Born with an anomalous heart: Stephanie’s story

Stephanie Scott and husband.
Stephanie Scott, a long-time patient of Dr. Brickner, was born with a congenital heart condition called dextro-transposition of the great arteries.

I’ve known Stephanie Scott since she was 20 years old – more than two decades now.

Stephanie was born with a congenital heart condition known as dextro-transposition of the great arteries, or d-TGA, a defect in which the aorta and the pulmonary artery arise from the wrong pumping chambers. Normally, oxygen-depleted blood enters the heart from the body and is pumped by the right side of the heart to the lungs, where it gathers oxygen. Then, the oxygen-rich blood returns to the left side of the heart and is pumped out to the body.

In a child born with d-TGA, the low-oxygen blood returning from the body is pumped back to the body without ever making a pass through the lungs to pick up oxygen, while oxygen-rich blood coming back from the lungs gets pumped back to the lungs. Without some type of connection between the two pumping circuits, the child will die. Typically, the child will have blue-tinged skin because the body is not getting enough oxygen.

When Stephanie was young, she had a life-saving surgery called the “Mustard operation,” an older procedure that redirects blood returning to the heart from the body out to the lungs, allowing oxygen-rich blood to be directed out to the body.

After this operation, patients typically feel great. Of course, the anatomy of their heart is still very different from that of the normal heart – which is where I come in.

When heart disease grows up

Pediatric cardiologists who treat congenital heart disease patients like Stephanie understand the specific details of the various congenital heart defects and the care required for them, but as these patients age out of pediatric care, it can be hard for them to find a doctor who likewise understands the aberrations in the workings of such patients’ hearts. As a specialist in treating adults born with congenital heart disease, knowing how to care for the unique challenges of these patients is precisely my area of expertise.

In 1992, Stephanie had a child. Her son was born healthy, but the pregnancy put a terrific stress on her unusual heart, leaving her hospitalized and unable to care for her child for many, many months. It was during this long, dark period that I met Stephanie. I quickly came to admire this young woman, for she has a lively tongue and a strong spirit. I remember her saying to me, “Don’t hide anything from me. Tell me the truth.”

After the birth, Stephanie was left with a very weak heart, but working together, we got her back on her feet, and she returned to her home in Waco, where she raised her son. I remained her cardiologist, and she regularly came to Dallas for her medical visits. Over the years, we got to know each other well.

Beth Brickner, M.D.
Beth Brickner, M.D.

Personal care – anytime, anywhere

Stephanie was stable and able to lead a normal life for a long time, but in 2009 her condition began to deteriorate. When I noted that the pumping function of her heart was worsening (despite good medications), I suggested that she have an implantable defibrillator placed to be able to treat life-threatening rhythm problems. Several years later, Stephanie passed out several times and her husband took her to the local emergency room, where it was found her defibrillator had fired twice, saving her twice from death.

Stephanie’s husband is very protective of her, and the two of us were her “team.” I gave her husband my cellphone number, and he tells me that ER physicians in Waco were stunned when he dialed me late one night and reached me directly rather than an answering service. But that was the plan – to always be there for her. Together, we were able to keep Stephanie mostly out of the hospital for a half-dozen years. 

By 2015, though, her situation was grave, and it was time for a transplant. Stephanie got her new heart on Jan. 22, 2016. She’s done very well since then. She and her husband go for long walks every day, both as therapy and for pleasure.

Because her new heart has normal anatomy, Stephanie doesn’t need my special expertise anymore. But I do still follow her, and Stephanie will always be a special patient for me.