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Affairs of the Heart
Mending a Broken HeartRobot Heart SurgeryThe Heart FactoryHow's Your Heart?
 
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Duotone of zach smiling baby pict.

Rob and Karen Bartholet remember the events of February 19, 1998 all too well. Twenty-five weeks into her second pregnancy, Karen underwent a routine ultrasound at a private hospital in Charlottesville, VA. As Rob recalls, the procedure took a long time, and though Rob couldn't make much sense out of the image on the screen, he could tell that the technician was lingering over the flickering that could only be the unborn baby's heart.
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DIAGNOSIS

"I knew right away something was wrong," says Rob.

In fact, there was a long list of things wrong with the boy's heart: Double outlet right ventricle, pulmonary atresia, criss-crossed atrial-ventricle blood flow, inferior/superior ventricles, straddling mitral valve, hypoplastic tricuspid valve, atrial septal defect and ventricular septal defect.

"I'm a nurse, and I didn't know half of the diagnoses," says Karen. "They were not even in my book."


"I knew right away something was wrong."

 

About eight out of every thousand babies are born with a heart defect, but the Bartholet baby's was an extremely rare diagnosis. Fewer than five percent of children with heart defects have conditions so complex, according to Dr. Richard Jonas, Cardiovascular Surgeon-in-Chief at Boston's Children's Hospital and a professor of Surgery at Harvard Medical School.

Normally, oxygen-poor blood, or "blue" blood, returns from the body to the heart, which sends it to the lungs to pick up more oxygen. At the same time, freshly aerated "red" blood returns from the lungs to the heart, which pumps it out to the body again. The four separate chambers of the heart keep the used blue blood separate from the fresh red blood.

Graphic of heart
  In newborn Zach's heart, the red and blue blood could intermix, unlike in this healthy heart

In the Bartholet baby, the septal and valve defects meant the used up blood could mix freely with the fresh blood, lowering the percentage of oxygen saturated in the boy's blood. But the pulmonary atresia was perhaps the most dire diagnosis, meaning the vessel between the boy's heart and lungs was closed off. The baby's life depended on surgery to bypass this dead-end.

"Pulmonary atresia used to be a very, very bad thing to have," says Dr. Richard Jonas, "It was fatal in the majority of children."

"We came out in a total state of shock," Rob recalls.

The couple was sent to the teaching hospital at the University of Virginia, where the doctors outlined a course of action to take once the baby was born. Until then, Karen's heart would do the work for the developing baby and she could expect an uneventful pregnancy.

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