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Introduction: Extracorporeal membrane oxygenation (ECMO) is an invaluable rescue technique for critically ill children with imminent or present cardiopulmonary collapse. However, medical team expertise to optimize results and decrease complications is scarce. Telemedicine can be used to enhance the delivery of quality interventions., Materials and Methods: This is a retrospective review of pediatric patients assisted with ECMO in the cardiac intensive care unit (CICU) at Fundación Cardiovascular de Colombia from July 2011 to June 2015 (telemedicine) compared with similar patients from a previous period (pretelemedicine). Collected information included demographic data, cardiac diagnosis, risk adjustment for congenital heart surgery (RACHS-1), hospital mortality, CICU and hospital length of stay (LOS), ECMO type, and ECMO run hours as well as specific telemedicine information., Results: Fifty-seven patients in the pretelemedicine and 109 in the telemedicine periods were included in the analysis. Forty-nine teleconsulted patients received 218 teleconsultations, with a recommendation for diagnostic or interventional catheterization in 38 patients (77.5%). A surgical procedure for significant residual lesions was recommended in 30 patients (61.2%). Patients in the telemedicine period were older (4.7 months vs. 1.6 months, p = 0.006), more likely to receive operating room ECMO (43.1% vs. 24.6%, p = 0.02), and had a higher proportion of patients with two-ventricle physiology (73.4% vs. 54.4%, p = 0.013). Hospital survival was higher during the telemedicine period (54.1% vs. 29.8%, p = 0.002), with a longer hospital LOS (67 days vs. 28 days, p < 0.001)., Conclusion: The implementation of telemedicine-assisted interventions in a pediatric ECMO program delivered valuable diagnostic and therapeutic advice, was associated with significant changes in selection criteria and model of care, and an increased hospital survival.

Background: Newborns with congenital heart disease have associated brain damage that affects short-and long-term neurodevelopment. Several neuronal biomarkers exist that could predict brain damage. We investigated the pattern of neuron-specific enolase (NSE) and s100B levels after cardiopulmonary bypass surgery in neonates with congenital heart disease., Methods: We completed a prospective observational study of neonates with congenital heart disease who were undergoing cardiopulmonary bypass surgery. NSE and s100B levels were measured from serum samples obtained preoperatively, immediately postoperatively, and once daily on postoperative days one to seven. Cranial ultrasounds were obtained preoperatively and postoperatively and findings were scored using an internally developed scoring system., Results: Eighteen neonates were included. Immediate postoperative and peak levels of both NSE (58.0 [21.6] and 68.1 [55.7] μg/L) and s100B (0.14 [0.3] and 0.14 [0.3] μg/L) were significantly increased when compared with preoperative levels (34.0 [21.6] μg/L; P < 0.01 and 0.08 [0.1] μg/L; P < 0.02). By postoperative day seven, NSE and s100B levels were lower than preoperative levels: NSE (18 [5.7]; P = 0.09) and s100B (0.03 [0.05]; P < 0.01). Postoperative s100B levels were negatively correlated with age at surgery and positively correlated with circulatory arrest time. Although there was no significant correlation between either NSE or s100B levels and intensive care unit length of stay, hospital length of stay, and pediatric cerebral performance category score, there was a negative correlation between postoperative levels of NSE and ventriculomegaly., Conclusions: NSE and s100B levels increase after bypass surgery and return below preoperative baseline levels by postoperative day seven. The levels of s100B were positively correlated with circulatory arrest time and negatively correlated with age at time of surgery. This finding may be supportive of pre-existing prenatal brain injury that could be enhanced by longer surgical times but also of some brain protection effect associated with longer wait until surgery., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Objective: To test for associations between abnormal respiratory ciliary motion (CM) and brain abnormalities in infants with congenital heart disease (CHD) STUDY DESIGN: We recruited 35 infants with CHD preoperatively and performed nasal tissue biopsy to assess respiratory CM by videomicroscopy. Cranial ultrasound scan and brain magnetic resonance imaging were obtained pre- and/or postoperatively and systematically reviewed for brain abnormalities. Segmentation was used to quantitate cerebrospinal fluid and regional brain volumes. Perinatal and perioperative clinical variables were collected., Results: A total of 10 (28.5%) patients with CHD had abnormal CM. Abnormal CM was not associated with brain injury but was correlated with increased extraaxial cerebrospinal fluid volume (P < .001), delayed brain maturation (P < .05), and a spectrum of subtle dysplasia including the hippocampus (P < .0078) and olfactory bulb (P < .034). Abnormal CM was associated with higher composite dysplasia score (P < .001), and both were correlated with elevated preoperative serum lactate (P < .001)., Conclusions: Abnormal respiratory CM in infants with CHD is associated with a spectrum of brain dysplasia. These findings suggest that ciliary defects may play a role in brain dysplasia in patients with CHD and have the potential to prognosticate neurodevelopmental risks., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Objective: Efficient diagnosis of an underlying genetic aetiology in a patient with congenital heart disease is essential to optimising clinical care. Copy number variants are one aetiology of congenital heart disease; the majority are identifiable by targeted fluorescence in situ hybridisation or array comparative genomic hybridisation, not by classical cytogenetic analysis. This study assessed the utility of array comparative genomic hybridisation as a first-tier diagnostic test for neonates with congenital heart disease. Study design A prospective chart review of neonates with congenital heart disease in the Cardiac Intensive Care Unit at Children's Hospital of Pittsburgh of UPMC was performed. Patients were tested by array comparative genomic hybridisation and classical cytogenetic analysis simultaneously. Data collected included all chromosome abnormalities detected, physical examination findings, and imaging results. McNemar's test was used to compare detection of array comparative genomic hybridisation and classical cytogenetic analysis., Results: Of 45 patients, three (6.7%) had an abnormality detected by classical cytogenetic analysis and an additional 10 (22.2%) had a copy number variant detected by array comparative genomic hybridisation, highlighting an increased detection rate (p=0.008). Several of these copy number variants had unclear clinical significance, requiring additional investigation. The prevalence of dysmorphology and/or comorbidity in this population was 72%. Identification of dysmorphic features was greater when assessed by a geneticist than by providers of different subspecialties., Conclusions: Array comparative genomic hybridisation has significant clinical utility as a first-tier test in this population, but it carries the potential for incidental findings and results of uncertain clinical significance. Collaboration between cardiologists and medical geneticists is essential to providing optimal clinical care.

Objectives: In children with cardiac disease, common indications for extracorporeal membrane oxygenation (ECMO) include refractory cardiopulmonary resuscitation (E-CPR), failure to separate from cardiopulmonary bypass (OR-ECMO), and low cardiac output syndrome (LCOS-ECMO). Despite established acceptance, ECMO outcomes are suboptimal with a survival between 38% and 55%. We evaluated factors associated with significantly increased survival in cardiac patients requiring ECMO., Methods: We conducted a retrospective investigation of consecutive patients undergoing ECMO between 2006 and 2010. Demographic, pre-ECMO, ECMO, and post-ECMO parameters were analyzed. Neurologic outcomes were assessed with the pediatric overall performance category scale at the latest follow-up., Results: There were 3524 admissions, 95 (3%) of which necessitated ECMO; 40 (42%) E-CPR, 31 (33%) OR-ECMO, and 24 (25%) LCOS-ECMO. The overall hospital survival was 73%. The within-groups hospital survival was 75% in E-CPR, 77% OR-ECMO and 62% LCOS-ECMO. In the multivariable logistic regression analysis, chromosomal anomalies (odds ratio [OR], 8; 95% confidence interval [CI], 2-35), single ventricle (OR ,6; 95% CI, 3-33), multiple ECMO runs (OR, 15; 95% CI, 4-42), higher 24-hour ECMO flows (OR, 8; 95% CI, 4-22), decreased lung compliance (OR, 5; 95% CI, 2-16), and need for plasma exchange (OR, 5; 95% CI, 3-18) were all significant factors associated with mortality. From the univariate analysis, a common parameter associated with mortality within all groups was intracranial hemorrhage. At 1.9 years (0.9, 2.9) of follow-up, 66% were still alive, and 89% of survivors had normal function or only mild neurodevelopmental disability., Conclusions: ECMO was successfully used in children with cardiac disease with 73% and 66% short- and intermediate-term survival, respectively. The majority of the survivors had normal function or only a minimal neurodevelopmental deficit., (Copyright © 2013 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Objective.  Infants with critical congenital heart disease (CHD) can have genetic and other extracardiac malformations, which add to the short- and long-term risk of morbidity and perhaps mortality. We sought to examine our center's practice of screening for extracardiac anomalies and to determine the yield of these tests among specific cardiac diagnostic categories. Design.  Retrospective review of infants admitted to the cardiac intensive care unit with a new diagnosis of CHD. Subjects were categorized into six groups: septal defects (SD), conotruncal defects (CTD), single-ventricle physiology (SV), left-sided obstructive lesions (LSO), right-sided obstructive lesions (RSO), and "other" (anomalous pulmonary venous return, Ebstein's anomaly). Screening modalities included genetic testing (karyotype and fluorescent in situ hybridization for 22q11.2 deletion), renal ultrasound (RUS), and head ultrasound (HUS). Results.  One hundred forty-one patients were identified. The incidence of cardiac anomalies was: CTD (36%), SD (18%), SV (18%), LSO (14%), RSO (3%), and "other" (8%). Overall 14% had an abnormal karyotype, 5% had a deletion for 22q11.2, 28% had an abnormal RUS and 22% had abnormal HUS. Patients in SD and SV had the highest incidence of abnormal karyotype (36% and 17%); 22q11.2 deletion was present only in CTD and LSO groups (9% and 7%, respectively); abnormal RUS and HUS were seen relatively uniformly in all categories. Premature infants had significantly higher incidence of renal 43% vs. 24%, and intracranial abnormalities 46% vs. 16%. Conclusion.  Infants with critical CHD and particularly premature infants have high incidence of genetic and other extracardiac anomalies. Universal screening for these abnormalities with ultrasonographic and genetic testing maybe warranted because early detection could impact short and long-term outcomes., (© 2011 Wiley Periodicals, Inc.)

Objective: Advancements in the preoperative management of patients with single-ventricle physiology continue to evolve. Previous reports have questioned the benefit of using inhaled nitrogen in single-ventricle patients, suggesting that this therapeutic modality may not provide adequate systemic cardiac output. The objective of this study was to review our institutional experience managing preoperative patients with single-ventricle physiology using a combination of afterload reduction and inhaled hypoxemic therapy., Design, Setting, and Patients: This is a retrospective review of 49 consecutive single-ventricle patients admitted preoperatively between July 2004 and January 2009, to the cardiac intensive care unit at Children's Hospital of Pittsburgh who underwent single-ventricle palliation, and treated preoperatively with milrinone and inhaled nitrogen. Therapeutic interventions and indirect indicators of cardiac output were collected on day of admission (time 0) and compared with those collected on the morning of surgery (time 1); data included clinical assessment, hemodynamic measurements, and laboratory values., Results: When comparing time 0 to time 1, there was a statistically significant decrease in lactate (from 2.2 to 1.8 mEq/L [P < 0.001]) and an increase in pH (from 7.36 to 7.41 [P < 0.001]), serum bicarbonate (from 24.16 to 27.55 mmol/L [P < 0.001]) and arterial PaO2 (from 38.10 to 41.82 mm Hg [P = 0.027]). Preoperatively, there were no deaths, and only two patients had an evidence of multiorgan dysfunction on day of surgery (time 1)., Conclusion: Our results suggest that a combination of afterload reduction and hypoxemic therapy was able to maintain an appropriate distribution of the cardiac output in the majority of preoperative patients with single-ventricle physiology. An adequate balance of systemic and pulmonary blood flow was successfully achieved with an increase in arterial PaO2 values., (© 2011 Wiley Periodicals, Inc.)

Objective: Assessment of electrocardiographic (ECG) effects of dexmedetomidine., Design: Prospective observational study including children 0-17 years of age with congenital heart disease (CHD) and children following cardiothoracic surgery. Patients who did not receive dexmedetomidine were used as a control group. All patients had two ECGs: one baseline, pre-dexmedetomidine (T1) and one during dexmedetomidine infusion (T2)., Measurements and Results: Fifty-one patients, median age of 0.5 years (IQR = 3.4), and 25 patients, age 0.25 (IQR = 2.9), were included in the dexmedetomidine and control groups, respectively. Forty received a dexmedetomidine-loading dose of 1 microg/kg (IQR = 0.5). At T2, the dexmedetomidine infusion was 1 microg/kg/h (IQR = 0.5). In the dexmedetomidine group, heart rate (HR) decreased from 140 +/- 22 to 115 +/- 23 (P < 0.001); PR, PRc and PR index changed from 115 +/- 28 to 122 +/- 29 ms (P = 0.01), 174 +/- 38 to 167 +/- 35 ms (P = 0.07) and 15,882 +/- 3,565 to 13,792 +/- 3,311 (P < 0.001), respectively. QRS decreased from 84 +/- 21 to 80 +/- 21 ms (P = 0.02), and QTc had no change (433 +/- 47 to 435 +/- 36 ms). When compared to the control group, none of the ECG intervals had any difference other than a trend towards lower HR (P = 0.08). Neonates and infants had a bigger drop in the HR compared to older children (P < 0.001), while other parameters were similar. At T2 none of the dexmedetomidine group patients had atrioventricular block or other arrhythmia. Four patients in the control group had accelerated junctional rhythm., Conclusions: Use of dexmedetomidine in patients with CHD and patients following cardiothoracic surgery is not associated with any significant ECG interval abnormalities other than a trend towards lower HR.

Objective: To assess clinical response of dexmedetomidine alone or in combination with conventional sedatives/analgesics after cardiac surgery., Design: Retrospective study., Setting: Pediatric cardiac intensive care unit., Patients: Infants and neonates after cardiac surgery., Measurements and Main Results: We identified 80 patients including 14 neonates, at mean age and weight of 4.1 +/- 3.1 months and 5.5 +/- 2 kg, respectively, who received dexmedetomidine for 25 +/- 13 hours at an average dose of 0.66 +/- 0.26 microgxkgxhr. Overall normal sleep to moderate sedation was documented 94% of the time and no pain to mild pain for 90%. Systolic blood pressure (SBP) decreased from 89 +/- 15 mm Hg to 85 +/- 11 mm Hg (p = .05), heart rate (HR) from 149 +/- 22 bpm to 129 +/- 16 bpm (p < .001), and respiratory rate (RR) remained unchanged. When baseline arterial blood gases were compared with the most abnormal values, pH decreased from 7.4 +/- 0.07 to 7.37 +/- 0.05 (p = .006), Po2 from 91 +/- 67 mm Hg to 66 +/- 29 mm Hg (p = .005), and CO2 increased from 45 +/- 8 mm Hg to 50 +/- 12 mm Hg (p = .001). At the beginning of the study, 37 patients (46%) were mechanically ventilated; and at 48 hours, 13 patients (16%) were still intubated and five patients failed extubation. Three groups of patients were identified: A, dexmedetomidine only (n = 20); B, dexmedetomidine with sedatives/analgesics (n = 38); and C, dexmedetomidine with both sedatives/analgesics and fentanyl infusion (n = 22). The doses of dexmedetomidine and rescue sedatives/analgesics were not significantly different among the three groups but duration of dexmedetomidine was longer in group C vs. A (p = .03) and C vs. B (p = .002). Pain, sedation, SBP, RR, and arterial blood gases were similar. HR was higher in group C vs. B (p = .01). Comparison between neonates and infants showed that infants required higher dexmedetomidine doses, 0.69 +/- 25 microgxkgxhr, and vs. 0.47 +/- 21 microgxkgxhr (p = .003) and had lower HR (p = .01), and RR (p = .009), and higher SBP (p < .001)., Conclusions: Dexmedetomidine use in infants and neonates after cardiac surgery was well tolerated in both intubated and nonintubated patients. It provides an adequate level of sedation/analgesia either alone or in combination with low-dose conventional agents.

Background: The use of large-dose aprotinin during cardiopulmonary bypass (CPB) in adult patients has been linked to postoperative renal dysfunction, but its effect on the pediatric population undergoing complex congenital cardiac operations is not well defined., Methods: We used a retrospective cohort analysis to evaluate children undergoing cardiac surgery requiring CPB between July 2004 and July 2006. Demographic data and surgical risk quantified by the Aristotle surgical complexity level were analyzed as covariates. Renal dysfunction was defined according to the RIFLE criteria, an international consensus classification which defines three grades of increasing severity of acute kidney injury: risk (Class R), injury (Class I), and failure (Class F) based on serum creatinine values. A univariate and multivariate logistic regression analysis and a propensity score were used to analyze the data. The propensity score was developed using pretreatment covariates associated with the administration of aprotinin. A multivariate logistic regression was then used with the propensity score and intraoperative measures as covariates. A P value <0.05 was considered statistically significant., Results: Among 395 patients who underwent cardiac surgery, 55% received aprotinin and 45% did not. Thirty-one percent of the cohort had previous cardiac surgery; 17% were neonates. According to the RIFLE criteria, 80 of the patients (20.3%) had acute kidney injury in the postoperative period; 53 (13.4%) had risk of renal dysfunction with 23 (5.8%) having injury and four patients (0.7%) having failure. Those receiving aprotinin had a higher incidence of previous cardiac surgery (54.1% vs 5%), sepsis (6.9% vs. 0.0%), heart failure (24.8% vs 12.4%), mechanical ventilation (25.2% vs 2.8%), or mechanical circulatory support (6.0% vs. 0.6%). More patients had an Aristotle level of 4 (26.6% vs 2.8%) and were treated with diuretics (63.8% vs 26.6%), angiotensin converting enzyme inhibitors (21.1% vs 7.9%), milrinone (25.7% vs 4.5%), and inotropic support (16.1% vs 2.3%). Although there was a significant difference in the unadjusted risk of renal dysfunction, adjustment with the preoperative propensity score revealed that there was no association between aprotinin and renal dysfunction (OR 1.32; 95% CI 0.55-3.19). The duration of CPB was the only independent variable associated with the development of renal dysfunction (OR 1.0; 95% CI 1.009-1.014)., Conclusions: Patients who receive aprotinin are more likely to present with preoperative risk factors for the development of postoperative renal dysfunction. However, when associated risk factors are properly considered, the use of aprotinin does not seem to be associated with a higher risk of developing renal dysfunction in the immediate postoperative period in children.

Objective: In this report, we describe the use of dexmedetomidine as the primary sedative agent while performing invasive procedures in infants and toddlers with congenital heart disease who are breathing spontaneously., Design: Retrospective case review., Setting: University Hospital, pediatric cardiac intensive care unit., Patients: Six spontaneously breathing children, five infants and one toddler, all with congenital heart disease, who received dexmedetomidine as the primary sedative agent while undergoing an invasive procedure., Interventions: None., Measurements and Main Results: Six patients with congenital heart disease, age 3 days-29 months were included. Five of the patients were <6 months of age. Each patient underwent an invasive procedure including central venous line placement, chest tube insertion, fiberoptic bronchoscopy, and femoral cut-down for Broviac placement. All patients were breathing spontaneously throughout their procedure. Dexmedetomidine was used as the primary sedative agent during the procedure with additional sedation provided with low dose ketamine for patient movement in three of the six patients. The average dexmedetomidine dose used was 1.5 microg/kg (1-3 microg/kg). An additional low dose of ketamine, 0.7 mg/kg (0.3-1.5 mg/kg), was used in 50% of the patients. All patients breathed spontaneously without significant desaturation throughout the procedure, and although there was a trend toward lower blood pressure and heart rate, all patients remained warm and well perfused. Each of the six procedures was successfully completed without any associated complications., Conclusions: Our experience suggests that invasive procedures can be successfully performed in spontaneously breathing infants and toddlers with congenital heart disease using dexmedetomidine alone or in combination with low dose ketamine.