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For Release 2005-11-15

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- Lucile Packard Children's Hospital at Stanford cardiac surgeon Frank L. Hanley, MD, reported this week that many patients with a complex congenital heart defect can benefit from one early, marathon surgery that corrects all the anatomical abnormalities in one fell swoop. His analysis of more than 300 consecutive pediatric patients over a 12-year period debunks the idea that the traditional multi-step, developmentally staged surgery is the best way to correct the problem. The data was reported on Monday (14) as part of the American Heart Associations annual meeting in Dallas, Texas.

This is the most complicated operation I do, said Hanley, chief of pediatric cardiothoracic surgery at Packard Childrens and the Lawrence Crowley, MD, Endowed Professor in Child Health at the Stanford University School of Medicine. It can take up to ten hours in the operating room. But the data show that, if attempted early in infancy, many of the children can get a total repair and recover with nearly normal pulmonary blood pressures. Abnormally high blood pressures in the lungs a side effect of the cardiac condition can quickly and irreversibly damage the delicate tissue and cause lifelong problems for the child.

The median age of Hanleys patients over the twelve-year time period covered by the study was eight months, although he prefers to tackle the problem in children as young as three months in an attempt to limit the ongoing lung damage. Early mortality for the complex operation is two percent, reports Hanley, and ten year survival rates are around 86 percent.

Congenital heart defects are not uncommon. About one in 100 newborns have some type of abnormality. But candidates for Hanleys marathon surgeries belong to a select group. They have a combination of problems known as Tetralogy of Fallot, or Tet, complicated by pulmonary atresia their pulmonary artery is missing or malformed and extra vessels called major aortopulmonary collaterals, or MAPCAs. About 3000 babies are born each year in this country with Tet, and about one quarter of these have pulmonary atresia. One half to one third of these have MAPCAs. Hanley performs the operation on about 75 kids each year at Packard Children's.

Although developmentally abnormal, the MAPCAs serve an important function in keeping these kids alive. They siphon off some of the blood rushing from the aorta to the body and deliver it instead to the lungs. A hole between the two sides the heart, another one of the defects of Tet, allows blood to escape from the dead-ended right side into the left and out the aorta.  Blood circulating to the body is only partially oxygenated, which compromises a childs growth and can cause a condition known as blue baby syndrome. In addition, the rerouted blood from the aorta enters the lungs at a much higher pressure than normal, irreversibly damaging the delicate tissue.

During the surgery, called unifocalization, Dr. Hanley detaches the collateral arteries from the aorta and gathers them together like the stems of a bouquet. He then stitches them together to form a larger vessel, which is then attached to the right side of the heart where the pulmonary artery would normally be.

At this point, Hanley has a decision to make. Sealing the hole in the heart and correcting the other defects of Tet would leave the child with normal circulation, and the hope of a normal future. But if the pulmonary pressure is still too high, going ahead with internal repairs to the heart at that time can cause the heart to fail. These children benefit from some additional time to grow and develop before attempting further repair.

Its a very important decision, said Hanley. If a patient should not have the intracardiac repair done, and you go ahead and do it anyway, there is lots of morbidity. Conversely, there can also be adverse consequences if you dont do the repair when you should. Rather than assessing this subjectively, we developed a way to measure right in the operating room how the blood is flowing in the new vessels.

If the flow and pressure are satisfactory, Hanley goes ahead with the rest of the operation, if not, he ends the procedure and plans another surgery when the complete repair will be better tolerated by the child. About 60 percent of Hanleys unifocalization patients end up having the complete, one-stage procedure including the closure of the hole in the heart, while the other 40 percent receive only the unifocalization of the MAPCAs initially, and the hole in the heart is repaired later.

We dont treat everyone the same way, said Hanley. We have very specific management protocols based on each childs particular physiology.

 We have a very large experience with this complex problem, and our management approach is somewhat unique, said Hanley. We have been pleased with how well these patients have done and felt it was the right time to report the experience.
About Lucile Packard Children's Hospital
Ranked as one of the nation's top 10 pediatric hospitals by U.S. News & World Report, Lucile Packard Children's Hospital at Stanford is a 264-bed hospital devoted to the care of children and expectant mothers. Providing pediatric and obstetric medical and surgical services and associated with the Stanford School of Medicine, Packard Children's Hospital offers patients locally, regionally and nationally the full range of health-care programs and services from preventive and routine care to the diagnosis and treatment of serious illness and injury. For more information, visit