Mortality after total cavopulmonary connection in children with the down syndrome.

A total cavopulmonary connection (Fontan surgery) is rarely performed in a child with trisomy 21 (Down syndrome) for a univentricular heart, and the outcomes after surgery are not well defined, but the incidence of mortality has been reported to be higher. To determine the mortality rate and contributing factors after Fontan surgery in children with Down syndrome, mortality data after Fontan surgery from the Pediatric Cardiac Care Consortium Registry were evaluated. Among Fontan procedures (n = 2,853), all patients with Down syndrome (n = 17) were selected, of whom 13 had hemodynamic data available. Thirteen children without chromosomal aberrations were then selected as a control group, matched 1 to 1 for gender, age, weight, lesion, and type of Fontan procedure. The following variables were evaluated: pulmonary artery pressure and vascular resistance, weight, hemoglobin, degree of atrioventricular regurgitation, previous Glenn operation, fenestration, and length of stay in the hospital. In children with Down syndrome, mortality after the Glenn operation was 28%. Mortality after the Fontan operation was increased significantly (p = 0.001) in children with Down syndrome (35%) compared with those without Down syndrome (10%). Between patients with Down syndrome and controls, there were no significant differences in the perioperative parameters evaluated. Almost all mortality was in the early postoperative period in children with Down syndrome. The relative risk ratio of mortality was 2.5 (95% confidence interval 0.63 to 10). In conclusion, Down syndrome was found to be an independent parameter associated with a significantly higher risk for mortality in the early postoperative period after Fontan surgery.

Pediatric Cardiac Care Consortium: an instrument for evidence-based clinical decision support.

Pediatric Cardiac Care Consortium is a registry of cardiac catheterizations, surgical operations, and autopsies performed for infants, children, and adults with congenital heart disease. Four examples of use of PCCC data to evaluate variability in morphology, management, and outcomes for the procedures are described. Consideration is given to the following clinical problems: (1) the experience with surgical heart block in operative closure of perimembranous VSD, (2) the transition away from atrial baffle operations to the arterial switch operation for simple transposition of the great arteries, (3) the experience of planned 3 stage palliation of hypoplastic left heart syndrome, and (4) the identification of a high risk combination of cardiovascular anomalies in Williams syndrome. Analysis of registry outcomes allows ongoing quality improvement at a cardiac center to consider not only its own experience but that of the overall group. The PCCC data can be used to personalize management of rare congenital cardiac anomalies and combinations of anomalies. The PCCC registry allows longitudinal consideration of issues such as staged repairs and incidence of unplanned reoperation. In future years, the PCCC can facilitate investigations into the etiology of congenital heart disease.