult for her to part with a part of her,” Melissa says. “Yet, the chance to see her old heart, go over findings with the pathologist, and receive the 3-D model afterward gave her some closure.”

Successful heart transplant surgery despite complications

The family heard the news that a donor heart was matched at lunchtime, and by that evening Zoë was prepped for heart transplant surgery. Dr. Ma performed the transplant, and everything went as planned.

“Dr. Ma came out and told us that it went well, but the moment we got up to the CVICU after surgery, she coded,” Melissa says.

Zoë had an uncommon occurrence of primary graft dysfunction. If heart doctors are unfamiliar with the nuances of treating it, a patient may not survive.

“Primary graft dysfunction (PGD) can happen in five percent of heart transplants. Our goal is to support kids on lifesaving extracorporeal membrane oxygenation (ECMO)—a heart-lung bypass machine—as quickly as possible while their heart gets back on track,” Dr. Hollander says.

PGD tends to happen with more complex patients. Since Stanford Children’s is known for taking on higher-risk, more difficult heart transplant patients, the heart team sees a higher rate of PGD. The team has seen a few cases in the last few years, but they’ve been able to salvage the heart transplant with expert, coordinated, calm action between heart surgeons, heart transplant/heart failure specialists, and critical care specialists all working side-by-side to save a child’s life.

“Zoë coded for nine minutes, but the remarkable efforts by the CVICU team to resuscitate Zoë, then Dr. Ma’s team expediting placement of ECMO, kept her blood pressure from dropping the entire time. From a nurse’s point of view, that’s incredible,” Melissa adds.

Only large pediatric heart centers tend to offer ECMO. Stanford Medicine is proud to be a gold-level Extracorporeal Life Support Organization (ELSO) Center of Excellence.

“To some, ECMO can seem like something to only use as a last resort, but we are not afraid to use ECMO and other advanced techniques early on. We act sooner than later, and we have the expertise to carry it out. It’s probably one of the reasons why our heart transplant patients often end up doing fine,” Dr. Ma says.

Caring for her whole body, brain, and well-being

When a child codes, protecting the brain from injury is paramount. The brain requires 15% to 20% of the heart’s output, and it demands a consistent flow of oxygen-filled blood. The CVICU team took heroic steps to protect Zoë’s brain, using advanced protocols and the latest guidelines to ensure that she was fine.

“I know Stanford Children’s mission is to provide “extraordinary care,” but to see the CVICU and heart teams really carry that out was unbelievable to me. I am still blown away by the care they provided,” Melissa says.

She recalls nurses taking time to play card games with Zoë and paint her nails. One night nurse came in with a flashlight to braid her hair so she’d be more presentable, even though she was unconscious on ECMO. Later, the Child Life team helped Zoë, who loves to draw, host drawing games on television with other kids in the hospital, and her doctors, fellows, and nurses joined in on the fun.

“The care was amazing. And it wasn’t just because I am an employee. We saw that every family got the same care,” Melissa says.

Home and back to living life

After nearly six months in the hospital, Zoë is home sleeping in her own soft wonderful bed, getting homeschooled, taking long walks, and hanging out with her family, including her dad, David, and her cousins. Because she attended the Hospital School, she stayed on track with learning and homework so that she was able to pick up right where she had left off.

“I got tired easily before. I’m feeling more energy now,” says Zoë. She is looking forward to going back to school in the fall for her eighth-grade year and getting back to praise band, volleyball, and golf. The family plans to celebrate Zoë’s one-year heart transplant anniversary with a trip to Hawaii.

Her heart model sits in a prominent spot on the family’s mantle. It reminds Zoë of how far her old heart took her. She sometimes misses it, but she’s grateful to be getting back to life with her new heart.