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.

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.

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.

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.

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.

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.

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.

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.

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.

Early Experience With Reverse Double Switch Operation for the Borderline Left Heart.

This study reviews our early experience with the "reverse" double switch operation (R-DSO) for borderline left hearts. A retrospective review of children with borderline left hearts who underwent R-DSO between 2017 and 2021 was conducted. Patient characteristics and early hemodynamic and clinical outcomes were collected. R-DSO was performed in 8 patients with no operative or postoperative deaths; 5 underwent decompressing bidirectional Glenn. Left ventricular (LV) poor-compliance was the dominant pathophysiology. Four patients had undergone staged LV recruitment but were not candidates for anatomical biventricular circulation due to LV hypoplasia and/or diastolic dysfunction. 7/8 patients had risk factors for Fontan circulation including pulmonary vein stenosis, pulmonary hypertension, and pulmonary artery stenosis. Median age at R-DSO was 3.7 years (19 months-12 years). All patients were in sinus rhythm at discharge. At median follow-up of 15 months (57 days-4.1 years) no mortalities, reoperations or heart transplants had occurred. All patients had normal morphologic LV systolic function. In one patient, pre-existing pulmonary hypertension (HTN) resolved after R-DSO. Reinterventions included transcatheter mitral valve replacement for residual mitral stenosis and neo-pulmonary balloon valvuloplasty. In 4 patients follow-up catheterization done at a median of 519 days (320 days-4 years) demonstrated median cardiac index of 3.2 L/min/m2 (2.2-4); median sub-pulmonary left ventricular end diastolic pressure was 9 mm Hg (7-15); median inferior vena cava/baffle pressure was 8 mm Hg (7-13). R-DSO is an alternative to anatomical biventricular repair or single ventricle palliation in patients with borderline left hearts and can result in low inferior vena cava pressures and favorable early results. This approach can also relieve pulmonary HTN and allow future transplant candidacy.

Staged Ventricular Septation in Double-Inlet Ventricle - A Strategy to Avoid Fontan?

Single-stage ventricular septation for double-inlet left or right ventricle (DILV or DIRV) has historically been associated with poor outcomes. We hypothesize that staged ventricular septation may demonstrate favorable clinical outcomes to be an alternative to Fontan palliation. This single-center retrospective study reviewed patients with DILV or DIRV who underwent staged ventricular septation between 2015-2021. The strategy involves pulmonary artery banding or Norwood procedure during infancy (stage 1), followed by partial ventricular septation to anchor the septum, while maintaining systemic RV pressure to avoid septal shift (stage 2). Residual septal defects are closed with pulmonary artery band removal at stage 3. Results are reported as median (interquartile range). Twelve patients underwent partial ventricular septation. At a median follow-up time of 17 months (8-30) after stage 2, there were no interstage deaths or cardiac transplants; LV dysfunction was observed in one patient. Hemodynamic evaluation after stage 2 demonstrated median left atrial pressure of 9.5 mm Hg (8.9-11.5), cardiac index of 3.4 L/min/m2 (3.2-3.6), and RV and LV indexed end-diastolic volumes of 52 ml/m2 (41-67) and 105 ml/m2 (81-115), respectively. Five patients have progressed to stage 3; one required pacemaker for complete heart block. Unplanned reintervention was required in 4 patients after stage 1, 2 patients after stage 2, and 3 patients after stage 3. Staged ventricular septation is an alternative to single-ventricle palliation in a subset of double-inlet ventricle patients and is associated with acceptable early outcomes. Further studies are necessary to determine long-term outcomes.

Intraoperative conduction mapping in complex congenital heart surgery.

Objective: Postoperative heart block is a significant problem in congenital heart surgery because of the unpredictability and variability of conduction tissue location in complex congenital heart defects. A novel technique for intraoperative conduction system mapping during complex congenital heart surgery is described. Methods: Intraoperative conduction system mapping was performed utilizing a high-density multielectrode grid catheter to collect intracardiac electrograms on open, beating hearts during repair of complex congenital heart defects. Electrograms were interpreted by electrophysiologists, and conduction tissue location was communicated in real time to the surgeon. After localizing conduction tissue, the heart was arrested and the repair was completed taking care to avoid injury to the mapped conduction system. Results: Two patients with complex heterotaxy syndrome underwent intraoperative conduction mapping during biventricular repair. Mapping accurately identified the location of conduction tissue thereby enabling avoidance of conduction system injury during surgery. Notably, conduction was unexpectedly found to be located inferiorly in a patient with L-looped ventricles. Successful biventricular repair was accomplished in both patients without injury to the conduction system. Conclusions: Intraoperative conduction mapping can effectively localize the conduction system during surgery and enable the surgeon to avoid its injury. This can lower the risk of heart block requiring pacemaker in children undergoing complex congenital heart surgery.

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.

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.

Capturing Adult Congenital Heart Disease: Framework for Development of an Adult Congenital Heart Disease Mortality Risk Model.

BACKGROUND: As a formative step toward development of adult congenital heart disease (ACHD) risk-adjusted metrics, we describe The Society of Thoracic Surgeons (STS) ACHD population, procedural frequency, and early mortality. METHODS: Adults (≥18 years) with CHD (2014-2019) were identified in the STS Adult Cardiac Surgery (ASCD) and the Congenital Heart Surgery (CHSD) Databases. After deduplication, variable mapping, data concatenation, and harmonization of preprocedure factors, procedures were grouped, and unadjusted mortality was catalogued for the overall cohort and the cohort excluding patients with an isolated bicuspid aortic valve (BAV). RESULTS: Among 171 186 ACSD and 18 281 CHSD records, 152 731 unique records met inclusion criteria. Twenty-eight congenital diagnoses accounted for 86% of the overall cohort, and prevalence of ACHD increased over the study time frame. ACHD patients underwent operations to treat both acquired and CHD. Most common procedures overall and after excluding isolated BAV were aortic valve replacement (AVR), ascending aortic surgery, and coronary artery bypass grafting (CABG). After excluding isolated BAV, major procedure combinations occurred in 46% (31% had 2 major procedures and 3% had ≥4), and the most prevalent combinations included AVR + CABG (n = 2352), AVR + subaortic stenosis repair (n = 1481), and AVR + ascending aortic surgery (n = 1239). Unadjusted 30-day mortality was procedure dependent, 2.8% overall and 3.6% with isolated BAV excluded. CONCLUSIONS: The ACHD surgical population is heterogenous, and patients undergo surgery for CHD-related and adult/acquired procedure combinations. Early mortality is variable and influenced by surgical complexity. Excluding isolated BAV patients and developing procedure-based ACHD mortality risk models may be ideal but will require empirically derived grouping and collaboration.

Pulmonary vein stenosis: Anatomic considerations, surgical management, and outcomes.

OBJECTIVE: The study objective was to evaluate outcomes of pulmonary vein stenosis repair in a large single-center cohort. METHODS: Clinical data from a pulmonary vein stenosis registry were retrospectively reviewed identifying patients who underwent pulmonary vein stenosis repair. The primary/index operation was defined as the patient's first pulmonary vein stenosis operation during the study period. RESULTS: Between January 2007 and August 2019, 174 patients underwent pulmonary vein stenosis repair. Bilateral pulmonary vein stenosis occurred in 111 patients (64%); 71 patients (41%) had 4-vessel disease. Fifty-nine patients (34%) had primary pulmonary vein stenosis. Median age was 9 months (interquartile range, 5-27) and weight was 6.5 kg (4.7-10.2). Surgical techniques evolved and included ostial resection, unroofing, reimplantation, sutureless, modified sutureless, and a newer anatomically focused approach of pulmonary vein stenosis resection with lateralization or patch enlargement of the pulmonary vein-left atrium connection. Twenty-three patients (13%) required reoperation. Cumulative 2-year incidence of postoperative transcatheter intervention (balloon dilation ± stenting) was 64%. One-, 2-, and 5-year survivals were 71.2%, 66.8%, and 60.6%, respectively. There was no association between surgery type and reoperation rate (hazard ratio, 2.38, P = .25) or transcatheter intervention (hazard ratio, 0.97, P = .95). The anatomically focused repair was associated with decreased mortality on univariate (hazard ratio, 0.38, P = .042) and multivariable analyses (hazard ratio, 0.19, P = .014). Antiproliferative chemotherapy was also associated with decreased mortality (hazard ratio, 0.47, P = .026). CONCLUSIONS: This large single-center surgical pulmonary vein stenosis experience demonstrates encouraging midterm results. A new anatomically focused repair strategy aims to alleviate pulmonary vein angulation to minimize turbulence and shows promising early outcomes. Continued follow-up is required to understand longer-term outcomes for this surgical approach.

Single-Leaflet Aortic Valve Reconstruction Utilizing the Ozaki Technique in Patients With Congenital Aortic Valve Disease.

Evaluate outcomes of single leaflet aortic valve reconstruction using Ozaki sizer and template. Single institute retrospective analysis between August 2015 and August 2019. Thirty-three patients, median age 9.3 years and weight 29.2 kg underwent single leaflet Ozaki repair. Preoperative indications were: AR (n = 17), AS (n = 3) or AS/AR (n = 13). Baseline anatomy was unicuspid (n = 15), bicuspid (n = 9) or tricuspid (n = 9). Two patients had endocarditis. Prior interventions included balloon valvuloplasty (n = 22) and aortic valve repair (n = 9). Pre-op average native annulus diameter was 19.6 mm and peak echo gradient was 36 mm Hg. Autologous pericardium, Photofix and CardioCel bovine pericardium were used in 26, 5, and 2 patients. Non-coronary sinus enlargement was required in 3 and aortic root reduction in 9 patients. Single leaflet reconstruction was done for the right coronary cusp (n = 25), non-coronary cusp in (n = 6) and left coronary cusp (n = 2). Additional procedures were done in 30 patients. Median ICU and hospital LOS were 2.1 and 6.3 days. There were no early re-interventions or conversions to valve replacement and one unrelated mortality.en At discharge, all patients had < moderate AR and/or AS with average peak gradients of 15 mm Hg. The median follow-up was 1.1 year, (IQR 0.7-1.8 years). Freedom from ≥ moderate AR and AS at 2 years was 76% and 86%. One patient required surgical re-intervention for severe AR 1.5 years after surgery for inflammatory infiltrate with calcification and fibrosis. Single-leaflet aortic valve leaflet reconstruction utilizing the Ozaki technique has promising early results and can be considered in patients when there are acceptable native leaflets.

Pulmonary Vein Stenosis-Evolving Surgical Management of a Challenging Disease.

Pulmonary vein stenosis (PVS) is an extremely challenging clinical problem in congenital heart disease. It has traditionally required multimodal therapy given its complex underlying pathophysiology. As with other modalities, surgical therapy has undergone tremendous evolution since the 1950s. These evolving strategies have been based upon an improved understanding of the substrates that cause PVS and recurrent vein obstruction. More recent anatomic-based surgical strategies have focused on the pulmonary vein course, and how adjacent mediastinal structures can create a fulcrum effect on the pulmonary veins as they pass from the lung parenchyma to the left atrium. The consequent angulation of pulmonary veins creates altered wall shear stress and likely serves as a nidus for recurrent PVS. Encouraging early results suggest that eliminating pulmonary vein angulation and shortening/straightening the pulmonary vein course may prove effective in surgically managing PVS.