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Patient is "living to live instead of living to survive," thanks to heart repair surgery

Congenital heart defects, abnormalities in the heart that occur during fetal development, are the most common type of birth defect, with the Centers for Disease Control and Prevention estimating that one million American adults are currently living with one. Some types of congenital heart defects can be mild and require no medical treatment, while others such as transposition of the great arteries were once fatal and today require lifelong cardiac care.

In transposition of the great arteries (TGA), the aorta and pulmonary artery, two major vessels that carry blood away from the heart are transposed. This causes blood from the lungs to flow back into the lungs and blood from the body to flow back into the body without getting the proper amount of oxygen. Brooke Stone was diagnosed with the condition as a newborn and, like many infants, underwent a complex surgery to correct her blood flow. While Stone's congenital heart defect was successfully repaired in infancy, the surgical fix meant she had to sit out of gym class as a child, and it produced some deadly complications for her later in life.

But thanks to a second life-saving surgery performed last September at Lucile Packard Children's Hospital, Stone can now enjoy a full life. In today's Inside Stanford Medicine, my colleague offers a closer look at Stone's story and how Frank Hanley, MD, director of the Children's Heart Center at Packard Children's, is pioneering a new approach to improve surgical repairs for TGA:

Instead of backing away from the [conventional approach, which had poor patient outcomes], he looked closer to understand its limitations. "The idea that everyone who needed the procedure could just be slam-dunked into the arterial switch was wrong," he said. "We focused on setting rigid criteria for accepting people into the program, and setting up a five-point report card after left-ventricle training to ensure that we were selecting appropriate patients who would have good outcomes."

Today, Hanley may be the only surgeon in the United States doing the procedure. A careful process of multiyear monitoring, patient selection and rigorous evaluation is key to his successful approach. Over the past 15 years, as the criteria for selection and the procedure have evolved, the survival rate for Hanley's patients has grown to exceed 90 percent. So far, Hanley has managed 36 patients with a failing Mustard/Senning procedures, and estimates that thousands more in the United States may still need lifesaving intervention of some kind.

The story goes on to explain how Packard Children's is working in tandem with the multidisciplinary Adult Congenital Heart Program at Stanford to better provide monitoring and care of survivors of congenital heart surgery.

Previously: Advancing heart surgery for the most fragile babies and Little hearts, big tools
Photo by Chris Conroy

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