New book captures the manifestations of adult congenital heart disease

Patient and doctor collaborate to create a book on congenital heart disease for the layperson.
July 2nd, 2019
women snowshoeing up a mountain
Stephania Bunka on Colorado’s Stanley Mountain this winter. The engineer-turned-author has continued to be, as she put it, “super-active” despite adult congenital heart disease. (Linda Wolgemuth photo; courtesy Stephania Bunka)

When Stephania Bunka couldn’t find the perfect book about adult congenital heart disease, she quit her engineering job to write one herself – with a little help from her doctor at UCHealth.

If the old adage “write what you know” holds water, Bunka is poised for success. She was born blue in Sudbury, Ontario, and it had much more to do with oxygenation than with her mood at the time. She had a congenital heart disease called tetralogy of Fallot, one of many types of heart problems that surprisingly many babies are born with – roughly one in 100 births come with a heart defect, says Dr. Joseph Kay, the doctor who’s coauthoring the book on congenital heart disease with Bunka.

“It’s the most common birth defect,” he said, with about 40,000 babies added to the rolls each year. About two million people in the United States have congenital heart disease, half of whom are now adults. Perhaps 20 to 30% of those with adult congenital heart disease (ACHD) are getting the specialist care they need, Kay adds.

Kay is a prominent example of such a specialist. He is director of Colorado’s Adult and Teen Congenital Heart (CATCH) program, a collaboration between the Adult Congenital Heart Disease Clinic at UCHealth University of Colorado Hospital at the Anschutz Medical Campus and the congenital heart disease program at Children’s Hospital Colorado. For 15 years now, the CU School of Medicine cardiologist has kept an eye on hundreds of patients who, thanks to miraculous heart surgeries on babies and infants, lived on into adulthood and old age.

Bunka, 46, is among them. Tetralogy of Fallot involves four heart problems – a narrowing of the exit from the right ventricle (which pumps blood to the lungs); a hole in the wall between the right and left ventricles; a thickening of the muscle in the right ventricle; and what’s called an overriding aorta, which lets blood from both ventricles into the aorta (the left ventricle alone normally feeds the aorta, the heart’s exit door for oxygen-rich blood to the body). Bunka had open-heart surgery in Toronto when she was two years old and has gone on to live an active life – hiking (she has climbed Longs Peak twice now), biking (road and, more recently, mountain), and running half marathons and the Bolder Boulder. But she had, and always will have, adult congenital heart disease.

Fixes need fixes

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Bunka aims to create a layman’s guide replete with human stories related to ACHD and its treatment. (courtesy Stephania Bunka)

Why, if doctors fixed Bunka’s heart, does she still have heart disease, congenital or otherwise? Adult congenital heart disease takes many forms – though most commonly manifests in heart rhythm problems and heart failure symptoms, Kay says. But fixes – be they automotive repairs or intricate heart surgeries – are not forever.

Bunka became a patient of Kay’s shortly after her arrival in Colorado in 2003. The trained engineer had come to work as a turbine generator maintenance specialist with Xcel Energy. For about a decade, the appointments with Kay were routine. Then it became clear that heart trouble was brewing.

The tetralogy of Fallot surgery done back in 1974 left Bunka’s pulmonary valve – which, when healthy, opens and closes with each heartbeat, sending low-oxygen blood from the right ventricle to the lungs – open. That caused the right ventricle to bloat like a water balloon. In Bunka’s case, her heart ended up nearly twice its normal size, Kay says. To repair the old repair, UCHealth and CU School of Medicine cardiac surgeon Dr. David Campbell installed a new pulmonary valve in an open-heart surgery, and six weeks later, his cardiac electrophysiologist colleague Dr. Duy Nguyen added an implantable cardioverter defibrillator (ICD) to quell heart-rhythm problems that had cropped up.

Bunka transferred to a desk job with Xcel – given the potential electrical issues with her heart, being off in remote locations in the field was deemed too risky – and resumed what she described as a “super-active” life. But a seed had been planted, and in October 2018, she left Xcel to tour Central America and then to pursue her book idea.

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Adult congenital heart disease specialist Dr. Joseph Kay is both Bunka’s doctor and coauthor of her book on congenital heart disease.

She’s thousands of words and dozens of interviews into the project now. It involves a combination of layperson-friendly medical descriptions and, most importantly to Bunka, interviews with a dozen or so adult congenital heart disease patients from Colorado and elsewhere. Kay is contributing expertise as well as reaching out to patients for potential interviews. Bunka aims to write about the spectrum of adult congenital heart disease defects, but also cover the socioeconomic bases, she says.

“I want to capture all the different manifestations of adult congenital heart disease, and among people in different life situations,” Bunka said.

Shelf life

On the medical side, she’ll explain everything from what sorts of medical testing is done and why, what treatment options are available, how those options have changed over time, and where medical science and technology looks to be headed with respect to the disease she knows so well. Technological advancement stands to benefit Bunka one day, too: her prosthetic heart valve will wear out one day; its replacement could be delivered via catheter rather than an open procedure.

The book’s overriding message, she says, is that adult congenital heart disease repairs have a shelf life.

“A lot of ACHD patients who were operated on as infants were sent home and told they were fixed and fine for life,” Bunka said. “But they in fact need lifelong follow-up by a congenital heart specialist. We want to take every opportunity to get that message out there.”