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Introduction: Children with trisomy 21 are prone to postoperative chylothorax, caused by malformation of the lymphatic system, after cardiac surgery. The clinical course of patients diagnosed with postoperative chylothorax and trisomy 21 was compared to that of patients without dysmorphic syndromes. Additionally, differences between the groups in composition, amount, and duration of chyle were analyzed to better understand chylothorax in patients with trisomy 21., Materials and Methods: Retrospective cohort study using inpatient clinical databases during a 10-year period., Results: A total of 2255 patients underwent cardiac operations during the period, of whom 160 (7.1%) patients were diagnosed with trisomy 21. Chylothorax developed in 122 children; 89 patients were included in our study. Of 160 trisomy 21 patients, 27 (16.9%) developed postoperative chylothorax compared to 62 (3%) of 2095 patients without dysmorphic syndromes (p = <0.001). Time on ventilation, stay in intensive care, hospital stay, mortality, and composition of chylous effusion did not differ between groups. The rate of thrombosis was significantly lower (p = 0.02) in the trisomy 21 group., Conclusion: Children with trisomy 21 and congenital heart disease are more prone to developing chylothorax after heart surgery than those without dysmorphic syndromes. However if they develop this postoperative complication, mortality, chylous composition, time in ICU, and duration of hospital stay is not different to from that of other infants or children with this complication. This is important information for the medical specialists involved and is helpful in counseling parents of children with trisomy 21 undergoing heart surgery., Level of Evidence: This is a treatment study evidence level III., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Objectives: To evaluate the predictive value of pre- and postoperative amplitude-integrated electroencephalography (aEEG) on neurodevelopmental outcomes in children operated for congenital heart disease (CHD)., Study Design: Prospectively enrolled cohort of 60 infants with CHD who underwent cardiac surgery with cardiopulmonary bypass in the first 3 months of life. Infants with a genetic comorbidity were excluded. aEEG was assessed for 12 hours pre- and 48 hours postoperatively. Background pattern was classified by the use of standard categories, and the presence of seizures and sleep-wake cycles (SWCs) was noted. Outcome at 1 and 4 years of age was assessed with standardized developmental tests., Results: Preoperatively, infants either showed continuous normal voltage (n = 56) or discontinuous normal voltage (n = 4). Postoperatively, abnormal background pattern (flat trace, burst suppression, or continuous low voltage) was detected in 7 (12%), discontinuous normal voltage in 37 (61%), and continuous normal voltage in 16 (27%) infants. Nineteen infants (32%) did not return to normal SWCs within the recording period. Seizures were detected in 4 infants preoperatively and in another 4 postoperatively. After we controlled for surgical and postoperative risk factors, abnormal postoperative background pattern and lack of return to SWCs independently predicted poorer intelligence quotient at 4 years (P = .03 and P = .04 respectively) but was not related to motor outcome., Conclusion: aEEG is a useful bedside tool that helps to predict outcome in infants undergoing open-heart surgery for CHD. Abnormal postoperative background pattern and lack of return to SWCs are markers for subsequent impaired cognitive development., (Copyright © 2016. Published by Elsevier Inc.)

Objectives: To study the long-term neurodevelopmental effects of hyperglycemia in infant bypass surgery for congenital heart disease (CHD)., Study Design: Prospective cohort study on neurodevelopmental outcome after infant cardiac bypass surgery., Exclusion Criteria: age older than 1 year at first surgery, genetic comorbidity, and birth weight <2000 g. Of 167 eligible infants, follow-up examination at 4 years was completed in 150 children (90%). Intraoperative and postoperative highest and lowest glucose levels within 24 hours after bypass surgery were prospectively collected. Neurodevelopmental outcome at 4 years of age was assessed using standardized IQ tests and the Movement Assessment Battery for Children-second version for motor outcome assessment., Results: Mean age at surgery was 2.8 months (0.1-10.7 months), 33% of children had an acyanotic CHD and 67% a cyanotic CHD. Glucose levels were elevated (>8 mmol/L) in 21 (14%) children in the first 24 postoperative hours. Glucose levels normalized in all children within 48 hours, 7 children (4%) received insulin infusions. Mean total IQ was within the normal range, but significantly lower than the normal population (92.5 [SD 15.0], P < .001). Higher postoperative glucose levels were related to better cognitive performance in the univariate analysis (P < .03), but not when other risk factors were taken into account. Independent risk factors for adverse outcome were lower socioeconomic status, higher risk adjustment for congenital heart surgery score, and longer duration of intensive care stay., Conclusion: Hyperglycemia is common in the early postoperative course after infant bypass surgery for CHD and normalizes within 48 hours. Hyperglycemia has no adverse effect on long-term neurodevelopmental outcome., (Copyright © 2015 Elsevier Inc. All rights reserved.)

The aim of this study is to describe the effect of sedation and analgesia on postoperative amplitude-integrated EEG (aEEG) in newborns with congenital heart disease (CHD) undergoing heart surgery. This is a consecutive series of 26 newborns with CHD of which 16 patients underwent cardiopulmonary bypass (CPB) surgery and 10 patients did not. aEEG was monitored for at least 12 h preoperatively and started within the first 6 h postoperatively for 48 h. Outcome was assessed at 1 year of age. All 26 patients showed a normal preoperative continuous cerebral activity with sleep-wake cycles (SWC). The postoperative duration to return to normal background activity with SWC was similar for both groups. Independent of group assignment, patients requiring midazolam had a significantly later onset of a normal SWC than those without midazolam (p=0.03). Three patients in the CPB group and two in the non-CPB group showed continuous low voltage or flat trace after administration of fentanyl. These changes did not correlate with neurodevelopmental outcome. Sedation with midazolam has a transient effect on the background activity, whereas fentanyl can induce a severe pathologic background pattern. The significance of these changes on outcome is not yet clear. Thus, more attention should be paid to these effects when interpreting aEEG in this population.

The aim of this prospective study was to examine the determinants of growth failure and the association with neurodevelopmental outcome in infants undergoing open-heart surgery. In 107 infants undergoing open-heart surgery for congenital heart disease (CHD), we evaluated weight at birth, at surgery, and at one year of age (expressed as z-scores). Neurodevelopmental status was assessed before surgery and at one year of age. Median age at surgery was 3.9 months (range: 0.1-10.2). Mean [+/-standard deviation (S.D.)] weight z-score at birth was -0.27 (+/-1.45), before surgery -1.34 (+/-1.45) (P<0.001 vs. birth weight), and at one year -0.86 (+/-1.35), (P<0.001 vs. weight at surgery). Poor preoperative weight (<10th percentile) was associated with genetic disorders [odds ratio (OR) 5.9, P<0.001], preoperative neurological abnormalities (OR 3.41, P<0.05), and older age at surgery (OR 1.01, P<0.05). Weight <10th percentile at one year was associated with the same factors as poor preoperative weight, however, also with risk adjustment for congenital heart surgery-1 (RACHS) score >3 (OR 3.22, P<0.05). Neurodevelopmental outcome at one year was not determined by growth failure. In conclusion, impaired body weight gain before surgery is followed by a catch-up growth after surgery. However, there is no relationship to neurodevelopmental outcome. Genetic comorbidity is the most significant factor for poor weight gain.

Objective: Diaphragmatic paralysis (DP) caused by phrenic nerve injury is potentially life-threatening in infants. Phrenic nerve injury due to thoracic surgery is the most common cause of DP in children. We retrospectively analyzed incidence, surgical details, management and follow-up of our patients with DP after cardiac surgery to develop an algorithm for the management and follow-up., Methods: Retrospective analysis of 43 patients with DP after cardiac surgery performed between 1996 and 2000., Results: Median age at cardiac surgery was 1 month (range 3 days to 9 years). Incidence of DP was 5.4%. A trend towards higher incidences of DP were observed after arterial switch operation (10.8%, P=0.18), Fontan procedure (17.6%, P=0.056) and Blalock-Taussig Shunt (12.8%, P=0.10). Median time from cardiac surgery to surgical plication was 21 days (range 7-210 days). Transthoracic diaphragmatic plication was performed in 29/43 patients, no plication was done in 14/43 patients. Patients in whom diaphragmatic plication was required were younger (median age 2 months, range 21 days to 53 months versus 17.5 months, range 4 days to 110 months; P<0.001). Indications for plication were failure to wean from ventilator (n=22), respiratory distress (n=4), cavopulmonary anastomosis (n=2), and failure to thrive (n=1). All these symptoms resolved after diaphragmatic plication, however, 8/29 patients with plication and 2/14 without plication died. Cause of death was not related to diaphragmatic plication in any patient. Position of plicated diaphragm was normal in 18/21 surviving patients 1 month after plication. In 2/12 surviving patients without plication hemidiaphragm showed a normal position 1 year after surgery. The rate of pulmonary infections was not significantly different during 12-60 months follow-up., Conclusions: DP is an occasional complication of cardiac surgery. High incidences of DP were seen after arterial switch operation, Fontan procedure and Blalock-Taussig shunt (BT). Respiratory insufficiency requires diaphragmatic plication in most infants with DP whereas older children may tolerate DP. Transthoracic diaphragmatic plication is an effective treatment of DP and achieves relief of respiratory insufficiency in most patients. Spontaneous recovery from postsurgical DP is rare.