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Congenital heart surgery has improved the survival of patients with even the most complex defects, but the long-term survival after these procedures has not been fully described.The purpose of this study was to evaluate the long-term survival of patients (age 21 years) who were operated on for congenital heart defects (CHDs).This study used the Pediatric Cardiac Care Consortium data, a U.S.-based, multicenter registry of pediatric cardiac surgery. Survival analysis included 35,998 patients who survived their first congenital heart surgery at 21 years of age and had adequate identifiers for linkage with the National Death Index through 2014. Survival was compared to that in the general population using standardized mortality ratios (SMRs).After a median follow-up of 18 years (645,806 person-years), 3,191 deaths occurred with an overall SMR of 8.3 (95% confidence interval [CI]: 8.0 to 8.7). The 15-year SMR decreased from 12.7 (95% CI: 11.9 to 13.6) in the early era (1982 to 1992) to 10.0 (95% CI: 9.3 to 10.8) in the late era (1998 to 2003). The SMR remained elevated even for mild forms of CHD such as patent ductus arteriosus (SMR 4.5) and atrial septal defects (SMR 4.9). The largest decreases in SMR occurred for patients with transposition of great arteries (early: 11.0 vs. late: 3.8; p 0.05), complete atrioventricular canal (31.3 vs. 15.3; p 0.05), and single ventricle (53.7 vs. 31.3; p 0.05).In this large U.S. cohort, long-term mortality after congenital heart surgery was elevated across all forms of CHD. Survival has improved over time, particularly for severe defects with significant changes in their management strategy, but still lags behind the general population.

L-Carnitine has been shown to protect the myocardium from ischemic injury, and also improves cardiac function in cardiomyopathies associated with carnitine deficiency and diabetes. However, the exact mechanism whereby carnitine exerts its effect is not fully understood. The present study was designed to investigate the effect of carnitine on the regulation of glucose utilization in the heart. These studies were performed by determining the effect of L-carnitine (5 mM) on the 14 CO 2 release from [1- 14 C]pyruvate (an index of pyruvate dehydrogenase complex, PDH), [2- 14 C]pyruvate (an index of acetyl-CoA flux through Kreb's cycle), and [6- 14 C]glucose (an index of the oxidative utilization of glucose). in rat myocytes. Substrate Rate of Oxidation (nmol/mg protein130 min) Control L-Carnitine [1- 14 C]pyruvate 141.80 ± 8.50 212 ± 28.80 * [2- 14 C]6436pyruvate 39.40 ± 7.90 21.1 ± 4.40 * [6- 14 C]glucose 1.56 ± 0.05 0.95 ± 0.05 * Values are presented as the mean ± SD of at least three different experiments. Asterisks represent a p l 0.05 of the effect of carnitine vs control. These data show that L-carnitine stimulates PDH activity by 50%. However, the flux of acetyl-CoA and glucose through Kreb's cycle was significantly decreased by this compound. These results suggest that L-carnitine enhances the removal of acetyl-CoA produced from glucose metabolism out of the mitochondrial matrix, thereby, activating the PDH complex.