Transposition of the great arteries--outcomes and time interval of early neonatal repair.

BACKGROUND: This study evaluates the relationship of morbidity and resource utilization with the timing of early neonatal repair of transposition of the great arteries and intact ventricular septum (d-TGA/IVS). METHODS: All patients with d-TGA/IVS who underwent arterial switch in the first 14 days of life, between January 2000 and May 2011, were reviewed. Patients undergoing repair at ≤ 4 days of age were categorized as group I, 5 to 7 days as group II, and 8 to 14 days as group III. Outcomes included mortality, morbidity, and resource utilization. RESULTS: Hospital survival was 69 (98.6%) of 70. The length of stay (LOS) and total charges were lowest in group I--15.5 days compared to group II--18.0 days and group III--23.5 days (P = .005); group I--US$128,219 compared to group II--US$141,729 and group III--US$217,427 (P = .0006). Using regression analysis to account for potentially confounding effects of multiple variables and treating time as a continuous variable demonstrated that age at surgery was significantly associated with total LOS (P = .029), hospital charges (P = .029) and intensive care unit charges (P = .002). Younger age at repair was not associated with worse outcomes for any measure of morbidity. CONCLUSIONS: Earlier repair of d-TGA/IVS was associated with decreased resource utilization and no detriment to clinical outcomes. Further analysis based on a larger cohort of patients is needed to verify these results that have important implications for improving the value of care.

Thromboelastography in the assessment of bleeding following surgery for congenital heart disease.

BACKGROUND: Perioperative bleeding is common in pediatric cardiac surgery patients. Traditional laboratory tests do not adequately characterize coagulation derangements in patients with bleeding. We sought to establish preoperative thromboelastography parameters in children prior to cardiopulmonary bypass, to compare thromboelastography assessment with standard coagulation parameters postoperatively, and to assess thromboelastography in children with significant hemorrhage. METHODS: Sixty patients requiring cardiopulmonary bypass were enrolled in a prospective observational study of perioperative thromboelastography. Thromboelastography measures were obtained preoperatively, intraoperatively after protamine administration, upon admit to the intensive care unit, and when patients were treated for bleeding. Thromboelastography measures were not used for clinical care. Postoperative thromboelastography measurements were compared with the standard coagulation parameters. Intraoperative thromboelastography, postoperative thromboelastography, and clinical outcomes were compared among patients who did and did not have significant postoperative bleeding. RESULTS: Preoperative thromboelastography parameters were similar to other published normal values for pediatric patients. Transfusion recommendations based on thromboelastography measurements were significantly different from those based on the standard coagulation testing. Thromboelastography measures after initial protamine administration were significantly different in patients with postoperative bleeding. This difference was not present upon arrival to the intensive care unit. Patients with significant bleeding tended to cease bleeding when clinical interventions were in agreement with recommendations based on thromboelastography. CONCLUSIONS: Pediatric patients with significant postoperative bleeding after surgery are more likely to have abnormal thromboelastography early after cessation of cardiopulmonary bypass. Thromboelastography illustrates derangements in the coagulation system and may aid in the treatment of postoperative bleeding.

A Gender Assessment Team: experience with 250 patients over a period of 25 years.

PURPOSE: To describe a Gender Assessment Team that has provided a multidisciplinary approach to the diagnosis, medical and surgical treatment, genetic counseling, and psychosocial support of patients with ambiguous genitalia, intersex disorders, and other genital anomalies, collectively termed disorders of sex development; and to determine the major diagnostic categories and approach. METHODS: A retrospective review of 250 patients evaluated by the Team at Children's Hospital and Regional Medical Center in Seattle, WA, from January 1981 through December 2005. The Team included the following specialties: medical genetics, cytogenetics, gynecology, pediatric urology, endocrinology, and psychiatry. RESULTS: Of the subjects, 177 were infants, 46 were children or adolescents, and 27 had a multisystem genetic condition. The most common diagnoses were congenital adrenal hyperplasia (14%), androgen insensitivity syndrome (10%), mixed gonadal dysgenesis (8%), clitoral/labial anomalies (7%), hypogonadotropic hypogonadism (6%), and 46,XY small-for-gestational-age males with hypospadias (6%). CONCLUSION: The six most common diagnoses comprised 50% of the cohort. The expertise of a multidisciplinary team allowed for integrated care for patients with disorders of sex development and identification of novel conditions. Geneticists play an important role in a team approach through knowledge of genetic testing options and diagnosis of patients with karyotypic abnormalities and syndromes with genital anomalies.