A gravely ill two-year-old boy is recovering at Seattle Children's Hospital after receiving the Berlin Heart, a mechanical heart designed specifically for children.

The boy, called Angel, is the first child under five in the Northwest to receive a mechanical heart.

Angel was admitted to Children's Hospital in January with dilated cardiomyopathy, an enlargement of the heart that affects 1 in 10,000 children and can be caused by viral infections, or metabolic and genetic defects.

He was kept alive with extracorporeal membrane oxygenation (ECMO), a temporary mechanical support of heart and lung function which required him to be sedated and paralyzed with medication. ECMO can provide sufficient oxygenation for several weeks.

Meanwhile, doctors at Children's petitioned the U.S. Food and Drug Administration (FDA) for permission to use the Berlin Heart. Although the device is available in Europe, it does not yet have FDA approval for use in the U.S.

The Berlin Heart, the size of a tennis ball, is also known as a ventricular assist device, or VAD. It is similar to adult VADs but is small enough for use in children. Produced in Germany, the computerized pump system extends outside the chest and connects to the heart via tubes implanted in the child's chest.

"Before, the only option for children under five years of age with heart failure was ECMO then a heart transplant," said Dr. Gordon Cohen, chief of pediatric cardiothoracic surgery and co-director of Children's Heart Center. "The Berlin Heart allows us to buy more time to keep these children alive and bridge them to transplant with a higher quality of life."

In addition to its use as a bridge to transplantation, the Berlin Heart is sometimes an option for patients who are not eligible for transplantation. In approximately 15 percent of cases, it allows the heart time to recover and transplantation can be avoided altogether.

On Angel's 13th day receiving ECMO, surgeons received FDA approval on a one-time compassionate use basis and implanted the device in a five-hour procedure on Feb. 7.

"Angel had been in heart failure, on ECMO, and was paralyzed. His condition was deteriorating at a rate that was putting him at risk for organ failure within days," said Dr. Cohen. "The Berlin Heart allows him to be awake, and to resume normal toddler activities while he waits at the hospital for a heart transplant."

Angel was moved out of the intensive care unit on Feb. 28 and will remain at Children's until a donor heart becomes available.

"Even with the FDA emergency approval, there is a shortage of Berlin Heart devices in the U.S.," said Dr. Cohen. "We were lucky, to get this one, usually there is waiting period because it is in limited supply."

According to Berlin Heart Inc., 234 children have been implanted with the device since its invention. Some children have survived up to 16 months before transplant. In the U.S. only 60 children have received the device, Angel is the first person in the Northwest to use one.

Nationally, 110 children under five years of age are currently on the waiting list for donor hearts. The average waiting time for a donor heart for a child less than five years of age was 78.5 days according to the United Network for Organ Sharing. In 2006, seven children under five years of age received heart transplants at Children's.

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