Philadelphia, Pa. - Survival rates have been steadily improving for children who undergo surgery as infants for a life-threatening congenital condition called hypoplastic left heart syndrome (HLHS). Drawing on 15 years of experience with the surgery at The Children's Hospital of Philadelphia, physicians found significant improvements in survival, both early on and years after the reconstructive surgery. Late deaths - those occurring after hospital discharge following the multistage surgery - were uncommon, as was the need for a heart transplant.
The article reported on one of the largest group of patients operated on for HLHS at one institution: 840 infants who received the multistage surgery between 1984 and 1999. The study appeared in a November supplement of Circulation, the journal of the American Heart Association.
In hypoplastic left heart syndrome (HLHS), 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, HLHS is the second most common congenital heart defect presenting in the first week of life, and is invariably fatal unless surgery is performed. Surgical intervention for HLHS was first performed in the early 1980s. Currently, HLHS surgery is performed in three stages: the first on newborns, followed by an intermediate operation at about 6 months of age, and a third stage, the Fontan procedure, between one and two years of age.
The Children's Hospital researchers found that rates for hospital survival after the first stage of HLHS surgery improved from 56 percent in 1984 - 1989 to 71 percent in 1995 - 1998. According to William Mahle, M.D., attending cardiologist at the Cardiac Center of The Children's Hospital of Philadelphia and first author of the study, unpublished data show even better results, an 86 percent survival rate, for January 1999 through April 2000.
Furthermore, said Dr. Mahle, "The study found that overall three-year survival rates improved even more dramatically, from 28 percent in 1984 to 1988, to 66 percent in 1995 to 1998." Dr. Mahle added, "Until now, relatively little has been known about longer-term survival of patients with HLHS, but our study shows very few late deaths among patients discharged after the third stage," said Dr. Mahle.
Among the reasons for the improved survival rates were refinements in surgical techniques and in pre- and post-surgical management of children with HLHS, according to Bernard J. Clark III, M.D., an attending cardiologist at Children's Hospital and senior author of the study. Another reason was the introduction of the intermediate surgical procedure in 1989, performed at age 6 months, between the stage I newborn surgery and the Fontan procedure. An additional factor is experience: "High-volume medical institutions such as The Children's Hospital of Philadelphia tend to have better survival rates for HLHS surgeries," he adds, "because of greater experience, and the presence of comprehensive cardiac centers." The Cardiac Center at Children's Hospital performs over 1,000 cardiothoracic surgeries per year, including approximately one operation per week on newborns with HLHS.
In addition to Drs. Mahle and Clark, co-authors of the study, all from the Cardiac Center at Children's Hospital, are Thomas L. Spray, M.D., Gil Wernovsky, M.D., and J. William Gaynor, M.D.
Founded in 1855 as the nation's first pediatric hospital, The Children's Hospital of Philadelphia is recognized today as one of the leading treatment and research facilities in the world. Through its long-standing commitment to providing exceptional patient care, training new generations of pediatric healthcare professionals and pioneering major research initiatives, Children's Hospital has fostered discoveries that have benefited children worldwide. Its pediatric research program is among the largest in the country, ranking second in National Institutes of Health funding. In addition, its unique outreach and public service programs have brought the 381-bed hospital recognition as a leading advocate for children from before birth through age 19.