The primary objective of this study was to describe the impact of 22q11.2 deletion (del22q11) on the clinical characteristics, postoperative course, and short-term outcomes of children undergoing surgery for congenital heart disease. The charts of all children ages 1 day-18 years who received cardiac surgery for interrupted aortic arch (IAA), tetralogy of Fallot (TOF), or truncus arteriosus (TA) repair from 1 January 2001 to 31 December 2011 were retrospectively reviewed. The patients were divided into two groups: the 22q11 group including children with del22q11 undergoing surgery for TOF, IAA, or TA and the non-22q11 or control group including children with no chromosomal or genetic abnormality undergoing surgery for TOF, IAA, or TA. Demographic information, cardiac diagnoses, noncardiac abnormalities, preoperative factors, intraoperative details, surgical procedures performed, postoperative complications, and in-hospital deaths were collected. The outcome data collected included days of inotrope use, need for dialysis, length of mechanical ventilation, intensive care unit (ICU) length of stay (LOS), hospital LOS, and mortality. The study enrolled 173 patients: 65 patients in the 22q11 group and 108 patients in the control group. Of the 65 patients in the 22q11 group, 36 (55 %) underwent repair for TOF, 13 (20 %) for IAA, and 16 (25 %) for TA. The two groups did not differ in terms of age or weight. The preexisting conditions were similar in the two groups. Unplanned noncardiac operations were more common in the children with del22q11, but delayed chest closure was similar in the two groups. The incidence of postoperative noncardiac complications such as reintubation, vocal cord paralysis, and diaphragmatic paralysis was similar in the two groups. However, increasing numbers of patients in del22q11 group needed dialysis in one form or the other during the immediate postoperative stay. The incidence of fungal infection and wound infection was higher in the del22q11 group than in the control group. Duration of mechanical ventilation, ICU LOS, and hospital LOS were similar in the two groups, except in certain subgroups. Mortality did not differ significantly between the two groups. In conclusion, children with del22q11 have a higher risk of postoperative complications after cardiac surgery, with no difference in length of mechanical ventilation, ICU LOS, hospital LOS, or mortality. However, short-term outcomes may differ in certain subgroups.

This study aimed to identify the prevalence, etiology, and outcomes of extubation failure in children after complete repair for tetralogy of Fallot at a single tertiary-care, academic children's hospital. The secondary aim of this study was to determine the cardiorespiratory effects of the transition from positive-pressure ventilation to spontaneous breathing in children with extubation success and extubation failure. For this study, extubation was defined as the need for reintubation within 96 h after extubation. Demographics as well as pre-, intra-, post-, and periextubation data were collected in a retrospective observational format for patients who underwent complete repair for tetralogy of Fallot during the period January 2001-June 2011. Patients with multiple aortopulmonary collateral arteries or associated complete atrioventricular septal defects were excluded from the study. The cardiorespiratory variables collected before and immediately after extubation included heart rate, respiratory rate, mean arterial blood pressure, central venous pressures, near-infrared spectroscopy, oxygen saturations, and lactate levels. The clinical outcomes evaluated included the success or failure of extubation and the hospital length of stay. Descriptive and univariate statistics were used to compare the group with extubation failure and the group with extubation success. Extubation failure occurred for 7 % (12/164) of the 164 eligible patients during the study period. The median age of the patients at surgery was 200 days (range 98-356 days), and their median weight was 6.8 kg (range 5.2-8.5 kg). For 6 % (10/164) of the patients, intubation was performed before surgery. The median duration of mechanical ventilation was 33 h (range 19.5-73 h), and the median hospital stay was 10 days (range 7-15 days). Of the 12 patients with extubation failure, 2 had extubation failure in first 2 h after extubation, 6 had failure in 2-24 h, 3 had failure in 24-48 h, and 1 had failure in 48-96 h. The patients in the extubation success and extubation failure groups were similar in age, sex, and body weight at the time of surgery. All preexisting conditions also were similar in the two groups. The intraoperative variables and postoperative complications did not differ between the two groups. The hospital stay was longer for the children with extubation failure (p < 0.001). The partial pressure of oxygen in arterial blood (PaO2), tachycardia, mean arterial blood pressure, and inotrope score improved significantly at conversion from positive-pressure ventilation to spontaneous ventilation in the patients with extubation success. This study demonstrated that extubation failure in patients after complete repair for tetralogy of Fallot is low and that the etiology is diverse. The majority of extubation failures in these patients occurred in the first 24 h. Extubation success in the children after repair for tetralogy of Fallot was associated with improvement in PaO2, tachycardia, and mean arterial pressure, with a decrease in inotrope score. Extub...

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Introduction: The development of atherosclerotic cardiovascular disease begins in childhood. The American Academy of Pediatrics (AAP) endorsed guidelines recommending universal hyperlipidemia screening of children ages 9–11 and again at 17–21 years. An AAP Periodic Survey of Fellows demonstrated less than half of pediatricians report adherence to these guidelines. This quality improvement initiative’s objective was to improve compliance with AAP hyperlipidemia guidelines in an outpatient pediatric cardiology clinic at a single academic center to 80% over a 2-month time frame. Methods: We report the results of an IRB-approved chart review at a single-center outpatient pediatric cardiology practice. We defined pediatric cardiologists’ compliance as documented prior lipid screening, ordering a lipid panel, or documented recommendation for follow-up screening. Two plan-do-study-act (PDSA) cycles were undertaken. The first intervention included an informational session to provide pediatric cardiologists with AAP recommendations. The second intervention involved weekly email reminders and a statement for physicians in the electronic medical record. Results: We collected data from 600 individual charts of patients seen over 35 clinic days. We received charts before the first PDSA intervention. Baseline compliance with outpatient hyperlipidemia screening was 0%. After the first PDSA cycle, the average screening rate improved to 49%. After the second PDSA cycle, the average screening rate improved to 89%, and there was a centerline shift in the data, indicating improvement. Conclusion: We improved the pediatric cardiologists’ compliance with the AAP-recommended hyperlipidemia screening guidelines from 0% to 89% through 2 intervention cycles. Further efforts may be required to sustain this change.