Neurodevelopmental Outcomes for Individuals With Congenital Heart Disease: Updates in Neuroprotection, Risk-Stratification, Evaluation, and Management: A Scientific Statement From the American Heart Association.

Over the past decade, new research has advanced scientific knowledge of neurodevelopmental trajectories, factors that increase neurodevelopmental risk, and neuroprotective strategies for individuals with congenital heart disease. In addition, best practices for evaluation and management of developmental delays and disorders in this high-risk patient population have been formulated based on literature review and expert consensus. This American Heart Association scientific statement serves as an update to the 2012 statement on the evaluation and management of neurodevelopmental outcomes in children with congenital heart disease. It includes revised risk categories for developmental delay or disorder and an updated list of factors that increase neurodevelopmental risk in individuals with congenital heart disease according to current evidence, including genetic predisposition, fetal and perinatal factors, surgical and perioperative factors, socioeconomic disadvantage, and parental psychological distress. It also includes an updated algorithm for referral, evaluation, and management of individuals at high risk. Risk stratification of individuals with congenital heart disease with the updated categories and risk factors will identify a large and growing population of survivors at high risk for developmental delay or disorder and associated impacts across the life span. Critical next steps must include efforts to prevent and mitigate developmental delays and disorders. The goal of this scientific statement is to inform health care professionals caring for patients with congenital heart disease and other key stakeholders about the current state of knowledge of neurodevelopmental outcomes for individuals with congenital heart disease and best practices for neuroprotection, risk stratification, evaluation, and management.

Defining the optimal historical control group for a phase 1 trial of mesenchymal stromal cell delivery through cardiopulmonary bypass in neonates and infants.

OBJECTIVE: The Mesenchymal Stromal Cell Delivery through Cardiopulmonary Bypass in Pediatric Cardiac Surgery study is a prospective, open-label, single-centre, dose-escalation phase 1 trial assessing the safety/feasibility of delivering mesenchymal stromal cells to neonates/infants during cardiac surgery. Outcomes will be compared with historical data from a similar population. We aim to define an optimal control group for use in the Mesenchymal Stromal Cell Delivery through Cardiopulmonary Bypass in Pediatric Cardiac Surgery trial. METHODS: Consecutive patients who underwent a two-ventricle repair without aortic arch reconstruction within the first 6 months of life between 2015 and 2020 were studied using the same inclusion/exclusion criteria as the Phase 1 Mesenchymal Stromal Cell Delivery through Cardiopulmonary Bypass in Pediatric Cardiac Surgery trial (n = 169). Patients were allocated into one of three diagnostic groups: ventricular septal defect type, Tetralogy of Fallot type, and transposition of the great arteries type. To determine era effect, patients were analysed in two groups: Group A (2015-2017) and B (2018-2020). In addition to biological markers, three post-operative scoring methods (inotropic and vasoactive-inotropic scores and the Pediatric Risk of Mortality-III) were assessed. RESULTS: All values for three scoring systems were consistent with complexity of cardiac anomalies. Max inotropic and vasoactive-inotropic scores demonstrated significant differences between all diagnosis groups, confirming high sensitivity. Despite no differences in surgical factors between era groups, we observed lower inotropic and vasoactive-inotropic scores in group B, consistent with improved post-operative course in recent years at our centre. CONCLUSIONS: Our studies confirm max inotropic and vasoactive-inotropic scores as important quantitative measures after neonatal/infant cardiac surgery. Clinical outcomes should be compared within diagnostic groupings. The optimal control group should include only patients from a recent era. This initial study will help to determine the sample size of future efficacy/effectiveness studies.

Impact of Cardiopulmonary Bypass on Neurogenesis and Cortical Maturation.

OBJECTIVE: Neurodevelopmental delays and frontal lobe cortical dysmaturation are widespread among children with congenital heart disease (CHD). The subventricular zone (SVZ) is the largest pool of neural stem/progenitor cells in the postnatal brain. Our aim is to determine the effects of cardiopulmonary bypass (CPB) on neurogenesis and cortical maturation in piglets whose SVZ development is similar to human infants. METHODS: Three-week-old piglets (n = 29) were randomly assigned to control (no surgery), mild-CPB (34°C full flow for 60 minutes) and severe-CPB groups (25°C circulatory-arrest for 60 minutes). The SVZ and frontal lobe were analyzed with immunohistochemistry 3 days and 4 weeks postoperatively. MRI of the frontal lobe was used to assess cortical development. RESULTS: SVZ neurogenic activity was reduced up to 4 weeks after both mild and severe CPB-induced insults. CPB also induced decreased migration of young neurons to the frontal lobe, demonstrating that CPB impairs postnatal neurogenesis. MRI 4 weeks after CPB displayed a decrease in gyrification index and cortical volume of the frontal lobe. Cortical fractional anisotropy was increased after severe CPB injury, indicating a prolonged deleterious impact of CPB on cortical maturation. Both CPB-induced insults displayed a significant change in densities of three major inhibitory neurons, suggesting excitatory-inhibitory imbalance in the frontal cortex. In addition, different CPB insults altered different subpopulations of inhibitory neurons. INTERPRETATION: Our results provide novel insights into cellular mechanisms contributing to CHD-induced neurological impairments. Further refinement of CPB hardware and techniques is necessary to improve long-term frontal cortical dysmaturation observed in children with CHD. ANN NEUROL 2021;90:913-926.

The evolution of cardiac care for children in Washington, DC.

Cardiac surgery for CHD was pioneered in Washington, DC by Charles Hufnagel and Edgar Davis working at Georgetown University and Children's Hospital of the District of Columbia. Children's Hospital, now Children's National Hospital, had been established just 5 years after the end of the Civil War. In the 1950s, Davis and Hufnagel undertook many open-heart operations using the technique of surface cooling, hypothermia, and circulatory arrest. Hufnagel and Lewis Scott, who founded the cardiology department at Children's, were trained in Boston by Gross and Nadas. Judson Randolph, also a trainee of Gross, introduced cardiac surgery using cardiopulmonary bypass and established the General Pediatric Surgery department at Children's in the 1960s. The transition of hospital staffing from community-based private physicians to full-time hospital employees was often controversial but was complete by the turn of the millennium. The 21st century has seen continuing growth of the new Children's National Heart Institute and consolidation of several congenital cardiac programmes in Washington, DC.

Neurodevelopmental Abnormalities and Congenital Heart Disease: Insights Into Altered Brain Maturation.

In the past 2 decades, it has become evident that individuals born with congenital heart disease (CHD) are at risk of developing life-long neurological deficits. Multifactorial risk factors contributing to neurodevelopmental abnormalities associated with CHD have been identified; however, the underlying causes remain largely unknown, and efforts to address this issue have only recently begun. There has been a dramatic shift in focus from newly acquired brain injuries associated with corrective and palliative heart surgery to antenatal and preoperative factors governing altered brain maturation in CHD. In this review, we describe key time windows of development during which the immature brain is vulnerable to injury. Special emphasis is placed on the dynamic nature of cellular events and how CHD may adversely impact the cellular units and networks necessary for proper cognitive and motor function. In addition, we describe current gaps in knowledge and offer perspectives about what can be done to improve our understanding of neurological deficits in CHD. Ultimately, a multidisciplinary approach will be essential to prevent or improve adverse neurodevelopmental outcomes in individuals surviving CHD.

Effect of Obesity and Underweight Status on Perioperative Outcomes of Congenital Heart Operations in Children, Adolescents, and Young Adults: An Analysis of Data From the Society of Thoracic Surgeons Database.

BACKGROUND: Extreme body mass index (BMI; either very high or very low) has been associated with increased risk of adverse perioperative outcome in adults undergoing cardiac surgery. The effect of BMI on perioperative outcomes in congenital heart disease patients has not been evaluated. METHODS: A multicenter retrospective cohort study was performed studying patients 10 to 35 years of age undergoing a congenital heart disease operation in the Society of Thoracic Surgeons Congenital Heart Surgery Database between January 1, 2010, and December 31, 2015. The primary outcomes were operative mortality and a composite outcome (1 or more of operative mortality, major adverse event, prolonged hospital length of stay, and wound infection/dehiscence). The associations between age- and sex-adjusted BMI percentiles and these outcomes were assessed, with adjustment for patient-level risk factors, with multivariate logistic regression. RESULTS: Of 18 337 patients (118 centers), 16% were obese, 15% were overweight, 53% were normal weight, 7% were underweight, and 9% were severely underweight. Observed risks of operative mortality (P=0.04) and composite outcome (P<0.0001) were higher in severely underweight and obese subjects. Severely underweight BMI was associated with increased unplanned cardiac operation and reoperation for bleeding. Obesity was associated with increased risk of wound infection. In multivariable analysis, the association between BMI and operative mortality was no longer significant. Obese (odds ratio, 1.28; P=0.008), severely underweight (odds ratio, 1.29; P<0.0001), and underweight (odds ratio, 1.39; P=0.002) subjects were associated with increased risk of composite outcome. CONCLUSIONS: Obesity and underweight BMI were associated with increased risk of composite adverse outcome independently of other risk factors. Further research is necessary to determine whether BMI represents a modifiable risk factor for perioperative outcome.

Risk Factors for Development of Ectopic Atrial Tachycardia in Post-operative Congenital Heart Disease.

Ectopic atrial tachycardia (EAT) is common in surgically repaired congenital heart disease (CHD) and carries the potential for significant hemodynamic compromise. Our objective was to determine the incidence, and risk factors of EAT after CHD surgery. Prospective study of patients that underwent surgery for CHD from February to October 2016 was performed. Demographic, perioperative and electrophysiologic data were collected. Sustained EAT (> 30 s) was documented by telemetry or electrocardiogram and confirmed by a pediatric electrophysiologist. All patients were followed through index hospitalization. During the study period, 17/204 (8%) of patients developed EAT with median time-to-event of 14 days. 15/17 (88%) received anti-arrhythmic therapy for sustained EAT. By univariate analysis, younger age (5 vs. 284 days, P < .001), lower weight (3.2 vs. 7.5 kg, P < .001), single ventricle physiology (P = .05), longer cardiopulmonary bypass time (176 vs. 94 min, P < .001), need for delayed sternal closure (P < .001), and higher STAT category (P < .001) were associated with EAT. Incidence among single ventricle patients was 7/44 (16%), and of those 7/13 (54%) were < 30 days of age. Multivariable Cox regression analysis confirmed age at surgery < 30 days (hazard ratio = 11.7, P = .002) and use of milrinone (hazard ratio = 4.4, P = .007) as independent predictors of EAT. Post-operative EAT is frequent following surgery for CHD especially in neonates. Further study is warranted, specifically in the single ventricle population, given the high potential risk for arrhythmia-induced hemodynamic compromise in this vulnerable population.

Surgery for Congenital Heart Disease: Improvements in Outcomes.

Cardiac surgery for congenital heart disease has changed dramatically since the first surgery in 1938. During the early era, children underwent surgery at older ages often with palliative procedures before their corrective operation. Not surprisingly, in the early era, there was considerably higher early and late mortality, including the additive risks of having more than one procedure and a long period of living with an unphysiological palliated circulation. Over time with advances in noninvasive diagnosis, surgical approach, cardiopulmonary bypass techniques, and team-based care, outcomes have improved. Children now undergo corrective surgery at a younger age and have fewer palliative procedures. Short-term outcome as measured by the commonly used metric "procedural early mortality" (i.e., death before hospital discharge or less than 30 days following a surgical procedure) is now as low as 1 or 2% for many low-to-moderate complexity procedures. Late outcomes have also improved with long-term survival of hospital survivors for simple lesions being close to population controls. Late outcomes for more complex defects still show diminishing survival relative to a control population. Examination of the causes of death provides insights into areas in which clinical improvements may further improve the outlook for children with complex congenital heart disease.

Risk of tumor transmission after thoracic allograft transplantation from adult donors with central nervous system neoplasm-A UNOS database study.

BACKGROUND: We analyzed the UNOS database to better define the risk of transmission of central nervous system (CNS) tumors from donors to adult recipients of thoracic organs. METHODS: Data were procured from the Standard Transplant Analysis and Research dataset files. Donors with CNS tumors were identified, and recipients from these donors comprised the study group (Group I). The remaining recipients of organs from donors who did not have CNS tumors formed the control group (Group II). Incidence of recipient CNS tumors, donor-related malignancies, and overall survival were calculated and compared in addition to multivariable logistic regression. RESULTS: A cohort of 58 314 adult thoracic organ recipients were included, of which 337 received organs from donors who had documented CNS tumors (Group I). None of these recipients developed CNS tumors at a median follow-up of 72 months (IR: 30-130 months). Although overall mortality in terms of the percentage was higher in Group I than Group II (163/320=51% vs 22 123/52 691=42%), Kaplan-Meier curves indicate no significant difference in the time to death between the two groups (P=.92). CONCLUSIONS: There is little risk of transmission of the common nonaggressive CNS tumors to recipients of thoracic organs.

Perioperative Near-Infrared Spectroscopy Monitoring in Neonates With Congenital Heart Disease: Relationship of Cerebral Tissue Oxygenation Index Variability With Neurodevelopmental Outcome.

OBJECTIVES: To evaluate the value of perioperative cerebral near-infrared spectroscopy monitoring using variability analysis in the prediction of neurodevelopmental outcomes in neonates undergoing surgery for congenital heart disease. DESIGN: Retrospective cohort study. SETTING: Urban, academic, tertiary-care children's hospital. PATIENTS: Neonates undergoing surgery with cardiopulmonary bypass for congenital heart disease. INTERVENTIONS: Perioperative monitoring of continuous cerebral tissue oxygenation index by near-infrared spectroscopy and subsequent neurodevelopmental testing at 6, 15, and 21 months of age. MEASUREMENTS AND MAIN RESULTS: We developed a new measure, cerebral tissue oxygenation index variability, using the root mean of successive squared differences of averaged 1-minute cerebral tissue oxygenation index values for both the intraoperative and first 24-hours postoperative phases of monitoring. There were 62 neonates who underwent cerebral tissue oxygenation index monitoring during surgery for congenital heart disease and 44 underwent subsequent neurodevelopmental testing (12 did not survive until testing and six were lost to follow-up). Among the 44 monitored patients who underwent neurodevelopmental testing, 20 (45%) had abnormal neurodevelopmental indices. Patients with abnormal neurodevelopmental indices had lower postoperative cerebral tissue oxygenation index variability when compared with patients with normal indices (p = 0.01). Adjusting for class of congenital heart disease and duration of deep hypothermic circulatory arrest, lower postoperative cerebral tissue oxygenation index variability was associated with poor neurodevelopmental outcome (p = 0.02). CONCLUSIONS: We found reduced postoperative cerebral tissue oxygenation index variability in neonatal survivors of congenital heart disease surgery with poor neurodevelopmental outcomes. We hypothesize that reduced cerebral tissue oxygenation index variability may be a surrogate for impaired cerebral metabolic autoregulation in the immediate postoperative period. Further research is needed to investigate clinical implications of this finding and opportunities for using this measure to drive therapeutic interventions.

Bridging together: teamwork in caring for the family touched by CHD.

In the opening plenary address of the 2017 7th World Congress of Pediatric Cardiology and Cardiac Surgery the author, who represented the World Society for Pediatric and Congenital Heart Surgery at the Congress and is currently the Society's president, described the history of the formation of the World Society. He listed accomplishments of the World Society including publication of the only journal devoted to congenital cardiac surgery, development of a global database, and convening several international conferences dating back to the inaugural conference in Washington, DC in 2007. The general theme of the presentation is the importance of teamwork in managing patients and families with CHD. Challenges facing congenital heart teams are discussed including the fragility of cardiac programmes, that can be heavily influenced by the administrative structure of a paediatric hospital; the difficulty of recruiting skilled surgeons into the field as training in general cardiothoracic surgery contracts and general surgery becomes predominantly laparoscopic with few open procedures; and increasing barriers to the international movement of surgeons including the opportunities for United States of America-based surgeons to acquire international experience at leading global centres. Finally, the author focusses on the danger that the team approach poses to maintaining empathy and emotional support for the family with CHD undergoing a stressful hospitalisation. He discusses strategies to optimise holistic support of the child and family.

International quality improvement initiatives.

Across the globe, the implementation of quality improvement science and collaborative learning has positively affected the care and outcomes for children born with CHD. These efforts have advanced the collective expertise and performance of inter-professional healthcare teams. In this review, we highlight selected quality improvement initiatives and strategies impacting the field of cardiovascular care and describe implications for future practice and research. The continued leveraging of technology, commitment to data transparency, focus on team-based practice, and recognition of cultural norms and preferences ensure the success of sustainable models of global collaboration.

Myocardial cytochrome oxidase activity increases with age and hypoxemia in patients with congenital heart disease.

BACKGROUND: Myocardial tolerance to ischemia is influenced by age and preoperative cyanosis through unknown mechanisms and significantly affects postoperative outcomes. Cytochrome c oxidase (CcOx), the terminal enzyme of the mitochondrial electron transport chain, may play a role in the susceptibility to ischemic-reperfusion (IR) injury. Our study aimed at investigating changes in human myocardial CcOx activity based on age and preoperative oxygen saturation to understand its role in transition from neonatal to mature myocardium and hypoxic conditions. METHODS: The right atrial appendage from patients undergoing first time surgical repair/palliation of congenital heart defects was analyzed for steady state CcOx activity by oxidation of ferrocytochrome c via spectrophotometry and steady state CcOx subunit I protein content by protein immunoblotting. Student's t-test compared CcOx activity and protein levels between patients with preoperative hypoxia and normoxia. Multiple linear regression analysis was used to assess the effects of age and preoperative arterial oxygen saturations (SaO2) on CcOx protein activity and protein content. RESULTS: Thirty-two patients with a median (interquartile range) age of 83 days (8-174) and preoperative oxygen saturation 98% (85-100%) were enrolled. Independent of age, preoperative SaO2 ⩽90% was associated with significantly greater CcOx steady state activity (p=0.004). Additionally, older age itself was associated with increased CcOx steady state activity (p=0.022); the combination of preoperative SaO2 and age account for 33% of the variation in CcOx steady state activity (R2=0.332). There was no increase in the CcOx subunit I protein content with either age or preoperative hypoxia. CONCLUSIONS: In patients with congenital heart disease, an increase in CcOx steady state activity is seen with increasing age. Hypoxia leads to upregulation of CcOx steady state activity without an increase in the amount of enzyme protein itself. Higher CcOx activity in older and cyanotic patients may indicate CcOx-dependent reactive oxygen species as the mechanism for IR injury.

Outcomes of Patients with Pulmonary Atresia and Major Aortopulmonary Collaterals Without Intervention in Infancy.

Treatment of pulmonary atresia with major aortopulmonary collaterals (PA MAPCAs) remains a challenge. Despite variations in surgical technique, contemporary strategies all include initial intervention in the first year of life. However, a subset of patients presents later in life, and contemporary outcomes of this group have not been reported previously. We performed a retrospective case series of consecutive cases of PA MAPCAs who were seen at our center between January 2001 and February 2016, who had not undergone surgery before the age of 1 year. We describe their presenting characteristics, operative and transcatheter interventions, and outcomes. A total of eight cases were identified from 76 children with PA MAPCAs treated over the study period. Median age at presentation was 5.9 years. Seventy-five percent had confluent pulmonary arteries with a median Nakata index of 113 mm(2)/m(2). Operative intervention was performed in 5/6 cases. Two are awaiting intervention. The combination of operative and transcatheter interventions allowed for ventricular septal defect closure in 60 % of cases, all of whom had subsystemic right ventricular pressures. Operative intervention is possible in some older cases with PA and MAPCAs. Though multiple operations and transcatheter therapies are necessary, some can achieve operative correction of serial circulation with tolerable physiology. Subjects with ventricular hypoplasia and those without confluent pulmonary arteries are more challenging.

Diagnosis and treatment of fetal cardiac disease: a scientific statement from the American Heart Association.

BACKGROUND: The goal of this statement is to review available literature and to put forth a scientific statement on the current practice of fetal cardiac medicine, including the diagnosis and management of fetal cardiovascular disease. METHODS AND RESULTS: A writing group appointed by the American Heart Association reviewed the available literature pertaining to topics relevant to fetal cardiac medicine, including the diagnosis of congenital heart disease and arrhythmias, assessment of cardiac function and the cardiovascular system, and available treatment options. The American College of Cardiology/American Heart Association classification of recommendations and level of evidence for practice guidelines were applied to the current practice of fetal cardiac medicine. Recommendations relating to the specifics of fetal diagnosis, including the timing of referral for study, indications for referral, and experience suggested for performance and interpretation of studies, are presented. The components of a fetal echocardiogram are described in detail, including descriptions of the assessment of cardiac anatomy, cardiac function, and rhythm. Complementary modalities for fetal cardiac assessment are reviewed, including the use of advanced ultrasound techniques, fetal magnetic resonance imaging, and fetal magnetocardiography and electrocardiography for rhythm assessment. Models for parental counseling and a discussion of parental stress and depression assessments are reviewed. Available fetal therapies, including medical management for arrhythmias or heart failure and closed or open intervention for diseases affecting the cardiovascular system such as twin-twin transfusion syndrome, lung masses, and vascular tumors, are highlighted. Catheter-based intervention strategies to prevent the progression of disease in utero are also discussed. Recommendations for delivery planning strategies for fetuses with congenital heart disease including models based on classification of disease severity and delivery room treatment will be highlighted. Outcome assessment is reviewed to show the benefit of prenatal diagnosis and management as they affect outcome for babies with congenital heart disease. CONCLUSIONS: Fetal cardiac medicine has evolved considerably over the past 2 decades, predominantly in response to advances in imaging technology and innovations in therapies. The diagnosis of cardiac disease in the fetus is mostly made with ultrasound; however, new technologies, including 3- and 4-dimensional echocardiography, magnetic resonance imaging, and fetal electrocardiography and magnetocardiography, are available. Medical and interventional treatments for select diseases and strategies for delivery room care enable stabilization of high-risk fetuses and contribute to improved outcomes. This statement highlights what is currently known and recommended on the basis of evidence and experience in the rapidly advancing and highly specialized field of fetal cardiac care.

Magnesium Lowers the Incidence of Postoperative Junctional Ectopic Tachycardia in Congenital Heart Surgical Patients: Is There a Relationship to Surgical Procedure Complexity?

Magnesium sulfate was given to pediatric cardiac surgical patients during cardiopulmonary bypass period in an attempt to reduce the occurrence of postoperative junctional ectopic tachycardia (PO JET). We reviewed our data to evaluate the effect of magnesium on the occurrence of JET and assess a possible relationship between PO JET and procedure complexity. A total of 1088 congenital heart surgeries (CHS), performed from 2005 to 2010, were reviewed. A total of 750 cases did not receive magnesium, and 338 cases received magnesium (25 mg/kg). All procedures were classified according to Aristotle score from 1 to 4. Overall, there was a statistically significant decrease in PO JET occurrence between the two groups regardless of the Aristotle score, 15.3 % (115/750) in non-magnesium group versus 7.1 % (24/338) in magnesium group, P < 0.001. In the absence of magnesium, the risk of JET increased with increasing Aristotle score, P = 0.01. Following magnesium administration and controlling for body weight, surgical and aortic cross-clamp times in the analyses, reduction in adjusted risk of JET was significantly greater with increasing Aristotle level of complexity (JET in non-magnesium vs. magnesium group, Aristotle level 1: 9.8 vs. 14.3 %, level 4: 11.5 vs. 3.2 %; odds ratio 0.54, 95 % CI 0.31-0.94, P = 0.028). Our data confirmed that intra-operative usage of magnesium reduced the occurrence of PO JET in a larger number and more diverse group of CHS patients than has previously been reported. Further, our data suggest that magnesium's effect on PO JET occurrence seemed more effective in CHS with higher levels of Aristotle complexity.

A contemporary, single-institutional experience of surgical versus expectant management of congenital heart disease in trisomy 13 and 18 patients.

The objective of this study was to examine a large institutional experience of patients with trisomy 13 and trisomy 18 in the setting of comorbid congenital heart disease and present the outcomes of surgical versus expectant management. It is a retrospective single-institution cohort study. Institutional review board approved this study. Thirteen consecutive trisomy 18 patients and three consecutive trisomy 13 patients (sixteen patients in total) with comorbid congenital heart disease who were evaluated by our institution's Division of Cardiovascular Surgery between January 2008 and December 2013 were included in the study. The primary outcome measures evaluated were operative mortality (for patients who received surgical management), overall mortality (for patients who received expectant management), and total length of survival during follow-up. Of the thirteen trisomy 18 patients, seven underwent surgical management and six received expectant management. With surgical management, operative mortality was 29 %, and 80 % of patients were alive after a median follow-up of 116 days. With expectant management, 50 % of patients died before hospital discharge. Of the three patients with trisomy 13, one patient underwent surgical management and two received expectant management. The patient who received surgical management with complete repair was alive at last follow-up over 2 years after surgery; both patients managed expectantly died before hospital discharge. Trisomy 13 and trisomy 18 patients with comorbid congenital heart disease can undergo successful cardiac surgical intervention. In this population, we advocate that nearly all patients with cardiovascular indications for operative congenital heart disease intervention should be offered complete surgical repair over palliative approaches for moderately complex congenital cardiac anomalies.

Choice of Peripheral Venoarterial Extra-Corporeal Membrane Oxygenation Cannulation Site in Patients Above 15 kilograms.

BACKGROUND: Extracorporeal membrane oxygenation (ECMO) is a life-saving measure for pediatric patients with cardiopulmonary failure. The option of cannulating neck vessels versus those of the groin exists for patients over 15 kg; however, each carries the risk for complications. We present a single-center experience comparing the risks and benefits of these alternate peripheral ECMO cannulation sites. METHODS: All pediatric patients supported by venoarterial (VA) ECMO via peripheral cannulation from January 2006 to May 2013 were retrospectively reviewed, excluding those weighing less than 15 kg. We compared complications of neck versus groin cannulation sites, including neurologic dysfunction, limb ischemia, and in-hospital mortality. Univariate analysis and multivariable logistic regression were performed to identify factors for complications including mortality. RESULTS: Thirty-six patients (median age 10.8 years, interquartile range: 6.0-15.8) were cannulated for VA ECMO. Forty-four percent were cannulated via neck vessels and 47% were cannulated via groin. Nine percent were cannulated at both sites. Overall survival was 72% at decannulation and 67% at hospital discharge. No statistically significant survival difference between groin and neck cannulation subgroups was found. Rate of neurologic injury was higher in neck (25%) versus groin (12%) cannulation, but this was not statistically significant (p = 0.52). Extremity ischemia occurred in five patients having groin cannulation (29%, p = 0.04). CONCLUSIONS: Neck cannulation is a valuable alternative to groin vessels in patients above 15 kg. Risk of limb ischemia with groin cannulation is significant, and must be considered when choosing the cannulation site for ECMO support.