Congenital heart disease and heterotaxy: upper gastrointestinal fluoroscopy can be misleading and surgery in an asymptomatic patient is not beneficial.

PURPOSE: Heterotaxy syndrome is associated with intestinal abnormalities. We sought to define the gastrointestinal anatomy and determine both the risk of volvulus and benefit of screening upper gastrointestinal fluoroscopy (UGI) in these patients. METHODS: Medical records from 2003 until 2011 at Children's Hospital Los Angeles were reviewed in patients with heterotaxy for cardiovascular diagnosis, gastrointestinal symptoms, imaging and surgical arrangement of viscera, perioperative morbidities, and overall mortality. RESULTS: 224 patients were identified. Fifteen had polysplenia, 41 had asplenia, 50 had normal splenic morphology, 13 had inversus, and 104 were uncharacterized. UGI was performed in 4 patients for suspected volvulus and 20 for obstructive symptoms. Sixty-two had "screening" UGIs. Of 138 asymptomatic patients without imaging, none developed volvulus during the study period. In 30 patients with duodenojejunal malposition (DJM) who underwent surgery, none had malrotation or narrow mesentery. Eleven developed complications, with 8 requiring reoperation for obstruction. Of 8 patients with malrotation, 7 received a Ladd's procedure, and 2 had volvulus with viable bowel. One patient required reoperation and resection for obstruction. CONCLUSION: While rotational abnormalities are common in heterotaxy, risk of volvulus is low. Following operation, the risk of bowel obstruction and of need for reoperation is higher. We advocate avoiding operation in the asymptomatic patient.

Intraoperative hypercyanosis in a patient with pulmonary artery band: case report and review of the literature.

A case of intraoperative cyanosis in a patient with a common atrioventricular canal palliated with a pulmonary artery (PA) band is presented. The patient's physiology was consistent with cyanosis due to inadequate pulmonary blood flow, and responded quickly to typical interventions used for a hypercyanotic episode in a patient with unrepaired Tetralogy of Fallot. Differences and similarities in the physiology of PA banding compared with Tetralogy of Fallot are presented, including a rationale for treatment options for hemodynamic decompensation occurring in the setting of anesthesia and surgery.

Validation of nutrient intake using an FFQ and repeated 24 h recalls in black and white subjects of the Adventist Health Study-2 (AHS-2).

OBJECTIVE: To validate a 204-item quantitative FFQ for measurement of nutrient intake in the Adventist Health Study-2 (AHS-2). DESIGN: Calibration study participants were randomly selected from the AHS-2 cohort by church, and then subject-within-church. Each participant provided two sets of three weighted 24 h dietary recalls and a 204-item FFQ. Race-specific correlation coefficients (r), corrected for attenuation from within-person variation in the recalls, were calculated for selected energy-adjusted macro- and micronutrients. SETTING: Adult members of the AHS-2 cohort geographically spread throughout the USA and Canada. SUBJECTS: Calibration study participants included 461 blacks of American and Caribbean origin and 550 whites. RESULTS: Calibration study subjects represented the total cohort very well with respect to demographic variables. Approximately 33 % were males. Whites were older, had higher education and lower BMI compared with blacks. Across fifty-one variables, average deattenuated energy-adjusted validity correlations were 0.60 in whites and 0.52 in blacks. Individual components of protein had validity ranging from 0.40 to 0.68 in blacks and from 0.63 to 0.85 in whites; for total fat and fatty acids, validity ranged from 0.43 to 0.75 in blacks and from 0.46 to 0.77 in whites. Of the eighteen micronutrients assessed, sixteen in blacks and sixteen in whites had deattenuated energy-adjusted correlations >or=0.4, averaging 0.60 and 0.53 in whites and blacks, respectively. CONCLUSIONS: With few exceptions validity coefficients were moderate to high for macronutrients, fatty acids, vitamins, minerals and fibre. We expect to successfully use these data for measurement error correction in analyses of diet and disease risk.