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To investigate the influence of hypothermic cardiopulmonary bypass (HCPB) at 25 degrees C and circulatory arrest at 18 degrees C on the global and regional cerebral blood flow (CBF) during pulsatile perfusion, we performed the following studies in a neonatal piglet model. Using a pediatric physiologic pulsatile pump, we subjected six piglets to deep hypothermic circulatory arrest (DHCA) and six other piglets to HCPB. The DHCA group underwent hypothermia for 25 min, DHCA for 60min, cold reperfusion for 10 min, and rewarming for 40 min. The HCPB group underwent 15 min of cooling, followed by 60 min of HCPB, 10min of cold reperfusion, and 30 min of rewarming. The following variables remained constant in both groups: pump flow (150 ml/kg/min), pump rate (150 bpm), and stroke volume (1 ml/kg). During the 60-min aortic crossclamp period, the temperature was kept at 18 degrees C for DHCA and at 25 degrees C for HCPB. The global and regional CBF (ml/100g/min) was assessed with radiolabeled microspheres. The CBF was 48% lower during deep hypothermia at 18degrees C (before DHCA) than during hypothermia at 25 degrees C (55.2 +/- 14.3ml/100g/min vs 106.4 +/- 19.7 ml/100 g/min; p < 0.05). After rewarming, the global CBF was 45% lower in the DHCA group than in the HCPB group 48.3 +/- 18.1 ml/100g/min vs (87 +/- 35.9ml/100g/min; p < 0.05). Fifteen minutes after the termination of CPB, the global CBF was only 25% lower in the DHCA group than in the HCPB group (42.2 +/- 20.7 ml/100 g/min vs 56.4 +/- 25.8ml/100g/min; p = NS). In the right and left hemispheres, cerebellum, basal ganglia, and brain stem, blood flow resembled the global CBF. In conclusion, both HCPB and DHCA significantly decrease the regional and global CBF during CPB. Unlike HCPB, DHCA has a continued negative impact on the CBF after rewarming. However, 15 min after the end of CPB, there are no significant intergroup differences in the CBF. [ABSTRACT FROM AUTHOR]

We investigated the effects of two new hollow-fiber membrane oxygenators, the Capiox SX10 and the Lilliput 901, on pulsatile versus nonpulsatile perfusion in an in vitro model designed to simulate a 3 kg infant. The experiments were divided into eight groups (six pulsatile and two nonpulsatile), according to the equipment and settings used. Each group included six tests. In all experiments, the pseudo-patient's mean arterial pressure was 40 mmHg, and the pump flow rate ws 550 ml/min. During pulsatile cardiopulmonry bypass, the pump's base flow was set at 30%, and the pump rate was set at 80, 100, 120, 140, 150 beats/min. The PUMP START and PUMP STOP timing points were adjusted to produce different pulse-width settings. We were especially interested in evaluating the pre- and postoxygenator extracorporeal circuit pressure (ECP), the oxygenator pressure drop, and the precannula ECP. When used with a pulsatile roller pump, th Capiox produced a significantly lower preoxygenator ECP than the Lilliput (p < 0.001); moreover, the Capiox yielded a significantly lower oxygenator pressure drop (p < 0.001). During nonpulsatile perfusion, the Capiox again produced a lower preoxygenator ECP than the Lilliput (p < 0.001). These results suggest that the Capiox may be more suitable than the Lilliput when the pulsatile flow is employed, and pulsatile flow does not increase the ECP with either oxygenator. [ABSTRACT FROM AUTHOR]

Background: Aspirin is frequently utilized for antiplatelet therapy in children with congenital heart disease (CHD). Patients who are unresponsive to aspirin, as measured by aspirin reaction units (ARU), are at higher risk for thrombotic events. It is undetermined if dose modification of aspirin results in adequate responsiveness in these patients. This study evaluates the prevalence and risk factors for aspirin nonresponsiveness and the results of dose escalation in this population., Methods: This is a retrospective review of patients cared for in the cardiac care unit at a quaternary care academic congenital heart center who received aspirin and had responsiveness evaluated between January 2018 and January 2023. Patient demographics and clinical characteristics were extracted from the medical record. Descriptive, parametric, and nonparametric univariate analysis were employed., Results: A total of 142 patients (69 [49%] female, 45 [32%]Non-Hispanic White, and 63 [44%] Hispanic]) were identified. Median age at first aspirin responsiveness assessment was 54 [interquartile range, IQR: 23.3-411.5] days with a median weight of 5.2 [IQR: 3.64-9.29] kg. Of these, 32/142 (22.5%) were nonresponsive on their initial testing. Of these patients, 23/32 (72%) had follow-up testing with 19/23 (83%) subsequently becoming therapeutic. This was achieved with an increased dose in 12/19 (63%) patients and increased duration of therapy in 7/19 (37%) patients. Seventeen of 142 (12%) patients experienced a thrombotic event, 13/17 (77%) of which were therapeutic on initial responsiveness assessment., Conclusions: It is common for CHD patients to be aspirin nonresponsive with initial weight-based dosing. If aspirin is used in this population, it is necessary to evaluate ARUs on all patients as underdosing is not uncommon with current weight-based dosing methods., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objective: The population of adults with single-ventricle congenital heart disease (CHD) is growing. This study explores their lived experiences through an adult developmental psychology framework., Methods: Individuals aged 18 and older with single-ventricle CHD participated in Experience Group sessions and 1:1 interviews. Sessions were transcribed and analysed thematically. Themes were categorized by developmental domains and age group., Results: Of the 29 participants, 18 (62%) were female, 10 (35%) were emerging (18-29 years), 13 (45%) were established (30-45 years), and 6 (21%) were midlife adults (46-60 years). Emerging adults expressed reluctance to initiate romantic relationships and fear of burdening partners, while established adults reported strong relationships with partners deeply involved in caregiving. Emerging adults struggled with finding fulfilling work that meets their health needs, whereas established and midlife adults faced unemployment or early retirement due to health limits. Family dynamics shifted, with established and midlife adults educating their children to become caregivers. Physical limitations and low self-rated health were consistent across life stages, and midlife adults did not worry about traditional chronic conditions. Mental health concerns, including anxiety and depression, persisted across all life stages, but resiliency and positive affect were also evident., Conclusion: Adults with single-ventricle CHD experience developmental milestones differently, indicating the need for early anticipatory guidance in these domains to achieve optimal outcomes in adulthood.

Objectives: Patients with dextro-transposition of the great arteries (d-TGA) frequently undergo balloon atrial septostomy (BAS) prior to the arterial switch operation (ASO) to promote atrial-level mixing. Balloon atrial septostomy has inherent risks as an invasive procedure and may not always be necessary. This study revisits the routine utilization of BAS prior to ASO., Methods: Single-center, retrospective review of d-TGA patients undergoing the ASO from July 2018 to March 2023. Preoperative patient characteristics, pulse oximetry oxygen saturations (SpO 2 ), cerebral/renal near-infrared spectroscopy (NIRS) readings along with prostaglandin status at the time of the ASO were analyzed with descriptive and univariate statistics., Results: Thirty patients underwent the ASO. Of these, 7 (23%) were female, 25 (83%) were white, and median weight at ASO was 3.2 kg (range 0.8-4.2). Twenty-two (73%) patients underwent BAS. There were no demographic differences between BAS and no-BAS patients. Of those who underwent BAS, there was a significant increase in SpO 2 (median 83% [range 54-92] to median 87% [range 72-95], P  = .007); however, there was no change in NIRS from pre-to-post BAS. Six (27%) patients in the BAS group were prostaglandin-free at ASO. Balloon atrial septostomy patients underwent the ASO later compared with no-BAS patients (median 8 [range 3-32] vs 4 [range 2-10] days old, P  = .016) and had a longer hospital length of stay (median 13 [range 7-43] vs 10 [range 8-131] days, P  = .108)., Conclusions: While BAS is an accepted preoperative procedure in d-TGA patients to improve oxygen saturations, it is also an additional invasive procedure, does not guarantee prostaglandin-free status at the time of ASO, and may increase the interval to ASO. Birth to direct early ASO, with prostaglandin support, should be revisited as an alternative, potentially more expeditious strategy., Competing Interests: Declaration of Conflicting InterestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Nickel is a component of nitinol, an alloy used in several medical devices. Allergy to nickel may place patients at a high risk for severe hypersensitivity reactions. We report a rare case of a patient who developed severe ulcerative colitis ten years following closure of an atrial septal defect with the Amplatzer Septal Occluder device., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

We present the case of a 4 year old child who developed cardiogenic shock due to permanent junctional reciprocating tachycardia, requiring left ventricular assist device (LVAD) support. Despite the initial critical clinical presentation, successful myocardial recovery was achieved over 5 months, allowing for successful LVAD explantation. The patient's young age and behavioral issues were constraining factors for a prolonged LVAD wean trial. A modified wean protocol over a 3 day period with parameters for assessment of cardiac recovery before LVAD explantation is described., Competing Interests: Disclosure: The authors have no conflicts of interest to report., (Copyright © ASAIO 2024.)

Background: Iatrogenic complete atrioventricular block (ICAVB) has long been noted as a major complication after congenital heart surgery (CHS), and it contributes to complex postoperative care and potentially affects patients' outcomes., Methods: This study is a retrospective review of the Pediatric Health Information System database from January 1, 2004 to September 30, 2023. All patients who underwent The Society of Thoracic Surgeons benchmark procedures were included. International Classification of Diseases (ICD) 9th and 10th editions were used to identify diagnoses and procedures. All patients with a diagnosis of complete atrioventricular block and placement of a permanent pacemaker after CHS but in the same hospitalization were identified as having ICAVB., Results: A total of 42,332 patients were identified, with 17,106 (41%) female and 23,042 (55%) non-Hispanic White and with a median age of 5.4 months [interquartile range, 0.4-25.8 months]. Of those patients, 246 (0.6%) had ICAVB. The procedure with the highest incidence of ICAVB was the arterial switch operation with ventricular septal defect (VSD) repair (74 of 1552; 4.5%). On multivariable analysis, the arterial switch operation with VSD repair had the highest adjusted odds of ICAVB (odds ratio, 5.41; 95% CI, 3.57-8.19; P < .001) when compared with isolated VSD repair. A diagnosis of endocarditis was significantly associated with ICAVB. Center volume was not associated with ICAVB. ICAVB was associated with a 121% (95% CI, 98.5%-146.8%) increase in length of stay (P < .001) and increased in-hospital mortality (odds ratio, 2.26; 95% CI, 1.34-3.82; P < .001)., Conclusions: The overall incidence of ICAVB after CHS is low. However, certain procedures have incidences as high as 4.5%. ICAVB is associated with increased postoperative mortality and length of stay. Further work is needed to identify drivers of variation among centers to improve overall outcomes., Competing Interests: Disclosures The authors have no conflicts of interest to disclose., (Copyright © 2024 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Postoperative critical care management of congenital heart disease patients requires prompt intervention when the patient deviates significantly from clinician-determined vital sign and hemodynamic goals. Current monitoring systems only allow for static thresholds to be set on individual variables, despite the expectations that these signals change as the patient recovers and that variables interact. To address this incongruency, we have employed statistical process monitoring (SPM) techniques originally developed to monitor batch industrial processes to monitor high-frequency vital sign and hemodynamic data to establish multivariate trajectory maps for patients with d-transposition of the great arteries following the arterial switch operation. In addition to providing multivariate trajectory maps, the multivariate control charts produced by the SPM framework allow for assessment of adherence to the desired trajectory at each time point as the data is collected. Control charts based on slow feature analysis were compared with those based on principal component analysis. Alarms generated by the multivariate control charts are discussed in the context of the available clinical documentation., Competing Interests: The authors declare no conflicts of interest., (© 2024 The Authors. Bioengineering & Translational Medicine published by Wiley Periodicals LLC on behalf of American Institute of Chemical Engineers.)

Background: The Berlin Heart EXCOR® (BHE) can bridge children with severe heart failure to transplantation, but some are successfully weaned and spared transplantation. This study seeks to identify characteristics of children amenable to successful explantation with BHE support., Methods: Preferred Reporting Items for Systematic reviews and Meta-Analyses 2020 guidelines were used. Five databases were screened for original, English articles measuring BHE support in patients <18 years old based on title and abstract. Exclusion criteria were applied: full-text availability, <10 total pediatric BHE patients, zero successful explantations from BHE, nonprimary literature, adult and pediatric results that could not be separated, and studies with overlapping patient information. Studies were analyzed with descriptive statistics., Results: From 41 857 potential studies, 14 were analyzed with data from 58 hospitals on four continents from 1990 to 2020. There were 984 BHE patients. The most common diagnosis was dilated cardiomyopathy (n = 318, 32.3%), followed by congenital heart disease (n = 249, 25.3%). There were 85 (8.6%) children explanted with favorable outcomes. The underlying diagnosis was known in 44 (51.8%) cases: 14 (8.4%) of 166 cardiomyopathies, 17 (48.6%) of 35 myocarditis, and 12 (16.7%) of 72 with congenital heart disease were explanted. When the type of support was known, the rate of LVAD patients explanted was 21.3% (n = 19/89) and 2.4% (n = 1/42) of BiVAD patients were explanted., Conclusion: Explantation from BHE is not uncommon at 8.6%, but significant variation exists in the explantation data reported. Myocarditis and LVAD support may be populations suitable for weaning. Standardization of reporting measures and prospective registries may help identify patients suitable for this alternative to transplant and help develop weaning protocols., (© 2024 International Center for Artificial Organ and Transplantation (ICAOT) and Wiley Periodicals LLC.)

Background: CHD care is resource-intensive. Unwarranted variation in care may increase cost and result in poorer health outcomes. We hypothesise that process variation exists within the pre-operative evaluation and planning process for children undergoing repair of atrial septal defect or ventricular septal defect and that substantial variation occurs in a small number of care points., Methods: From interviews with staff of an integrated congenital heart centre, an initial process map was constructed. A retrospective chart review of patients with isolated surgical atrial septal defect and ventricular septal defect repair from 7/1/2018 through 11/1/2020 informed revisions of the process map. The map was assessed for points of consistency and variability., Results: Thirty-two surgical atrial septal defect/ventricular septal defect repair patients were identified. Ten (31%) were reviewed by interventional cardiology before surgical review. Of these, 6(60%) had a failed catheter-based closure and 4 (40%) were deemed inappropriate for catheter-based closure. Thirty (94%) were reviewed in case conference, all attended surgical clinic, and none were admitted prior to surgery. The process map from interviews alone identified surgery rescheduling as a point of major variability; however, chart review revealed this was not as prominent a source of variability as pre-operative interventional cardiology review., Conclusions: Significant variation in the pre-operative evaluation and planning process for surgical atrial septal defect/ventricular septal defect patients was identified. If such process variation is widespread through CHD care, it may contribute to variations in outcome and cost previously documented within CHD surgery. Future research will focus on determining whether the variation is warranted or unwarranted, associated health outcomes and cost variation attributed to these variations in care processes.

Objective: The pathway to become a congenital heart surgeon (CHS) is challenging and unpredictable. Previous voluntary manpower surveys have shed partial light on this problem but have not included all trainees. We believe that this arduous journey merits more attention., Methods: To examine the real-life challenges of recent participants in Accreditation Council for Graduate Medical Education-accredited CHS training programs, we conducted phone interviews with all graduates of approved programs from 2021 to 2022. This institutional review board approved survey focused on issues including preparation, length of training, debt burden, and employment., Results: All 22 (100%) graduates during the study period were interviewed. Age at fellowship completion was a median 37 years (range, 33-45 years). Pathways to fellowship included traditional general surgery with adult cardiac (43%), abbreviated general surgery ("4 + 3," 19%) and integrated-6 (38%). Time spent on any pediatric related rotation before CHS fellowship was a median 4 months (range, 1-10 months). During CHS fellowship, graduates reported medians of 100 (range, 75-170) total cases and 8 (range, 0-25) neonatal cases as the primary surgeon. Debt burden at completion was a median of $179,000 (range, $0-$550,000). Maximal financial compensation during training before and during CHS fellowship were medians of $65,000 (range, $50,000-$100,000) and $80,000 (range, $65,000-$165,000), respectively. Six (27.3%) are currently in roles in which they cannot practice independently (5 [22.7%] faculty instructors, 1 [4.5%] CHS clinical fellowship). Median salary in first job is $450,000 (range, $80,000-$700,000)., Conclusions: Graduates of CHS fellowships are old, and training is highly variable. Aptitude screening and pediatric-focused preparation are minimal. Debt burden is onerous. Further attention to refining training paradigms and compensation are justified., (Copyright © 2023 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Background: Neonatal congenital atrioventricular block (nCAVB) is rare, causes bradycardia, confers high mortality, and frequently requires pacing. In-hospital outcomes and pacemaker management in nCAVB are limited., Objectives: The purpose of this study was to analyze pacing and outcomes of nCAVB with and without congenital heart disease (CHD) using a multicenter database., Methods: A Pediatric Health Information System database review from January 1, 2004, to June 30, 2022. Patients <31 days of age with a nCAVB International Classification of Diseases-9th/10th Revision diagnosis code and no cardiac surgeries except pacemaker were included. Pacing and in-hospital mortality were analyzed using univariate and multivariable logistic statistics and competing risk and event-free survival models., Results: Of 1,146 patients with nCAVB, 659 (57.5%) were girls and 506 (44.2%) were premature. Among the 326 (28.4%) with CHD, 134 (41.1%) underwent pacemaker insertion as initial intervention and 56 (17.2%) had temporary pacing wires. In-hospital mortality occurred in 118 (36.2%), with increased adjusted odds with temporary pacing wires placed at 0 to 1 or 2 to 7 days of age relative to no wires, and with decreased odds among pacemakers placed at 2 to 7 or 8+ days of age relative to no pacemaker. Of 820 (71.6%) without CHD, 334 (40.7%) underwent pacemaker insertion as the initial intervention and 81 (9.9%) had temporary pacing wires. In-hospital mortality occurred in 69 (8.4%) with increased adjusted odds in prematurity and decreased odds among pacemaker placement at 2 to 7 days of age relative to no pacemaker., Conclusions: Over 18.5 years, in-hospital mortality occurred in 36.2% of nCAVB patients with CHD and 8.4% with non-CHD. Associations with increased in-hospital mortality included CHD and prematurity and decreased with pacemaker placement. Prospective registries are needed to better characterize and standardize management of this rare but high-mortality disease., Competing Interests: Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2023 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background The lifetime journey of patients with single-ventricle congenital heart disease is characterized by long-term challenges that are incompletely understood and still unfolding. Health care redesign requires a thorough understanding of this journey to create and implement solutions that improve outcomes. This study maps the lifetime journey of individuals with single-ventricle congenital heart disease and their families, identifies the most meaningful outcomes to them, and defines significant challenges in the journey. Methods and Results This qualitative research study involved experience group sessions and 1:1 interviews of patients, parents, siblings, partners, and stakeholders. Journey maps were created. The most meaningful outcomes to patients and parents and significant gaps in care were identified across the life journey. A total of 142 participants from 79 families and 28 stakeholders were included. Lifelong and life-stage specific journey maps were created. The most meaningful outcomes to patients and parents were identified and categorized using a " capability (doing the things in life you want to), comfort (experience of physical/emotional pain/distress), and calm (experiencing health care with the least impact on daily life) " framework. Gaps in care were identified and classified into areas of ineffective communication, lack of seamless transitions, lack of comprehensive support, structural deficiencies, and insufficient education. Conclusions There are significant gaps in care during the lifelong journey of individuals with single-ventricle congenital heart disease and their families. A thorough understanding of this journey is a critical first step in developing initiatives to redesign care around their needs and priorities. This approach can be used for people with other forms of congenital heart disease and other chronic conditions. Registration URL: https://www.clinicaltrials.gov; Unique identifier: NCT04613934.

Background: Pericardial effusion (PCE) is a significant complication after pediatric cardiac surgery. This study investigates PCE development after the arterial switch operation (ASO) and its short-term and longitudinal impacts. Methods: A retrospective review of the Pediatric Health Information System database. Patients with dextro-transposition of the great arteries who underwent ASO from January 1, 2004, to March 31, 2022, were identified. Patients with and without PCE were analyzed with descriptive, univariate, and multivariable regression statistics. Results: There were 4896 patients identified with 300 (6.1%) diagnosed with PCE. Thirty-five (11.7%) with PCE underwent pericardiocentesis. There were no differences in background demographics or concomitant procedures between those who developed PCE and those who did not. Patients who developed PCE more frequently had acute renal failure (N = 56 (18.7%) vs N = 603(13.1%), P  = .006), pleural effusions (N = 46 (15.3%) vs N = 441 (9.6%), P  = .001), mechanical circulatory support (N = 26 (8.7%) vs N = 199 (4.3%), P  < .001), and had longer postoperative length of stay (15 [11-24.5] vs 13 [IQR: 9-20] days). After adjustment for additional factors, pleural effusions (OR = 1.7 [95% CI: 1.2-2.4]), and mechanical circulatory support (OR = 1.81 [95% CI: 1.15-2.85]) conferred higher odds of PCE. There were 2298 total readmissions, of which 46 (2%) had PCE, with no difference in median readmission rate for patients diagnosed with PCE at index hospitalization (median 0 [IQR: 0-1] vs 0 [IQR: 0-0], P  = .208). Conclusions: PCE occurred after 6.1% of ASO and was associated with pleural effusions and mechanical circulatory support. PCE is associated with morbidity and prolonged length of stay; however, there was no association with in-hospital mortality or readmissions.

Background: Open intrauterine fetal myelomeningocele repair has demonstrated decreased ventriculoperitoneal shunting and improved motor outcomes despite maternal and fetal risks. Few data directly compare the safety of open vs endoscopic approaches., Objective: This study aimed to analyze in-hospital maternal and fetal outcomes of pregnant patients undergoing open vs endoscopic fetal myelomeningocele repair using a large, multi-center database., Study Design: This was a review of the Pediatric Health Information System database from October 1, 2015, to December 31, 2021. All patients who underwent open or endoscopic fetal myelomeningocele repair according to the International Classification of Diseases, Tenth Revision, were identified. Demographics, gestational age, and outcomes were analyzed. Descriptive and univariate statistics were used., Results: A total of 378 pregnant patients underwent fetal myelomeningocele repair. The approach was endoscopic in 143 cases (37.8%) and open in 235 cases (62.2%). Overall postprocedural outcomes included no maternal in-hospital mortalities or intensive care unit admissions, a median length of stay of 4 days (interquartile range, 4-5), 14 cases (3.7%) of surgical and postoperative complications, 6 cases (1.6%) of intrauterine infections, 12 cases (3.2%) of obstetrical complications (including preterm premature rupture of membranes), 3 cases (0.8%) of intrauterine fetal demise, and 16 cases (4.2%) of preterm delivery. Compared with an open approach, the endoscopic approach occurred at a later gestational age (25 weeks [interquartile range, 24-25] vs 24 weeks [interquartile range, 24-25]; P<.001) and had an increased rate of intrauterine infection (6 [4.2%] cases vs 0 [0%] case; P=.002). There was no difference between approaches in the rates of surgical complications, obstetrical complications, intrauterine fetal demise, or preterm deliveries., Conclusion: Compared with an open approach, endoscopic fetal myelomeningocele repair displays a comparable rate of fetal complications, including intrauterine fetal demise and preterm delivery, and a similar in-hospital maternal safety profile despite an association with increased intrauterine infection., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Objectives: Currently, surgical repair of tetralogy of Fallot (TOF) is associated with an 1.1% 30-day mortality rate. Those with junctional ectopic tachycardia (JET) and restrictive right ventricular physiology have poorer outcomes. Routine postoperative adrenergic or inodilator therapy has been reported, while beta-blockade following cardiopulmonary bypass has not. This study evaluated routine perioperative treatment with esmolol in infants undergoing TOF repair., Design: Retrospective chart review of the perioperative course following TOF repair., Setting: Single-center case series describing perioperative management of TOF in a cardiac ICU., Patients: This study reviewed all patients less than 18 months old who underwent TOF repair, excluding cases of TOF with absent pulmonary valve or atrioventricular septal defect, at our institution from June 2018 to April 2021., Interventions: This review investigates the hemodynamic effects of esmolol following cardiopulmonary bypass for TOF repair., Measurements and Main Results: Preoperative clinical characteristics and perioperative course were extracted from the medical record. Descriptive statistics were used. Twenty-six patients receiving perioperative esmolol after TOF repair were identified and included. Postoperative hemodynamic parameters were within a narrow range with minimal vasoactive support in most patients. Three of 26 patients experienced JET, and one of 26 of whom had a brief cardiac arrest. Median and interquartile range (IQR) for hospital and postoperative length of stay was 7 days (IQR, 6-9 d) and 6 days (IQR, 5-8 d), respectively. There were no 30-day or 1-year mortalities., Conclusions: In this infant cohort, our experience is that the routine use of postoperative esmolol is associated with good cardiac output with minimal requirement for vasoactive support in most patients. We believe optimal postoperative management of infant TOF repair requires a meticulous multidisciplinary approach, which in our experience is enhanced with routine postoperative esmolol treatment., Competing Interests: The authors have disclosed that they do not have any potential conflicts of interest., (Copyright © 2022 by the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies.)

Arrhythmias account for 55 per 100,000 patient evaluations in pediatric emergency departments. Most arrhythmias in children are amenable to medical management or cardioversion. Rarely, arrhythmias lead to significant hemodynamic instability requiring extracorporeal membrane oxygenation (ECMO) support. This study seeks to evaluate children under 1 year of age with a structurally normal heart requiring ECMO for an arrhythmia. This is a retrospective review of the Extracorporeal Life Support Organization Registry. All patients less than 1 year of age between 2009 and 2019 with a diagnosis of arrhythmia and without a diagnosis of structural heart malformation were included. Demographics, clinical characteristics, and outcomes were assessed with descriptive statistics and univariate and multivariable analyses. A total of 140 eligible patients were identified from the dataset. The most common arrhythmia was supraventricular tachycardia (SVT) in 70 (50%) patients. ECMO complications occurred in 106 (76.3%) patients and survival to discharge was achieved in 120 (85.7%) patients. In-hospital mortality was associated with neuromuscular blockade prior to ECMO [aOR 10.0 (95% CI 2.95-41.56), p < 0.001], neurologic ECMO complication [aOR 28.1 (95% CI 6.6-155.1), p < 0.001], and race with white race being protective [aOR 0.13, (95% CI 0.02-0.21), p = 0.002]. Similar survival and complication rates were found in subgroup analysis of SVT arrhythmias alone. Arrhythmias necessitating ECMO support in infants without structural congenital heart disease is a rare occurrence. However, survival to hospital discharge is favorable at greater than 85%. Given the favorable survival, earlier and more aggressive utilization of ECMO may result in improved outcomes., (© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

The survival of congenital heart disease (CHD) patients with single-ventricle (SV) physiology has markedly increased as a result of advances in operative techniques and postsurgical management. Nonetheless, these patients remain highly susceptible to end-stage heart failure requiring cardiac replacement therapies at early ages. Given a worldwide shortage of transplantable organs, mechanical circulatory support (MCS) represents an alternative treatment option. The significant heterogeneity of the SV population presents unique indications for MCS that have begun to be evaluated. This case study describes a 12-year-old female with heterotaxy syndrome and an SV condition, previously palliated with a Fontan operation at another institution. The patient was placed on veno-arterial (VA) extracorporeal membrane oxygenation (ECMO) during prolonged cardiopulmonary resuscitation, and later underwent HeartWare ventricular assist device (HVAD) implantation as a bridge to transplantation (BTT). A novel method was chosen to optimize careful de-airing of the heart through a minimized cardiopulmonary bypass (CPB) setup, during full ECMO support and surgical insertion of the HeartWare. The ascending aorta was vented proximal to the HVAD outflow graft anastomosis through a minimized CPB circuit at <10% of the ECMO flow rate. This circuit adaption allowed for euvolemic resuscitation via connection from the minimized CPB circuit to the venous limb of the ECMO circuit. The transition from VA-ECMO to the HeartWare was well tolerated despite a challenging sternotomy and cardiac anomaly. A minimized bypass circuit proved efficacious for the benefit of volume resuscitation and safe de-airing of the HVAD while on ECMO support. The literature is limited concerning safe practices for implantation of durable VADs in complex SV patients coupled with those transitioning from varying modalities of MCS. As SV survivability regresses to heart failure, it is essential that we share techniques that aim to improve the long-term outcomes for successful BTT or bridge to decision (BTD)., (© Copyright 2021 AMSECT.)

Background: We aimed to describe our experience with patients with sickle cell trait (SCT) and undergoing surgery on cardiopulmonary bypass (CPB)., Methods: Data on all patients with SCT or sickle-α thalassemia who underwent surgery on CPB were collected (1996-2017)., Results: Overall, 46 patients were included, 37 (80%) had SCT and 9 (20%) had sickle-α thalassemia. A total of 4 (9%) developed a potential sickle cell-related complication. Patients with sickle cell-related complications were significantly older (median 14 years vs 14 months, P = .037) and heavier (median 54 kg vs 9 kg, P = .041). Complications occurred, although without statistical significance, in patients who underwent longer median CPB times (249 minutes vs 137 minutes, P = .069), lower median temperature (31.7 °C vs 33.3 °C, P = .094), and a higher percentage underwent deep hypothermic circulatory arrest (50% vs 7%, P = .053). A total of 30 (65%) patients underwent exchange transfusion (ET) pre-bypass. Patients who underwent ET were significantly older (median 4 years vs 7 months, P = .003) and heavier (median 16 kg vs 6 kg, P = .015) than patients who did not undergo ET. The incidence of complications was comparable between patients who underwent ET (10%) and those who did not (6%)., Conclusions: In this retrospective, single-center study, it has been shown that cardiac surgery requiring CPB in patients with SCT and sickle-α thalassemia had a low risk for sickle cell-associated complications. In this cohort of patients, older age, longer CPB times, lower median temperature, and the utilization of deep hypothermic circulatory arrest appear to play an important role in the development of complications.

We aimed to describe the short- and long-term outcomes of patients after an arterial switch operation (ASO) at a single institution during a 23-year period. A retrospective chart review of all patients <18 months of age who underwent an ASO between January 1995 and March 2018 at Texas Children's Hospital, Houston, TX was performed. Primary endpoints include mortality and reintervention. Perioperative mortality was defined as mortality occurring in-hospital and/or <30 days after surgery. Survival and freedom-from-reintervention were analyzed using Kaplan-Meier method, log-rank tests, and Cox regression models. The cohort included 394 patients. Diagnoses included 204 patients (52%) with intact ventricular septum, 137 (35%) with a ventricular septal defect, 17 (4%) with a ventricular septal defect and left ventricular outflow tract obstruction (LVOTO), and 36 (9%) with Taussig-Bing anomaly. Median age at surgery was 8 days (range: 1 day to 17 months) and median weight was 3.4 (range: 0.8-12.0) kg. Overall perioperative mortality was 1.3% (n = 5), 0.3% (n = 1) since 1999. Overall survival at 5, 10, and 15 years was 98.2%, 97.8%, and 97.8%, respectively. Perioperative morality was associated with prematurity (P = 0.012), <2.5 kg (P< 0.001), and longer circulatory arrest (P = 0.024) after univariate analysis. Reintervention was associated with a longer cross-clamp time (P < 0.001), <2.5 kg (P = 0.009), LVOTO resection (P = 0.047), and genetic syndrome (P= 0.011) after multivariable analysis. Current ASO expectations should include a perioperative mortality risk of <1% and good long-term survival. Reinterventions are more frequent in patients <2.5 kg, concomitant LVOTO resection, a genetic syndrome, and longer cross-clamp time., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: Intraseptal anomalous aortic origin of a coronary artery is considered a benign condition. However, there have been case reports of patients with myocardial ischemia, arrhythmia, and sudden cardiac death. The purpose of this study was to determine the clinical presentation, myocardial perfusion on provocative stress testing, and management of children with anomalous aortic origin of a coronary artery with an intraseptal course in a prospective cohort., Methods: Patients with anomalous aortic origin of a coronary artery and intraseptal course were prospectively enrolled from December 2012 to May 2019, evaluated, and managed following a standardized algorithm. Myocardial perfusion was assessed using stress imaging. Fractional flow reserve was performed in patients with myocardial hypoperfusion on noninvasive testing. Exercise restriction, β-blockers, and surgical intervention were discussed with the families., Results: Eighteen patients (female 6, 33.3%), who presented with no symptoms (10, 55.6%), nonexertional (4, 22.2%), and exertional symptoms (4, 22.2%), were enrolled at a median age of 12.4 years (0.3-15.9). Perfusion imaging was performed in 14/18 (77.8%) and was abnormal in 7/14 (50%); fractional flow reserve was positive in 5/8 (62.5%). All 4 patients with exertional symptoms and 3/10 (30%) with no or nonexertional symptoms had myocardial hypoperfusion. Coronary artery bypass grafting was performed in a 4-year-old patient; β-blocker and exercise restriction were recommended in 4 patients not suitable for surgery. One patient had nonexertional chest pain and 17 were symptom-free at median follow-up of 2.5 years (0.2-7.1)., Conclusions: Up to 50% of patients with intraseptal anomalous aortic origin of a coronary artery had inducible myocardial hypoperfusion during noninvasive provocative testing. Long-term follow-up is necessary to understand the natural history of this rare anomaly.

Background: Anomalous aortic origin of a coronary artery (CA) is the second leading cause of sudden cardiac death in young athletes. Management is controversial and longitudinal follow-up data are sparse. We aim to evaluate outcomes in a prospective study of anomalous aortic origin of CA patients following a standardized algorithm., Methods: Patients with anomalous aortic origin of a CA were followed prospectively from December 2012 to April 2017. All patients were evaluated following a standardized algorithm, and data were reviewed by a dedicated multidisciplinary team. Assessment of myocardial perfusion was performed using stress imaging. High-risk patients (high-risk anatomy-anomalous left CA from the opposite sinus, presence of intramurality, abnormal ostium-and symptoms or evidence of myocardial ischemia) were offered surgery or exercise restriction (if deemed high risk for surgical intervention). Univariate and multivariable analyses were used to determine predictors of high risk., Results: Of 201 patients evaluated, 163 met inclusion criteria: 116 anomalous right CA (71%), 25 anomalous left CA (15%), 17 single CA (10%), and 5 anomalous circumflex CA (3%). Patients presented as an incidental finding (n=80, 49%), with exertional (n=31, 21%) and nonexertional (n=32, 20%) symptoms and following sudden cardiac arrest/shock (n=5, 3%). Eighty-two patients (50.3%) were considered high risk. Predictors of high risk were older age at diagnosis, black race, intramural course, and exertional syncope. Most patients (82%) are allowed unrestrictive sports activities. Forty-seven patients had surgery (11 anomalous left CA and 36 anomalous right CA), 3 (6.4%) remained restricted from sports activities. All patients are alive at a median follow-up of 1.6 (interquartile range, 0.7-2.8) years., Conclusions: In this prospective cohort of patients with anomalous aortic origin of a CA, most have remained free of exercise restrictions. Development of a multidisciplinary team has allowed a consistent approach and may have implications in risk stratification and long-term prognosis.

Background: This single-institution study assessed the midterm outcomes of patients undergoing complete vascular ring (CVR) repair and the need for reintervention., Methods: The study included all patients who underwent surgical repair of an isolated CVR from 1996 to 2018 at our institution. Patients who underwent concomitant intracardiac repair were excluded. Data analysis included demographics, type of anomaly, other congenital heart disease, clinical symptomatology, operative technique, perioperative outcomes, reoperation rates, and mortality., Results: CVR repair through open thoracotomy was performed in 148 patients (80 boys [54%]), median age, 1.04 years (interquartile range, 0.4-5.2 years), and median weight, 12.8 kg (interquartile range, 7.5-26.5 kg). The cohort included 72 patients with double aortic arch (DAA), 69 with right aortic arch (RAA) with aberrant left subclavian artery and left ligamentum arteriosum (LLA), 5 with RAA with left descending aorta and LLA, and 2 with RAA with mirror-image branching and LLA. There was 1 outpatient perioperative death (0.7%) 15 days postoperatively. Perioperative complications occurred in 20 patients (14%): 18 (12%) with chylothorax (3 required reintervention), 1 pneumothorax, and 1 vocal cord paresis. Two of 36 patients (5.5%) without primary diverticulum resection required reoperation and subclavian reimplantation at 3 and 4 years, and 1 patient required aortic translocation 9 years later for persistent symptoms., Conclusions: Freedom from reoperation after CVR repair was 93% at 5 years and 86% at 10 years. A small proportion of patients who do not undergo diverticulum resection and aberrant left subclavian artery reimplantation at the time of CVR repair will require reintervention in the future., (Copyright © 2020 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The Fontan procedure is the accepted standard for single-ventricle palliation. The goal of this study was to determine short- and midterm outcomes of patients undergoing a Fontan operation at a single institution and to identify contemporary risk factors for acute and chronic failure., Methods: All patients undergoing a Fontan operation between 1995 and 2016 were included. Failure was defined as death, transplantation, Fontan takedown or revision, fenestration creation or enlargement, plastic bronchitis, protein-losing enteropathy, or major perioperative reintervention. Multivariable logistic and Cox regression models were used to identify risk factors for acute (perioperative) and chronic failure (after hospital discharge or 30 days postoperatively, or both)., Results: The cohort included 610 patients. Median age at surgery was 4 years. Median follow-up was 6.8 years. Trends showed increasing use of extracardiac conduits, nonfenestrated Fontan, and extubation in the operating room. Perioperative mortality was 0.5% (n = 3). Transplant-free survival at 5, 10, and 15 years was 97%, 94%, and 92%, respectively; freedom from failure was 91%, 89%, and 87%, respectively. Extubation in the operating room was associated with lower risk of acute failure (odds ratio, 0.30; 95% confidence interval [CI], 0.11 to 0.87). Independent risk factors for chronic failure included genetic syndrome (hazard ratio [HR], 2.54; 95% CI, 1.11 to 5.83), ventricular dysfunction (HR, 3.86; 95% CI, 1.81 to 8.24), cardiopulmonary bypass time in 30-minute intervals (HR, 1.242; 95% CI, 1.100 to 1.402), and persistent pleural effusions (HR, 4.26; 95% CI, 2.25 to 8.07). Moderate or severe atrioventricular valve regurgitation (HR, 2.61; 95% CI, 1.13 to 6.02) and cardiopulmonary bypass time (HR, 1.22; 95% CI, 1.03 to 1.45) were associated with reduced long-term transplant-free survival., Conclusions: Contemporary midterm outcomes for Fontan patients are reassuring. Lifelong follow-up is mandatory to determine long-term outcomes and need for additional surgery as patients reach adulthood., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background/purpose: Elective ambulatory surgical care traditionally involves three independent visits. Single-Visit Surgery (SVS) is an alternative surgical model that consolidates care into one visit. Evaluation of the effect of this novel program on hospital operations is limited. The objective of this study was to analyze SVS from an institutional perspective., Methods: We retrospectively reviewed patients scheduled for SVS at a freestanding children's hospital between January 2016 and August 2017. Data collected included clinic "no show" rates, operating room (OR) utilization, reimbursement rates, and postoperative visits., Results: There were 89 patients scheduled for SVS, of which 63% (n=56) were male, and the median age was 6 years [IQR, 4-9]. The SVS clinic "no show" rate was 2% (n=2) compared to the pediatric surgery clinic "no show" rate of 11% (p=0.01). The SVS OR block utilization rate was 90%. Payment was received from third-party payors for 92% of consultations and 100% of operative procedures without securing prior authorization. Postoperatively 25% (n=17) of patients presented to clinic for follow-up, and one child presented to the emergency department for vomiting. There were no hospital admissions., Conclusion: Single-Visit Surgery is an alternative model of ambulatory surgical care that improves institutional efficiency while also enhancing the patient experience., Type of Study: Retrospective cohort review LEVEL OF EVIDENCE: III., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: There has been increasing use of durable ventricular assist devices (VAD) in children as a bridge to transplantation (BTT). The Berlin Heart investigational device exemption (IDE) trial was the first pediatric VAD trial to demonstrate excellent survival outcomes as a BTT., Objectives: Our aim was to compare the expanded post-transplant outcomes for children enrolled in the Berlin Heart IDE trial to a matched Pediatric Heart Transplant Study (PHTS) cohort not requiring mechanical circulatory support (MCS)., Setting: University Hospitals., Methods: This was a retrospective review of linked PHTS and Berlin Heart IDE databases for pediatric (≤18 years) recipients transplanted from 2007-2011. Subjects with <5 years of follow up were excluded. VAD supported patients were matched 1:2 to non-VAD supported controls from the PHTS database., Results: Among 109 Berlin Heart IDE study enrollees, 83 were merged with the PHTS database and matched to 166 non-MCS supported patients. There was no difference in diagnosis, status at listing, and age between groups with the expected difference in inotrope use in the non-MCS supported patients. Compared to their matched cohort, there was no statistical difference in 5-year patient survival between VAD and non-VAD patients (81% vs 88%; p = 0.09) nor was there a difference in freedom from rejection or infection., Conclusions: This data suggests that children supported with a Berlin Heart VAD had similar survival, infection and rejection rates compared to those not requiring MCS support. Continued surveillance of the Berlin Heart IDE trial population post heart transplantation is warranted., (Copyright © 2018 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

Background: Our institutional policy is to continue centrifugal-flow ventricular assist device support for 3 months or more without activation on the transplant wait-list for physical recovery and assessment of possible myocardial recovery. We evaluated our single-institutional outcomes with centrifugal-flow ventricular assist device support in children., Methods: Prospectively collected outcomes data in consecutive patients aged 18 years or less with centrifugal-flow ventricular assist device support were reviewed., Results: There were 40 implantations in 39 patients (28 with cardiomyopathy, 11 with congenital heart disease, including 3 with univentricular physiology). The median support was 8 months (range, 1-79), with 13 patients (33%) supported for 12 months or more and a cumulative duration of 41 patient-years. The median age and weight at implantation were 11 (4-18) years and 35 (14-98) kg, respectively. The median body surface area was 1.1 (0.7-2.2) m 2 , with 16 patients (40%) having a body surface area less than 1.0 m 2 . Thirty-four patients (85%) had Interagency Registry for Mechanically Assisted Circulatory Support 1 or 2. Children with congenital heart disease were significantly smaller (P < .01) and had more prior cardiac interventions (P < .01) than those with cardiomyopathy. There were 2 early mortalities (5%) in children with cardiomyopathy. Of the 38 patients with successful implantations, 36 (95%) were discharged home and managed as outpatients. Overall adverse event rates were 5.1 (bleeding), 0.8 (device malfunction), 6.1 (infection), 3.9 (neurologic dysfunction), and 1.0 (renal dysfunction) (per 100 patient-month). In the 21 patients with cardiomyopathy supported for 3 months or more, 5 (24%) experienced normalization of left ventricular function; 4 underwent successful explantation, and 1 remains on support., Conclusions: This study demonstrates favorable outcomes of centrifugal-flow ventricular assist device support in children, including those with congenital heart disease, with an increased incidence of cardiac recovery., (Copyright © 2018 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Mortality after surgery for congenital heart disease (CHD) in Mexico is significantly higher than in high-income countries due to structural, medical, and financial factors. In Mexico, public hospitals have a large volume of patients but inadequate quality control systems, whereas private hospitals, although having higher quality control systems, have an insufficient number of patients to build programs of excellence. We describe the creation of a novel hybrid private-public program in Mexico that leverages the advantages of both sectors while establishing an integrated multidisciplinary unit that has allowed us to improve the quality of care for patients with CHD.

Background: We reviewed our single institutional experience with pediatric ventricular assist device (VAD) support over the last 2 decades, with an aim to improve our current management and gain an insight into the future direction., Methods: A retrospective review was conducted on all patients that had undergone VAD support between 1996 and 2017. Outcomes were analyzed based on the type of VADs, whether temporary or durable devices. Primary end points were positive outcomes, including bridge-to-transplantation, bridge-to-recovery, alive on device, and bridge-to-bridge to another VAD, or negative outcomes, including death during VAD support or in-hospital death after bridge-to-recovery. The Pediatric Interagency Registry for Mechanical Circulatory Support definition was used to classify adverse events., Results: Overall, 201 VADs were implanted in 159 patients, with 82 (41%) and 119 (59%) being temporary and durable support, respectively. There has been a trend toward an increasing annual implant volume both with temporary and durable VADs. Positive outcomes were achieved in 80% (66 of 82) of those with temporary support, with bridge-to-recovery (53% [35 of 66]) and bridge-to-bridge to another VAD (38% [25 of 66]) being the predominant outcomes. Of those on durable support, 84% (100 of 119) achieved positive outcomes, with bridge-to-transplant (66% [78 of 119]) being the leading destination. The most notable change during the study period was the introduction of implantable continuous-flow VADs, resulting in outpatient management becoming a routine practice. No patients were discharged on VAD support before 2004, but 85% were discharged on VADs with discharge capability after 2013., Conclusions: The present study has demonstrated the evolutional changes of pediatric VAD support and their effect on clinical outcomes over the last 2 decades., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objective: The exact incidence and risk factors for reoperation in partial and transitional atrioventricular septal defects are unclear. The goal of this study was to assess risk factors for left atrioventricular valve and left ventricular outflow tract reoperation in partial and transitional atrioventricular septal defects., Methods: All patients undergoing partial and transitional atrioventricular septal defects repair between 1995 and 2017 were reviewed. Patients were classified as infants (<1 year), toddlers (1-3 years), children (3-17 years), and adults (≥18 years). Survival and reoperation were assessed using log-rank test and Cox models for univariate and multivariable analyses, respectively., Results: Overall, 265 patients underwent partial and transitional atrioventricular septal defects repair (partial: 177 [67%]). Median age was 2 years. The cohort included 73 infants (28%), 85 toddlers (32%), 94 children (35%), and 13 adults (5%). Trisomy 21 was present in 76 patients (29%), and in 216 patients (83%), the zone of apposition was completely closed. Perioperative mortality was 0.8%. Complete heart block did not develop in any patients. Ten-year survival and freedom from reoperation were 98% and 81%, respectively. On multivariable analysis, trisomy 21 (hazard ratio [HR], 0.16) and older age compared with infants (toddlers: HR, 0.35; children: HR, 0.25) were protective for any reoperation, whereas heterotaxy (HR, 3.43) was a risk factor. For left atrioventricular valve reoperation, toddlers (HR, 0.35), children (HR, 0.25), and trisomy 21 (HR, 0.16) remained protective, whereas left atrioventricular valve anomaly was a risk factor (HR, 2.61). Likewise, for left ventricular outflow tract reoperation, toddlers (HR, 0.24) and children (HR, 0.06) were protective., Conclusions: Mortality after partial and transitional atrioventricular septal defects repair is minimal, yet reoperation for left atrioventricular valve disease and left ventricular outflow tract obstruction remains significant. Patients requiring repair during infancy are at higher risk of reoperation., (Copyright © 2018. Published by Elsevier Inc.)

Background: Contemporary outcomes of complete atrioventricular septal defect (CAVSD) repair, particularly for defects with associated abnormalities, is unclear. The goal of this study is to report an all-inclusive experience of CAVSD repair using a consistent surgical approach., Methods: All patients undergoing CAVSD repair between 1995 and 2016 at our institution were included. Patients were divided into 2 groups: isolated and complex (tetralogy of Fallot, aortic arch repair, double outlet right ventricle, and total anomalous pulmonary venous return). Survival and reoperation were analyzed using log-rank test and Gray's test, respectively. Multivariable analysis was performed with Cox regression., Results: Overall, 406 patients underwent repair: 350 (86%) isolated and 56 (14%) complex CAVSD (tetralogy of Fallot: 34, double outlet right ventricle: 7, aortic arch repair: 12, total anomalous pulmonary venous return: 3). Median age at repair was 5 months (range, 10 days to 16 years); 339 (84%) had trisomy 21. A 2-patch repair was used in 395 (97%) and the zone of apposition was completely closed in 305 (75%). Perioperative mortality was 2% and 4% in the isolated and complex groups, respectively. Perioperative mortality since 2006 was 0.9%. Median follow-up was 7 years. Overall 10-year survival and incidence of any reoperation were 92% and 11%, respectively. Complex anatomy was not a risk factor for mortality (p = 0.35), but it was for reoperation (hazard ratio [HR]: 2.6; p < 0.01). Risk factors for left atrioventricular valve reoperation were a second bypass run (HR: 2.7) and preoperative moderate or worse regurgitation (HR: 2.3)., Conclusions: Mortality after CAVSD repair is low, yet reoperation remains a significant problem. Repair of complex CAVSD can be performed with similar mortality rates., (Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Development of pediatric ventricular assist devices (VADs) has significantly lagged behind that of adult devices. This frustrating reality is reflected by the fact that the Berlin Heart EXCOR VAD is currently the only approved pediatric-specific device in the USA. An alternative option is an off-label use of adult continuous-flow VADs, such as HeartMate II (HMII), which inevitably causes patient-device size mismatch in small children. We sought to conduct in vitro hemocompatibility testing in a pediatric flow condition, with a specific aim to provide benchmark values for future pediatric device development. Given the aforementioned fact that both pulsatile and continuous-flow devices are being used in the pediatric population, we opted to test both types of devices in the present study. The EXCOR and HMII blood pumps were tested using bovine blood under constant hemodynamic conditions (flow rate, Q = 2.5 ± 0.25L/min; differential pressure across the pump, ΔP = 68 ± 5mm Hg). Hemolysis was measured by Harboe assay. There was a steady increase in plasma free hemoglobin during in vitro testing, with a statistically significant difference between 5 and 360 min for both EXCOR (P < 0.0001) and HMII (P < 0.001). However, the degree of an increase in plasma free hemoglobin was more significant with HMII (P < 0.001). Normalized index of hemolysis for EXCOR and HMII were 0.003 ± 0.0026g/100 L and 0.085 ± 0.0119g/100 L, respectively. There was also a steady increase in platelet activation detected by CAPP2A antibody using flow cytometry, with a statistically significant difference between 5 and 360 min for both devices (P < 0.05). The degree of an increase in platelet activation was similar between the two devices (P = 0.218). High molecular weight von Willebrand factor (HMW vWF) multimer degradation measured by immunoblotting was evident for both devices, however, it was more pronounced with the EXCOR. EXCOR blood samples from all three time points (120, 240, and 360 min) were significantly different from the baseline (5 min), whereas only 360 min samples had a significant difference from the baseline with the HMII. In conclusion, we have observed similarities and differences in hemocompatibility profiles between the EXCOR and HMII, both of which are commonly used in the pediatric population. We anticipate the benchmark values in the present study will facilitate future pediatric VAD development., (© 2018 International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc.)

Objective: There are limited studies analyzing pulsatile Glenn as a long-term palliation strategy for single ventricle patients. This study sought to determine their outcomes at a single institution., Design: A retrospective review was performed., Setting: Study performed at a single pediatric hospital., Patients: All single ventricle patients who underwent pulsatile Glenn from 1995 to 2016 were included., Outcome Measures: Pulsatile Glenn failure was defined as takedown, transplant, or death. Further palliation was defined as Fontan, 1.5, or biventricular repair. Risk factors were assessed by Cox multivariable competing risk analyses., Results: Seventy-eight patients underwent pulsatile Glenn at age 9 months (interquartile range, 5-14). In total, 28% had heterotaxy, 18% had a genetic syndrome, and 24% had an abnormal inferior vena cava. There were 3 (4%) perioperative mortalities. Further palliation was performed in 41 (53%) patients with a median time-to-palliation of 4 years (interquartile range, 3-5). Pulsatile Glenn failure occurred in 10 (13%) patients with 8 total mortalities. Five- and 10-year transplant-free survival were 91% and 84%, respectively. At a median follow-up of 6 years (interquartile range, 2-8), 27 patients (35%) remained with PG (age 7 years [interquartile range, 3-11], oxygen saturation 83% ± 4%). Preoperative moderate-severe atrioventricular valve regurgitation (AVVR) (hazard ratio 7.77; 95% confidence interval 1.80-33.43; P =.005) and higher pulmonary vascular resistance (hazard ratio 2.59; 95% confidence interval 1.08-6.15; P =.031) were predictors of pulsatile Glenn failure after adjusting for covariates. Reaching further palliation was less likely in patients with preoperative moderate-severe AVVR (hazard ratio 0.22, 95% confidence interval 0.08-0.59; P =.002)., Conclusion: Pulsatile Glenn can be an effective tool to be used in challenging circumstances, these patients can have a favorable long-term prognosis without reducing their suitability for further palliation., (© 2018 Wiley Periodicals, Inc.)