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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., (Copyright © 2013 Elsevier Inc. All rights reserved.)

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., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Background: Preoperative laboratory examination of patients undergoing elective surgical procedures has been routinely performed for decades. Although there is a large body of literature concerning the appropriate preoperative assessment of adult patients, corresponding literature for the pediatric population is not as well defined. Children and young adults with cardiac disease are a particularly vulnerable subset of patients who often undergo an extensive battery of preoperative laboratory testing. We examined the serum chemistry profiles for children with cardiac disease presenting for outpatient surgery. The investigation aims to define the effectiveness of preoperative electrolyte determination in this population of children and young adults., Methods: A retrospective chart review of all children presenting as outpatients to a tertiary care, freestanding children's hospital for elective cardiac surgery between January 1, 2000 and January 31, 2003 was performed. All patient charts in which the admission date matched the cardiac surgical date were examined. Patients were excluded if the preoperative laboratory evaluation was performed outside of our facility, preoperative laboratory investigation was not performed, or the patient was transported by medical transport to our hospital. Patients were grouped according to three methods: the number of cardiac medications (none to four), and cardiac medications, noncardiac medications, and no medications. The presence of electrolyte abnormalities was also examined in the context of cardiac medications with various pharmacologic effects. The primary outcome measure was the incidence of abnormal laboratory values for children taking various cardiac medications., Results: Of the 933 initial entries found, 774 met the investigational criteria and were included in the analysis. Although statistically significant differences in preoperative electrolytes were associated with the use of cardiac and noncardiac medication, there was no clinical value to this correlation. The data demonstrate a very low incidence of hypokalemia and hypomagnesemia in the entire study population., Conclusion: Preoperative electrolyte disturbances in children and young adults presenting for cardiac surgery are uncommon. The concern of hypokalemia or hypomagnesemia important in the adult population taking cardiac medications was not identified in the pediatric population. These data do not support the need for routine preoperative electrolyte evaluation in children taking cardiac medications.

Implications: A premature infant undergoing ligation of a patent ductus arteriosus had arterial desaturation during surgical retraction, which was caused by a previously unrecognized vascular ring anomaly.

Esophageal electrocardiography (EsECG) is a useful adjunct in the diagnosis of dysrhythmias that are difficult to diagnose with a conventional ECG. This study was designed to evaluate which type of dysrhythmias required the EsECG for proper diagnosis and what factors produced the rhythm problems. Sixty-eight pediatric patients undergoing cardiac surgery were studied. After release of the aortic cross-clamp, the cardiac rhythm was analyzed by a standard limb-lead ECG at five-minute intervals. Twenty-six of 68 patients exhibited rhythms during reperfusion that required the EsECG for definitive diagnosis. This group of patients was younger, had longer durations of cardiopulmonary bypass and aortic occlusion, and required more time and interventions to achieve a normal sinus rhythm. The likelihood of difficult dysrhythmias was not related to the type of surgical procedure performed.

Minimum alveolar concentration (MAC) was determined in intact dogs (N = 10 for each anesthetic) to be 2.12 +/- 0.04 vol% for enflurane (ENF), 1.28 +/- 0.04 vol% for isoflurane (ISO), and 0.94 +/- 0.03 vol% for halothane (HAL). Then, the direct cardiac effects of these three anesthetics were studied at 0.36, 0.6, 1.0, and 1.2 MAC in the dog heart-lung preparation (HLP): an in situ whole heart preparation devoid of major extracardiac influences and reflex control. All three agents produced concentration-dependent decreases in heart rate (HR) that became significantly different from control at 0.6 MAC. HAL and ISO reduced +dP/dtmax by the same degree at all MAC levels, becoming statistically significant at 0.6 MAC, while a significant reduction in +dP/dtmax for ENF occurred first at 1.0 MAC. Marked increases in left atrial pressure (LAP) were observed at 1.0 MAC for all anesthetics and the first significant depression of systemic output (SO) occurred at 1.0 MAC. Each agent produced significant shifts of the left ventricular function curves (LVFC) to the right with each consecutive MAC fraction. Marked reductions in the slope of the LVFC were first observed at 1.0 MAC, and this change in slope was more pronounced with ENF. At 1.2 MAC, ENF seemed to produce a more severe cardiodepression than HAL or ISO, as suggested by a greater incidence of cardiac failure; however, this was not statistically significant. In general, the data suggest that at MAC fractions up to 0.6, ENF is less cardiodepressant than ISO or HAL, but that ENF has a tendency to be more depressant than HAL or ISO at concentrations higher than 1.0 MAC.

Cardiopulmonary bypass in children with congenital heart disease is associated with significant morbidity manifested by increased complement degradation products, heightened pulmonary vascular activity, and coagulopathy. In adults with cardiac disease, the prostaglandins (eicosanoids) have been shown to contribute to the pathophysiologic response to extracorporeal circulation. This study assessed the effect of cardiopulmonary bypass in infants and children on two potent eicosanoids: thromboxane, a vasoconstrictor and platelet aggregating agent, and prostacyclin, a vasodilator and platelet disaggregating agent. The biochemical profiles of thromboxane and prostacyclin were evaluated in temporal relationship to selected parameters of platelet loss and pulmonary vascular hemodynamics during and after cardiopulmonary bypass. Twenty-one children, aged 3 days to 9 years, with congenital heart defects who were undergoing repair with cardiopulmonary bypass were studied. Nine pediatric patients undergoing palliative heart operations with no cardiopulmonary bypass served as the control group. In the group having cardiopulmonary bypass, the thromboxane concentration significantly increased during bypass (195 +/- 10 to 910 +/- 240 pg/ml, +/- standard error of the mean, p less than 0.005), whereas the control group demonstrated no significant change in thromboxane concentration. The highest thromboxane values were seen in the youngest patients (p less than 0.002). There was no significant correlation between thromboxane changes with alterations in pulmonary vascular resistance, platelet loss, duration of cardiopulmonary bypass or aortic cross-clamping. Prostacyclin levels rose significantly in both the bypass group (100 +/- 20 to 570 +/- 80 pg/ml, p less than 0.01) and in the control group (109 +/- 44 to 589 +/- 222 pg/ml, p less than 0.01), which apparently is due to surgical manipulation of vascular endothelium. These data show that eicosanoid production is significantly altered in children during cardiopulmonary bypass. Although thromboxane, a potent vasoconstrictor, is produced in significant amounts during and after cardiopulmonary bypass, our data show that thromboxane does not directly mediate changes in pulmonary artery hypertension and is not quantitatively related to platelet loss during pediatric cardiovascular operations.

Recovery from the cardiac depressant effects of enflurane and halothane was examined in the dog heart-lung preparation (HLP) and in right ventricular muscle isolated from guinea pig hearts. In the HLP. recovery was studied under two conditions: (1) After a two-hour exposure to anesthetic concentrations increasing from 0.36 to 1.2 MAC, and (2) after a one-hour exposure to a single concentration that raised the left atrial pressure (LAP) to 9 to 10 mmHg. Under either condition, +dP/dtmax. was significantly less depressed with enflurane and returned to preanesthetic control levels, while recovery with halothane remained significantly below control. Following the longer exposure. recovery of the LAP and left ventricular function curves (LVFC) was significantly less with halothane; however, this difference was not observed after the shorter exposure period. In electrically paced, isometrically contracting right ventricular strips exposed for one hour to 2.25 vol% enflurane (a concentration that reduced contractility by 45%), force development returned within 60 minutes to values above preanesthetic control values. After an identical depression for one hour with halothane (0.80 vol%), force development recovered to values less than those observed following enflurane. These data indicate that the recovery from anesthetic-induced negative inotropic effects in isolated cardiac preparations is better with enflurane than halothane.