Operative and nonoperative outcomes in patients with trisomy 13 and 18 with congenital heart disease.

Objective: To evaluate the short- and long-term outcomes of cardiac repair versus nonoperative management in patients with trisomy 13 and trisomy 18 with congenital heart disease. Methods: An institutional review board-approved, retrospective review was undertaken to identify all patients admitted with trisomy 13/18 and congenital heart disease. Patients were divided into 2 cohorts (operated vs nonoperated) and compared. Results: Between 1985 and 2023, 62 patients (34 operated and 28 nonoperated) with trisomy 13 (n = 9) and trisomy 18 (n = 53) were identified. The operated cohort was 74% girls, underwent mainly The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery mortality category 1 procedures (n = 24 [71%]) at a median age of 2.5 months (interquartile range [IQR], 1.3-4.5 months). This compares with the nonoperative cohort where 64% (n = 18) would have undergone The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery mortality category 1 procedures if surgery would have been elected. The most common diagnosis was ventricular septal defect. Postoperative median intensive care unit stay was 6.5 days (IQR, 3.7-15 days) with a total hospital length of stay of 15 days (IQR, 11-49 days). Thirty-day postoperative survival was 94%. There were 5 in-hospital deaths in the operated and 7 in the nonoperated cohort. Median follow-up was 15.4 months (IQR, 4.3-48.7 months) for the operated and 11.2 months (IQR, 1.2-48.3 months) for the nonoperated cohorts. One-year survival was 79% operated versus 51.5% nonoperated (P < .003). Nonoperative treatment had an increased risk of mortality (hazard ratio, 3.28; 95% CI, 1.46-7.4; P = .004). Conclusions: Controversy exists regarding the role of primary cardiac repair in patients with trisomy 13/18 and congenital heart disease. Cardiac repair can be performed safely with low early mortality and operated patients had higher long-term survival compared with nonoperated in our cohort.

Outcomes of Patients Undergoing Surgery for Complete Vascular Rings.

BACKGROUND: Few studies describe outcomes after complete vascular ring surgery in a comprehensive manner. OBJECTIVES: This study sought to describe the clinical presentation, diagnostic work-up, operative approach, and outcomes in children undergoing surgery for complete vascular rings. METHODS: This single-center retrospective cohort study includes consecutive patients (January 1990 through September 2023) undergoing primary surgery for complete vascular rings, or rerepair after primary surgery elsewhere. The primary outcome of interest was complete (as distinct from partial) symptom resolution at latest clinic follow-up. Our current preference is to pursue a comprehensive initial operation including adjunctive vascular and airway procedures targeting common causes of residual aerodigestive symptoms, such as Kommerell diverticulum resection and tracheobronchopexy. Preoperative work-up routinely involved computed tomographic angiography, dynamic bronchoscopy, and laryngoscopy. RESULTS: Of 515 patients (including 39 rerepairs), the most common diagnoses were right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum (n = 323, 62.7%) and double aortic arch (n = 174, 33.8%). There was no perioperative mortality. Chylothorax occurred in 28 patients (5.4%), vocal cord dysfunction in 22 patients (4.3%), and diaphragm paralysis in 2 patients (0.4%). Follow-up was available on 453 patients (88.0%) with a median duration of 3.0 years (Q1-Q3: 0.6-9.2 years). At latest clinic follow-up, 429 patients (94.7%) reported complete symptom resolution. The risk of reoperation for residual or recurrent aerodigestive symptoms was 9.6% (95% CI: 5.7%-13.5%) at 10 years and 12.4% at 20 years (95% CI: 6.9%-17.8%). CONCLUSIONS: Surgery for complete vascular rings provides good symptomatic relief with low risk of complications, whereas reoperations for aerodigestive symptoms are infrequent.

Reverse Double Switch Operation for the Borderline Left Ventricle.

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

Short-Term Results With Ozaki Valved Conduit-A Simple Solution for Patients Needing Right Ventricle to Pulmonary Artery Conduit in a Low-Resource Setting.

BACKGROUND: The repair of certain types of complex congenital cardiac defects may require a right ventricle-pulmonary artery (RV-PA) conduit. Using the Ozaki Aortic valve neocuspidization (AVNeo)technique, a valved RV-PA conduit was constructed with an Ozaki valve inside a Dacron graft. This study aims to evaluate the short-term outcome of the Ozaki valved RV-PA conduit. MATERIAL/METHOD: A total of 22 patients received the Ozaki valved RV-PA conduit from November 2019 until December 2023. The median age was 12 years (interquartile range [IQR], 5.5-21), median body weight was 35 kg (IQR, 15.8-48.5). The conduit was used in 16 patients (72.7%) under 18 years of age. Indications for conduit placement included: anatomic repair of corrected transposition of the great arteries, ventricular septal defect/pulmonary stenosis, conduit replacement, pulmonary atresia with associated anomalies, pulmonary artery aneurysm with dysplastic pulmonary valve, tetralogy of Fallot with coronary artery crossing the right ventricular outflow tract, bioprosthetic pulmonary valve regurgitation, and rheumatic heart disease. Native pericardium was used for the Ozaki valve in 12 patients and bovine pericardium for 10 patients. Conduit sizes ranged from 18 mm to 30 mm. RESULT: The median intensive care unit stay was 4 (IQR, 2-6) days and the median hospital stay was 9 (IQR, 5.5-13.5) days. There were two perioperative mortalities (9.1%) both unrelated to the conduit. The median follow-up was 12.3 (IQR, 4.43-21.2) months. There was no infective endocarditis of the conduit. The median peak gradient across the conduit was 22 mm Hg (range 0-44 mm), and all were competent with trivial regurgitation on follow up. CONCLUSION: Creation of an Ozaki valved conduit is an attractive option due to low cost, reproducibility, and excellent hemodynamics. Longer-term studies are needed to confirm the durability.

State of the Art Review: Aortic Valve Repair in Infants and Children.

BACKGROUND: Congenital aortic and truncal valve disease is challenging in infants and children given the lack of available prostheses in very small sizes and the limited durability of homograft aortic valve replacement. METHODS: A comprehensive literature search was performed using the PubMed database. Studies were included either if the report included patients less than 1 year of age or if the technique was tailored to accommodate for somatic growth. RESULTS: Techniques for aortic and truncal valve repair addressing each aspect of the aortic valve complex - the aorta, aortic annulus, commissures, and cusps - were reviewed. The incidence of reoperation following aortic or truncal valve repair is significant at 10-years ranging from 30% to 70% depending on the underlying diagnosis and the repair technique utilized. A significant challenge in interpreting the published literature relates to the lack of anatomic data available in the publications limiting the ability to make direct comparisons between operative techniques and also limits the ability to draw conclusions regarding these techniques as applied to varied etiologies. CONCLUSIONS: A comprehensive understanding of the aortic valve complex is necessary to achieve adequate results in pediatric aortic valve repair given the high variability in these valves.

Clinical and 2D/3D-Echo Cardiography Determinants of Mitral Valve Reoperation in Children With Congenital Mitral Valve Disease.

Background: Congenital mitral valve disease (CMVD) presents major challenges in its medical and surgical management. Objectives: The purpose of this study was to investigate the value of 3-dimensional echocardiography (3DE) and identify associations with MV reoperation in this setting. Methods: All children <18 years of age who underwent MV reconstruction for CMVD in 2002 to 2018 were included. Preoperative and postoperative 2-dimensional echocardiography (2DE) and 3DE data were collected. Competing risks and Cox regression analysis were used to identify independent associations with MV reoperation. Receiver operating characteristic and decision-tree analysis were implemented for comparison of 3DE vs 2DE. Results: A total of 206 children underwent MV reconstruction for CMVD (mitral stenosis, n = 105, mitral regurgitation [MR], n = 75; mixed disease, n = 26); 64 (31%) required MV reoperation. Variables independently associated with MV reoperation were age <1 year (HR: 2.65; 95% CI: 1.13-6.21), tethered leaflets (HR: 2.00; 95% CI: 1.05-3.82), ≥ moderate 2DE postoperative MR (HR: 4.26; 95% CI: 2.45-7.40), changes in 3D-effective orifice area (3D-EOA) and in 3D-vena contracta regurgitant area (3D-VCRA). Changes in 3D-EOA and 3D-VCRA were more strongly associated with MV reoperation than changes in mean gradients (area under the curve [AUC]: 0.847 vs AUC: 0.676, P = 0.006) and 2D-VCRA (AUC: 0.969 vs AUC: 0.720, P = 0.012), respectively. Decision-tree analysis found that a <30% increase in 3D-EOA had 80% accuracy (HR = 8.50; 95% CI: 2.9-25.1) and a <40% decrease in 3D-VCRA had 93% accuracy (HR: 22.50; 95% CI: 2.9-175) in discriminating MV reoperation for stenotic and regurgitant MV, respectively. Conclusions: Age <1 year, tethered leaflets, 2DE postoperative MR, changes in 3D-EOA and 3D-VCRA were all independently associated with MV reoperation. 3DE parameters showed a stronger association than 2DE. 3DE-based decision-tree algorithms may help prognostication and serve as a support tool for clinical decision-making.

Development of a Simple Analytical Model to Facilitate Preoperative Surgical Planning in Valve-Sparing Aortic Root Replacement.

PURPOSE: Valve-sparing root replacement (VSRR) is attractive for aortic root dilation as it preserves the native aortic valve (AoV). Low effective height (eH) after reconstruction is a risk factor for repair failure and reoperation. We developed and validated a quantitative AoV repair strategy to reliably restore normal valve proportions to promote long-term function. METHODS: Normal AoV proportions were used to derive geometric relationships for sinotubular junction diameter (DSTJ), free edge length (FEL), free edge angle, and commissure height. These relationships informed two models for predicting eH following VSRR: (1) assuming valve symmetry and (2) accounting for valve asymmetry. Porcine heart (n = 6) ex vivo validation was performed under 4 VSRR scenarios: "Ideal" (tube graft size targeting FEL/DSTJ = 1.28), "Oversized" (one graft size larger than Ideal), "Undersized" (two sizes smaller), and "Undersized + Plicated" (FEL/DSTJ = 1.28 restored with leaflet plication). RESULTS: Our analytical models predicted eH using preoperative measurements and estimated reconstructed dimensions. The Oversized graft exhibited similar eH to Ideal but higher regurgitation in the ex vivo model, whereas the Undersized graft demonstrated lower eH and regurgitation. Plication in the Undersized graft restored valve function (regurgitation & eH) similar to Ideal in the ex vivo model and above Ideal in the analytical models. Both analytical models predicted ex vivo eH well except in the Oversized and Undersized + Plicated conditions. CONCLUSION: Utilizing measurements from preoperative imaging and simple mathematical models, patient-specific operative plans for VSRR can be created by estimating valve dimensions necessary to achieve favorable valve features post-repair. Clinical application of this approach promises to improve consistency in achieving optimal long-term dimensions and durability.

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.

Systematic Analysis of PTFE Monocusp Leaflet Design in a Patient-Based 3D in-Vitro Model of Tetralogy of Fallot.

PURPOSE: Pulmonary valve (PV) monocusp reconstruction in transannular patch (TAP) right ventricular outflow tract (RVOT) repair for Tetralogy of Fallot has variable clinical outcomes across different surgical approaches. The study purpose was to systematically evaluate how monocusp leaflet design parameters affect valve function in-vitro. METHODS: A 3D-printed, disease-specific RVOT model was tested under three infant physiological conditions. Monocusps were sewn into models with the native main pulmonary artery (MPA) forming backwalls that constituted 40% and 50% of the reconstructed circumference for z-score zero PV annulus and MPA diameters (native PV z-score - 3.52 and - 2.99 for BSA 0.32m2). Various leaflet free edge lengths (FEL) (relative to backwall), positions (relative to PV STJ), and scallop depths were investigated across both models. Pressure gradient, regurgitation, and coaptation were analyzed with descriptive statistics and regression models. RESULTS: Increasing FEL beyond 100% of the MPA backwall decreased gradient but mildly increased regurgitation to a peak of 25%. Positioning the free edge 2 mm past the STJ mildly increased gradient for each FEL without significantly changing regurgitation compared to STJ placement. Scalloping leaflets trivially affected performance. Pre-folding leaflets improved mobility and slightly reduced gradient. CONCLUSIONS: Balancing gradient, regurgitation, and oversizing for growth, a set of leaflet designs have been selected for pre-clinical evaluation. Designs with leaflet widths 140-160% in the 40% backwall model (110-120% in the 50% backwall), positioned at or 2 mm past the STJ, demonstrated the best results. The next stage of ex-vivo testing will additionally consider native RVOT distensibility, native leaflet interactions, and TAP characteristics.

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.

Right Heart Remodeling After Pulmonary Valve Replacement in Patients With Pulmonary Atresia or Critical Stenosis With Intact Ventricular Septum.

Background Patients with pulmonary atresia or critical pulmonary stenosis with intact ventricular septum (PA/IVS) and biventricular circulation may require pulmonary valve replacement (PVR). Right ventricular (RV) remodeling after PVR is well described in tetralogy of Fallot (TOF); we sought to investigate RV changes in PA/IVS using cardiac magnetic resonance imaging. Methods and Results A retrospective cohort of patients with PA/IVS who underwent PVR at Boston Children's Hospital from 1995 to 2021 with cardiac magnetic resonance imaging before and after PVR was matched 1:3 with patients with TOF by age at PVR. Median regression modeling was performed with post-PVR indexed RV end-diastolic volume as the primary outcome. A total of 20 patients with PA/IVS (cases) were matched with 60 patients with TOF (controls), with median age at PVR of 14 years. Pre-PVR indexed RV end-diastolic volume was similar between groups; cases had higher RV ejection fraction (51.4% versus 48.6%; P=0.03). Pre-PVR RV free wall and left ventricular (LV) longitudinal strain were similar, although LV midcavity circumferential strain was decreased in cases (-15.6 versus -17.1; P=0.001). At a median of 2 years after PVR, indexed RV end-diastolic volume was similarly reduced; cases continued to have higher RV ejection fraction (52.3% versus 46.9%; P=0.007) with less reduction in RV mass (Δ4.5 versus 9.6 g/m2; P=0.004). Post-PVR, RV and LV longitudinal strain remained unchanged, and LV circumferential strain was similar, although lower in cases. Conclusions Compared with patients with TOF, patients with PA/IVS demonstrate similar RV remodeling after PVR, with lower reduction in RV mass and comparatively higher RV ejection fraction. Although no differences were detected in peak systolic RV or LV strain values, further investigation of diastolic parameters is needed.

The Yasui operation: A single institutional experience over 30 years.

Objective: The Yasui operation was introduced in 1987 for patients with 2 adequate ventricles, a ventricular septal defect, and aortic atresia or interrupted aortic arch. Despite promising early outcomes, left ventricular outflow tract obstruction (LVOTO) remains a long-term concern. The purpose of this study is to report our institutional experience with the Yasui operation. Methods: We retrospectively reviewed all patients undergoing the Yasui operation between 1989 and 2021. Results are reported as median with interquartile range (IQR). Results: Twenty-five patients underwent a Yasui operation (19 primary), at 11 days (IQR, 7-218 days) of life and weight of 3 kg (IQR, 2.8-4.1 days). Fundamental diagnosis was ventricular septal defect/interrupted aortic arch in 11 patients and ventricular septal defect/aortic atresia in 14. Follow-up was 96% (24 out of 25) at 5 years (IQR, 1.4-14.7) with 92% survival. Freedom from LVOTO reoperation was 91% at late follow-up with 2 patients requiring baffle revision at 6 and 9 years. Latest echocardiogram showed 100% of patients had normal biventricular function and 87% (20 out of 23) less than mild LVOTO at 5 years (IQR, 2.3-14.9). Diagnosis, aortic valve morphology, and material used were not predictors of LVOTO. Freedom from right ventricle-to-pulmonary artery conduit reoperation was 48% at a median of 5 years (IQR, 1.4-14.7). Conduit type was not a predictor of reintervention. Conclusions: The Yasui operation can be performed with low morbidity and mortality in patients with 2 acceptable-size ventricles and aortic atresia or interrupted aortic arch with severe LVOTO. Despite some burden of reoperation, midterm reoperation for LVOTO is not common and ventricular function is preserved.

Transcatheter Pulmonary Artery Banding in High-Risk Neonates: In-Vitro Study Provoked by Initial Clinical Experience.

PURPOSE: Very high-risk, ductal-dependent or complex two-ventricle patients with associated comorbidities often require pulmonary blood flow restriction as bridge to a more definitive procedure, but current surgical options may not be well-tolerated. An evolving alternative utilizes a fenestrated Micro Vascular Plug (MVP) as a transcatheter, internal pulmonary artery band. In this study, we report a case series and an in-vitro evaluation of the MVP to elicit understanding of the challenges faced with device implantation. METHODS: Following single-center, retrospective review of eight patients who underwent device placement, an in-vitro flow study was conducted on MVP devices to assess impact of device and fenestration sizing on pulmonary blood flow. A mathematical model was developed to relate migration risk to vessel size. Results of the engineering analysis were compared to the clinical series for validation. RESULTS: At median follow-up of 8 months (range 1-15), survival was 63% (5/8), and 6 (75%) patients underwent subsequent target surgical intervention with relatively low mortality (1/6). Occluder-related challenges included migration (63%) and peri-device flow, which were evaluated in-vitro. The device demonstrated durability over normal and supraphysiologic conditions with minimal change in fenestration size. Smaller vessel size significantly increased pressure gradient due to reduced peri-device flow and smaller effective fenestration size. CONCLUSION: Device oversizing, with appropriate adjustment to fenestration size, may reduce migration risk and provide a clinically appropriate balance between resulting pressure gradient and Qp:Qs. Our results can guide the interventionalist in appropriately selecting the device and fenestrations based on patient-specific anatomy and desired post-implantation flow characteristics.

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.

Pacemaker lead insertion sites contribute to abnormalities of myocardial function and histopathology.

BACKGROUND: Ventricular pacing can cause myocardial dysfunction, but how lead anchoring to the myocardium affects function has not been studied. OBJECTIVE: The purpose of this study was to evaluate patterns of regional and global ventricular function in patients with a ventricular lead using cine cardiac computed tomography (CCT) and histology. METHODS: This was a single-center retrospective study with 2 groups of patients with a ventricular lead: (1) those who underwent cine CCT from September 2020 to June 2021 and (2) those whose cardiac specimen was analyzed histologically. Regional wall motion abnormalities on CCT were assessed in relation to lead characteristics. RESULTS: For the CCT group, 122 ventricular lead insertion sites were analyzed in 43 patients (47% female; median age 19 years; range 3-57 years). Regional wall motion abnormalities were present at 51 of 122 lead insertion sites (42%) in 23 of 43 patients (53%). The prevalence of a lead insertion-associated regional wall motion abnormality was higher with active pacing (55% vs 18%; P < .001). Patients with lead insertion-associated regional wall motion abnormalities had a lower systemic ventricular ejection fraction (median 38% vs 53%; P < .001) than did those without regional wall motion abnormalities. For the histology group, 3 patients with 10 epicardial lead insertion sites were studied. Myocardial compression, fibrosis, and calcifications were commonly present directly under active leads. CONCLUSION: Lead insertion site-associated regional wall motion abnormalities are common and associated with systemic ventricular dysfunction. Histopathological alterations including myocardial compression, fibrosis, and calcifications beneath active leads may explain this finding.