Gastrostomy Button Placement in Infants With Cyanotic Versus Acyanotic Congenital Heart Disease.

BACKGROUND: Infants with congenital heart disease (CHD) may exhibit increased metabolic demands, and many will undergo placement of a gastrostomy to achieve adequate nutritional intake. There is a paucity of data, however, comparing the operative risks and overall complications of gastrostomy placement in cyanotic versus acyanotic infants with CHD. We hypothesized that patients with cyanotic CHD would have a higher rate of gastrostomy-associated complications than infants with acyanotic CHD. METHODS: We retrospectively reviewed patients who underwent gastrostomy button placement after cardiac surgery for CHD between 2013 and 2018. Patients were stratified into cyanotic CHD and acyanotic CHD cohorts. Patient data were extracted from the Society of Thoracic Surgeons database and merged with clinical data related to gastrostomy placement and complications from chart review. Unadjusted analyses were used to find covariates associated with cyanotic CHD and acyanotic CHD, using a t-test or Wilcoxon rank-sum test for continuous data, depending on normalcy, and χ2 or Fisher's exact tests for categorical data depending on the distribution. RESULTS: There were 257 infants with CHD who underwent gastrostomy placement during the study period, of which 86 had cyanotic CHD. There were no significant differences in baseline weight or preoperative albumin levels between the two groups. Patients with cyanotic CHD had a lower incidence of comorbid syndromes (P = 0.0001), higher Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery scores (P < 0.0001), and higher postoperative mortality rate (P = 0.0189). There was a higher rate of granulation tissue formation in patients with acyanotic CHD (48.5% versus 22.1%, P < 0.0001). There were no differences in other gastrostomy button-related complications, including leakage, wound infection, or dislodgement. CONCLUSIONS: Patients with acyanotic CHD demonstrated a higher incidence of granulation tissue. We found no difference in gastrostomy-specific complication rates between the two groups, with the notable exception of granulation tissue formation. Based on this study, the diagnosis of cyanotic CHD does not increase the risk of gastrostomy-related complications.

The Nissen Unwrapped: Predictors and Outcomes of Nissen Fundoplication in Congenital Heart Disease.

BACKGROUND: Infants with congenital heart disease (CHD) often require the placement of a gastrostomy button to ensure proper nutrition. Some also require a Nissen fundoplication (NF) to further improve nutrition capabilities in the setting of reflux, however, the clinical and diagnostic imaging characteristics that support NF are variable. The aims of this study were as follows: (1) identify the factors associated with NF in patients with CHD and (2) determine the incidence of NF complications in patients with CHD. METHODS: All patients with CHD who underwent cardiac repair and subsequent creation of a gastrostomy at a single institution between 6/1/2013 and 9/1/2018 were included. We then identified which patients underwent NF. RESULTS: Two-hundred fifty-seven CHD patients who had a gastrostomy button placed after CHD repair, with 17% undergoing a simultaneous NF or an NF at a later time. The presence of acyanotic heart disease, neurologic comorbidities, and vocal cord dysfunction was not univariately associated with a higher likelihood of NF. On multivariable model, only prematurity was significantly associated with NF (P = 0.022). Abnormal findings on imaging studies (upper gastrointestinal series, gastric emptying studies, motility studies, upper endoscopies, swallow studies, and pH probe studies) were not associated with an NF (all P's > 0.05). The overall complication rate was 23%. CONCLUSIONS: Prematurity was the only factor associated with an NF. Surprisingly, cyanotic heart disease, neurologic comorbidities, age at first cardiac surgery, and vocal cord dysfunction were not associated with an NF. We identified an area for quality improvement at our institution given the lack of standardized work-up for the NF in this high-risk population.

Achieving Sustainability in Reducing Unplanned Extubations in a Pediatric Cardiac ICU.

OBJECTIVES: To determine the incidence of unplanned extubations in a pediatric cardiac ICU in order to prove sustainability of our previously implemented quality improvement initiative. Additionally, we sought to identify risk factors associated with unplanned extubations as well as review the overall outcome of this patient population. DESIGN: Retrospective chart review. SETTING: Pediatric cardiac ICU at Children's Hospital of Colorado on the Anschutz Medical Center of the University of Colorado. PATIENTS: Intubated and mechanically ventilated patients in the cardiac ICU from July 2011 to December 2017. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: A total of 2,612 hospitalizations for 2,067 patients were supported with mechanical ventilation. Forty-five patients had 49 episodes of unplanned extubations (four patients > 1 unplanned extubation). The average unplanned extubation rate per 100 ventilator days was 0.4. Patients who had an unplanned extubation were younger (0.09 vs 5.45 mo; p < 0.001), weighed less (unplanned extubation median weight of 3.0 kg [interquartile range, 2.5-4.5 kg] vs control median weight of 6.0 kg [interquartile range, 3.5-13.9 kg]) (p < 0.001), and had a longer length of mechanical ventilation (8 vs 2 d; p < 0.001). Patients who had an unplanned extubation were more likely to require cardiopulmonary resuscitation during their hospital stay (54% vs 18%; p < 0.001) and had a higher likelihood of in-hospital mortality (15% vs 7%; p = 0.001). There was a significant difference in surgical acuity as denoted by The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery score and patients with an unplanned extubation had a higher Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery category (p = 0.019). Contributing factors associated with unplanned extubation were poor endotracheal tube tape integrity, inadequate tube securement, and/or inadequate sedation. A low rate of unplanned extubation was maintained even in the setting of increasing patient complexity and an increase in patient volume. CONCLUSIONS: A low rate of unplanned extubation is sustainable even in the setting of increased patient volume and acuity. Additionally, early identification of patients at higher risk of unplanned extubation may also contribute to decreasing the incidence of unplanned extubation.

Delirium is a Common and Early Finding in Patients in the Pediatric Cardiac Intensive Care Unit.

OBJECTIVE: To determine incidence, associated risk factors, and characteristics of delirium in a pediatric cardiac intensive care unit (CICU). Delirium is a frequent and serious complication in adults after cardiac surgery, but there is limited understanding of its impact in children with critical cardiac disease. STUDY DESIGN: Single-center prospective observational study of CICU patients ≤21 years old. All were screened for delirium using the Cornell Assessment for Pediatric Delirium each 12-hour shift. RESULTS: Ninety-nine patients were included. Incidence of delirium was 57%. Median time to development of delirium was 1 day (95% CI 0, 1 days). Children with delirium were younger (geometric mean age 4 vs 46 months; P < .001), had longer periods of mechanical ventilation (mean 35.9 vs 8.8 hours; P = .002) and had longer cardiopulmonary bypass times (geometric mean 126 vs 81 minutes; P = .001). Delirious patients had longer length of CICU stay than those without delirium (median 3 (IQR 2, 12.5) vs 1 (IQR1, 2) days; P < .0001). A multivariable generalized linear mixed model showed a significant association between delirium and younger age (OR 0.35 for each additional month, 95% CI 0.19, 0.64), need for mechanical ventilation (OR 4.1, 95% CI 1.7, 9.89), and receipt of benzodiazepines (OR 3.78, 95% CI 1.46, 9.79). CONCLUSIONS: Delirium is common in patients in the pediatric CICU and is associated with longer length of stay. There may be opportunities for prevention of delirium by targeting modifiable risk factors, such as use of benzodiazepines.

Morphine Pharmacokinetics in Children With Down Syndrome Following Cardiac Surgery.

OBJECTIVES: To assess if morphine pharmacokinetics are different in children with Down syndrome when compared with children without Down syndrome. DESIGN: Prospective single-center study including subjects with Down syndrome undergoing cardiac surgery (neonate to 18 yr old) matched by age and cardiac lesion with non-Down syndrome controls. Subjects were placed on a postoperative morphine infusion that was adjusted as clinically necessary, and blood was sampled to measure morphine and its metabolites concentrations. Morphine bolus dosing was used as needed, and total dose was tracked. Infusions were continued for 24 hours or until patients were extubated, whichever came first. Postinfusion, blood samples were continued for 24 hours for further evaluation of kinetics. If patients continued to require opioid, a nonmorphine alternative was used. Morphine concentrations were determined using a unique validated liquid chromatography tandem-mass spectrometry assay using dried blood spotting as opposed to large whole blood samples. Morphine concentration versus time data was modeled using population pharmacokinetics. SETTING: A 16-bed cardiac ICU at an university-affiliated hospital. PATIENTS: Forty-two patients (20 Down syndrome, 22 controls) were enrolled. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The pharmacokinetics of morphine in pediatric patients with and without Down syndrome following cardiac surgery were analyzed. No significant difference was found in the patient characteristics or variables assessed including morphine total dose or time on infusion. Time mechanically ventilated was longer in children with Down syndrome, and regarding morphine pharmacokinetics, the covariates analyzed were age, weight, presence of Down syndrome, and gender. Only age was found to be significant. CONCLUSIONS: This study did not detect a significant difference in morphine pharmacokinetics between Down syndrome and non-Down syndrome children with congenital heart disease.

Suspected necrotising enterocolitis after surgery for CHD: an opportunity to improve practice and outcomes.

Infants with CHD are at increased risk of necrotising enterocolitis, which can interfere with the achievement of adequate nutrition and, ultimately, growth and development. Necrotising enterocolitis is classified by severity as suspected, confirmed, and advanced. We sought to quantify the incidence of all types of necrotising enterocolitis among infants who underwent surgery, with a particular focus on suspected necrotising enterocolitis. This is a retrospective review of all infants <6 months of age who underwent cardiac surgery during 2012 and 2013 at Children's Hospital Colorado. We examined the hospital course of 265 hospitalisations (n=251 patients) and found 18 patients (19 hospitalisations) with suspected necrotising enterocolitis and 16 patients (16 hospitalisations) with confirmed or advanced necrotising enterocolitis. Single-ventricle physiology, lower weight, and younger age were associated with necrotising enterocolitis. Patients with all types of necrotising enterocolitis experienced prolonged length of hospital stay. We found suspected necrotising enterocolitis to be as common as confirmed necrotising enterocolitis, and it frequently occurred early in the post-operative course. We speculate that suspected necrotising enterocolitis may often be overlooked in research owing to a reliance on billing codes. Nevertheless, suspected necrotising enterocolitis poses a substantial barrier to post-operative progression of the CHD patient, as does confirmed necrotising enterocolitis. Following the diagnosis of all types of necrotising enterocolitis, there was wide variability in practice patterns. In response to this variability, we developed care guidelines for the diagnosis and treatment of necrotising enterocolitis in this population.

Clinical Associations of Early Dysnatremias in Critically Ill Neonates and Infants Undergoing Cardiac Surgery.

Dysnatremias (DN) are common electrolyte disturbances in cardiac critical illness and are known risk factors for adverse outcomes in certain populations. Little information exists on DN in children with cardiac disease admitted to the cardiac intensive care unit (CICU) after undergoing cardiac surgery, either corrective or palliative. The aim was to determine the incidence and adverse outcomes associated with DN in neonates and infants undergoing cardiac surgery. Retrospective cohort and single center study performed at Children's Hospital Colorado from May 2013 to May 2014, in children under 1 year old admitted to the CICU after undergoing surgery for congenital or acquired cardiac disease. 183 subjects were analyzed. EXCLUSIONS: subjects that demonstrated DN before surgery. Serum sodium levels were recorded for the first 72 h post-operatively. DN was present in 54% of the subjects (98/183): hypernatremia in 60 (33%), hyponatremia in 38 (21%). Multivariate analysis revealed that mild hypernatremia (146-150 mmol/dl) and moderate hypernatremia (151-155 mmol/dl) were associated with longer hospital length of stay (LOS, p < 0.05) and ventilation times (p < 0.05). No association was shown between mild/moderate hyponatremia (125-134 mmol/dl) with either outcome. Hours to DN were significantly lower in hypernatremic (median = 5.8 h) than hyponatremic (median = 43.8 h) patients (p < 0.001). Children younger than 30 days presented DN at an earlier stage than those 31 days-1 year old (median +2.2 vs. 17.3 h). No associations present between DN and the class of diuretic (loop vs. thiazide) administered, or the route of administration (intravenous bolus vs. constant infusion). Total median sodium bicarbonate administration was associated with hypernatremia, as was exposure to vasopressin within the first 72 h post-operatively. Dysnatremias are common in the early post-operative period in neonates and infants undergoing cardiac surgery. Mild to moderate hypernatremia, but not hyponatremia, is associated with longer LOS and longer ventilation time in infants undergoing cardiovascular surgery. Hypernatremia is also associated with younger infants, a higher surgical complexity, administration of bicarbonate and exposure to vasopressin. Diuretic type or interval timing of intravenous delivery did not demonstrate any effect. Prospective studies are needed in this population, in order to determine how DN, particularly hypernatremia, contributes to adverse outcomes, whether this association is independent of illness severity, and what may be safe treatments and interventions for these disorders.

Tachyarrhythmias after the Norwood procedure: relationship and effect of vasoactive agents.

Tachyarrhythmias are common after the Norwood stage I palliation. The effects of vasoactive medications on the development of post-operative tachyarrhythmias have not been studied. The primary objective was to identify associations between postoperative tachyarrhythmias and vasoactive medication usage after Norwood stage 1 palliation. Secondary objectives included evaluation for morbidities of tachyarrhythmias and for associations with anatomical or surgical variables. Retrospective chart review was performed on all patients who underwent the Norwood stage 1 palliation at Children's Hospital Colorado between January 2008 and June 2012. Primary outcomes were development of postoperative tachyarrhythmias and the effects of vasoactive medications. Dopamine, epinephrine, milrinone, and vasopressin duration, cumulative dose, highest dose, and dose at onset of tachyarrhythmia were identified. The effects of surgical variables and anatomy were also studied. Sixty-six patients underwent the Norwood procedure, and 33 (50 %) of these patients had postoperative tachyarrhythmias. Patients with tachyarrhythmias had longer ICU stays (p = 0.02) and hospital stays (p < 0.01), but no change in mortality (p = 1.0). Multivariate Cox regression analysis showed that the right ventricle to pulmonary artery shunt (p < 0.01), longer duration of epinephrine treatment (p = 0.02), and higher milrinone dose (p = 0.002) were associated with tachyarrhythmias. Postoperative tachyarrhythmias are common after the Norwood procedure and are associated with longer ICU and hospital stays. High doses of milrinone, longer duration of epinephrine treatment, and the right ventricle to pulmonary artery shunt were associated with for the development of tachyarrhythmias. Further studies are required to determine the effects of anatomy on post-operative tachyarrhythmias.

Hyponatremia in children with bronchiolitis admitted to the pediatric intensive care unit is associated with worse outcomes.

OBJECTIVE: To characterize the relationship between hyponatremia (serum sodium <135 mEq/L) and clinical outcomes in children ages 1 month to 2 years admitted to the pediatric intensive care unit (PICU) with bronchiolitis. STUDY DESIGN: Single-center retrospective cohort study comprising children who were admitted to the PICU between January 2009 and April 2011. Serum sodium concentrations, collected within the first 2 hours after admission to the PICU, were recorded and associations with clinical outcomes were calculated. Quantitative data are presented as mean ± SD or percentage. Student t-test, Fisher exact test, and χ(2) analyses were performed as appropriate. Subjects were excluded if they were previously diagnosed with chronic disease that would affect initial serum sodium concentration. RESULTS: Children with bronchiolitis were enrolled (n = 102; age = 10.7 ± 6.7 months). Twenty-three patients (22%) were diagnosed with hyponatremia within 2 hours of admission. Mortality (13% vs 0%; P = .011), ventilator time (8.41 ± 2 days vs 4.11 ± 2 days; P = .001), duration of stay in the PICU (10.63 ± 2.5 days vs 5.82 ± 2.09 days; P = .007), and noninvasive ventilator support (65% vs 24%; P = .007) were significantly different between subjects with hyponatremia vs those without. There were no differences in the number of patients with seizures, bronchodilator use, steroid use, intubation requirement, oxygen use at discharge, or hospital readmission. CONCLUSIONS: Pediatric patients diagnosed with bronchiolitis who present with a serum sodium concentration less than 135 mEq/L within 2 hours of admission to the PICU fare worse than their cohorts with normonatremia. A prospective study to evaluate the effects of hyponatremia appears justified.

Kinetics of procalcitonin and C-reactive protein and the relationship to postoperative infection in young infants undergoing cardiovascular surgery.

BACKGROUND: The utility of procalcitonin (PCT) and C-reactive protein (CRP) as infectious biomarkers following infant cardiothoracic surgery is not well defined. METHODS: We designed a prospective cohort study to evaluate PCT and CRP after infant cardiothoracic surgery. PCT and CRP were drawn preoperatively and 24/72 h postoperation or daily in delayed sternal closure patients. Presence of infection within 10 d of surgery, vasoactive-inotropic scores at 24 and 72 h, and length of intubation, intensive care unit stay, and hospital stay were documented. RESULTS: PCT and CRP were elevated at 24 h. PCT then decreased while CRP increased in patients undergoing delayed sternal closure or cardiopulmonary bypass. In the delayed sternal closure group, PCT was significantly higher on postoperative days 2-5 in patients who ultimately developed infection. Higher PCT was independently associated with increased vasoactive-inotropic score at 72 h. CRP did not correlate with infection or postoperative support. CONCLUSION: PCT rises after cardiothoracic surgery in infants but decreases by 72 h while CRP remains elevated. Sternal closure may affect CRP but not PCT. PCT is independently associated with circulatory support requirements at 72 h postoperation and with development of infection. PCT may have greater utility as a biomarker in this population.

Extended survival and re-hospitalisation among paediatric patients requiring extracorporeal membrane oxygenation for primary cardiac dysfunction.

BACKGROUND: Although survival to hospital discharge among children requiring extracorporeal membrane oxygenation support for medical and surgical cardio-circulatory failure has been reported in international registries, extended survival and re-hospitalisation rates have not been well described in the literature. MATERIAL AND METHODS: This is a single-institution, retrospective review of all paediatric patients receiving extracorporeal membrane oxygenation for primary cardiac dysfunction over a 5-year period. RESULTS: A total of 74 extracorporeal membrane oxygenation runs in 68 patients were identified, with a median follow-up of 5.4 years from hospital discharge. Overall, 66% of patients were decannulated alive and 25 patients (37%) survived to discharge. There were three late deaths at 5 months, 20 months, and 6.8 years from discharge. Of the hospital survivors, 88% required re-hospitalisation, with 63% of re-admissions for cardiac indications. The median number of hospitalisations per patient per year was 0.62, with the first re-admission occurring at a mean time of 9 months after discharge from the index hospitalisation. In all, 38% of patients required further cardiac surgery. CONCLUSIONS: Extended survival rates for paediatric hospital survivors of cardiac extracorporeal membrane oxygenation support for medical and post-surgical indications are encouraging. However, re-hospitalisation within the first year following hospital discharge is common, and many patients require further cardiac surgery. Although re-admission hospital mortality is low, longer-term follow-up of quality-of-life indicators is required

Seizures and methemoglobinemia in an infant after excessive EMLA application.

Topical anesthetics are commonly used in many health care settings and for many clinical conditions. However, there are a number of potential adverse effects associated with their use. Their widespread administration can convey a false sense of security and failure to appreciate possible complications. We present the case of an infant with extensive vascular malformations treated with EMLA cream who developed seizures and methemoglobinemia from lidocaine and prilocaine toxicity. We describe the pathophysiology of these morbidities, the use of pulse oximetry in this setting, and the clinical presentation and treatment of methemoglobinemia.

Alkaline phosphatase activity after cardiothoracic surgery in infants and correlation with post-operative support and inflammation: a prospective cohort study.

INTRODUCTION: Limited evidence suggests that serum alkaline phosphatase activity may decrease after cardiac surgery in adults and children. The importance of this finding is not known. Recent studies, however, have identified a potential role for alkaline phosphatase as modulator of inflammation in multiple settings, including during adult cardiopulmonary bypass. We sought to describe the change in alkaline phosphatase activity after cardiothoracic surgery in infants and to assess for a correlation with intensity and duration of post-operative support, markers of inflammation, and short-term clinical outcomes. METHODS: Sub-analysis of a prospective observational study on the kinetics of procalcitonin in 70 infants (≤ 90 days old) undergoing cardiothoracic surgery. Subjects were grouped based on the use of cardiopulmonary bypass and delayed sternal closure. Alkaline phosphatase, procalcitonin, and C-reactive protein (CRP) levels were obtained pre-operation and on post-operative day 1. Mean change in alkaline phosphatase activity was determined in each surgical group. Generalized linear modeling and logistic regression were employed to assess for associations between post-operative alkaline phosphatase activity and post-operative support, inflammation, and short term outcomes. Primary endpoints were vasoactive-inotropic score at 24 hours and length of intubation. Secondary endpoints included procalcitonin/CRP levels on post-operative day 1, length of hospital stay, and cardiac arrest or death. RESULTS: Mean decrease in alkaline phosphatase was 30 U/L (p = 0.01) in the non-bypass group, 114 U/L (p < 0.0001) in the bypass group, and 94 U/L (p < 0.0001) in the delayed sternal closure group. On multivariate analysis, each 10 U/L decrease in alkaline phosphatase activity on post-operative day 1 was independently associated with an increase in vasoactive-inotropic score by 0.7 (p < 0.0001), intubation time by 6% (p < 0.05), hospital stay by 5% (p < 0.05), and procalcitonin by 14% (P < 0.01), with a trend towards increased odds of cardiac arrest or death (OR 1.3; p = 0.06). Post-operative alkaline phosphatase activity was not associated with CRP (p = 0.7). CONCLUSIONS: Alkaline phosphatase activity decreases after cardiothoracic surgery in infants. Low post-operative alkaline phosphatase activity is independently associated with increased procalcitonin, increased vasoactive/inotropic support, prolonged intubation time, and prolonged hospital stay. Alkaline phosphatase may serve as a biomarker and potential modulator of post-operative support and inflammation following cardiothoracic surgery in infants.

Acute kidney injury after cardiac surgery in infants and children: evaluation of the role of angiotensin-converting enzyme inhibitors.

Children with congenital heart disease who undergo cardiac surgery are vulnerable to acute kidney injury (AKI). This study sought to evaluate the role of angiotensin-converting enzyme (ACE) inhibitors and other nephrotoxic medications in the risk for the development of AKI in neonates and children undergoing cardiac surgery. A retrospective review of all patients younger than 2 years admitted to the cardiac intensive care unit after cardiac surgery from March 2007 to September 2008 was conducted. Patients were included in the review if they received furosemide alone or in combination with an ACE inhibitor. Creatinine clearance was calculated, and the patient's maximal degree of AKI was classified by pRIFLE. A P value less than 0.05 was considered significant. Of the 319 patients who met the inclusion criteria, 149 (47%) received furosemide therapy alone and 170 (53%) received a combination of furosemide and an ACE inhibitor. Patients in the furosemide-only group (age, 5 months) were older than the patients who received both furosemide and an ACE inhibitor (age, 3.8 months; P = 0.024). Despite statistically higher Aristotle scores in the ACE-inhibitor group, the intraoperative variables did not differ between the two groups. Postoperatively, the ACE-inhibitor group had a decreased creatinine clearance (55.3 ml/min/1.73 m(2)) compared with the furosemide group (64.4 ml/min/1.73 m(2); P = 0.015) and an increased incidence of a pRIFLE maximal score of "F" (odds ratio [OR], 1.75; P = 0.033). However, after adjustment for additional risk factors, no difference in the occurrence of AKI resulted (OR, 0.939; P = 0.85) when patients received an ACE inhibitor. More than half of the study population received ACE inhibitors, but this treatment was not associated with an increase in AKI.

Oral feeding outcomes in neonates with congenital cardiac disease undergoing cardiac surgery.

BACKGROUND: Achievement of adequate oral nutrition is a challenging task after early neonatal cardiac surgery. This study aims to describe predictors of oral feeding outcomes for neonates after early surgical interventions. MATERIALS AND METHODS: A retrospective review of neonates admitted with congenital cardiac disease over a period of 1 year. We analysed predictors of the need for a feeding tube at discharge and the amount taken at each feeding. Multilevel modelling was used to look at individual change over time predicting oral amount at each feeding. RESULTS: We identified 56 neonates. Diagnoses were heterogeneous; 23% of the infants had associated genetic syndromes and 45% required pre-operative mechanical ventilation. The median time from birth to surgery was 8.4 days, with 29 infants fed orally before surgery. The mean time from surgery to first oral feeding attempt was 12 hours. Time from surgery to oral feeding, the amount taken with first feeding, and cross-clamp times were significant predictors of oral feeding success, whereas the presence of a comorbidity--genetic abnormality--and longer ventilator dependency predicted failure. Almost half of the neonates required a feeding tube upon discharge, and no infant discharged was solely breastfed. Discharge with a feeding tube was associated with greater weight gain at that time. CONCLUSIONS: Neonates with congenital cardiac disease face significant barriers to successfully achieving oral feeding on hospital discharge. Enteral feeding guidelines focus on physiological stabilisation and do not always address the developmental milestones necessary to support oral feeding. Future prospective studies are necessary to identify multimodal strategies to optimise early feeding.

Enteral feeding in neonates with prostaglandin-dependent congenital cardiac disease: international survey on current trends and variations in practice.

BACKGROUND: The benefits of early enteral feeding in neonates are well known and yet the optimal pre-operative nutrition of prostaglandin-dependent infants with congenital cardiac disease remains ill-defined. This survey delineates international nutritional practices and trends with this population. MATERIALS AND METHODS: Paediatric practitioners responded to an Internet-based survey that explored assessment of feeding practices, criteria for feeding readiness, medication dosing, concurrent feeding with umbilical catheters, and the observed incidence of feeding intolerance. Documented nutritional strategies were not correlated with patient outcomes. RESULTS: A total of 200 caregivers responded to the survey. Fewer United States caregivers (56%) reported routine pre-operative enteral feeding in prostaglandin-dependent infants when compared with caregivers outside the United States of America (93%). Of those respondents willing to feed, approximately two-thirds did not base their decision on the ductal flow direction. Numerous and heterogeneous parameters were reported to assess feeding readiness. Many caregivers report scepticism with regard to enteral feeding safety in neonates with an umbilical artery catheter, and to a lesser extent in the presence of an umbilical venous catheter. In summary, there is a prevailing lack of consensus regarding pre-operative enteral nutrition to prostaglandin-dependent neonates. CONCLUSIONS: This survey demonstrates noticeable variations in pre-operative nutritional practices between providers from around the world. Arguments that support or refute this practice have little support in the medical literature. Future studies should aim to demonstrate the safety of such practice and compare the outcomes of prostaglandin-dependent neonates who were pre-operatively enterally fed with those who were not.

Coarctation of aorta and vein of Galen aneurysmal malformation in a neonate.

PURPOSE OF REVIEW: A case report of a newborn with coarctation of aorta and severe vein of Galen malformation. RECENT FINDINGS: This case report reviews literature on congenital heart disease and intracranial arteriovenous malformations. Emphasis is laid on diagnostic challenges and management options. SUMMARY: Congenital heart diseases with arteriovenous malformations carry a high risk of mortality. Thorough physical examination and high index of suspicion can lead to early diagnosis, and interdisciplinary coordination leads to better outcomes.

Propofol as a bridge to extubation for high-risk children with congenital cardiac disease.

BACKGROUND: Children with congenital cardiac defects may have associated chromosomal anomalies, airway compromise, and/or pulmonary hypertension, which can pose challenges to adequate sedation, weaning from mechanical ventilation, and successful extubation. Propofol, with its unique properties, may be used as a bridge to extubation in certain cardiac populations. MATERIALS AND METHODS: We retrospectively reviewed 0-17-year-old patients admitted to the Cardiac Intensive Care Unit between January, 2007 and September, 2008, who required mechanical ventilation and received a continuous infusion of propofol as a bridge to extubation. Medical charts were reviewed for demographics, associated comorbidities, as well as additional sedation medications and haemodynamic trends including vital signs and vasopressor support during the peri-infusion period. Successful extubation was defined as no re-intubation required for respiratory failure within 48 hours. Outcomes measured were successful extubation, evidence for propofol infusion syndrome, haemodynamic stability, and fluid and inotropic requirements. RESULTS: We included 11 patients for a total of 12 episodes. Propofol dose ranged from 0.4 to 5.6 milligram per kilogram per hour with an average infusion duration of 7 hours. All patients were successfully extubated, and none demonstrated worsening metabolic acidosis suggestive of the propofol infusion syndrome. All patients remained haemodynamically stable during the infusion with average heart rates and blood pressures remaining within age-appropriate ranges. One patient received additional fluid but no increase in vasopressors was needed. CONCLUSIONS: This study suggests that propofol infusions may allow for successful extubation in a certain population of children with congenital cardiac disease. Further studies are required to confirm whether propofol is an efficient and safe alternative in this setting.

The use of Arginine Vasopressin in neonates following the Norwood procedure.

BACKGROUND: Following the Norwood palliation, neonates may require an escalation of inotropic and vasoactive support. Arginine Vasopressin may be uniquely useful in supporting this population. MATERIALS AND METHODS: A retrospective evaluation of neonates at this institution between November, 2007 and October, 2010 who received Arginine Vasopressin following the Norwood procedure. Data were recorded from the patient records at one hour prior to, and then 1, 2, 3, 4, 6, and 24 hours following Arginine Vasopressin initiation. RESULTS: We included 28 neonates. The mean dose of Arginine Vasopressin was 0.0005 plus or minus 0.0003 units per kilogram per minute. There was an early response (less than 6 hours) characterised by an 8% increase in systolic blood pressure (p = 0.0004), a 100% increase in urine output (p = 0.02), and a 29% decrease in total fluid administration (p = 0.04). The late response (at 24 hours) revealed further increases in systolic blood pressure and urine output as well as a 53% decrease in serum lactate (p = 0.007) and increase in arterial pH from 7.36 to 7.45 (p less than 0.0001). These changes were not accompanied by increases in heart rate or inotrope score. CONCLUSIONS: The initiation of Arginine Vasopressin in post-operative Norwood patients was temporally associated with an improvement in markers of perfusion including systolic blood pressure, urine output, lactate, and pH. Further studies are required to ascertain the efficacy of Arginine Vasopressin in this population.