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Child/Adolescent Health

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Children receiving surgery for hypoplastic left heart syndrome have better outcomes at hospitals that perform more such surgeries

Children born with the congenital heart defect hypoplastic left heart syndrome (HLHS) usually die within the first days or months of life without treatment. In this condition, the entire left half of the heart (aorta, aortic valve, left ventricle, and mitral valve) is underdeveloped (hypoplastic). Treatment options for children with HLHS include staged surgical palliation (usually shortly after birth), orthotopic heart transplantation, or comfort care. Children with HLHS who underwent stage 1 palliation surgery in teaching hospitals in 1997 were 2.6 times more likely to die than those who underwent the surgery at nonteaching hospitals. This was after accounting for hospital palliation surgery volume (usually indicative of outcomes) and severity of the child's condition.

However, by 2000, palliation surgery was centralized at teaching hospitals, with only 2 percent of surgeries performed at nonteaching hospitals. This centralization of surgery, along with advances in postoperative medical and surgical care for these children, was associated with an overall decrease in mortality from 28 to 24 percent. Yet mortality rates continued to approach 50 percent at hospitals that conducted only one or two of these surgeries a year (compared with 19 percent for high-volume hospitals), according to the study supported in part by the Agency for Healthcare Research and Quality (AHRQ) (HS11826).

Researchers retrospectively examined in-hospital mortality rates for 754 children with HLHS in 1997 and 880 children with HLHS in 2000 using the Kids' Inpatient Database (created as part of AHRQ's Healthcare Cost and Utilization Project). Over one-fourth (28 percent) of the children in 1997 and 24 percent in 2000 died in the hospital. Limited access to higher volume hospitals and patient preference for local health care may be contributing factors for the continued use of low-volume hospitals in 2000.

See "In-hospital mortality for children with hypoplastic left heart syndrome after stage 1 surgical palliation: Teaching versus nonteaching hospitals," by Jay G. Berry, M.D., Collin G. Cowley, M.D., Charles J. Hoff, Ph.D., and Rajendu Srivastava, M.D., F.R.C.P.C., M.P.H., in the April 2006 Pediatrics 117(4), pp. 1307-1313.

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