Lexi Miller – Tetralogy of Fallot

Learn more about Lexi's experience with Tetralogy of Fallot at Children's Hospital.

Whoever thought open-heart surgery could be a cool experience? When Lexi Miller, 22, describes her 2018 heart valve replacement that way, the excitement in her voice makes you believe it.

It was the most recent of three invasive heart procedures doctors at UPMC Children’s Hospital of Pittsburgh Heart Institute used to treat Lexi’s tetralogy of Fallot, a congenital condition that affects how the heart pumps blood. And it exemplifies UPMC Children’s Hospital’s commitment to care for the region’s children from infancy through young adulthood and far, far beyond.

Lexi credits a lifelong relationship with her cardiologist, Johanna Drickman, MD, Division of Pediatric Cardiology, for her positive outlook, even before the latest surgery. “Dr. Drickman made me feel safe and helped me understand that the doctors at UPMC Children’s were going to take good care of me. She just took the worry out of my mind,” she says.

Heart anomalies

Tetralogy of Fallot appears in an estimated 1,660 babies born in the United States each year, according to the Centers for Disease Control and Prevention. The condition is known as a tetralogy for the perfect storm of four heart defects that commonly occur together.

“Frequently, there is also an atrial septal defect — a hole between the upper chambers of the heart. Sometimes the condition is called pentalogy of Fallot,” says Dr. Drickman. Lexi was born with this additional hole in the heart.

These defects can be life-threatening if not addressed in infancy. “Without surgical intervention the prognosis is not good. With surgical intervention, children can do very well, although they often require a second surgery or procedure,” says Dr. Drickman. Tetralogy of Fallot is a common cause of cyanotic episodes, making a child appear blue when oxygen-poor blood is pumped to the body instead of the lungs.

Lexi didn’t exhibit blue symptoms as a baby. A newborn screening pointed to the heart defects — a frightening diagnosis for young parents. “When she was born I found out [about her diagnosis] before I even got to bring her home,” says her mother, Suzette Miller. “It was awful. I was terrified that she had something wrong with her heart.”

Dr. Drickman became Lexi’s cardiologist soon after she was born. “I remember counseling her parents on symptoms to watch for. Our goal with treatment then, as now, is to hopefully be able to send children for surgery before they have blue spells, because that can be a very scary experience,” says Dr. Drickman, who joined UPMC Children’s in 1991.

Treatment begins early

In 1997, when she was just 6 months old, Lexi underwent her first open-heart surgery. The pulmonary stenosis was relieved by removing some of the muscle below the pulmonic valve that was causing the narrowing, enlarging the pulmonic valve and using a transannular patch to enlarge the pulmonary artery. The hole between the ventricles — the ventricular septal defect — was closed with a patch, which also directs the blue blood to the pulmonary artery and the red blood to the aorta.

“I have learned not to worry about things and put things in God’s hands. I knew that with Dr. Drickman and UPMC Children’s, Lexi was in good hands. I just had the faith that she was going to be OK,” says Mrs. Miller. “Early on I really liked Dr. Drickman. She’s so honest and when you need to know something, she tells you. I really think she’s amazing.”

In 2008, at age 11, Lexi had a device inserted into her heart to address the atrial septal defect. Jacqueline Kreutzer, MD, FAAC, FSCAI, now chief of the Division of Pediatric Cardiology and medical director of the Heart Institute, advanced the double-disk device through a catheter inserted into a blood vessel in the groin to the right upper chamber of the heart, where the inner skin of the heart could grow around it.

The device made the atrial septal defect smaller, but it did not completely close it. “That helped some. It may have delayed the time that we needed to put the new valve in. It got her through high school,” says Dr. Drickman.

Lexi never let her congenital heart condition slow her down — except one time when she felt winded on the basketball court and decided that sport wasn’t for her. She played competitive softball until she was 18 years old, and continues to exercise and eat healthy as a senior at Indiana University of Pennsylvania.

“Other than (basketball) I have lived a normal and full life with no restrictions, and I’m able to do pretty much whatever I want. Sometimes I even forget I have heart problems or I’ve had open heart surgery,” says Lexi, who lives in Robinson.

When echocardiogram and magnetic resonance imaging tests during periodic check-ups began to reveal an enlarged heart on the side where it was working harder, Dr. Drickman told Lexi and her parents, Suzette and Scott Miller, that it was time to consider a heart valve replacement. “We follow patients closely to catch problems before they develop symptoms. That’s what we did in Lexi’s case. She didn’t have any symptoms, but we could see the changes starting to take place,” Dr. Drickman says.

“Lexi’s right ventricle was getting progressively more dilated and her pulmonary valve was incompetent. This long-standing combination can eventually put her at risk for worsening right heart failure and fatal arrhythmias,” says Melita Viegas, MD, Division of Pediatric Cardiothoracic Surgery, who performed Lexi’s second open-heart surgery. “We work together with the cardiologists to time the surgery appropriately to decrease this risk.”

“That’s when her mother was diagnosed with breast cancer. We were able to postpone Lexi’s surgery until her mother completed her treatment for breast cancer and was well enough to help her daughter face surgery,” says Dr. Drickman. “We just don’t treat a person — we treat whole families. In this case, I don’t think it hurt Lexi to wait another year.”

Mrs. Miller received her cancer diagnosis in March 2017, and underwent a year of chemotherapy and radiation treatments. “Dr. Drickman wanted to wait so that I could be strong for my daughter,” she recalls.

“Having a support system during recovery is key. There is a lot of hard work that goes into the hospital stay, but even more once patients go home. Lexi’s mom is part of her support system. Making sure she was healthy was an extremely important part of Lexi’s recovery,” adds Dr. Viegas.

The pig valve

In June 2018 — 21 years after Lexi’s first open-heart surgery — her leaky pulmonic valve was replaced with a bioprosthetic porcine valve. Dr. Viegas also closed the residual hole from the atrial septal defect during the two-hour surgery.

Her doctors had considered using a mechanical valve that tends to last longer than a bioprosthetic, which can last 10 to 20 years depending on the individual. Ultimately Lexi was not a candidate, but the upside is a porcine valve typically requires a less severe anticoagulant protocol following surgery — long-term use of aspirin rather than much stronger blood thinners that could force activity limits.

She received her pig valve on a Wednesday, and was discharged the following Saturday. “They had me out of bed and sitting up in a chair not even 24 hours after surgery,” she says.

Dr. Drickman recalls Lexi’s first follow-up appointment two weeks after surgery. “Her first question was, ‘When can I go back to the gym?’ I think the reason she did so well is because she was in very good condition going into this surgery. It really made a difference. She recovered very quickly,” says Dr. Drickman.

Even after repairs, patients who have been diagnosed with tetralogy of Fallot require lifelong follow-up care. In a few years, Dr. Drickman will pass Lexi’s care to specialists at the Adult Congenital Heart Disease Center at UPMC Children’s. The center is designed to help patients transition from pediatric to adult care; provide complete long-term care to adults with congenital heart disease to help them lead happy and fruitful lives; and counsel and assist the safe and successful care of adults with inborn heart disease who want to have children.

“She’s doing really well. She’s on her way to becoming a great young adult, because she’s been a great young person all this time. Right now, I wouldn’t limit her from any of the activities that she was doing before this,” says Dr. Drickman.

Dr. Viegas adds: “If she needs another intervention on her valve, it is our hope that it can be done in the cardiac catheterization lab with a Melody™ valve. Saving her another open-heart surgery would be great.”

After a tumultuous few years of medical treatments for daughter and mother, they’re both upbeat. With a repaired heart and a cancer-free bill of health, the family is looking ahead to a bright future. “2019 is our year,” says Lexi. And she means it.