Baby MJ Battles an Extremely Rare, Strange Heart Defect and Wins

The Egan family of Portola Valley, California, has an inspiring motto. During dark days, it kept them focused on what was more important than anything else: their daughter MJ’s survival.

“Our entire family—grandparents, aunts, uncles—joined in each morning to say, ‘Every day Mckenna Jay (MJ) wakes up to fight, we will fight with her.’ She has a strong crew supporting and praying for her,” says Michelle Egan.

MJ’s name has special meaning. Mckenna is inspired by her maternal grandfather, and Jay is the name of her great-grandfather with whom she shares a birthday. While the Egans were at Stanford Medicine Children’s Health when MJ was being born, the rest of their family was celebrating Jay’s 90th birthday.

Learning that the baby they were expecting had an extremely rare heart condition 

At her 21-week ultrasound, Michelle said, the ultrasound technician went quiet. Then the doctor came in to explain that their daughter had a serious congenital heart defect, and it was a wonder that she was still alive. Things heated up quickly from there.

“From the second we received the news, we felt like we got first-class care from Stanford Children’s,” Michelle says. 

The couple was quickly scheduled with Fetal Cardiology for counseling and an echocardiogram. MJ was diagnosed with an extremely rare version of absent pulmonary valve syndrome. In this syndrome, the pulmonary valve—the valve that regulates blood flow from the heart to the lungs—is missing or is so poorly formed that it’s essentially useless.

MJ’s large team of expert heart doctors at the nationally leading Betty Irene Moore Children’s Heart Center hadn’t seen anything like MJ’s particular heart malformations before.

“In my 30 years as a fetal cardiologist, I haven’t encountered anything quite like it. After doing comprehensive research and connecting with colleagues across the nation, we identified three similar cases in the last five years in the United States,” says Theresa Tacy, MD.

With MJ’s condition (absent pulmonary valve with intact ventricular septum), the ductus arteriosus (a normal connection during fetal life between the artery that carries blood from the heart to the lungs and the artery that carries blood to the body) shunts blood backward from the body, through the ductus, and into the right ventricle (which normally pumps blood from the heart to the lungs).

“MJ’s case was really rare, and no parent wants to hear that. We provided a lot of fetal counseling prenatally to help Michelle and Dan cope with the uncertainty of it all,” Dr. Tacy says. “Can you imagine wondering from minute to minute if your baby is going to pass away in utero, or have to be delivered extremely prematurely? I give them a lot of credit for how well they handled things.”

“We cried a lot throughout the pregnancy because we didn’t know if our daughter would ultimately survive,” Michelle says. “Dr. Tacy took us under her wing.”

Living in the gray area when your child is sick is hard. You simply want answers. Michelle and Dan were devastated, but they jumped in to help, researching and sharing information with the doctors.

“We appreciated their honesty about how rare and challenging her condition was, and we valued how they included us as parents. When they had more information available about her condition or care, they would share it with us, unfiltered,” says Dan.

Michelle remembers being 30 weeks pregnant and talking with Dr. Tacy about what kind of future MJ could have. Michelle asked, “Will she be able to play sports? Or be able to have children when she grows up?”

“Dr. Tacy stayed positive the entire time. She told us it would be hard, but that the team would carry us through all of the way, and they did,” Michelle adds.

Michelle was nearing full term, and an entire team of doctors was lined up and ready to deliver and support her and MJ. Two side-by-side operating rooms were planned—one to perform Michelle’s cesarean section and the other to provide immediate life-saving heart surgery for MJ.

MJ’s heart slows down, and Michelle delivers urgently over Thanksgiving

Then, at 38 weeks along, MJ’s heart rate slowed for four minutes. Because of this slowing of the heart, MJ had to be delivered urgently, that very same day. It was Thanksgiving weekend, which meant a whole new team of doctors for the family.  

“Everyone on the original team was out for the holiday. We had a brand-new cardiologist and heart surgeon, but because of the strength of the entire Heart Center, and the thorough preparation that was done, MJ survived,” Dan says. “All of the doctors at Stanford Children’s dedicate their lives to their patients. It’s the reason MJ is here.”

Michelle remembers Elisabeth Martin, MD, a cardiac surgeon, walking into her room just five minutes after her maternal-fetal medicine doctor, Yasser El-Sayed, MD, who was off for the holiday and monitoring Michelle and MJ’s condition remotely, called to say she had to deliver immediately.

“Dr. Martin was a calming, reassuring presence. She came into our room and described in detail what she would do for MJ. It was incredible. She is brilliant and confident, and just a kind human,” Michelle says.

Side-by-side operating rooms for mom and baby

A team of doctors and caregivers quickly assembled for MJ’s birth and heart surgery. There was “Team Mom” and “Team Baby,” and they worked in lockstep. Team Mom included doctors from high-risk pregnancy obstetrics (MFMs) and obstetric anesthesiology. Team Baby included doctors from neonatology, cardiac anesthesia, cardiology, and heart surgery, and an extracorporeal membrane oxygen (ECMO) perfusionist. Michelle’s cesarean section, performed by Amy Judy, MD, the maternal-fetal medicine specialist on call, went well, and Team Baby was ready and waiting for MJ in the operating room next door.

“Everything went smoothly with the surgery. It took a lot of logistics, but we have the infrastructure for that and a fantastic team,” Dr. Martin says. “We had talked over the plan before MJ’s surgery, so we were all ready.”

When Dr. Martin opened MJ’s chest, she found a heart that was enlarged on the right side and a shockingly large pulmonary artery that was compressing the rest of her chest, including her airway. The large pulmonary vessels resulted from the extra flow going back and forth from her right ventricle and pulmonary arteries. This enlargement was identified while MJ was still in the uterus (womb) when MJ also had an MRI to help the care team understand the impact that these enlarged vessels might have on her airways.

“MJ’s pulmonary artery was 10 times larger than it should’ve been—the size of a football rather than, say, a celery branch,” Dr. Martin says. 

During the surgery, Dr. Martin removed excess tissue in the pulmonary arteries to ease pressure and closed the ductus arteriosus to make for more efficient blood flow in the heart and lungs. The surgery gave MJ a near-normal heart with normal blood flow.

“Her enlarged pulmonary artery was compressing the rest of her chest, so I was very delicate in surgery so I wouldn’t disturb her too much. The goal was to get her through without extracorporeal membrane oxygen (ECMO),” Dr. Martin says.  

ECMO acts as a heart-lung bypass machine, which oxygenates the blood and then pumps it back into the patient’s body. It is used as a lifesaving device, supporting someone until their body recovers enough to function on its own. But it’s not without risk, especially for premature newborn babies.

A long, scary first night of life

Cardiologist Stephen Roth, MD, described MJ’s first night of life as “just flying above the trees.” Because MJ went straight to surgery, she didn’t get a chance to recover from birth, which can make for a rocky start. And things got rocky.

MJ required minute-by-minute care for the first 48 hours of her life. Dr. Martin and Kathleen Ryan, MD, cardiac intensive care doctor, sat by her side. They worked together to protect her newly repaired heart, take care of her lungs, and make sure her brain got enough oxygen, all without ECMO.

“All night long we were right there running lab work and making decisions with her chest open and the cannula ready for ECMO,” Dr. Ryan says. “We put everything we had into her to get her where she is now—home, thriving, and healthy.”

The Stanford Children’s heart teams are experts in caring for premature babies with highly complex heart defects. It was this deep knowledge that guided them through the new territory of a very rare condition that was acting strangely. It’s why MJ had such a great outcome.

“MJ’s heart was responding differently than expected, so we listened to her and paid close attention to the signals she was sending to guide our next steps,” Dr. Ryan says. “At no point did she need chest compression (CPR), or lose circulation, or need ECMO, or go so far beyond the pale that we couldn’t get her back.”

It was an incredible victory. And it was the ultimate in personalized care.

“Our team has a huge depth of experience, so even when a baby has an extremely rare defect and is not acting predictably, we still have the experience and nimbleness to respond,” Dr. Tacy says.

“MJ should not have survived that night. She shouldn’t have even survived being born, but because of Stanford Children’s, she is here today, smiling,” Michelle says.

Six months in cardiac intensive care

In her first six months of life, MJ needed constant care. She needed medications to help her heart squeeze, a tracheostomy—an opening in her windpipe with a breathing tube—to help her airway remain open so she could receive enough oxygen, and nutrition to support her growing body and brain. For all of this, she needed 24/7 monitoring in the Cardiovascular Intensive Care Unit (CVICU).

“Dr. Tacy told us, ‘Your daughter will go home,’ and that became our mantra in the CVICU. She even made us a quilt to remind us that she would go home,” Michelle says.

Michelle and Dan were not thrilled to hear that MJ needed a tracheostomy, but “it was ultimately the best decision we have ever made,” Michelle says. MJ’s lungs were small, and they needed support to grow from the Pulmonary, Asthma, and Sleep Medicine Center. And MJ needed to put her energy toward growing rather than breathing. Plus, her airway remained compressed from her oversized pulmonary arteries in utero.

“The tracheostomy and ventilator liberated her from the hospital. It’s what got her home and kept her home with few readmissions,” says Michael Tracy, MD, pulmonologist.

To perform the tracheostomy and place the breathing tube, MJ received care from the Aerodigestive and Airway Reconstruction Center, which regularly partners with the Moore Children’s Heart Center to care for babies. Following the tracheostomy, she was cared for on a regular basis in Pediatric Otolaryngology (ENT), the Aerodigestive Center, and the Center for Pediatric Voice and Swallowing Disorders. Due to routine monitoring and close care, when tracheostomy changes became difficult, MJ was treated with a small surgery from Douglas Sidell, MD, to remove a shelf of skin that had grown inside of her stoma (the opening for the tracheostomy). These small procedures have kept MJ safe and healthy, and they often help children to avoid other, larger operations.

Home and living a full life

Having the tracheostomy was the final boost that MJ needed to turn the corner. Today, she is an active, happy 15-month-old, something her parents marvel at every day. Because the tracheostomy needs ongoing surveillance, MJ and her family have regular joint appointments with Dr. Tracy and Dr. Sidell.

“Removing the tracheostomy and weaning her off of the ventilator is a slow process, but we do expect a full recovery, because she’s growing and developing well,” Dr. Sidell says.

Someday, MJ will likely need another heart surgery to place a new pulmonary valve, but not yet. For now, MJ and her family are relishing their time together and are grateful for the tremendous outpatient support MJ is receiving. She is catching up with her milestones learning to crawl, and she is cruising along, despite having to bring along her ventilator and breathing tube.

“Her heart ventricle function is normal, which is unbelievable,” Dan says. “The fact that she’s here is a miracle, and it’s because of the enormous team at Stanford Children’s that did crazy things to give her a chance at a full life.”

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