"In hypoplastic left heart syndrome (HLHS), babies are born basically missing the left side of their heart and would die within the week without treatment," said Dr. Bacha, surgical director of the Congenital Heart Center at the University of Chicago. "The heart's main pumping chamber, the left ventricle, and the body's primary blood vessel, the aorta, are not properly formed. As a result, the heart is unable to pump blood effectively to the body."
"Approximately one percent of babies are born with some kind of heart abnormality, which can range from very minor to very serious," said Dr. Bacha "HLHS accounts for 7 to 9 percent of all congenital heart disease diagnosed in the first year of life."
Dr. Bacha spoke today at the AMA's 23rd Annual Science Reporters Conference in Washington, D.C.
The standard treatment for these infants has been a series of three open-heart surgeries the first one (called the Norwood after the surgeon who developed it) is performed within the first few days of life. Additional open-heart surgeries are conducted at approximately six months and two years of age. The new approach being pioneered by Dr. Bacha and Dr. Hijazi eliminate the initial open-heart surgery, which carries the greatest risk of serious side effects and death.
"The initial surgery has roughly a 20 percent mortality rate," Dr. Bacha said. "This is a very dangerous surgery for these tiny infants. We have to put them on cardio-pulmonary bypass, cool the patient and do deep hypothermic circulatory arrest. On a five or six day old, who may be premature, it's not surprising that the outcomes in terms of the brain ar
Contact: John Easton
American Medical Association