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Even with advances in surgery, deaths among children with single ventricle congenital heart disease remain high

Children with two types of congenital heart disease, double-inlet left ventricle (DILV) and tricuspid atresia with transposed great arteries (TA-TGA), often have an associated aortic arch anomaly and may develop pulmonary vascular disease due to excessive pulmonary blood flow. Even with improved surgery, the death rate continues to be high among patients with a single-ventricle heart, according to a study supported in part by the Agency for Healthcare Research and Quality (HS13217).

Ruey-Kang Chang, M.D., M.P.H., of the University of California, Los Angeles, and colleagues reviewed the outcomes of 140 pediatric patients with DILV or TA-TGA who underwent surgery at one hospital between 1983 and 2002. They examined multiple factors to assess the risk of death or the need for orthotopic heart transplantation (OHT) among 105 patients with DILV and 35 patients with TA-TGA.

The overall mortality rate, including patients who would have died without OHT, was 29 percent. Patients with DILV had a lower mortality rate than patients with TA-TGA (23 vs. 49 percent, respectively). The predicted survival rates at 5, 15, and 25 years were 89 percent, 80 percent, and 63 percent, respectively, for patients with DILV, and 68 percent, 63 percent, and 26 percent, respectively, for patients with TA-TGA. Even with improvements in surgery, only 52 percent of patients with a single left ventricle were expected to survive beyond 25 years.

The presence of arrhythmia and the need for a pacemaker were associated with increased mortality, whereas pulmonary atresia or stenosis and pulmonary artery banding were associated with decreased mortality. Sex, era of birth, aortic arch anomaly, and systemic outflow obstruction (as long as it was recognized and relieved early) were not risk factors for death.

See "Outcome of patients with double-inlet left ventricle or tricuspid atresia with transposed great arteries," by Yueh-Tze Lan, M.D., Dr. Chang, and Hillel Laks, M.D., in the January 7, 2004, Journal of the American College of Cardiology 43(1), pp. 113-119.

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