Mitral valve orifice area predicts outcome after biventricular repair in patients with hypoplastic left ventricles.

BACKGROUND: Identification of risk factors for biventricular (BiV) repair in children with hypoplastic left ventricles (HLV) has been challenging. We sought to identify preoperative cardiovascular magnetic resonance (CMR) predictors of outcome in patients with HLVs who underwent BiV repair, with a focus on the mitral valve (MV). METHODS: Single-center retrospective analysis of preoperative CMRs on patients with HLV (≤50 mL/m2) and no endocardial fibroelastosis who underwent BiV repair from 2005-2022. CMR measurements included MV orifice area in diastole. The primary composite outcome included time to death, transplant, BiV takedown, heart failure admission, left atrial decompression, or unexpected reoperation; and the secondary outcome included more than or equal to moderate mitral stenosis and/or regurgitation. RESULTS: Median follow-up was 0.7 (interquartile range 0.1, 2.2) years. Of 122 patients [59 atrioventricular canal (AVC) and 63 non-AVC] age 3 ± 2.8 years at the time of BiV repair, freedom from the primary outcome at 2 years was 53% for AVC and 69% for non-AVC (log rank p = 0.12), and freedom from the secondary outcome at 2 years was 49% for AVC and 79% for non-AVC (log rank p < 0.01). Independent predictors of primary outcome for AVC patients included MV orifice area z-score <-2 and transitional AVC; for non-AVC patients, predictors included MV orifice area z-score <-2, abnormal MV anatomy, and conal-septal ventricular septal defect. Independent predictors of secondary outcome for AVC patients included older age at surgery, transitional AVC, and transposition of the great arteries. CONCLUSION: In children with HLV, low MV orifice area and pre-existing MV pathology are risk factors for adverse outcome after BiV repair.

Insight into the Role of the Child Opportunity Index on Surgical Outcomes in Congenital Heart Disease.

OBJECTIVE: To use neighborhood-level Child Opportunity Index (COI) measures to investigate disparities in congenital heart surgery postoperative outcomes and identify potential targets for intervention. STUDY DESIGN: In this single-institution retrospective cohort study, children <18 years old who underwent cardiac surgery between 2010 and 2020 were included. Patient-level demographics and neighborhood-level COI were used as predictor variables. COI-a composite US census tract-based score measuring educational, health/environmental, and social/economic opportunities-was dichotomized as lower (<40th percentile) vs higher (≥40th percentile). Cumulative incidence of hospital discharge was compared between groups using death as a competing risk, adjusting for clinical characteristics associated with outcomes. Secondary outcomes included hospital readmission and death within 30 days. RESULTS: Among 6247 patients (55% male) with a median age of 0.8 years (IQR, 0.2-4.3), 26% had lower COI. Lower COI was associated with longer hospital lengths of stay (adjusted HR, 1.2; 95% CI, 1.1-1.2; P < .001) and an increased risk of death (adjusted OR, 2.0; 95% CI. 1.4-2.8; P < .001), but not hospital readmission (P = .6). At the neighborhood level, lacking health insurance coverage, food/housing insecurity, lower parental literacy and college attainment, and lower socioeconomic status were associated with longer hospital length of stay and increased risk of death. At the patient-level, public insurance (adjusted OR, 1.4; 95% CI, 1.0-2.0; P = .03) and caretaker Spanish language (adjusted OR 2.4; 95% CI, 1.2-4.3; P < .01) were associated with an increased risk of death. CONCLUSIONS: Lower COI is associated with longer length of stay and higher early postoperative mortality. Risk factors identified including Spanish language, food/housing insecurity, and parental literacy serve as potential intervention targets.

Associations With Extubation Failure and Predictive Value of Risk Analytics Algorithms With Extubation Readiness Tests Following Congenital Cardiac Surgery.

OBJECTIVES: Extubation failure is associated with morbidity and mortality in children following cardiac surgery. Current extubation readiness tests (ERT) do not consider the nonrespiratory support provided by mechanical ventilation (MV) for children with congenital heart disease. We aimed to identify factors associated with extubation failure in children following cardiac surgery and assess the performance of two risk analytics algorithms for patients undergoing an ERT. DESIGN: Retrospective cohort study. SETTING: CICU at a tertiary-care children's hospital. PATIENTS: Children receiving MV greater than 48 hours following cardiac surgery between January 1, 2017, and December 31, 2019. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Six hundred fifty encounters were analyzed with 49 occurrences (8%) of reintubation. Extubation failure occurred most frequently within 6 hours of extubation. On multivariable analysis, younger age (per each 3-mo decrease: odds ratio [OR], 1.06; 95% CI, 1.001-1.12), male sex (OR, 2.02; 95% CI, 1.03-3.97), Society of Thoracic Surgery-European Association for Cardiothoracic Surgery category 5 procedure (p equals to 0.005), and preoperative respiratory support (OR, 2.08; 95% CI, 1.09-3.95) were independently associated with unplanned reintubation. Our institutional ERT had low sensitivity to identify patients at risk for reintubation (23.8%; 95% CI, 9.7-47.6%). The addition of the inadequate delivery of oxygen (IDO2) index to the ERT increased the sensitivity by 19.0% (95% CI, -2.5 to 40.7%; p = 0.05), but the sensitivity remained low and the accuracy of the test dropped by 8.9% (95% CI, 4.7-13.1%; p < 0.01). CONCLUSIONS: Preoperative respiratory support, younger age, and more complex operations are associated with postoperative extubation failure. IDO2 and IVCO2 provide unique cardiorespiratory monitoring parameters during ERTs but require further investigation before being used in clinical evaluation for extubation failure.

Expandable Valves, Annuloplasty Rings, Shunts, and Bands for Growing Children.

In congenital heart surgery, the surgeon must constantly consider how a palliative or corrective procedure could be impacted by the child's somatic growth. Within pediatric valve surgery, existing valve repair techniques lack growth-accommodating prostheses. Valve replacement options are fixed in size and unable to grow with the child, thus subjecting children to repeated valve reoperations. When creating a systemic-to-pulmonary artery shunt, replacing a branch pulmonary artery or conduit, creating an extracardiac Fontan pathway, or banding the pulmonary artery, the implant size must factor in both the child's current size and his or her anticipated growth. A variety of growth-accommodating technologies have been developed to fill this unmet need. Some devices have reached the clinical arena, while several are in preclinical development. The purpose of this review is to characterize the clinical need for growing device technology, and then review established and developing technologies for growth accommodation in congenital heart surgery.

Intraoperative Coronary Artery Imaging for Planning.

A clear understanding of coronary anatomy is critical in congenital heart surgery, especially when a ventriculotomy is planned, as in right ventricular outflow tract reconstructions and biventricular repairs. Typically, major epicardial coronary arteries are easily identified during first-time operations. However, in reoperative surgery, epicardial adhesions can obscure coronary vessels making their identification challenging. This paper describes the application of intraoperative fluorescence imaging in congenital heart surgery to obtain real-time coronary artery visualization for operative planning in order to avoid coronary injury. The fluorescence imaging utilizes indocyanine green (ICG). ICG is diluted to 0.28 mg/mL, and 0.1-1.0 mL of ICG solution is delivered via the cardioplegia cannula into the coronaries. A handheld imaging probe illuminates the field with near-infrared laser light, which excites ICG fluorescence. The fluorescence is captured by a camera, and the signal is transmitted to a monitor for real-time viewing. The imaging probe shows the precise coronary anatomy, enabling identification of the optimal ventriculotomy site. Intraoperative fluorescence imaging is a safe and effective technique for characterizing coronary anatomy. This technique enhances procedural planning and helps minimize the risk of coronary injury during reoperative congenital heart surgery.

Creation of nonischemic functional mitral regurgitation by annular dilatation and nonplanar modification in a chronic in vivo swine model.

BACKGROUND: Mechanisms and treatments of nonischemic functional mitral regurgitation (NIMR) are not fully established, in part, because of a lack of proper large animal models. We developed a novel technique of NIMR creation in a swine model by making multiple small incisions in the mitral annulus. METHODS AND RESULTS: Ex vivo experiments using isolated swine hearts (n=10) showed a 15% increase in annular area (6.8-7.8 cm(2)) after 16 incisions were made along the posterior mitral annulus of a pressurized left ventricle. In an in vivo swine model (n=7; 46.4 ± 2.2 kg), NIMR was created by making fourteen to twenty-six 2-mm incisions in the atrial aspect of the mitral annulus using a cardioport video-assisted imaging system in the beating heart. Animals were euthanized at 4 weeks (n=4) and 6 weeks (n=3). Three-dimensional (3D) echocardiography was obtained before and immediately after NIMR creation and at euthanasia; vena contracta area, mitral annular dimension, left ventricular volume, and inter-papillary muscle distance were measured. The mitral annular incisions resulted in mild to moderate mitral regurgitation and an increased vena contracta area. NIMR creation altered mitral valve geometry by decreasing mitral annular nonplanarity and increasing annular area, primarily in the anteroposterior dimension. NIMR creation did not significantly change left ventricular volume or inter-papillary muscle distance. Longer follow-up period did not significantly affect these outcomes. CONCLUSIONS: NIMR can successfully be created in a beating heart swine model and results in dilatation and 3D changes in mitral annular geometry. This model can enhance the experimental validation of new valve repair devices and techniques.

Postmortem imaging of antemortem myocardial ischaemia.

OBJECTIVES: To determine the minimum survival time for detection of antemortem myocardial ischaemia with postmortem imaging (PMI) techniques. METHODS: Nine pigs underwent ligation of the left anterior descending (LAD) (8) and/or right coronary artery (RCA) branch (4), and were killed 30 min-6 h after ligation. PMI (MRI and CT angiography) was performed 2-55 h after euthanasia. Signal intensity of myocardial segments was measured. The hearts were removed, the coronary arteries injected to mark perfused segments, and sections submitted for histology. RESULTS: MRI T2-weighted sequences showed the ischaemic area as hyperintense in 4/4 LAD ligations with ≥4 h of ischaemia but in 0/4 with <4 h. Histological evidence of ischaemia was present in 4/4 animals after 4 h. Right ventricular ischaemic myocardium was visible on MRI T2-weighted sequences after 6 h of ischaemia in one animal. CT angiography showed the occluded coronary artery in all cases. CONCLUSIONS: Ischaemic lesions of the left ventricle, but not of the right, at least 4 h old can be detected as hyperintense areas on T2-weighted postmortem MRI. This technique is most sensitive in the first 24 h after death. Other sequences did not enhance detection. KEY POINTS: • Left ventricular myocardial ischaemia/infarction can be demonstrated by postmortem imaging (PMI). • Ischaemia/infarction is better detected if survival time is at least 4 h. • Right ventricular ischaemia/infarction is not reliably detected by PMI. • Computed tomography angiography can demonstrate arterial occlusion.

Repeat Repair After Translocation of an Anomalous Left Coronary Artery.

We repaired a left anomalous aortic origin of coronary artery using coronary ostium translocation/reimplantation. Repeat repair was required due to suboptimal flow pattern in postoperative transesophageal echocardiography, which was confirmed with coronary angiography. We emphasize the importance of early recognition, diagnosis, and intervention to deal with a problem with the coronary repair.

Reverse double switch operation for the borderline left ventricle.

OBJECTIVE(S): This study investigates outcomes of the reverse double switch operation (R-DSO) and ventricular switch, novel approaches for patients with D-looped borderline left hearts that utilize the morphologic right ventricle as the systemic ventricle and the hypoplastic left ventricle as the subpulmonary ventricle. METHODS: This retrospective review analyzed early outcomes of children who underwent R-DSO/ventricular switch at a single institution between 2015 and 2023. Our primary end points were right ventricular (RV) function and tricuspid regurgitation. Secondary outcomes included mortality, reoperation, and perioperative complications. RESULTS: Twenty-eight patients underwent either R-DSO (n = 19) or ventricular switch (n = 9). In 19 patients, a decompressing bidirectional cavopulmonary shunt was utilized, creating a reverse 1.5 switch. Median age at R-DSO/ventricular switch was 3.1 years (range, 9 months-12 years). At a median follow-up of 1.0 year (range, 38 days-7.2 years), no mortalities or heart transplants had occurred. Mild-moderate or greater RV dysfunction was detected in 4 patients, and mild-moderate or worse tricuspid regurgitation was also detected in 4 patients. Three patients required reoperations. Preoperative RV ejection fraction <55% was associated with higher incidence of postoperative RV dysfunction. CONCLUSIONS: The R-DSO/VS strategy is an alternative to single-ventricle palliation or anatomic biventricular repair in the borderline left heart. Concerns for RV dysfunction and tricuspid regurgitation mandate close monitoring. Patients with preoperative RV dysfunction may be at risk for postoperative RV dysfunction. Further studies with longer follow-up are needed to delineate outcomes in comparison to the Fontan pathway and identify optimal candidates for this novel strategy.

Impact of permanent pacemaker for iatrogenic atrioventricular block on outcomes after congenital heart surgery.

OBJECTIVE: The need for permanent pacemaker (PPM) for iatrogenic atrioventricular block (AVB) after congenital heart surgery is approximately 1%. We aimed to evaluate the long-term outcomes of patients with PPM for iatrogenic AVB and compare them with patients with an optimal repair (trivial/no residual) Residual Lesion Score (RLS) Class 1 repair without PPM need. METHODS: We reviewed 183 patients discharged with PPM for iatrogenic AVB from 2011 to 2022. Patients who survived to discharge with >30 days of follow-up were matched 1:1 with a cohort of patients categorized as RLS Class 1 on the basis of fundamental diagnosis and primary procedure. RESULTS: Median age at PPM placement was 1.4 years (interquartile range, 4.3 months to 3.9 years). The cumulative incidence of moderate or greater ventricular dysfunction at 1 year and 5 years was 11% and 18% in patients with PPM, respectively, compared with 3% and 7% in patients categorized as RLS Class 1 (subdistribution hazard ratio, 2.6; 95% confidence interval, 1.2-6.1; P = .022). Independently, patients with PPM with hypoplastic left heart syndrome (P = .027) and who had undergone Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery Mortality Category 5 procedures (P = .033) were at greater risk of ventricular dysfunction. Transplant-free survival at 1 year and 5 years was 94% and 89% in patients with PPM, respectively, compared with 98% and 97% in patients categorized as RLS Class 1 (P = .044). In addition, patients with PPM with palliated circulation had significantly lower transplant-free survival (P < .001). CONCLUSIONS: Compared with patients with an optimal repair without PPM, patients with PPM for iatrogenic AVB are at greater risk of developing moderate or greater ventricular dysfunction and have lower transplant-free survival.

Cleft closure and other predictors of contemporary outcomes after atrioventricular canal repair in patients with parachute left atrioventricular valve.

OBJECTIVES: Parachute left atrioventricular valve (LAVV) complicates atrioventricular septal defect (AVSD) repair. We evaluate outcomes of AVSD patients with parachute LAVV and identify risk factors for adverse outcomes. METHODS: We evaluated all patients undergoing repair of AVSD with parachute LAVV from 2012 to 2021. The primary outcome was a composite of time-to-death, LAVV reintervention and development of greater than or equal to moderate LAVV dysfunction (greater than or equal to moderate LAVV stenosis and/or LAVV regurgitation). Event-free survival for the composite outcome was estimated using Kaplan-Meier methodology and competing risks analysis. Cox proportional hazards regression was used to identify predictors of the primary outcome. RESULTS: A total of 36 patients were included with a median age at repair of 4 months (interquartile range 2.3-5.5 months). Over a median follow-up of 2.6 years (interquartile range 1.0-5.6 years), 6 (17%) patients underwent LAVV reintervention. All 6 patients who underwent LAVV reintervention had right-dominant AVSD. Sixteen patients (44%) met the composite outcome, and all did so within 2 years of initial repair. Transitional AVSD (versus complete), prior single-ventricle palliation, leaving the cleft completely open and greater than or equal to moderate preoperative LAVV regurgitation were associated with a higher risk of LAVV reintervention in univariate analysis. In multivariate analysis, leaving the cleft completely open was associated with the composite outcome. CONCLUSIONS: Repair of AVSD with parachute LAVV remains a challenge with a significant burden of LAVV reintervention and dysfunction in medium-term follow-up. Unbalanced, right-dominant AVSDs are at higher risk for LAVV reintervention. Leaving the cleft completely open might independently predict poor overall outcomes and should be avoided when possible. CLINICAL TRIAL REGISTRATION NUMBER: IRB-P00041642.

Efficacy and safety of early postoperative ablation in patients with congenital heart disease.

BACKGROUND: Postoperative arrhythmias are most often transient and medically treated, but some patients may require electrophysiology study (EPS) and ablation. OBJECTIVE: The purpose of this study was to describe the efficacy and safety of early postoperative ablation. METHODS: This study presents a retrospective series of patients who underwent EPS within 12 months of surgery for congenital heart disease between 2000 and 2021. The procedural outcome included complete or partial success, empirical ablation or failure, and complications. The long-term outcome included arrhythmia recurrence and burden according to a 12-point clinical arrhythmia severity score (documented arrhythmia, arrhythmia severity, cardioversion, and antiarrhythmic medication). RESULTS: Among 28,902 operations during the study period, 24 patients (0.1%) underwent EPS within 3 months of surgery and 26 (0.1%) 3-12 months after surgery. Most patients had great (n = 27 [50%]) or moderate (n = 21 [42%]) congenital heart disease complexity. Mechanisms of arrhythmias included intra-atrial reentrant tachycardia (n = 23 [46%]), ectopic atrial tachycardia (n = 13 [26%]), accessory pathway (n = 6 [12%]), atrioventricular nodal reentrant tachycardia (n = 7 [14%]), twin atrioventricular node (n = 1 [2%]), atrial fibrillation (n = 1 [2%]), junctional ectopic tachycardia (n = 1 [2%]), and ventricular tachycardia (n = 2 [4%]). The procedure was acutely successful in 41 patients (82%), empirical in 5 (10%), and unsuccessful in 4 (8%). Complications occurred in 4 (8%) patients (major in 1, moderate in 1, and minor in 2). The recurrence of arrhythmia was documented in 27 patients (54%), although the burden of arrhythmia was significantly reduced. CONCLUSION: A minority of patients require early postoperative EPS and ablation. For those, the procedure can be performed with reasonable acute success and manageable morbidity even in critically ill patients with complex surgical anatomy.

Ventricular septation for double inlet ventricle: Avoiding conduction injury.

OBJECTIVES: Although conduction location can be reliably predicted in double inlet ventricle, ventricular septation continues to carry a significant risk of complete heart block. This study describes our experience using intraoperative conduction mapping during ventricular septation. METHODS: Patients undergoing ventricular septation from 2017 to 2023 were identified. Conduction mapping was performed on the open, decompressed, beating heart to identify the His bundle. Mapped His bundle locations were compared with those predicted from magnetic resonance imaging diagnoses by a senior pediatric cardiac electrophysiologist blinded to mapping results. RESULTS: Ventricular septation was performed in 31 patients, 25 with hypoplastic right ventricles and 6 with hypoplastic left ventricles. Two-stage septation was performed in 25 patients, and single-stage septation was performed in 6 patients. Mapped conduction location was performed in the last 25 consecutive patients. Mapped conduction location was concordant with expected location in 21 of 22 patients with predictions. Complete heart block requiring a permanent pacemaker occurred in 4 patients despite successful mapping, whereas 10 patients required other reoperations, most commonly to address residual atrioventricular valve regurgitation or subpulmonary obstruction from the ventricular septal defect patch. There has been no perioperative mortality, need for single-ventricle palliation, or heart transplantation. All 13 patients who have progressed to complete septation and 18 patients with interstage circulation have acceptable hemodynamics, preserved ventricular function, and no heart failure symptoms at latest follow-up. CONCLUSIONS: Ventricular septation represents an alternative to the Fontan that can be performed safely in a subset of patients with acceptable early outcomes. Conduction mapping is an adjunct strategy that may add precision to well-established rules for reliably predicting conduction location.

The Society of Thoracic Surgeons Clinical Practice Guidelines on the Management of Neonates and Infants With Coarctation.

BACKGROUND: Although coarctation of the aorta without concomitant intracardiac pathology is relatively common, there is lack of guidance regarding aspects of its management in neonates and infants. METHODS: A panel of experienced congenital cardiac surgeons, cardiologists, and intensivists was created, and key questions related to the management of isolated coarctation in neonates and infants were formed using the PICO (Patients/Population, Intervention, Comparison/Control, Outcome) Framework. A literature search was then performed for each question. Practice guidelines were developed with classification of recommendation and level of evidence using a modified Delphi method. RESULTS: For neonates and infants with isolated coarctation, surgery is indicated in the absence of obvious surgical contraindications. For patients with risk factors for surgery, medical management before intervention is reasonable. For those stable off prostaglandin E1, the threshold for intervention remains unclear. Thoracotomy is indicated when arch hypoplasia is not present. Sternotomy is preferable when arch hypoplasia is present that cannot be adequately addressed through a thoracotomy. Sternotomy may also be considered in the presence of a bovine aortic arch. Antegrade cerebral perfusion may be reasonable when the repair is performed through a sternotomy. Extended end-to-end, arch advancement, and patch augmentation are all reasonable techniques. CONCLUSIONS: Surgery remains the standard of care for the management of isolated coarctation in neonates and infants. Depending on degree and location, arch hypoplasia may require a sternotomy approach as opposed to a thoracotomy approach. Significant opportunities remain to better delineate management in these patients.

Left atrioventricular valve repair after primary atrioventricular canal surgery: Predictors of durability.

OBJECTIVE: Acute outcomes after atrioventricular canal defects (AVCD) surgery in the current era are excellent; yet despite surgical advances, ∼15% of patients require future left atrioventricular valve (LAVV) repair. Among patients with AVC who undergo LAVV repair after primary AVC surgery, we sought to characterize the durability of these repairs. Specifically, we aimed to determine predictors for reintervention following an LAVV repair in patients with repaired AVCD, with a focus on postoperative transesophageal echocardiography (TEE). METHODS: We reviewed all patients undergoing LAVV repair (after a primary AVCD surgery) at Boston Children's Hospital between 2010 and 2020. Competing risk analysis was performed to evaluate cumulative incidence of LAVV reinterventions. Predictors of LAVV reintervention were evaluated using multivariable Cox regression. RESULTS: A total of 137 LAVV repairs following primary AVCD surgery were performed in 113 patients. Median age and weight at LAVV repair were 25 months (interquartile range, 12-76 months) and 11.1 kg (interquartile range, 7.8-19.4 kg). Original anatomy was complete AVCD in 87 (63%), transitional AVCD in 27 (20%), and partial AVCD in 23 (17%) cases. Over a median follow-up of 12 months (interquartile range, 1.3 months-4 years), 47 (34%) of the LAVV repairs required LAVV reintervention. Reinterventions included a total of 27 LAVV re-repairs and 20 LAVV replacements. In multivariable analysis, age at LAVV repair younger than 72 months, partial AVCD anatomy, left ventricle dysfunction, mean LAVV stenosis gradient ≥5 mm Hg, and multiple jets of regurgitation on postoperative LAVV repair TEE were associated with LAVV reintervention. Grade of LAVV regurgitation on postoperative TEE was not an independent risk factor, but reintervention rates were high when residual LAVV stenosis gradient was ≥5 mm Hg and residual mild LAVV regurgitation was present on postoperative TEE (47%) and even higher when residual LAVV stenosis gradient was ≥5 mm Hg and LAVV regurgitation was greater than mild (73%). CONCLUSIONS: Reintervention rates remain high for LAVV repairs that occur after primary AVCD surgery, particularly for patients with LAVV stenosis gradient ≥5 mm Hg and mild or greater LAVV regurgitation on postoperative TEE.

Clinical risk prediction score for postoperative accelerated junctional rhythm and junctional ectopic tachycardia in children with congenital heart disease.

BACKGROUND: Accelerated junctional rhythm (AJR) and junctional ectopic tachycardia (JET) are common postoperative arrhythmias associated with morbidity/mortality. Studies suggest that pre- or intraoperative treatment may improve outcomes, but patient selection remains a challenge. OBJECTIVES: The purpose of this study was to describe contemporary outcomes of postoperative AJR/JET and develop a risk prediction score to identify patients at highest risk. METHODS: This was a retrospective cohort study of children aged 0-18 years undergoing cardiac surgery (2011-2018). AJR was defined as usual complex tachycardia with ≥1:1 ventricular-atrial association and junctional rate >25th percentile of sinus rate for age but <170 bpm, whereas JET was defined as a rate >170 bpm. A risk prediction score was developed using random forest analysis and logistic regression. RESULTS: Among 6364 surgeries, AJR occurred in 215 (3.4%) and JET in 59 (0.9%). Age, heterotaxy syndrome, aortic cross-clamp time, ventricular septal defect closure, and atrioventricular canal repair were independent predictors of AJR/JET on multivariate analysis and included in the risk prediction score. The model accurately predicted the risk of AJR/JET with a C-index of 0.72 (95% confidence interval 0.70-0.75). Postoperative AJR and JET were associated with prolonged intensive care unit and hospital length of stay, but not with early mortality. CONCLUSION: We describe a novel risk prediction score to estimate the risk of postoperative AJR/JET permitting early identification of at-risk patients who may benefit from prophylactic treatment.

Conduction mapping during complex congenital heart surgery: Creating a predictive model of conduction anatomy.

OBJECTIVES: The study objectives were to report on a growing experience of conduction system mapping during complex congenital heart surgery and create a predictive model of conduction anatomy. METHODS: Patients undergoing complex cardiac repair with conduction mapping were studied. Intraoperative mapping used a multielectrode catheter to collect His bundle electrograms in the open, decompressed, beating heart. Patient anatomy, operative details, His bundle location, and postoperative conduction status were analyzed. By using classification and regression tree analysis, a predictive model of conduction location was created. RESULTS: A total of 109 patients underwent mapping. Median age and weight were 1.8 years (range, 0.2-14.9) and 10.8 kg (range, 3.5-50.4), respectively. Conduction was identified in 96% (105/109). Median mapping time was 6 minutes (range, 2-33). Anatomy included atrioventricular canal defect, double outlet right ventricle, complex transposition of the great arteries, and multiple ventricular septal defects. By classification and regression tree analysis, ventricular looping and visceroatrial situs were the greatest discriminators of conduction location. A total of 94 of 105 patients (89.5%) were free of complete heart block. Only 1 patient (2.9%) with heterotaxy syndrome developed complete heart block. CONCLUSIONS: The precise anatomic location of the conduction system in patients with complex congenital heart defects can be difficult for the surgeon to accurately predict. Intraoperative conduction mapping enables localization of the His bundle and adds to our understanding of the anatomic factors associated with conduction location. Predictive modeling of conduction anatomy may build on what is already known about the conduction system and help surgeons to better anticipate conduction location preoperatively and intraoperatively.

Development of a Novel Society of Thoracic Surgeons Adult Congenital Mortality Risk Model.

BACKGROUND: Operative mortality risk models for adults with congenital heart disease (ACHD) undergoing cardiac operations are essential, given the growing population of these patients, yet they are currently unavailable. Existing adult Society of Thoracic Surgeons (STS) models exclude congenital procedures, whereas existing congenital models exclude operations for acquired disease. We aimed to develop an STS mortality risk model for ACHD patients undergoing cardiac operations. METHODS: Leveraging a comprehensive list of diagnostic and procedure codes, ACHD patients who underwent cardiac operations were identified from the STS Adult Cardiac Surgery Database (versions: v2.73, v2.81, and v2.9) between 2011 and 2019. The model was developed and validated in the ACHD population using a 60/40 development/validation split. Univariate analyses and clinical expertise informed the addition of ACHD-relevant procedure and diagnosis variables to existing STS adult risk model variables. Model performance was assessed overall and in 38 subgroups based on patient demographics, procedures, and diagnoses. RESULTS: Forty-seven procedure and diagnosis variables relevant to ACHD were added to existing STS adult risk model variables. The derived ACHD model for operative mortality was well calibrated within demographic, procedural, and diagnosis subgroups and the overall ACHD population, and discrimination in the validation cohort was excellent (C statistic, 0.815) compared with the model using only existing STS adult risk model variables (C statistic, 0.79; P < .0001). CONCLUSIONS: A novel, high-performing STS ACHD mortality risk model has been developed on the basis of contemporary patient data. The ACHD risk model represents an important expansion of the STS portfolio. Implementation with an online risk calculator is planned.

Report of the 2022 Society of Thoracic Surgeons Congenital Heart Surgery Practice Survey.

BACKGROUND: The Society of Thoracic Surgeons Workforce on Congenital Surgery performed a practice survey to analyze contemporary data. METHODS: An electronic survey was sent to congenital heart surgeons in North America. Details on demographics, training paradigm, clinical practice, and work satisfaction were queried, tabulated, and analyzed. RESULTS: Of 312 unique contacts, 201 (64.4%) responded. Of these, 178 (89%) were practicing. The median age was 52 years (interquartile range, 43, 59 years), and 157 (88%) were male. The number of female respondents increased from 12 (7%) in 2015 to 18 (11%) at present. Practice composition was predominantly mixed pediatric and adult (141; 79%), although 15 (8%) surgeons practiced exclusively pediatric surgery. Most surgeons (154; 87%) reported performing the Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery category 4 and 5 procedures. One-fourth (42; 24%) reported performing fewer than 50 pediatric cases per year, and 18 (10%) stated that their primary role was as a surgical assistant. Individual surgeon case volume was most commonly 100 to 149 total cases (29%). Although one-half (91; 51%) reported their volume as being "just right," 74 (42%) reported that their case volume was "too small." Seventy-six (43%) reported too many surgeons in their region. Of the 201 practicing surgeons, 30 (14.9%) plan retirement in the next 5 years. Most described career satisfaction, with 102 (57%) being very satisfied and 48 (27%) somewhat satisfied. CONCLUSIONS: Although most congenital heart surgeons in North America are satisfied with their careers, more than 40% believe that their caseload is inadequate and that there are too many surgeons in their region. Further analysis is warranted regarding career dissatisfaction and diversity.

Neonates With Right Aortic Arch Requiring Arch Reconstruction: A Single-Institution Experience.

BACKGROUND: Reconstruction of a right aortic arch is rarely required in the newborn period and has rarely been reported. METHODS: All patients who underwent a right aortic arch repair in the neonatal period from a single institution were retrospectively reviewed. The primary outcome measures included survival, complications, and reintervention. RESULTS: Between 1984 and 2020, 15 patients were identified. Nine patients (60%) presented with an interrupted aortic arch, 5 with a hypoplastic arch (33%), and 1 with anomalous origin of the brachiocephalic vessels (7%). All patients had associated complex congenital heart disease. Median age at surgery was 6 days (range, 2-29 days); median weight was 3.11 kg (range, 2.5-4.18 kg). Genetic syndromes were prevalent and 77% of interrupted aortic arch patients had DiGeorge syndrome. Surgical techniques included end-to-side (27%), end-to-end (27%), or side-to-side anastomosis (13%) and placement of an interposition graft (7%); 65% required patch augmentation. Median intensive care unit and total hospital length of stay were 20 days (range, 7-92 days) and 28 days (range, 10-240 days), respectively. At a median follow-up of 3.97 years (range, 0.19-36 years), 13 of 15 patients were alive (87%). We found vocal cord paralysis in 27%, hemidiaphragm paralysis in 13%, and considerable airway compression in 27%. Overall, 27% patients required reintervention on the aortic arch: 2 surgical and 2 percutaneous balloon dilation. CONCLUSIONS: Right aortic arch reconstruction in the newborn period is rare and associated with complex lesions with an acceptable reintervention rate.