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.

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.

Transcatheter Palliation With Pulmonary Artery Flow Restrictors in Neonates With Congenital Heart Disease: Feasibility, Outcomes, and Comparison With a Historical Hybrid Stage 1 Cohort.

BACKGROUND: Neonates with complex congenital heart disease and pulmonary overcirculation have been historically treated surgically. However, subcohorts may benefit from less invasive procedures. Data on transcatheter palliation are limited. METHODS: We present our experience with pulmonary flow restrictors (PFRs) for palliation of neonates with congenital heart disease, including procedural feasibility, technical details, and outcomes. We then compared our subcohort of high-risk single ventricle neonates palliated with PFRs with a similar historical cohort who underwent a hybrid Stage 1. Cox regression was used to evaluate the association between palliation strategy and 6-month mortality. RESULTS: From 2021 to 2023, 17 patients (median age, 4 days; interquartile range [IQR], 2-8; median weight, 2.5 kilograms [IQR, 2.1-3.3]) underwent a PFR procedure; 15 (88%) had single ventricle physiology; 15 (88%) were high-risk surgical candidates. All procedures were technically successful. At a median follow-up of 6.2 months (IQR, 4.0-10.8), 13 patients (76%) were successfully bridged to surgery (median time since PFR procedure, 2.6 months [IQR, 1.1-4.4]; median weight, 4.9 kilograms [IQR, 3.4-5.8]). Pulmonary arteries grew adequately for age, and devices were easily removed without complications. The all-cause mortality rate before target surgery was 24% (n=4). Compared with the historical hybrid stage 1 cohort (n=23), after adjustment for main confounding (age, weight, intact/severely restrictive atrial septum or left ventricle to coronary fistulae), the PFR procedure was associated with a significantly lower all-cause 6-month mortality risk (adjusted hazard ratio, 0.26 [95% CI, 0.08-0.82]). CONCLUSIONS: Transcatheter palliation with PFR is feasible, safe, and represents an effective strategy for bridging high-risk neonates with congenital heart disease to surgical palliation, complete repair, or transplant while allowing for clinical stabilization and somatic growth.

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.

Multidisciplinary approach to vascular rings and vascular-related aerodigestive compression: a clinical practice review.

Vascular rings, including double aortic arch and right aortic arch with aberrant left subclavian and left ligamentum, are part of a larger group of vascular-related aerodigestive compression syndromes that also includes innominate artery compression syndrome, dysphagia lusoria, aortic arch anomalies, and aneurysms of either the aorta or pulmonary artery. Additionally, post-surgical airway compression is a distinct entity in itself. The approach to the diagnosis and management of these varied phenomena has been streamlined by the multidisciplinary team at Boston Children's Hospital. Echocardiography, computed tomographic angiography, esophagram, and three-phase dynamic bronchoscopy are routinely performed in these patients in order to produce a comprehensive understanding of the unique anatomic challenges that each patient presents. Adjunctive diagnostic techniques include modified barium swallow, routine preoperative and postoperative screening of the vocal cords, and radiographic identification of the artery of Adamkiewicz. Beyond the vascular reconstruction, which ranges from subclavian-to-carotid transposition to descending aortic translocation, we liberally apply tracheobronchopexy and rotational esophagoplasty to relieve respiratory and esophageal symptoms. Due to the heightened risk for recurrent laryngeal nerve injury, intraoperative recurrent laryngeal nerve monitoring has become routine in these cases. The comprehensive care of these patients requires the coordinated efforts of a large team of dedicated personnel in order to achieve the optimal result.

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.

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.

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.

Outcomes of Repair of Congenital Aortic Valve Lesions Using Autologous Pericardium vs Porcine Intestinal Submucosa.

BACKGROUND: Outcomes following congenital aortic valve (AoV) repair are plagued by progressive dysfunction of currently available leaflet substitute materials. OBJECTIVES: We compared the long-term outcomes of congenital AoV repair using porcine intestinal submucosa vs autologous pericardium (AP). METHODS: This was a single-center retrospective review of all patients who underwent congenital AoV repair with either porcine intestinal submucosa or AP from October 2009 to March 2013. The primary outcome was postdischarge (late) unplanned AoV reintervention. Secondary outcomes included number of late AoV reinterventions and a composite of at least moderate aortic regurgitation or stenosis at latest follow-up or before the first reintervention. Associations between leaflet repair material and outcomes were assessed using multivariable regression models, adjusting for prespecified patient-related and operative variables. RESULTS: Of 26 porcine intestinal submucosa and 49 AP patients who met entry criteria, the median age was 11.0 years (IQR: 4.7-16.6 years). At a median follow-up of 8.5 years (IQR: 4.4-9.6 years), 17 (65.4%) porcine intestinal submucosa and 22 (44.9%) AP patients underwent at least 1 AoV reintervention. On multivariable analysis, porcine intestinal submucosa use was significantly associated with unplanned AoV reintervention (HR: 4.6; 95% CI: 2.2-9.8; P < 0.001), number of postdischarge AoV reinterventions (incidence rate ratio: 1.7; 95% CI: 1.0-2.9; P = 0.037), and at least moderate aortic regurgitation or stenosis at latest follow-up or before the first reintervention (OR: 5.0; 95% CI: 1.2-21.0; P = 0.027). CONCLUSIONS: Aortic valvuloplasty with porcine intestinal submucosa is associated with earlier time to reintervention compared with autologous pericardium. The search for the ideal AoV leaflet repair material continues.

Contemporary outcomes of the double switch operation for congenitally corrected transposition of the great arteries.

OBJECTIVE: To determine the contemporary outcomes of the double switch operation (DSO) (ie, Mustard or Senning + arterial switch). METHODS: A single-institution, retrospective review of all patients with congenitally corrected transposition of the great arteries undergoing a DSO. RESULTS: Between 1999 and 2019, 103 patients underwent DSO with a Mustard (n = 93) or Senning (n = 10) procedure. Segmental anatomy was (S, L, L) in 93 patients and (I, D, D) in 6 patients. Eight patients had heterotaxy and 71 patients had a ventricular septal defect. Median age was 2.1 years (range, 1.8 months-40 years), including 34 patients younger than age 1 year (33%). Median weight was 10.9 kg (range, 3.4-64 kg). Sixty-one patients had prior pulmonary artery bands for a median of 1.1 years (range, 14 days-12.9 years; interquartile range, 0.7-3.1 years). Median intensive care unit and hospital lengths of stay were 5 and 10 days, respectively. Median follow-up was 3.4 years (interquartile range, 1-9.8 years) and 5.2 years (interquartile range, 2.3-10.7 years) in 79 patients with >1 year follow-up. At latest follow-up, aortic, mitral, tricuspid valve regurgitation, and left ventricle dysfunction was less than moderate in 96%, 98%, 96%, and 93%, respectively. Seventeen patients underwent reoperation: neoaortic valve intervention (n = 10), baffle revision (n = 5), and ventricular septal defect closure (n = 4). At latest follow-up, 17 patients (17%) had a pacemaker and 27 (26%) had cardiac resynchronization therapy devices. There were 2 deaths and 2 transplants. Transplant-free survival was 94.6% at 5 years. Risk factors for death or transplant included longer cardiopulmonary bypass time and older age at DSO. CONCLUSIONS: The outcomes of the DSO are promising. Earlier age at operation might favor better outcomes. Progressive neoaortic regurgitation and reinterventions on the neo-aortic valve are anticipated problems.

Standardized Aortic Valve Neocuspidization for Treatment of Aortic Valve Diseases.

BACKGROUND: Aortic valve replacement is the traditional surgical treatment for aortic valve diseases, yet standardized aortic valve neocuspidization (AVNeo) is a promising alternative that is gaining popularity. The purpose of this article is to review the available published literature of AVNeo using glutaraldehyde-treated autologous pericardium, also known as the Ozaki procedure, including indications, outcomes, potential benefits, and modes of failure for the reconstructed valve. METHODS: A comprehensive literature search was performed using keywords related to aortic valve repair, AVNeo, or Ozaki procedure. All articles describing performance of AVNeo were reviewed. RESULTS: Reported early mortality after AVNeo varies from 0% to 5.88%. The largest cohort of patients in the literature includes 850 patients with an inhospital mortality rate of 1.88%. Cumulative incidence of aortic valve reoperation was 4.2% in the largest series. Reoperation was uncommon and mainly due to infective endocarditis or degeneration of the reconstructed valve (most commonly due to aortic valve regurgitation, rather than stenosis). CONCLUSIONS: Aortic valve neocuspidization is a versatile and standardized alternative to aortic valve replacement with a biological prosthesis. Early to midterm outcomes from a number of centers are excellent and demonstrate the safety and durability of the procedure. Long-term outcomes and clinical trial data are necessary to determine which patients benefit the most from this procedure.

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.

Tricuspid valve repair concomitant with the Norwood operation among babies with hypoplastic left heart syndrome.

OBJECTIVES: Among patients with hypoplastic left heart syndrome (HLHS), tricuspid valve regurgitation (TR) portends a poor prognosis. Our goal was to describe the outcomes of tricuspid valve reconstruction (TVR) concomitant with the Norwood operation and using two-dimensional echocardiography and evaluate the structural factors associated with successful functional correction. METHODS: We performed a retrospective, single-centre study of patients with HLHS undergoing TVR at the time of the Norwood operation. Structural echocardiographic parameters were compared between patients with successful correction (≤ mild TR) and those with ≥ moderate regurgitation at discharge. Preoperative dimensions of matched HLHS controls with ≤ trivial TR were used as a reference. RESULTS: Of 205 patients with HLHS undergoing the Norwood operation, 18 patients had a concomitant TVR. Ten (56%) patients had an improved TR grade postoperatively, 8 (44%) of whom had ≤ mild TR at discharge. Improvement in TR grade (P = 0.001) and having ≤ mild TR at discharge (P = 0.011) were associated with an improved reintervention and TR-free survival. Patients with successful functional correction had smaller preoperative tricuspid annulus lateral dimensions (P = 0.023), tricuspid valve area (P = 0.007) and right ventricle mid-width (P = 0.064). Preoperatively, the successful TVR cases tended to have had higher anterior leaflet excursion (80 ± 20 vs 55 ± 11, P = 0.010), and a higher proportion of anterior leaflet prolapse (63% vs 10%, P = 0.043) compared to cases where TVR was not successful. CONCLUSIONS: Patients with HLHS with significant tricuspid regurgitation undergoing the stage 1 operation were more likely to have successful concomitant tricuspid valve repair if they had less tricuspid annular dilation, less-severe RV enlargement and predominantly anterior leaflet prolapse. Successful tricuspid valve repair was associated with improved mid- and long-term outcomes.