Ron Winslow - Wall Street Journal
Surgeons at Boston Children’s Hospital have developed a way to help children born with half a heart to essentially grow a whole one—by marshaling the body’s natural capacity to heal and develop.
About 1,000 babies are born in the U.S. each year with a condition called hypoplastic left-heart syndrome, the result of a genetic anomaly that leaves them without a functioning left ventricle, the heart’s main pumping chamber. Without a surgical repair, the defect is almost always fatal.
A new surgical strategy helped 9-year-old Alexa Rand’s body to essentially grow half a heart into a whole one.
The standard treatment is a series of three open-heart operations to reroute circulation so that the right ventricle can take over pumping blood to the body’s organs and extremities. But the right ventricle “is meant to handle low-pressure blood flow to the lungs,” says Sitaram Emani, the surgeon heading the effort on the new approach. “Now you’re asking it to do the work of a high-pressure system and to do that work for many years. Eventually it fails.” That’s one reason why 30% of patients or more don’t survive to adulthood.
Dr. Emani and his colleagues devised a complex strategy to open obstructed valves and repair other malformations to direct blood flow to the left ventricle instead of away from it. That triggers biological processes that promote the heart’s growth.
Last month, after using the approach on 34 carefully selected patients over the past decade, the doctors reported in the Journal of the American College of Cardiology that 12 now have two working ventricles. One of them, 9-year-old Alexa Rand of Kings Park, N.Y., whose treatments began in utero, is thriving. She sings, dances and surprises doctors with how long she can walk on a treadmill, says her mother, Rosamaria Rand.
The main drawback: The strategy requires one more surgical procedure, on average, and significantly more days in the hospital than the conventional surgery. The hope is, Dr. Emani says, that the long-term benefits will outweigh the extra hospital time.
A version of this article appeared December 29, 2012, on page C2 in the U.S. edition of The Wall Street Journal
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