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.

Tracheobronchomalacia, Tracheobronchial Compression, and Tracheobronchial Malformations: Diagnostic and Treatment Strategies.

Tracheobronchomalacia (TBM) is an excessive dynamic narrowing of the airway that is greatest with increased mediastinal pressure such as coughing, Valsalva, and forced expiration. Airway compression and/or cartilage malformation is a fixed or static narrowing of the airway typically caused by great vessel malposition and/or abnormalities and may also contribute to airway narrowing. Although imprecise and misleading, the term TBM is often used to represent both problems, static and dynamic airway narrowing, which only serves to confuse and may mislead the treatment team into ineffective therapies. The consequences of airway narrowing caused by dynamic TBM and/or static compression includes a range of clinical signs and symptoms, depending on the location, extent, and severity of the airway collapse. All patients with mild to severe TBM benefit from medical management to optimize airway clearance of mucus. The milder cases of TBM may become asymptomatic with this therapy, allowing time for the child to grow and the airway to enlarge without the consequences of recurrent infections. In cases of more severe TBM with clinical sequelae, more aggressive management may be warranted. Multiple options for surgical intervention are available. This article discusses the details of clinical presentation, evaluation, diagnosis, and a variety of treatments.

Adjunct Targeted Biologic Inhibition Agents to Treat Aggressive Multivessel Intraluminal Pediatric Pulmonary Vein Stenosis.

OBJECTIVE: To evaluate the use of imatinib mesylate with or without bevacizumab targeting neoproliferative myofibroblast-like cells with tyrosine kinase receptor expression, as adjuncts to modern interventional therapies for the treatment of multivessel intraluminal pulmonary vein stenosis (PVS). We describe the 48- and 72-week outcomes among patients receiving imatinib mesylate with or without bevacizumab for multivessel intraluminal PVS. STUDY DESIGN: This single-arm, prospective, open-label US Food and Drug Administration approved trial enrolled patients with ≥2 affected pulmonary veins after surgical or catheter-based relief of obstruction between March 2009 and December 2014. Drug therapy was discontinued at 48 weeks, or after 24 weeks of stabilization, whichever occurred later. RESULTS: Among 48 enrolled patients, 5 had isolated PVS, 26 congenital heart disease, 5 lung disease, and 12 both. After the 72-week follow-up, 16 patients had stabilized, 27 had recurred locally without stabilization, and 5 had progressed. Stabilization was associated with the absence of lung disease (P = .03), a higher percentage of eligible drug doses received (P = .03), and was not associated with age, diagnosis, disease laterality, or number of veins involved. Survival to 72 weeks was 77% (37 of 48). Adverse events were common (n = 1489 total), but only 16 were definitely related to drug treatment, none of which were serious. CONCLUSION: Survival to 72 weeks was 77% in a referral population with multivessel intraluminal PVS undergoing multimodal treatment, including antiproliferative tyrosine kinase blockade. Toxicity specific to tyrosine kinase blockade was minimal.

Measurement of Dead Space Fraction Upon ICU Admission Predicts Length of Stay and Clinical Outcomes Following Bidirectional Cavopulmonary Anastomosis.

OBJECTIVES: Increased alveolar dead space fraction has been associated with prolonged mechanical ventilation and increased mortality in pediatric patients with respiratory failure. The association of alveolar dead space fraction with clinical outcomes in patients undergoing bidirectional cavopulmonary anastomosis for single ventricle congenital heart disease has not been reported. We describe an association of alveolar dead space fraction with postoperative outcomes in patients undergoing bidirectional cavopulmonary anastomosis. DESIGN: In a retrospective case-control study, we examined for associations between alveolar dead space fraction ([PaCO2 - end-tidal CO2]/PaCO2), arterial oxyhemoglobin saturation, and transpulmonary gradient upon postoperative ICU admission with a composite primary outcome (requirement for surgical or catheter-based intervention, death, or transplant prior to hospital discharge, defining cases) and several secondary endpoints in infants following bidirectional cavopulmonary anastomosis. SETTINGS: Cardiac ICU in a tertiary care pediatric hospital. PATIENTS: Patients undergoing bidirectional cavopulmonary anastomosis at our institution between 2011 and 2016. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Of 191 patients undergoing bidirectional cavopulmonary anastomosis, 28 patients were cases and 163 were controls. Alveolar dead space fraction was significantly higher in the case (0.26 ± 0.09) versus control group (0.17 ± 0.09; p < 0.001); alveolar dead space fraction at admission was less than 0.12 in 0% of cases and was greater than 0.28 in 35% of cases. Admission arterial oxyhemoglobin saturation was significantly lower in the case (77% ± 12%) versus control group (83% ± 9%; p < 0.05). Sensitivity and specificity for future case versus control assignment was best when prebidirectional cavopulmonary anastomosis risk factors, admission alveolar dead space fraction (AUC, 0.74), and arterial oxyhemoglobin saturation (AUC, 0.65) were combined in a summarial model (AUC, 0.83). For a given arterial oxyhemoglobin saturation, the odds of becoming a case increased on average by 181% for every 0.1 unit increase in alveolar dead space fraction. Admission alveolar dead space fraction and arterial oxyhemoglobin saturation were linearly associated with prolonged ICU length of stay, hospital length of stay, duration of mechanical ventilation, and duration of thoracic drainage (p < 0.001 for all). CONCLUSIONS: Following bidirectional cavopulmonary anastomosis, alveolar dead space fraction in excess of 0.28 or arterial oxyhemoglobin saturation less than 78% upon ICU admission indicates an increased likelihood of requiring intervention prior to hospital discharge. Increasing alveolar dead space fraction and decreasing arterial oxyhemoglobin saturation are associated with increased lengths of stay.

Pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: collateral vessel disease burden and unifocalisation strategies.

IntroductionThe optimal approach to unifocalisation in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (pulmonary artery/ventricular septal defect/major aortopulmonary collaterals) remains controversial. Moreover, the impact of collateral vessel disease burden on surgical decision-making and late outcomes remains poorly defined. We investigated our centre's experience in the surgical management of pulmonary artery/ventricular septal defect/major aortopulmonary collaterals.Materials and methodsBetween 1996 and 2015, 84 consecutive patients with pulmonary artery/ventricular septal defect/major aortopulmonary collaterals underwent unifocalisation. In all, 41 patients received single-stage unifocalisation (Group 1) and 43 patients underwent multi-stage repair (Group 2). Preoperative collateral vessel anatomy, branch pulmonary artery reinterventions, ventricular septal defect status, and late right ventricle/left ventricle pressure ratio were evaluated. RESULTS: Median follow-up was 4.8 compared with 5.7 years for Groups 1 and 2, respectively, p = 0.65. Median number of major aortopulmonary collaterals/patient was 3, ranging from 1 to 8, in Group 1 compared with 4, ranging from 1 to 8, in Group 2, p = 0.09. Group 2 had a higher number of lobar/segmental stenoses within collateral vessels (p = 0.02). Group 1 had fewer catheter-based branch pulmonary artery reinterventions, with 5 (inter-quartile range from 1 to 7) per patient, compared with 9 (inter-quartile range from 4 to 14) in Group 2, p = 0.009. Among patients who achieved ventricular septal defect closure, median right ventricle/left ventricle pressure was 0.48 in Group 1 compared with 0.78 in Group 2, p = 0.03. Overall mortality was 6 (17%) in Group 1 compared with 9 (21%) in Group 2.DiscussionSingle-stage unifocalisation is a promising repair strategy in select patients, achieving low rates of reintervention for branch pulmonary artery restenosis and excellent mid-term haemodynamic outcomes. However, specific anatomic substrates of pulmonary artery/ventricular septal defect/major aortopulmonary collaterals may be better suited to multi-stage repair. Preoperative evaluation of collateral vessel calibre and function may help inform more patient-specific surgical management.

Physiologic effects of delayed sternal closure following stage 1 palliation.

BACKGROUND: Following stage 1 palliation, delayed sternal closure may be used as a technique to enhance thoracic compliance but may also prolong the length of stay and increase the risk of infection. METHODS: We reviewed all neonates undergoing stage 1 palliation at our institution between 2010 and 2017 to describe the effects of delayed sternal closure. RESULTS: During the study period, 193 patients underwent stage 1 palliation, of whom 12 died before an attempt at sternal closure. Among the 25 patients who underwent primary sternal closure, 4 (16%) had sternal reopening within 24 hours. Among the 156 infants who underwent delayed sternal closure at 4 [3,6] days post-operatively, 11 (7.1%) had one or more failed attempts at sternal closure. Patients undergoing primary sternal closure had a shorter duration of mechanical ventilation and intensive care unit length of stay. Patients who failed delayed sternal closure had a longer aortic cross-clamp time (123±42 versus 99±35 minutes, p=0.029) and circulatory arrest time (39±28 versus 19±17 minutes, p=0.0009) than those who did not fail. Failure of delayed sternal closure was also closely associated with Technical Performance Score: 1.3% of patients with a score of 1 failed sternal closure compared with 18.9% of patients with a score of 3 (p=0.0028). Among the haemodynamic and ventilatory parameters studied, only superior caval vein saturation following sternal closure was different between patients who did and did not fail sternal closure (30±7 versus 42±10%, p=0.002). All patients who failed sternal closure did so within 24 hours owing to hypoxaemia, hypercarbia, or haemodynamic impairment. CONCLUSION: When performed according to our current clinical practice, sternal closure causes transient and mild changes in haemodynamic and ventilatory parameters. Monitoring of SvO2 following sternal closure may permit early identification of patients at risk for failure.

Truncus arteriosus versus tetralogy of Fallot with pulmonary atresia.

Truncus arteriosus and tetralogy of Fallot with pulmonary atresia may be difficult to differentiate prenatally. We present a case that, on newborn echocardiography, angiography, and intraoperative inspection, shared features of both diagnoses.

Surgical Treatment of Tracheobronchomalacia: A novel approach.

Tracheobronchomalacia, as a whole, is likely misdiagnosed and underestimated as a cause of respiratory compromise in pediatric patients. Currently, there is no standardized approach for the overall evaluation of pediatric tracheobronchomalacia (TBM) and the concept of excessive dynamic airway collapse (EDAC); no grading score for the evaluation of severity; nor a standardized means to successfully approach TBM and EDAC. This paper describes our experience standardizing the approach to these complex patients whose backgrounds include different disease etiologies, as well as a variety of comorbid conditions. Preoperative and postoperative evaluation of patients with severe TBM and EDAC, as well as concurrent development of a prospective grading scale, has allowed us to ascertain correlation between surgery, symptoms, and effectiveness on particular tracheal-bronchial segments. Long-term, continued collection of patient characteristics, surgical technique, complications, and outcomes must be collected given the overall heterogeneity of this particular population.

Accelerated degeneration of a bovine pericardial bioprosthetic aortic valve in children and young adults.

BACKGROUND: Experience with aortic valve replacement (AVR) with current-generation pericardial bioprostheses in young patients is limited. The death of a child with accelerated bioprosthetic aortic stenosis prompted enhanced surveillance of all such patients at our institution. METHODS AND RESULTS: We reviewed records of 27 patients who had undergone AVR (median follow-up, 13.7 months) with a bovine pericardial bioprosthesis at ≤30 years of age. In the Mitroflow LXA valve group (n=15), freedom from valve failure was 100% at 1 year, 53% (95% confidence interval, 12-82) at 2 years, and 18% (95% confidence interval, 1-53) at 3 years. No Magna/Magna Ease valves (n=12) failed by 3 years. Among valve failure patients, median age at AVR was 12 years (range, 10-21 years). Life-threatening prosthetic aortic stenosis was detected at a median of 6 months after prior echocardiograms showing mild or less gradients. Patients with Mitroflow LXA compared with Magna/Magna Ease valves were smaller (median body surface area, 1.42 versus 1.93 m(2); P=0.002) and younger (median age, 13.0 versus 20.9 years; P=0.02) at AVR. Pathology demonstrated diffuse intrinsic leaflet calcification, not associated with inflammation or infection, and virtually immobile leaflets in closed position. CONCLUSIONS: Young patients undergoing AVR with Mitroflow LXA pericardial valves are at high risk for rapid progression from mild or less to severe aortic stenosis over months, highlighting their need for heightened echocardiographic surveillance and suggesting that this aortic bioprosthesis should not be implanted in the young. Current data are insufficient to assess the safety of AVR with other pericardial bioprostheses in children and the youngest adults.

Transposition of the great arteries and sinus venosus defect with partially anomalous pulmonary venous return: physiological and anatomic considerations.

Transposition of the great arteries is a common congenital heart defect causing cyanosis in neonates, occurring in 0.2 per 1000 live births. It has been reported to occur with other associated congenital heart lesions. However, its association with a superior sinus venosus defect and partially anomalous pulmonary venous return has not been reported. We present a neonate with transposition of the great arteries, superior sinus venosus defect with partially anomalous pulmonary venous return that underwent successful complete neonatal repair, and discuss important physiological and anatomic considerations.

Hypercoagulability panel testing predicts thrombosis in neonates undergoing cardiac surgery.

Thrombosis contributes to morbidity and mortality in neonates following cardiac surgery. Alterations in hemostatic factors following cardiac surgery have been described, but there is no data correlating these changes with risk of thrombosis in neonates. The aim of this study is to predict thrombosis in neonates undergoing cardiac surgery by assessment of a panel of hypercoagulability markers. Neonates undergoing cardiac surgery were enrolled preoperatively and prospectively followed. Preoperative hypercoagulability panel testing included thrombin generation assay (TGA), immunoassays for antithrombin III, protein C, protein S, factor VIII, thrombin-activatable fibrinolytic inhibitor (TAFI), plasminogen activator inhibitor-1 (PAI-1), and cardiolipin antibody. Postoperative thrombosis was defined by clinical events (shunt thrombosis, limb ischemia, and stroke) or imaging (intravascular or intracardiac thrombus). Risk factors for thrombosis were assessed. One hundred neonates were enrolled in the study over a two-year period. The incidence of postoperative in-hospital thrombosis was 20%. The only significant clinical risk factor associated with thrombosis was the single ventricle physiology. Hypercoagulability factors associated with increased risk of thrombosis by univariate analysis were elevated PAI-1, TAFI, and TGA, and presence of anticardiolipin antibodies. Multivariable logistic regression analysis demonstrated that elevated PAI-1 (P = 0.015), TAFI (P = 0.028), and TGA (P = 0.007) were independent predictors of thrombosis. Hypercoagulability panel testing may help identify neonates at high risk for thrombosis following cardiac surgery. Future studies are warranted to determine if high risk patients benefit from targeted anticoagulation therapies.

Right ventricular papillary muscle approximation to the septum as an adjunct technique during tricuspid valve repair in congenital heart surgery.

OBJECTIVE: Tricuspid regurgitation is often caused by leaflet splaying from displaced papillary muscles or ventricular dilatation. Traditional annuloplasty may not address this mechanism. The present study describes a single institution's experience using right ventricular papillary muscle approximation for tricuspid valve repair. METHODS: Right ventricular papillary muscle approximation consists of suturing the anterior papillary muscle to a point of the septum (septum or septal papillary muscle) that optimizes leaflet coaptation. We describe our technique and analyze clinical data of patients undergoing tricuspid valve repair with right ventricular papillary muscle approximation during congenital heart surgery between 2012 and 2021. RESULTS: Right ventricular papillary muscle approximation was performed as an adjunct procedure in 207 of 825 tricuspid valve repairs (25.1%). Discharge tricuspid regurgitation grade was mild tricuspid regurgitation or less in 153 patients (73.9%), and 140 patients (67.6%) had mild tricuspid regurgitation or less at a median latest follow-up of 3.2 years (interquartile range, 0.7-6.8). Thirty patients (14.5%) underwent 11 early tricuspid valve reinterventions (3 due to right ventricular papillary muscle approximation dehiscence) and 21 late tricuspid valve reinterventions. Estimated 5-year freedom from tricuspid valve reintervention was 84% (95% CI, 77.0-89.2). Systemic right ventricle physiology (odds ratio, 2.88, P = .048) and multiple mechanisms of tricuspid regurgitation (odds ratio, 7.35, P = .038) were significant predictors of tricuspid valve reintervention on multivariable analysis. CONCLUSIONS: Tricuspid valve repair with right ventricular papillary muscle approximation demonstrates acceptable short-term durability, but similar to other tricuspid valve repair strategies is less durable in patients with systemic right ventricle pressure and multiple mechanisms of tricuspid regurgitation. Right ventricular papillary muscle approximation is a safe and effective adjunct technique that should be considered in patients with tricuspid regurgitation caused by leaflet splaying from displaced papillary muscles or right ventricle dilatation.

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.

Association of tetralogy of Fallot and complete atrioventricular canal: a single-centre 40-year experience.

OBJECTIVES: Outcome data in tetralogy of Fallot (ToF) and complete atrioventricular canal (CAVC) are limited. We report our experience for over 40 years in this patient population. METHODS: Single-centre, retrospective analysis of patients who underwent surgical repair with the diagnosis of ToF-CAVC from 1979 to 2022, divided into 2 different periods and compared. RESULTS: A total of 116 patients were included: 1979-2007 (n = 61) and 2008-2021 (n = 55). Balanced CAVC (80%) and Rastelli type C CAVC (81%) were most common. Patients in the later era were younger (4 vs 14 months, P < 0.001), fewer had trisomy 21 (60% vs 80%, P = 0.019) and fewer had prior palliative prior procedures (31% vs 43%, P < 0.001). In the earlier era, single-patch technique was more common (62% vs 16%, P < 0.001), and in recent era, double-patch technique was more common (84% vs 33%, P < 0.001). In the earlier era, right ventricular outflow tract was most commonly reconstructed with transannular patch (51%), while in more recent era, valve-sparing repairs were more common (69%) (P < 0.001). In-hospital mortality was 4.3%. The median follow-up was 217 and 74 months for the first and second eras. Survival for earlier and later eras at 2-, 5- and 10-year follow-up was (85.1%, 81.5%, 79.6% vs 94.2%, 94.2%, 94.2% respectively, log-rank test P = 0.03). CONCLUSIONS: The surgical approach to ToF-CAVC has evolved over time. More recently, patients tended to receive primary repair at younger ages and had fewer palliative procedures. Improved surgical techniques allowing for earlier and complete repair have shown a decrease in mortality, more valve-sparing procedures without an increase in total reoperations. Presented at the 37th EACTS Annual Meeting, Vienna, Austria.

Surgical Valve Choices for Pulmonary Valve Replacement.

The range of valve choices available to the cardiac surgeon for placement in the pulmonary position continues to expand. This article will provide a brief compendium of the most clinically relevant among these choices and review the contemporary literature regarding their relative durability as well as risk factors for structural valve deterioration and reintervention. The unique advantages and disadvantages of each of these valve choices will be discussed as they pertain to unique patient-specific factors, including patient size and the anatomy of the right ventricular outflow tract, that inform the choice of one prosthesis over another. Finally, general principles regarding the approach to valve choice, and future directions will be discussed.

A Risk Prediction Model for Reintervention After Total Anomalous Pulmonary Venous Connection Repair.

BACKGROUND: Outcomes after total anomalous pulmonary venous connection (TAPVC) repair remain suboptimal due to recurrent pulmonary vein (PV) obstruction requiring reinterventions. We sought to develop a clinical prediction rule for PV reintervention after TAPVC repair. METHODS: Data from consecutive patients who underwent TAPVC repair at a single institution from January 1980 to January 2020 were retrospectively reviewed after Institutional Review Board approval. The primary outcome was postdischarge (late) unplanned PV surgical or transcatheter reintervention. Echocardiographic criteria were used to assess PV residual lesion severity at discharge (class 1: no residua; class 2: minor residua; class 3: major residua). Competing risk models were used to develop a weighted risk score for late reintervention. RESULTS: Of 437 patients who met entry criteria, there were 81 (18.5%) reinterventions at a median follow-up of 15.6 (interquartile range, 5.5-22.2) years. On univariable analysis, minor and major PV residua, age, single-ventricle physiology, infracardiac and mixed TAPVC, and preoperative obstruction were associated with late reintervention (all P < .05). The final risk prediction model included PV residua (class 2: subdistribution hazard ratio [SHR], 4.8; 95% CI, 2.8-8.1; P < .001; class 3: SHR, 6.4; 95% CI, 3.5-11.7; P < .001), age <1 year (SHR, 3.3; 95% CI, 1.3-8.5; P = .014), and preoperative obstruction (SHR, 1.8; 95% CI, 1.1-2.8; P = .015). A risk score comprising PV residua (class 2 or 3: 3 points), age (neonate or infant: 2 points), and obstruction (1 point) was formulated. Higher risk scores were significantly associated with worse freedom from reintervention (P < .001). CONCLUSIONS: A risk prediction model of late reintervention may guide prognostication of high-risk patients after TAPVC repair.

Residual Lesion Severity Predicts Midterm Outcomes After Congenital Aortic Valve Repair.

BACKGROUND: We sought to validate the technical performance score (TPS) as a predictor of midterm outcomes after congenital aortic valve repair. METHODS: This was a single-center, retrospective review of consecutive patients who underwent aortic valve repair between January 1, 2011, and December 31, 2019. Predischarge echocardiograms were used to assign a TPS for each index operation as class 1, no aortic valve residua; class 2, minor aortic valve residua; or class 3, major aortic valve residua or predischarge reintervention for major residua. The primary outcome was postdischarge (late) unplanned aortic valve reintervention. Secondary outcomes included late mortality and at least moderate aortic regurgitation or stenosis at the latest follow-up or before the earliest reintervention. Associations between TPS and outcomes were assessed using competing risk, Cox proportional hazards, or logistic regression models, adjusting for preoperative patient- and procedure-related covariates. RESULTS: Of 507 patients, there were 110 (21.7%) reinterventions, 22 (4.3%) deaths, and 67 (13.2%) cases of at least moderate aortic regurgitation or stenosis at the latest follow-up or earliest reintervention. On multivariable analysis, class 3 patients had a greater risk of reintervention (subdistribution hazard ratio, 2.6; 95% CI, 1.3-5.1; P = .005) and mortality (hazard ratio, 5.3; 95% CI. 1.1-25.2; P = .038) compared with class 1 patients. Adjusting for duration of follow-up, class 3 patients also had a greater risk of at least moderate aortic regurgitation or stenosis at the latest follow-up or earliest reintervention (odds ratio, 7.7; 95% CI, 2.5-24.2; P < .001) vs class 1 patients. CONCLUSIONS: Patients with major residua after congenital aortic valve repair have significantly worse midterm outcomes compared with those with no residua, warranting closer follow-up.

Symmetric bicuspidizing repair for patients with congenital aortic or truncal valve disease.

OBJECTIVE: Symmetric bicuspidizing repair has been shown to be safe and effective in the short term in adults and children with unicuspid aortic valve. Outcomes of extending this technique to patients with other forms of aortic and truncal valve disease have not been reported. METHODS: We performed a retrospective review of patients who underwent the symmetric bicuspidizing repair at Boston Children's Hospital between December 2019 and June 2022 with a contemporary comparator group of patients who underwent other forms of bicuspidization. Survival, valve-related reoperation, and the development of moderate or greater aortic or truncal valve regurgitation were assessed. RESULTS: There were 23 patients who underwent symmetric bicuspidizing repair and 18 who underwent another form of bicuspidization. Preoperative aortic regurgitation was present in 87.0%. Patients who underwent symmetric bicuspidizing repair more commonly underwent suture annuloplasty (100% vs 55.6%; P = .002) and ascending aortoplasty (78.3% vs 27.8%; P = .004). There was 1 operative mortality (2.4%) in the entire cohort and 1 late mortality. Freedom from moderate aortic regurgitation was 87.5% at 21 months after symmetric bicuspidizing repair compared with 43.5% for patients who underwent other types of bicuspidization; P = .03. Freedom from valve-related reoperation was 100% in the symmetric bicuspidizing repair group compared with 64.4%; P = .02. CONCLUSIONS: The symmetric bicuspidizing repair may be safely extended to patients with various forms of congenital aortic and truncal valve disease. Longer term follow-up will be necessary to determine the comparative effectiveness of this technique compared with neocuspidization and the Ross procedure.

Prognostic utility of a risk prediction model for predischarge major residual lesions or unplanned reinterventions following congenital mitral valve repair.

OBJECTIVE: We sought to develop a risk prediction model for predischarge major mitral valve (MV) residual lesions or unplanned MV reinterventions following congenital MV repair. METHODS: Patients who underwent congenital MV repair (excluding primary repair, but including secondary repair, of canal-type defects) at a single institution from January 2000 to December 2020 and survived to discharge were retrospectively reviewed. The primary outcome was major MV residua (mean gradient >6 mm Hg or moderate or greater regurgitation on the discharge echocardiogram) or predischarge unplanned MV reintervention. Risk factors of interest included age, single-ventricle physiology, preoperative and intraoperative postrepair MV stenosis and regurgitation severity, MV annular diameter z score, systemic ventricle ejection fraction, unfavorable anatomy, concomitant left-heart procedure, and various technique-related categories. Logistic regression was used to develop a weighted risk score for the primary outcome. Internal validation using bootstrap-resampling was performed. RESULTS: Of 866 patients who underwent congenital MV repair at a median age of 2.7 years (interquartile range, 0.7-9.1 years), 202 (23.3%) patients developed the primary outcome. The final risk prediction model had a C-statistic of 0.82 (95% confidence interval, 0.78-0.85). A weighted risk score was formulated per the variables in this model. The median risk score was 8 (interquartile range, 6-11) points. Patients were categorized as low (score 0-5), medium (score 6-10), high (score 11-15), or very high (score ≥16) risk. The probability of the primary outcome was 5.0 ± 1.7%, 15.2 ± 6.7%, 45.9 ± 12.6%, and 76.7 ± 8.8% for low-, medium-, high-, and very-high-risk patients, respectively. CONCLUSIONS: Our risk prediction model may guide prognostication of patients following congenital MV repair.