Background:This study assessed postoperative outcomes by using markers of myocardial injury in infants who received del Nido vs institutional cardioplegia solution. Methods:Eighty-five infants undergoing cardiac surgery were enrolled (del Nido, n = 45; institutional, n = 40). Primary outcomes included serum troponin I, creatine kinase myocardial band (CK-MB), and heart fatty acid-binding protein on cardiothoracic intensive care unit (CTICU) admission and at 6, 12, and 24 hours postoperatively. Secondary outcomes included vasoactive inotropic score, arrhythmias, ventricular ejection fraction, ventilation hours, CTICU hours, hospital length of stay, and mortality. Results:Patients receiving del Nido cardioplegia solution were older (174 days vs 105 days) and had higher body weight (5.95 kg vs 5.05 kg). They received greater cardioplegia volume and had longer bypass times. The del Nido cohort showed higher initial postoperative troponin I (1.53 times at admission; P = .02) and CK-MB levels (1.61 times on admission; P = .01; 1.51 times at 6 hours; P = .03; 1.55 times at 12 hours; P = .02) before normalizing. No differences were found in heart fatty acid-binding protein levels. Median postoperative hospital length of stay (5 days vs 7 days; P = .2) and CTICU stay (45 hours vs 70 hours; P = .077) were shorter for patients receiving the del Nido cardioplegia, although this was not statistically significant. No differences were observed in arrhythmias, ventricular ejection fraction, intubation time, or vasoactive inotropic score. There was no in-hospital or 30-day mortality. Conclusions:The del Nido cardioplegia was associated with higher initial postoperative troponin I and CK-MB levels in infant cardiac surgery patients. However, no significant clinical outcome differences were found between the 2 solutions.

The hybrid pathway is an alternative surgical route for patients with hypoplastic left heart syndrome (HLHS) and patients with single-ventricle systemic obstructive physiology. Limited data exist on post-Fontan arrhythmias in these patients. This study aimed to assess the type and frequency of arrhythmias post-Fontan in hybrid pathway patients and compare them to the Single Ventricle Reconstruction Trial (SVRT). A retrospective chart review was performed on all patients with single-ventricle anatomy who underwent the hybrid procedure and subsequently completed the Fontan surgery. Exclusion criteria included incomplete pre- or post-operative Fontan data or lack of post-Fontan hospital discharge. Baseline demographics, pre-Fontan ECG, 6-year ECG and Holter, and cardiac medications were recorded. Arrhythmias were defined as isolated atrial or ventricular ectopy, non-sustained or sustained tachyarrhythmias. A total of 116 patients met criteria (HLHS n = 79, single-ventricle variants n = 37). Fontan age was 2.7 ± 0.7 years, post-Fontan ECG age was 6.4 ± 0.9 years, and Holter age was 7.0 ± 1.8 years. Mortality occurred in 3 patients and transplantation in 2 patients. Atrial arrhythmias appeared in 69

Background:This study aimed to investigate the impact of early extubation on stress response and neurodevelopmental outcomes after pediatric cardiac surgery utilizing cardiopulmonary bypass (CPB). Methods:In this single-center prospective pilot study, we attempted to study the impact of using dexmedetomidine as an adjunct to facilitate early extubation after pediatric cardiac surgery requiring CPB during the first year of life. The study was conducted between May 2014 and January 2020. Perioperative data and stress hormone levels were collected at different points during the perioperative period. In addition, neurodevelopmental outcome measures including cognitive composite score, language (expressive and receptive) and motor (fine and gross) composites were evaluated at five time points including prior to surgery and up to 1 year after the procedure. Two-sample t-tests and Kruskal-Wallis tests were used to compare continuous parametric and non-parametric outcomes, respectively. Fisher's exact or Chi-squared tests were used to compare categorical outcomes. Results:A total of 30 subjects were included in the final cohort of patients. Of the 30 subjects, 14 patients were randomized to the dexmedetomidine group (dexmedetomidine plus fentanyl) (DEX group) and 16 patients were randomized to the no dexmedetomidine group (fentanyl only) (no DEX group). With few exceptions, both groups demonstrated appropriate blunting of the stress response. There was a significant increase in the ratio of the pro-inflammatory interleukin-10 (IL-10) to the anti-inflammatory interleukin-6 (IL-6) for the no DEX group at the end of the procedure when compared to the DEX group (10 ± 9 vs. 5 ± 4, P = 0.04). When looking at the Bayley cognitive composite score, the DEX group scored better than the no DEX group during the second visit (102 ± 11 vs. 88 ± 17, P = 0.023). By the fifth visit, the two groups scored similarly (94 ± 12 vs. 94 ± 12, P = 0.9 for the no DEX and DEX groups, respectively). Conclusion:When looking at the neurodevelopmental outcome, both groups had no significant changes in their Bayley scores from baseline with blunting of most stress markers. This study offers possible evidence of the safety of early extubation after pediatric cardiac surgery while maintaining the goal of neuroprotection.

Background:The optimal timing of a balloon atrial septostomy (BAS) in patients with hypoplastic left heart syndrome (HLHS) undergoing hybrid stage 1 (HS1) palliation is unknown. We hypothesized that concomitant completion of the BAS and HS1 would decrease hospital stay, increase intensive care unit-free days, improve hemodynamic markers, and increase transplant-free survival. Methods:We retrospectively reviewed HLHS patients palliated with HS1 from January 2009 to July 2022 at our center. We excluded other single ventricle variants, those who did not have a BAS or had a BAS performed prior to the HS1, and those with an initial atrial septal intervention other than BAS. Patients were divided into 2 groups: (1) those with same-day BAS and HS1, and (2) BAS performed ≥1 day after the HS1. Results:Twenty patients had a BAS and HS1 on the same day, and 69 patients had delayed BAS, whereas 29 patients were excluded. The same-day cohort had significantly shorter hospital length of stay, higher recorded cerebral near infrared spectroscopy troughs, and lower 30-day post-BAS/discharge brain natriuretic peptide levels. There was no difference in transplant-free survival, and a greater proportion of the same-day group required atrial septal reintervention. Conclusions:Although there was no difference in transplant-free survival and higher rates of atrial septal reintervention, concomitant completion of BAS and HS1 in HLHS patients demonstrated shorter HS1 hospitalization and improved hemodynamic markers.

There are no widely accepted guidelines for mediastinal chest tube removal following cardiopulmonary bypass (CPB) surgery and management is highly variable. The aim of this retrospective study was to review our institutional routine of mediastinal chest tube removal on postoperative day (POD) one. We retrospectively reviewed patients undergoing CPB surgery from January 1, 2023 to December 31, 2023, with mediastinal chest tube removal on POD 1. Variables examined included age, operation, chest tube output, postoperative complications, hospital length of stay, discharge tests, and readmission within 30 days. Two hundred thirteen patients underwent CPB surgery of which 158 (74

BACKGROUNDSingle ventricle cardiac defects (SVCD) are one of the most healthcare-resource intensive congenital diseases. While traditionally palliated using the Norwood pathway, in the last two decades select programs have utilized the Hybrid strategy which redistributes operative and interstage risks. We sought to characterize resource utilization for our unique cohort of hybrid-palliated SVCD patients.METHODSAll SVCD patients palliated via the Hybrid strategy who followed exclusively at our institution from January 2008 to December 2021 were included. Endpoints were death, Norwood conversion, orthotopic heart transplant, 6 months post-Fontan, or 4 years of age in those who haven’t completed staged palliation. Primary endpoints included total days hospitalized, number of cardiology visits, echocardiograms, catheterizations, and advanced imaging performed.RESULTSOf 135 patients diagnosed with SVCD, 72 survived 6 months post-Fontan. Per patient-year for the entirety of the pathway, patients had a median LOS of 16 days (12-25), 7 cardiology visits (6-9), 8 echocardiograms (7-10), and 0.7 catheterizations (0.4-1.1). The interstage 1 period had the heaviest resource burden requiring intense cardiology follow-up and echocardiography surveillance. Cardiac catheterizations and advanced imaging were most prevalent during interstage 2 period with a median of 2 (1-2) catheterizations and 36 (40%) patients undergoing advanced imaging. Total median number of hospital days per patient was 63 (47-98.5).CONCLUSIONSResource utilization for the care of SVCD patients is significant. Intensity of surveillance decreases as patients progress through the pathway. In comparison to published Norwood pathway data, resource intensity and utilization patterns in Hybrid palliation are comparable.

OBJECTIVE:Our objective was to assess the predictive value of physiologic dead space fraction for mortality in patients undergoing the comprehensive stage 2 operation. METHODS:This was a single-centre retrospective observational study conducted at a quaternary free-standing children's hospital specialising in hybrid palliation of single ventricle cardiac disease. 180 patients underwent the comprehensive stage 2 operation. 76 patients (42%) underwent early extubation, 59 (33%) standard extubation, and 45 (25%) delayed extubation. We measured time to extubation, post-operative outcomes, length of stay and utilised Fine gray models, Youden's J statistic, cumulative incidence function, and logistic regression to analyse outcomes. RESULTS:Delayed extubation group suffered significantly higher rates of mortality (31.1% vs. 6.8%), cardiac arrest (40.0% vs. 10.2%), stroke (37.8% vs. 11.9%), and need for catheter (28.9% vs. 5.1%) and surgical intervention (24.4% vs. 8.5%) (P < 0.001). Physiologic dead space fraction was significantly higher in the delayed extubation group and in non-survivors with a value of 0.3, which was found to be the discriminatory point by Youden's J statistic. For a 0.1 unit increase in physiologic dead space fraction on post-operative day 1, the odds of a patient expiring increase by a factor of 2.26 (95% CI 1.41-3.97, p < 0.001) and by a factor of 3.79 (95% CI 1.65-11.7, p 0.01) on post-operative day 3. CONCLUSIONS:Delayed extubation impacts morbidity and mortality in patients undergoing the comprehensive stage 2 operation. Increased physiologic dead space fraction in the first 60 hours after arrival to the ICU is associated with higher mortality.

Background Pathogenic GATA6 variants have been associated with congenital heart disease (CHD) and a spectrum of extracardiac abnormalities, including pancreatic agenesis, congenital diaphragmatic hernia, and developmental delay. However, the comprehensive genotype-phenotype correlation of pathogenic GATA6 variation in humans remains to be fully understood. Methods Exome sequencing was performed in a family where four members had CHD. In vitro functional analysis of the GATA6 variant was performed using immunofluorescence, western blot, and dual-luciferase reporter assay. Results A novel, heterozygous missense variant in GATA6 (c.1403 G > A; p.Cys468Tyr) segregated with affected members in a family with CHD, including three with persistent truncus arteriosus. In addition, one member had childhood onset diabetes mellitus (DM), and another had necrotizing enterocolitis (NEC) with intestinal perforation. The p.Cys468Tyr variant was located in the c-terminal zinc finger domain encoded by exon 4. The mutant protein demonstrated an abnormal nuclear localization pattern with protein aggregation and decreased transcriptional activity. Conclusions We report a novel, familial GATA6 likely pathogenic variant associated with CHD, DM, and NEC with intestinal perforation. These findings expand the phenotypic spectrum of pathologic GATA6 variation to include intestinal abnormalities. Impact Exome sequencing identified a novel heterozygous GATA6 variant (p.Cys468Tyr) that segregated in a family with CHD including persistent truncus arteriosus, atrial septal defects and bicuspid aortic valve. Additionally, affected members displayed extracardiac findings including childhood-onset diabetes mellitus, and uniquely, necrotizing enterocolitis with intestinal perforation in the first four days of life. In vitro functional assays demonstrated that GATA6 p.Cys468Tyr variant leads to cellular localization defects and decreased transactivation activity. This work supports the importance of GATA6 as a causative gene for CHD and expands the phenotypic spectrum of pathogenic GATA6 variation, highlighting neonatal intestinal perforation as a novel extracardiac phenotype.

Objective: To determine patient characteristics and outcomes after Norwood versus comprehensive stage II (COMPSII) for infants with critical left heart obstruction who had prior hybrid palliation (bilateral pulmonary artery banding +/- ductal stent).Methods: From 23 Congenital Heart Surgeons' Society institutions (2005-2020), 138 infants underwent hybrid palliation followed by either Norwood (n = 73, 53%) or COMPSII (n = 65). Baseline characteristics were compared between Norwood and COMPSII groups. Parametric hazard model with competing risk methodology was used to determine risk and factors associated with outcomes of Fontan, transplantation, or death.Results: Infants who underwent Norwood versus COMPSII had a higher prevalence of prematurity (26% vs 14%, P = .08), lower birth weight (median 2.8 vs 3.2 kg, P < .01) and less frequent ductal stenting (37% vs 99%; P < .01). Norwood was performed at a median age of 44 days and median weight of 3.5 kg, versus COMPSII at 162 days and 6.0 kg (both P<.01). Median follow-up was 6.5 years. At 5 years after Norwood and COMPSII, respectively; 50% versus 68% had Fontan (P = .16), 3% versus 5% had transplantation (P = .70), 40% versus 15% died (P = .10), and 7% versus 11% are alive without transition, respectively. For factors associated with either mortality or Fontan, only preoperative mechanical ventilation occurred more frequently in the Norwood group.Conclusions: Higher prevalence of prematurity, lower birth weight, and other patient-related characteristics in the Norwood versus COMPSII groups may influence differences in outcomes that were not statistically significant for this limited risk-adjusted cohort. The clinical decision regarding Norwood versus COMPSII after initial hybrid palliation remains challenging. (J Thorac Cardiovasc Surg 2023;166:943-54)

NEONATE score > 17 has been proposed as a risk factor for interstage mortality/cardiac transplant (IM/T) for patients with single ventricle physiology. Hybrid procedure is assigned 6 points, the highest possible score for that surgical variable. Most centers reserve the hybrid procedure for high-risk patients. Goal of this study was to evaluate the NEONATE score at a center that routinely performs the hybrid procedure. Retrospective chart review of patients undergoing the hybrid procedure was performed (2008–2021). Demographics and variables used for the NEONATE score were collected. Maximization of Youden’s J Statistic used to determine cohort-specific optimal threshold for patients undergoing comprehensive Stage II procedure (H-CSII) versus those with IM/T (H-IM/T). Total of 120 patients met inclusion criteria (H-CSII = 105, H-IM/T = 15). Gestational age was median 39 weeks (IQR 38, 39) and birth weight was 3.18 kg (2.91, 3.57). No patient was discharged with opiates or required post-operative extracorporeal circulatory support. Optimal threshold, as selected by maximizing Youden’s J Statistic, was 22. Score > 22 had a positive predictive value of 0.33 (95

Objective: Pulmonary artery reconstruction during comprehensive stage 2 (CS2) procedure can be challenging. Since 2017, we have employed preemptive left pulmonary artery (LPA) stenting. We hypothesized that LPA stenting promotes adequate growth and without compromising Fontan candidacy. Herewith, we report our midterm results. Methods: From 2002 to 2020, 159 patients underwent CS2. Patients were divided as follows: no stent (n = 122; Group 1) and perioperative LPA stent (n = 37; Group 2). Group 2 was subdivided according to unplanned stent (n = 17; Group 2a) or preemptive stent (n = 20; Group 2b). Relevant perioperative data was reviewed. Nonparametric statistics were utilized. Results: Median age and weight at surgery and hospital length of stay after CS2 did not differ between groups. Median cardiopulmonary bypass and crossclamp times were significantly greater in Group 1 (265 vs 243 minutes [P = .021] and 46 vs 26 minutes [P = .008]). In-hospital mortality was similar between Groups 1 and 2 (9.0% vs 18.9%, respectively [P = .1348]). Group 2b demonstrated a superior survival compared to Group 2a (P = .0335) but not Group 1 (P > .9999). Preemptive stenting significantly increased median hilar LPA diameter at CS2 exit angiogram compared with no stenting (P < .0001). Groups 2a and 2b significantly increased the pre-Fontan diameter of the hilar LPA when compared with Group 1 (6.1 and 6.8 vs 5.7 mm, respectively [P < .0001]). A further 120 patients underwent Fontan operation (75%). Median follow-up for Groups 1 and 2 were 7.4 and 3.0 years, respectively. Conclusions: Perioperative LPA stenting during CS2 does not adversely affect pulmonary growth. Preemptive stenting seems advantageous for LPA growth in preparation for Fontan completion.

BACKGROUND Hybrid palliation (bilateral pulmonary artery banding with or without ductal stenting) is an initial management strategy for infants with critical left heart obstruction and serves as palliation until subsequent operations are pursued. OBJECTIVES This study sought to determine patient characteristics and factors associated with subsequent outcomes for infants who underwent hybrid palliation. METHODS From 2005 to 2019, 214 of 1,236 prospectively enrolled infants within the Congenital Heart Surgeons' Society's critical left heart obstruction cohort underwent hybrid palliation across 24 institutions. Multivariable hazard modeling with competing risk methodology was performed to determine risk and factors associated with outcomes of biventricular repair, Fontan procedure, transplantation, or death. RESULTS Preoperative comorbidities (eg, prematurity, low birth weight, genetic syndrome) were identified in 70% of infants (150 of 214). Median follow-up was 7 years, ranging up to 17 years. Overall 12-year survival was 55%. At 5 years after hybrid palliation, 9% had biventricular repair, 36% had Fontan procedure, 12% had transplantation, 35% died without surgical endpoints, and 8% were alive without an endpoint. Factors associated with transplantation were absence of ductal stent, older age, absent interatrial communication, smaller aortic root size, larger tricuspid valve area z-score, and larger left ventricular volume. Factors associated with death were low birth weight, concomitant genetic syndrome, cardiopulmonary bypass use during hybrid palliation, moderate to severe tricuspid valve regurgitation, and smaller ascending aortic size. CONCLUSIONS Mortality remains high after hybrid palliation for infants with critical left heart obstruction. None-theless, hybrid palliation may facilitate biventricular repair for some infants and for others may serve as stabilization for intended functional univentricular palliation or primary transplantation. (J Am Coll Cardiol 2023;82:1427-1441) (c) 2023 by the American College of Cardiology Foundation.

Objective: The prevalence of postoperative cardiac arrest (CA) increases with cardiothoracic surgical case complexity and is associated with a 40% % to 50% % mortality. Despite having a low overall surgical mortality rate at our center, our postoperative CA rates were higher than expected, with an observed-to-expected ratio of 2.6. Utilizing quality improvement methodology, we evaluated the influence of proactive risk mitigation on postprocedure CA in a high-risk cohort of pediatric cardiac patients. Methods: This single-center study utilized the Institute for Healthcare Improvement model. We created and implemented our Proactive Mitigation to Decrease Serious Adverse Events program in July 2020, prospectively enrolling preidentified fi ed high-risk patients. Enrolled patients underwent scheduled multidisciplinary reviews via virtual platform at 2 periprocedural time points with discussion of patient- specific risks and the subsequent development of proactive risk mitigation plans. Primary outcome measures were derived from the Pediatric Cardiac Critical Care Consortium national registry and included rate of postprocedure CA within 7 days and an institution-specific observed-to-expected ratio for postoperative CA. Results: Our baseline median number of high-risk cases between postprocedure CAs was 3. Following project initiation, median high-risk cases between events increased to 7. Our observed-to-expected ratio for postoperative CA decreased from 2.56 during the 12 months before Proactive Mitigation to Decrease Serious Adverse Events program implementation to 1.01 during the 12 months after Proactive Mitigation to Decrease Serious Adverse Events program implementation, and hospital length of stay decreased by 10 days. Conclusions: Implementation of periprocedure-related proactive risk mitigation strategies in high-risk pediatric cardiac patients led to improvement in postprocedure CA with a 133% % increase in high-risk cases between events. (JTCVS Open 2023;13:307-19)

Background : Neonates and infants undergoing cardiac surgery tend to receive high volumes of blood products. The use of rotational thromboelastometry (ROTEM ® ) has been shown to reduce the administration of blood products in adults after cardiac surgery. We sought to develop a targeted administration of blood products based on ROTEM ® to reduce blood product utilization during and after neonatal and infant cardiac surgery. Methods : We conducted a retrospective review of data from a single center for neonates and infants undergoing congenital cardiac surgery using cardiopulmonary bypass (CPB) from September 2018-April 2019 (control group). Then, using a ROTEM ® algorithm, we collected data prospectively between April-November 2021 (ROTEM group). Data collected included age, weight, gender, procedure, STAT score, CPB time, aortic cross-clamp time, volume, and type of blood products administered in the operating room and cardiothoracic intensive care unit (CTICU). In addition, ROTEM ® data, coagulation profile in CTICU, chest tube output at 6 and 24 hours, use of factors concentrate, and thromboembolic complications were recorded. Results : The final cohort of patients included 28 patients in the control group and 40 patients in the ROTEM group. The cohort included neonates and infants undergoing the following procedures: arterial switch, aortic arch augmentation, Norwood procedure, and comprehensive stage II procedure. There were no differences in the demographics or procedure complexity between the two groups. Patients in the ROTEM ® group received fewer platelets (36 ± 12 vs. 49 ± 27 mL/kg, p 0.028) and cryoprecipitate (8 ± 3 vs. 15 ± 10 mL/kg, p 0.001) intraoperatively when compared to the control group. Conclusion : The utilization of ROTEM ® may have contributed to a significant reduction in some blood product administration during cardiac surgery for infants and neonates. ROTEM ® data may play a role in reducing blood product administration in neonatal and infant cardiac surgery.

Purpose Donor-derived cell-free DNA (dd-cfDNA) is utilized by some adult lung transplant (LTX) centers as a biomarker of allograft injury or dysfunction. An elevated level of dd-cfDNA may be predictive of acute cellular rejection, antibody mediated rejection (AMR), chronic lung allograft dysfunction , or acute infection. Therefore, dd-cfDNA may have a role as a surrogate test for graft surveillance versus invasive methods including bronchoscopy with bronchoalveolar lavage (BAL) and transbronchial biopsy (TBBX). While there is an existing body of literature on the role of dd-cfDNA in adult LTX, there is limited understanding of its utility in pediatric patients. Non-invasive testing such as dd-cfDNA may be particularly helpful in young children with inherent challenges with TBBX. Methods Our center performed three bilateral LTX in pediatric patients in which we used dd-cfDNA as an adjunctive method of graft surveillance. All were females, ranging in age from 2-11 years. We obtained dd-cfDNA levels prior to bronchoscopy, BAL and TBBX . The % dd-cfDNA was evaluated in conjunction with clinical symptoms, BAL and TBBX results, and measurements of panel reactive antibody (cPRA) and donor specific antibodies. Results The specific diagnoses leading to transplant were interstitial lung disease related to juvenile idiopathic arthritis, post-infectious bronchiolitis obliterans, and alveolar capillary dysplasia. Results of data collected are summarized in Table 1. Conclusion In this small cohort of pediatric LTX recipients, we found consistently elevated dd-cfDNA levels. In two patients, dd-cfDNA levels did not correlate with histological evidence of graft dysfunction. However, in one patient, sustained high dd-cfDNA values may have signaled ongoing graft injury from AMR as a precedent to the onset of symptoms, subsequent respiratory failure, and ultimate clinical demise. Further investigation into dd-cfDNA as a biomarker of allograft injury in pediatric LTX recipients is needed.

OBJECTIVE:We sought to simplify reporting of outcomes in congenital heart surgery that compares well-defined patient groups and accommodates multiple stakeholder needs while being easily understandable. METHODS:We selected 19 commonly performed congenital heart surgeries ranging in complexity from repair of atrial septal defects to the Norwood procedure. Strict inclusion/exclusion criteria ensured the creation of 19 well-defined diagnosis/procedure cohorts. Preoperative, procedural, and postoperative data were collected for consecutive eligible patients from 9 centers between January 1, 2016, and December 31, 2021. Unadjusted operative mortality rates and hospital length of stay for each of the 19 diagnosis/procedure cohorts were summarized in aggregate and stratified by each center. RESULTS:Of 8572 eligible cases included, numbers in the 19 diagnosis/procedure cohorts ranged from 73 for tetralogy of Fallot repair after previous palliation to 1224 for ventricular septal defect (VSD) repair for isolated VSD. In aggregate, the unadjusted mortality ranged from 0% for atrial septal defect repair to 28.4% for hybrid stage I. There was significant heterogeneity in case mix and mortality for different diagnosis/procedure cohorts across centers (eg, arterial switch operation/VSD, n = 7-42, mortality 0%-7.4%; Norwood procedure, n = 16-122, mortality 5.3%-25%). CONCLUSIONS:Reporting of institutional case volumes and outcomes within well-defined diagnosis/procedure cohorts can enable centers to benchmark outcomes, understand trends in mortality, and direct quality improvement. When made public, this type of report could provide parents with information on institutional volumes and outcomes and allow them to better understand the experience of each program with operations for specific congenital heart defects.