Comparing apples to apples: Exploring public reporting of congenital cardiac surgery outcomes based on common congenital heart operations.

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.

Novel pathogenic GATA6 variant associated with congenital heart disease, diabetes mellitus and necrotizing enterocolitis.

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.

Outcomes After Hybrid Palliation for Infants With Critical Left Heart Obstruction.

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. Nonetheless, hybrid palliation may facilitate biventricular repair for some infants and for others may serve as stabilization for intended functional univentricular palliation or primary transplantation.

Utility of the NEONATE Score at an Institution that Routinely Performs the Hybrid Procedure for Hypoplastic Left Heart Syndrome.

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% CI 0.12-0.62), negative predictive values of 0.90 (95% CI 0.83-0.95), and accuracy of 0.83 (95% CI 0.75-0.90) for IM/T. At a center that routinely performs the hybrid procedure, value of > 22 had the highest accuracy. This suggests that the hybrid procedure is not necessarily intrinsically a risk-factor for IM/T, but rather patient selection for the hybrid procedure may play a larger role at centers that do not routinely perform this procedure.

A ROTEM-guided algorithm aimed to reduce blood product utilization during neonatal and infant cardiac surgery.

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.

Proactive risk mitigation for cardiac arrest prevention in high-risk patients with congenital heart disease.

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 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.

Preemptive stenting of the left pulmonary artery during comprehensive stage 2 procedure does not influence Fontan candidacy.

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.

Norwood operation versus comprehensive stage II after bilateral pulmonary artery banding palliation for infants with critical left heart obstruction.

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.

Management of Hypertrophic Cardiomyopathy in a Newborn with Dextro-Transposition of the Great Arteries.

Impaired maternal glucose metabolism during pregnancy can have significant effects on the cardiovascular system of the developing fetus. Early in pregnancy the teratogenic effects may lead to structural heart defects, while later in gestation a form of hypertrophic cardiomyopathy can develop due to overgrowth driven by fetal hyperinsulinism. We describe an infant with the uncommon combination of both dextro-transposition of the great arteries and hypertrophic cardiomyopathy. We emphasize the importance of a longitudinal multi-disciplinary approach, from fetal diagnosis to post-operative management, that allowed for an excellent outcome in this rare combination of severe cardiac malformations.

Post-operative Anticoagulation Strategy Following Comprehensive Stage 2 Procedure for Single Ventricle Physiology.

Thrombosis, especially thrombosis of the pulmonary artery, is a large contributor to morbidity and mortality following comprehensive stage 2 procedure for single ventricle cardiac physiology. A peri-operative management protocol was implemented at our institution in March 2010. It includes 6 weeks of therapeutic anticoagulation post-operatively to mitigate the thrombotic risks in this patient population. This is a retrospective study of hospitalized children who received post-operative anticoagulation following a comprehensive stage 2 procedure for single ventricle cardiac physiology at a free-standing children's hospital. The primary objectives are to describe our institution's anticoagulation strategy and report on the number of thromboses and major bleeding episodes in the 6 weeks post-operatively. Secondary objectives include the dose of enoxaparin required to obtain a therapeutic low-molecular weight anti-factor-Xa (AFXaLMWH) level, and the number of patients outside of the therapeutic range. A total of 71 infants were included in the final analysis. Four patients experienced a thrombosis episode and three patients experienced clinically significant bleeding. The mean dose of enoxaparin required to obtain a therapeutic AFXaLMWH level between 0.5-1 unit/mL was 1.23 mg/kg SQ every 12 h and 37% of patients achieved goal AFXaLMWH levels with the initial starting dose of enoxaparin 1 mg/kg SQ every 12 h. We describe a 9-year experience of anticoagulation after single ventricle palliation. Anticoagulation with therapeutic AFXaLMWH goals of 0.5-1 unit/mL may reduce the rates of clinically significant thrombosis post-operatively in this population and appears safe without increase in significant bleeding episodes when compared to a historical cohort. Further studies comparing this population to those who do not receive post-operative anticoagulation are warranted.

Mortality Prediction After Cardiac Surgery in Children: An STS Congenital Heart Surgery Database Analysis.

BACKGROUND: The Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database (CHSD) provides risk-adjusted operative mortality rates to approximately 120 North American congenital heart centers. Optimal case-mix adjustment methods for operative mortality risk prediction in this population remain unclear. METHODS: A panel created diagnosis-procedure combinations of encounters in the CHSD. Models for operative mortality using the new diagnosis-procedure categories, procedure-specific risk factors, and syndromes or abnormalities included in the CHSD were estimated using Bayesian additive regression trees and least absolute shrinkage and selector operator (lasso) models. Performance of the new models was compared with the current STS CHSD risk model. RESULTS: Of 98 825 operative encounters (69 063 training; 29 762 testing), 2818 (2.85%) STS-defined operative mortalities were observed. Differences in sensitivity, specificity, and true and false positive predicted values were negligible across models. Calibration for mortality predictions at the higher end of risk from the lasso and Bayesian additive regression trees models was better than predictions from the STS CHSD model, likely because of the new models' inclusion of diagnosis-palliative procedure variables affecting <1% of patients overall but accounting for 27% of mortalities. Model discrimination varied across models for high-risk procedures, hospital volume, and hospitals. CONCLUSIONS: Overall performance of the new models did not differ meaningfully from the STS CHSD risk model. Adding procedure-specific risk factors and allowing diagnosis to modify predicted risk for palliative operations may augment model performance for very high-risk surgical procedures. Given the importance of risk adjustment in estimating hospital quality, a comparative assessment of surgical program quality evaluations using the different models is warranted.

Reevaluating Congenital Heart Surgery Center Performance Using Operative Mortality.

BACKGROUND: The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) provides observed-to-expected (O/E) operative mortality ratios to more than 100 congenital heart centers in North America. We compared the current approach for estimating O/E ratios to approaches incorporating information on diagnosis as moderators of procedures, other unused risk factors, and additional variation in confidence interval construction to characterize center performance. METHODS: Bayesian additive regression trees (BART) and lasso models linked operative mortality to diagnosis-procedure categories, procedure-specific risk factors, and syndromes/abnormalities. Bootstrapping accounted for variation in the STS-CHSD (STS bootstrap) and lasso CIs. We compared O/E estimates, interquartile range of CI widths, and concordance of center performance categorizations (worse-than-, as-, or better-than-expected mortality) of the new approaches to the STS-CHSD. RESULTS: In 110 surgical centers including 98,822 surgical operative encounters, there were 2818 (2.85%) operative mortalities (center range, 0.37%-10%). Compared with the STS-CHSD, BART- and lasso-estimated O/E ratios varied more and had narrower confidence intervals (interquartile range of confidence interval: STS-CHSD = 1.11, STS bootstrap = 0.98; lasso = 0.80; BART = 0.96). Concordance of performance categorization with the STS-CHSD ranged from 84% (lasso) to 91% (STS Bootstrap); more than 70% of discordant centers improved categories. Discordant centers had smaller volumes, fewer operative mortalities, and treated more patients with congenital lung abnormalities. CONCLUSIONS: Relative to the STS-CHSD, up to 16% of hospitals changed performance categories, most improving performance. Given the significance of quality reports for congenital heart centers, inclusion of additional risk factors and unaddressed variation should be considered.

Incidence of and Risk Factors for Aortic Arch Interventions After the Comprehensive Stage II Procedure for Hypoplastic Left Heart Syndrome.

Minimal data exist about the incidence and risk factors for arch intervention after comprehensive stage II (CSII). Goal of this study was to document incidence of arch interventions after CSII and determine if any differences existed between those who underwent an arch intervention (aiCSII) versus those did not have an intervention. Single-center retrospective chart review of all hypoplastic left heart syndrome patients who underwent a CSII between 6/1/2005 and 2/1/2020 was performed. Univariate analysis was conducted in addition to principal components analysis (PCA). One hundred patients were evaluated. Sixteen patients underwent 24 arch interventions. Age at initial arch reintervention was 1.3 ± 1.2 years (median 1.0 years, range 0.5-2.2 years). Univariate analysis showed that the aiCSII group were more likely to be female, to have had a retrograde arch intervention post-hybrid procedure, and to be younger at time of CSII. On echocardiograms, aiCSII group had significantly higher pre-CSII patent ductus arteriosus velocities, arch velocities on their 1st post-operative and discharge study post-CSII, and arch velocities pre-Fontan. Gradients were higher in the aiCSII via pre-Fontan catheterization. With PCA, echocardiographic and catheterization data remained significantly associated with aiCSII versus those who did not undergo an arch intervention (OR = 4.5 (1.9, 19.8), p = 0.008). Incidence of arch intervention post-CSII was 16%. Echocardiographic arch velocities during the CSII hospitalization were the strongest predictors for subsequent aortic arch interventions. Further studies are needed to determine any modifiable variables that may reduce the incidence of arch interventions.

Preliminary Results With a Novel Expanded Polytetrafluoroethylene-based Pulmonary Valved Conduit.

BACKGROUND: A novel polymeric pulmonary valved conduit, resistant to calcification and structural valve deterioration, may provide a more durable therapy option for the pediatric population by preventing loss of right ventricular function and increasing freedom from valve-related reintervention and mortality. METHODS: This was a prospective, multicenter, single-arm study evaluating safety and performance of an investigational novel expanded polytetrafluoroethylene-based valve. Patients met study inclusion/exclusion criteria, had a signed informed consent, had pre- and postoperative evaluation via transthoracic echocardiography, and 6-month cardiac magnetic resonance imaging. RESULTS: Seventeen patients were enrolled from 3 sites. Median age was 12 years (range, 6-17 years) with 52.9% male. Body surface area ranged from 0.82 to 1.57 m2. There has been no mortality and 100% freedom from device related reinterventions. Baseline compared with 6-month cardiac magnetic resonance imaging (in 11 of 16 patients with available data) suggests favorable right ventricular remodeling (right ventricular end-diastolic volume, 123 ± 37 to 94 ± 25 mL/m2) with no significant change in ejection fraction. Through current follow-up, no patient has a right ventricular outflow tract gradient >20 mm Hg (mean, 11.2 ± 4.3 mm Hg). No evidence of worsening valvular insufficiency was observed throughout postoperative serial transthoracic echocardiogram evaluations. No pulmonary regurgitation above baseline (≤ mild) was observed. No patient developed endocarditis. No thrombus or calcification was identified. CONCLUSIONS: This preliminary evaluation of a novel expanded polytetrafluoroethylene-based valved conduit suggests promising valve function with no thromboembolic or infectious complications, no valve related reinterventions, no valve-related adverse events or unexpected findings, improved right ventricular volumes, and encouraging hemodynamic performance through current follow-up.

Impact of Viral PCR Positive Nasal Swabs (Non Covid-19) on Outcomes Following Cardiac Surgery.

Viral bronchiolitis is a relative contraindication to elective pediatric cardiac surgery. Nasopharyngeal swab utilizing polymerase chain reaction (PCR) screening for viruses known to cause bronchiolitis are commonly available. The objective of this study was to evaluate clinical outcomes in patients with nasopharyngeal viral PCR positive findings at the time of cardiac surgery. Retrospective review from January 2013 to May 2019 for patients with virus detected by PCR on nasopharyngeal swabs at the time of cardiac surgery. Single ventricle and two ventricle patients were compared to control group of age and procedure matched patients viral negative at the time of surgery. Outcome measures included OR extubation, reintubation, hospital length of stay, and mortality. For two ventricle patients (n = 81; control group = 165), there was no statistical difference in any outcome variable (OR extubation 74% vs 72%; p = 0.9; reintubation 9% vs 11% vs; p = 0.7; hospital length of stay 5 days (1-46) vs 4 days (2-131); p = 0.4; mortality 2 vs 1; p = 0.3). For single ventricle patients, there was no statistical difference in any outcome variable (OR extubation 81% vs 76%; p = 0.6; reintubation 14% vs 21% vs; p = 0.5; hospital length of stay 9.5 days (3-116) vs 15 days (2-241); p = 0.1; mortality 0 vs 3; (p = 0.6)). PCR is a sensitive test that fails to predict which patients will proceed to have a clinically significant infection. Viral bronchiolitis remains a relative risk factor for cardiac surgery; presence of detectable virus via nasopharyngeal swab with limited clinical symptoms may not be a contraindication to cardiac surgery.

Tricuspid Valve and Right Ventricular Function Throughout the Hybrid Palliation Strategy for Hypoplastic Left Heart Syndrome and Variants.

BACKGROUND: Tricuspid valve (TV) and right ventricular (RV) function are major determinants of morbidity and mortality in patients with hypoplastic left heart syndrome (HLHS). We sought to retrospectively evaluate these parameters throughout the hybrid palliation strategy. METHODS: From 2002 to 2018, 203 patients with HLHS and variants presented for hybrid stage I (HS1). Echocardiographic evaluation of tricuspid regurgitation (TR) and RV function was assessed at multiple time points. Clinical outcomes including tricuspid valvuloplasty, transplantation, and death were reviewed. RESULTS: The most prevalent HLHS subtype was aortic atresia/mitral atresia. The presence of significant TR and/or RV dysfunction was 14.78% and 9.36%, respectively, at the time of initial HS1. There were 185 survivors following HS1 (91.13%, n = 185/203), while 147 patients underwent comprehensive stage II or bidirectional Glenn shunt (72.41%, n = 147/203). Tricuspid valvuloplasty was undertaken in nine patients (4.86%, n = 9/185). Ultimately, 100 patients underwent the Fontan procedure. The odds of development of significant TR and/or RV dysfunction were not statistically different throughout the stages of palliation (TR: odds ratio [OR] = 0.14-0.25, P = .5260; RV dysfunction: OR = 0.02-0.13, P = .3992). However, the risk of death and/or transplant was 2.5- to 3.8-fold when either were present alone or in combination (TR: OR = 2.58, P = .0356; RV dysfunction: OR = 3.84, P = .0262). Transplant-free survival at 15 years was 44.8%. CONCLUSION: Following hybrid palliation for HLHS, the majority of survivors have normal RV and TV functions. Tricuspid valvuloplasty was required in few patients. Once significant TR and/or RV dysfunction ensues, there is a two- to three-fold risk of death and/or transplant.

Bloodless Arterial Switch Operation in a 2.7-kg Jehovah's Witness Patient.

Bloodless pediatric cardiac surgery requiring the use of cardiopulmonary bypass (CPB) remains a challenge for the entire operating room (OR) team. The amount of circulating blood volume to pump prime volume mismatch of small patients results in hemodilution that frequently results in transfusion of allogeneic blood products. Patients of families of the Jehovah's Witness (JW) faith reject the use of these products because of religious beliefs. Our institution is a referral center for children of JW families because we have developed techniques to minimize blood loss with the hope of performing bloodless pediatric cardiac surgery whenever possible. These techniques include preoperative treatment with erythropoietin, intraoperative acute normovolemic hemodilution, CPB circuit miniaturization, ultrafiltration during and after CPB, limiting blood gas analyses or other unnecessary blood draws, and using hemostatic agents during and after CPB. We present the case of a 4-day-old patient of the JW faith weighing 2.7 kg with transposition of the great arteries and an intact ventricular septum who underwent an arterial switch operation. The patient received no allogeneic blood product administration throughout the entire hospitalization. The patient's first hematocrit in the OR was 43%, lowest hematocrit on bypass was 15%, and first hematocrit in the cardiothoracic intensive care unit post-procedure was 21%. The patient was discharged on post-op day nine with a hematocrit of 36%.

Spontaneous reversal of stenosis in tissue-engineered vascular grafts.

We developed a tissue-engineered vascular graft (TEVG) for use in children and present results of a U.S. Food and Drug Administration (FDA)-approved clinical trial evaluating this graft in patients with single-ventricle cardiac anomalies. The TEVG was used as a Fontan conduit to connect the inferior vena cava and pulmonary artery, but a high incidence of graft narrowing manifested within the first 6 months, which was treated successfully with angioplasty. To elucidate mechanisms underlying this early stenosis, we used a data-informed, computational model to perform in silico parametric studies of TEVG development. The simulations predicted early stenosis as observed in our clinical trial but suggested further that such narrowing could reverse spontaneously through an inflammation-driven, mechano-mediated mechanism. We tested this unexpected, model-generated hypothesis by implanting TEVGs in an ovine inferior vena cava interposition graft model, which confirmed the prediction that TEVG stenosis resolved spontaneously and was typically well tolerated. These findings have important implications for our translational research because they suggest that angioplasty may be safely avoided in patients with asymptomatic early stenosis, although there will remain a need for appropriate medical monitoring. The simulations further predicted that the degree of reversible narrowing can be mitigated by altering the scaffold design to attenuate early inflammation and increase mechano-sensing by the synthetic cells, thus suggesting a new paradigm for optimizing next-generation TEVGs. We submit that there is considerable translational advantage to combined computational-experimental studies when designing cutting-edge technologies and their clinical management.

Hybrid Procedures: A Surgeon's Viewpoint on the Next 10 Years.

Many forces are once again bringing the congenital heart surgeon and interventional cardiologist, and the teams that support them, ever closer together in what has been deemed Hybrid Approaches to congenital heart disease. The goal of these hybrid approaches is to improve the quantity and quality of life for the patients we serve.