I.Q. and Neurologic Outcomes are Improving for Children Who Received Complex Heart Surgery as Infants; Pediatric Cardiologist from The Children's Hospital of Philadelphia To Present Recent Findings at Conference

02/16/2001

ORLANDO, Fla., Feb. 16 /PRNewswire/ -- Fetal diagnosis, early intervention, surgical refinements, and advanced medical techniques are helping more children survive congenital heart defects with fewer neurological complications, according to recent studies of infant heart surgery presented at Pediatric Cardiology 2001, a national conference sponsored by the Cardiac Center of The Children's Hospital of Philadelphia. The conference runs from Feb. 15 -18 in Orlando, Fla.

"As we move forward, our focus has shifted from minimizing mortality to maximizing long-term potential," said Gil Wernovsky, M.D., medical director of the Cardiac Intensive Care Unit at Children's Hospital, who spoke at the conference about the latest findings on neurologic outcomes in children who have undergone complex heart surgery. Dr. Wernovsky also co-authored an article on the same topic in the March 2001 issue of "Clinics in Perinatology," now in press.

"For example, we have found that fetal diagnosis of congenital heart problems allows better management of the newborn circulation immediately after birth until surgery, with the likelihood of fewer neurological problems," said Dr. Wernovsky. He added that tools such as fetal echocardiogram permit better diagnosis by enabling physicians to see images of circulation in the fetal heart.

Survival rates have increased dramatically over the past 20 years for children undergoing surgery for congenital heart defects. Medical advances and surgical advances have gradually permitted successful surgery at steadily earlier ages, including on newborns.

Therefore, physicians have been better able to concentrate on longer-term quality of life for children with congenital heart disease. While some of the survivors, particularly those from earlier periods when the surgery was performed, have long-term problems in neurodevelopment, such as lower IQs and impaired motor skills, "in general, preschool and school-age survivors with complex congenital heart defects can be expected to have normal I.Q. scores," says Dr. Wernovsky.

Better Results, Even for High-risk Conditions "In addition, still-emerging data from the current era of surgery show better neurological outcomes, even for higher-risk conditions," adds Dr. Wernovsky, who summarized such data in "Clinics in Perinatology," with co-author William T. Mahle, M.D., also a pediatric cardiologist at Children's Hospital.

One such heart condition having a higher risk of neurological problems is hypoplastic left heart syndrome (HLHS), in which a child is born with a deformed left ventricle that is unable to pump blood to the body. Occurring in one out of every 4,000 to 6,000 live births, it is the second most common congenital heart defect found in the first week of life, and is invariably fatal without surgery.

Surgery for HLHS was first performed in the early 1980s, and is currently performed in three stages during the first two years of life. The complex surgery requires a heart-lung bypass and is frequently performed in conjunction with a procedure called deep hypothermic circulatory arrest, in which blood circulation is stopped at very low temperatures. Both procedures carry a risk of neurological complications. In addition, congenital brain abnormalities found in some children with HLHS, and brain injury caused by poor circulation resulting from the heart defect, also raise the risk of neurological damage both before and after heart surgery.

Drs. Mahle and Wernovsky recently studied school-aged and adolescent children who had received surgery as infants for HLHS. They found a median I.Q. score of 86, significantly lower than that of the general population. Nevertheless, added Dr. Mahle, "most of the children are now in an age-appropriate grade in school and able to engage in normal childhood activities." The surgeries were performed between 1984 and 1991, and thus included some of the earliest children in the world to undergo surgical reconstruction for HLHS.

Also discussed at the conference were continued improvements in outcomes from a more recent period of surgery. For instance, pediatric cardiologists from the University of Michigan Congenital Heart Center found recently that preschool and early school-aged children who had undergone infant surgery for HLHS and related heart conditions had normal I.Q. scores, not significantly different from the general population. The Michigan study involved children who had heart surgeries between 1991 and 1997. "We expect that current advances in managing children with HLHS will continue to be reflected in better long-term outcomes in the years to come," said Dr. Mahle.

The Cardiac Center at The Children's Hospital of Philadelphia performs more than 1,000 cardiothoracic surgeries per year, including approximately one operation per week on newborns with HLHS. "We know from research that surgical mortality and complications are reduced in high-volume centers such as ours," said Dr. Mahle. "Greater experience and a comprehensive, multidisciplinary approach both contribute to better results."

Founded in 1855 as the nation's first pediatric hospital, The Children's Hospital of Philadelphia is ranked today as the best pediatric hospital in the nation by a comprehensive Child Magazine survey. Its pediatric research program is among the largest in the country, ranking second in National Institutes of Health funding.

NOTE TO REPORTERS: For details about press registration at the Pediatric Cardiology 2001 conference, or to arrange interviews before, during or after the conference with Drs. Wernovsky or Mahle, call The Children's Hospital of Philadelphia at 215-590-4092.

CONTACT: Cynthia Atwood of The Children's Hospital of Philadelphia, 215-590-4092 or Atwood@email.chop.edu.