Tetralogy of Fallot with absent pulmonary valve (TOF/APV) is a rare form of congenital heart disease that has both cardiac and respiratory implications. While some patients are asymptomatic at birth, others have significant airway disease requiring mechanical ventilation and urgent surgical repair. To compare the relationship between preoperative mechanical ventilation on durations of both total invasive mechanical ventilation and non-invasive ventilation in patients undergoing TOF/APV repair. Secondary aims include comparing echocardiographic parameters with clinical predictors and outcomes. Retrospective chart review of 23 neonates and infants less than one year-old undergoing initial repair for TOF/APV. Patients were stratified by whether they required mechanical ventilation for cardiopulmonary stabilization in the immediate preoperative period. Patients were assessed for the durations of invasive and non-invasive ventilation. Echocardiographic parameters including main pulmonary artery, branch pulmonary arteries, and pulmonary valve z-scores were compared to clinical outcomes as well. Patients intubated preoperatively had significantly longer initial intubations (383.28 [211.47, 500.30] vs. 33 [23.93, 140.0] hours, p < 0.001) and total duration of intubation (599.82 [441.83, 3324.22] vs. 33 [23.93, 141.17] hours, p < 0.001). Patients intubated preoperatively had significantly longer durations of non-invasive ventilation (173.33 [89.33, 585] vs. 0 [0, 72.57] hours, p = 0.004). Only LPA z-scores were associated with the need for preoperative intubation. Preoperative intubation was associated with longer CICU and hospital length of stay. In patients with TOF/APV, the need for preoperative intubation is a predictor of postoperative respiratory complexity and length of stay. Echocardiographic data must be interpreted within the broader clinical context. Recognizing the potential implications in patients requiring preoperative respiratory support may help clinical teams anticipate postoperative ventilatory needs, inform perioperative planning, and guide family counseling.

Objective:Patients with a left pulmonary artery (LPA) sling often require surgical reimplantation to address pulmonary flow maldistribution and tracheal compression. We developed a virtual modeling workflow to determine what length of LPA should be retained for reimplantation and provided these data for intraoperative guidance. Methods:From March 2024 to May 2025, 5 patients diagnosed with an LPA sling with (n = 3) or without (n = 2) tracheal stenosis underwent surgical LPA reimplantation with or without slide tracheoplasty at median age 1.8 years [range, 0.4-3.6 years]. All patients had preoperative surgical planning using measurements from a segmented 3-dimensional model using a digital workflow. Model measurements included distances from the main pulmonary artery reimplantation site to nearby landmarks and the length of pressurized, prestretched LPA that should be retained for reimplantation based on the distance between the reimplant site and anticipated extent of distal LPA mobilization. These measurements were used as intraoperative guidance. Results:The planned main pulmonary artery reimplant site varied across surgeons, and recommended LPA length ranged from 42% to 71% of the total native length. Model recommendations were closely followed in all patients with LPA sling. Median hospital length of stay was 7 days [6-15]. At discharge, 3 patients had no/trivial LPA stenosis and 2 had mild (peak gradient 23 mm Hg [20, 26]). At median follow-up of 17.6 months [6.5-22.1], all patients had no/trivial stenosis and were asymptomatic with 100% freedom from reintervention. Postoperative lung-perfusion and computed tomography scans demonstrated left/right split of 40/60% ± 2% (n = 3) and LPA diameter z score of -0.23 [-0.82, +0.31] (n = 2). Conclusions:Three-dimensional models provide a platform for quantitative surgical planning to help achieve targeted reconstruction with good initial clinical follow-up.

Objective: Pediatric aortic valve repair has traditionally been guided by qualitative assessment. In this study, we present our early experience using quantitative guidelines to inform repair. Methods: We present a simple model of normal aortic valve proportions and compare outcomes of valves repaired with and without model-based guidelines. In a secondary analysis of a subset of valves that preoperatively exhibited 2 or 3 distinct leaflets with normal commissures, we assess whether achieving target normal leaflet free edge lengths is associated with less regurgitation. Results: Valve repair aided by modeling exhibited similar rates of aortic regurgitation (AR) and aortic stenosis (AS) on discharge and follow-up echocardiograms to controls. The repair versus replacement rate increased significantly from 57% in controls to 71% during the period in which modeling was used (P = .009), and the proportion of nontricuspid valves and the severity of preoperative AR was higher in the modeling groups than in the controls (P = .049 and P = .001, respectively). In the secondary analysis, operatively changing relative free edge length toward normal target values was associated with significant decreases in AR (P < .001), with a clear boundary separating valves with moderate or severe AR from those with none or trivial. Conclusions: Use of quantitative repair guidelines achieved similar outcomes and higher repair rates despite higher preoperative valve complexity. Among valves with distinct leaflets and normal commissures, changes in AR from preoperation to postoperation closely tracked surgical changes to relative leaflet free edge length. Although initial results are promising, larger numbers and longer follow-up are needed.

Ebstein anomaly is associated with a broad spectrum of presentations. Many fetal factors have been associated with intrauterine fetal demise and early neonatal mortality. Little is described about how fetal factors may influence illness severity in the immediate postnatal period. To identify the relationship between fetal echocardiogram measures and severity of illness on admission utilizing pSOFA scores. Retrospective chart review of 32 neonates with prenatally diagnosed Ebstein anomaly. Fetal echocardiographic factors assessed included right ventricular pressure (RVp), presence of antegrade pulmonary blood flow, ductus arteriosus (DA) flow direction, and pulmonary regurgitation (PR). pSOFA score was calculated on admission from available data. Patients were evaluated for the composite postnatal outcome of cardiac arrest, need for ECMO, or mortality. Median RVp on the last fetal echocardiogram was 26 [22, 35] mmHg. In 40.6

Objective Patients with complex vascular ring anomalies can present with symptoms of esophageal and airway compression, occasionally persisting despite relief of the vascular ring, due to residual compression from the aorta. We report our early experience with descending aortic translocation to address aerodigestive vascular compression. Methods This is a retrospective single-center review of patients undergoing descending aortic translocation. The operation involves dividing the distal aortic arch on cardiopulmonary bypass, relocating the descending aorta beneath the carina and right pulmonary artery, and anastomosis to the posterior aspect of the ascending aorta, thereby entirely relieving compression on the aerodigestive tract. Concomitant esophageal mobilization and tracheopexies are performed as needed. Results From 2020 to 2023, 15 patients underwent descending aortic translocation. Anatomic variants included hairpin aorta (n = 2), circumflex aorta (n = 4), midline descending aorta (n = 7), and bowstring aorta (n = 2). Median age was 2 years. Preoperatively, most patients (80%) had mixed esophageal and airway symptoms and had undergone at least 1 prior operation (60%). Intraoperative bronchoscopy revealed improved airway patency for all patients. Postoperatively, 93% of patients had relief of respiratory symptoms, and 79% of patients had improved dysphagia. Postoperative complications included neurologic event (n = 1) with full resolution, right pulmonary artery compression requiring stenting (n = 1), and need for reoperation (n = 2). Conclusions Descending aortic translocation is a novel approach that can provide symptomatic relief of aerodigestive compression related to complex aortic anomalies. For patients with persistent symptoms after initial vascular ring repair, ongoing aortic compression may need to be addressed, thus also raising the question of whether descending aortic translocation should be considered as a first-line therapy.

OBJECTIVE:Durable and biocompatible pulmonary valved conduits remain a challenge in congenital cardiac surgery. The GORE PV1 device is a fully synthetic expanded polytetrafluoroethylene-based conduit designed to improve durability and reduce calcification. This study characterizes the gross and histopathologic findings in explanted PV1 conduits. METHODS:Six GORE PV1 conduits explanted from pediatric patients (median age: 13.5 years) were evaluated. All patients were part of a previously reported cohort. Clinical data, imaging, and operative notes were reviewed. Gross and histologic examinations assessed leaflet integrity, thrombus, inflammation, calcification, and tissue overgrowth. Sections were stained with hematoxylin and eosin and reviewed by an experienced pediatric cardiac pathologist. RESULTS:All patients had undergone multiple previous cardiac surgeries and received anticoagulation after conduit implantation. Explantation was prompted by thrombus formation, with pulmonary emboli in 4 of 6 patients (66.7%), including 2 with progression despite therapeutic anticoagulation. Leaflets appeared thin, transparent, and intact with no calcification or degeneration. Nonadherent thrombi, often filling the sinuses, were present in all conduits. Histology showed no leaflet inflammation, fibrosis, calcification, or endothelialization. Thrombi demonstrated laminated fibrin with inflammatory cells; 2 showed foreign-body giant cell reaction. Fibrous overgrowth along suture lines was minimal to moderate. CONCLUSIONS:The GORE PV1 conduit retained excellent structural integrity after midterm implantation. However, frequent nonadherent thrombi, despite anticoagulation, were associated with embolic events. These findings underscore the need for long-term monitoring and device design improvements, and may guide anticoagulation strategies in patients receiving synthetic valved conduits.

Aerodigestive compression syndromes of vascular origin can be broadly classified based on congenital and acquired causes. Congenital causes include various types of arch anomalies (vascular rings including double arches, right aortic arch with aberrant left subclavian artery, circumflex aorta, etc), arch branching anomalies (Left aortic arch with aberrant right subclavian artery, innominate artery related compression), aneurysms of either the aorta or main pulmonary artery or branch pulmonary arteries and any combinations of these. Acquired causes of aerodigestive compression are mostly post-surgical which is a distinct and challenging entity usually arising from arch reconstructive procedures, root amalgamation procedures, procedures involving the LeCompte maneuver and procedures involving PDA stenting. When there are associated thoracic cage and vertebral anomalies, an additional layer of complexity is added to an already challenging problem. At Boston Children’s Hospital, these patients are evaluated with a multi-disciplinary team using diagnostic studies including echocardiography, esophagram, computed tomographic angiography and three-phase dynamic bronchoscopy for a thorough understanding of anatomic and physiologic complexities. Surgical correction to relieve compression requires an individualized plan for each patient and 3D models are routinely employed. Posterior thoracotomy, sternotomy and video assisted thorocoscopy (VATs) are approaches to correct these problems with additional concomitant usage of aortopexy, tracheopexy and localized or complete esophageal mobilization. Intraoperative bronchoscopy is widely used both for surgical planning and for confirmation of relief of compression.

BACKGROUND:The study objective was to evaluate surgical outcomes of patients having had catheter-based pulmonary vein (PV) stents implanted with subsequent pulmonary vein stenosis (PVS) repair in a large, single-center cohort. METHODS:Clinical data were retrospectively reviewed from a PVS registry identifying patients who had prior PV stents placed, had in-stent stenosis, and required subsequent PVS repair. RESULTS:Between September 2007 and July 2023, 251 patients underwent surgical PVS repair, of whom 42 (17%) had prior PV stents implanted. Bilateral stents were present in 19 patients (45%). Thirty-one patients (74%) had at least 1 prior PV operation. Median follow-up was 8 months (interquartile range, 3-36 months). Thirty-one patients (74%) underwent postoperative catheter reintervention to maintain patency, and 6 patients (14%) underwent surgical reoperation. Overall, survival at discharge was 95%, and in the modern era, 65% survived at last follow-up. There were similar survival outcomes in PVS patients undergoing surgery with and without prior stents (P = .23). Of the individual veins in patients with prior stents, 84% (n = 97) of stented veins were patent at last follow-up. CONCLUSIONS:Increasing numbers of patients are undergoing catheter-based PV stent interventions for severe recurrent PVS. Despite this, there are reasons to be hopeful, with 84% of these veins patent at most recent follow-up, and no difference in survival of patients with or without preoperative stent placement.

Objective Supravalvular aortic stenosis (SVAS) repair carries a moderate risk for postoperative aortic regurgitation (AR). This may be related to the targeted sinotubular junction diameter z score (STJDZ) at reconstruction, traditionally sized to zero. We investigated relationships between achieved STJDZ and aortic valve function after repair. Methods Sixty-six children underwent patch repair from 2010 to 2024 at a median age of 1.2 years (interquartile range [IQR], 0.4, 3.5 years) via Brom (n = 53), Doty (n = 9), and McGoon (n = 4) techniques. Relationships between STJDZ and aortic valve function were assessed via Jonckheere-Terpstra nonparametric test for trend over median follow-up of 5.6 years (IQR, 2.4, 8.4 years). Results Preoperatively, AR was none/trivial in 52 (79%), left ventricular outflow tract obstruction (LVOTO) moderate or greater in 54 (82%), and median STJDZ −3.8 (IQR, −4.4, −2.9). At discharge, AR and LVOTO were mild or greater in 45 (68%) and 18 (27%), and median STJDZ was −0.3 (IQR, −1.3, −1.1). STJDZ increased by a median of 3.0 (IQR, 1.6, 4.4) preoperatively to postoperatively. Discharge AR was none/trivial at median STJDZ −1.3 (IQR, −1.8, −0.1) and worsened with increasing diameters (mild: 0.4 [IQR, −1.0, 1.8], mild+: 0.0 [IQR, −1.2, 1.5]; P = .038), with similar results at follow-up (P = .048). LVOTO worsened with lower median STJDZ (none/trivial: 0.2 [IQR, −1.2, 1.5], mild: −1.3 [IQR, −3.2, −0.1]; P = .015). Subanalysis of those with none/trivial preoperative AR (n = 52) showed that increase in STJDZ by ≥4 demonstrated worse follow-up AR (P = .016). Conclusions Achieving STJDZ zero using patch repair techniques to relieve SVAS obstruction demonstrated mild or greater postoperative AR compared with none/trivial regurgitation at median z score −1.3. Mild LVOTO was noted with median z score −1.3. Targeting STJDZ of −1 in pediatric SVAS repair appears appropriate to balance residual regurgitation and LVOTO. Further follow-up is required to understand the long-term impact of this target on aortic growth.

Congenital aortic and truncal valve diseases pose significant challenges due to their complex anatomy, diverse clinical presentations, and impact on long-term outcomes. Surgical management requires a tailored approach, balancing the need for immediate functional improvement with strategies to minimize reintervention, especially in growing children. This article outlines different surgical options to treat this pathology. Despite multiple choices, surgical decision-making remains complex, influenced by patient age, valve morphology, and comorbidities. Long-term outcomes depend on achieving a durable repair while preserving ventricular function and minimizing complications, emphasizing the need for multidisciplinary expertise in managing these challenging conditions.

BACKGROUND:The effect of anticoagulation on early postoperative outcomes after pulmonary valve replacement (PVR) with bioprosthetic valves and homografts is not yet defined. We hypothesized that short-term anticoagulation would be associated with improved valve durability. METHODS:Patients undergoing PVR or right ventricle-to-pulmonary artery conduits with a bioprosthetic or homograft valve >15 mm in diameter between January 2015 and April 2021 at Boston Children's Hospital were retrospectively compared by anticoagulation status. Survival, postoperative echocardiographic data, and the incidence of pulmonary valve reintervention and prosthetic valve dysfunction were compared using multivariable Cox and logistic regression analyses adjusted for a propensity score based on anticoagulation therapy. RESULTS:Among 531 patients with a median follow-up of 1.16 years, 130 received anticoagulation therapy. Freedom from reintervention was 99%, 92%, and 86% at 1, 3, and 5 years, respectively. Adjusted for propensity score, there was no association between reintervention and anticoagulation (hazard ratio, 0.78; 95% CI, 0.18-3.47; P = .75). The propensity score-adjusted rates of valve dysfunction were 9% in anticoagulated patients and 15% in non-anticoagulated patients (odds ratio, 0.54; 95% CI, 0.18-1.56; P = .25). There was no difference in early major bleeding rates between groups (3.4% vs 2.4%, P = .59) or in other postoperative outcomes. Only 1 of 18 (6%) of all bleeding events in the anticoagulated group occurred after starting anticoagulation. CONCLUSIONS:Anticoagulation therapy appeared safe with no association with major postoperative bleeding. However, additional follow-up is necessary to assess its impact on midterm to long-term valve durability after PVR.

OBJECTIVE:The impact of early age on outcomes for repair of complete atrioventricular canal defects (CAVCs) remains poorly defined. We evaluated young infants with CAVC, comparing those who underwent primary repair versus primary pulmonary artery banding (PAB) and results related to left atrioventricular valve (AVV) reintervention and survival. METHODS:Patients (age <60 days) with CAVC were evaluated (January 2005 to April 2022) at a single institution. Patients were categorized as having primary CAVC repair or PAB. Patients with complex unbalanced CAVC and severely hypoplastic ventricles and those not undergoing CAVC repair after PAB were excluded. Outcome measures included total number of operations, reoperation on the left AVV, hospital length of stay, and mortality. RESULTS:CAVC was identified in 135 patients, mean age 33 ± 19 days and weight 3.4 ± 0.7 kg at primary operation. Additional diagnosis included transposition of the great arteries (n = 4), tetralogy of Fallot (n = 9), transposition of the great arteries (n = 13), and total and partial anomalous pulmonary venous return (n = 7). Thirty-three patients required preoperative respiratory support. Primary CAVC repair was performed in 101 patients at 38 ± 16.6 days and 3.5 ± 0.7 kg, and primary PAB was performed in 34 patients at 16 ± 15 days and 3.2 ± 0.7 kg, of whom 62% (n = 21) underwent subsequent CAVC repair at 6.9 ± 4.7 months and 6.6 ± 2.3 kg. When we compared patients undergoing primary CAVC versus PAB; 55% versus 48% had preoperative mild and 39% versus 29% mild-moderate or greater atrioventricular valve regurgitation (AVVR). In patients who underwent CAVC repair, a 2-patch repair was used in 66% of cases and posterior left AVV annuloplasty in 34%. Predischarge reoperation for left AVVR was required in 13% (n = 14/101) patients whereas in patients who underwent PAB, it was required in 14% (n = 3/21). Hospital length of stay was shorter for primary CAVC (25 vs 41 days). Overall, median follow-up was 4.5 years. Patients undergoing primary CAVC had fewer total number of operations (1.3 vs 2.5, P < .001) and fewer reoperations on the left AVV (18% vs 24%, P = .56). Overall, freedom from reoperation in primary CAVC for left AVVR at 1 and 5 years was 85% and 82% compared with patients who underwent PAB (89% and 69%). At follow-up, 88% of patients undergoing primary CAVC repair had mild or less left AVVR, whereas 82% undergoing initial PAB had mild or less left AVVR. There were 10 deaths; overall mortality was 6% in patients who underwent primary CAVC and 19% in patients who underwent PAB. Similarly, follow-up rates of significant AVVR and mortality did not differ significantly between groups (P > .05). CONCLUSIONS:Definitive CAVC repair at ≤60 days can be performed with acceptable midterm survival. Primary CAVC repair versus primary PAB for young patients undergoing CAVC has a trend toward fewer total operations, fewer reoperations for AVVR, decreased hospital LOS, and less mortality. However, reoperation rates for AVVR and mortality were not statistically different, and pacemaker implantation occurred in 10% of patients who underwent primary repair. These results underscore the need for cautious interpretation, given the limitations of statistical power. Reoperation for left AVVR remains a challenge and occurs early after repair. Evolving surgical techniques to avoid postoperative left AVV dysfunction should further reduce early postoperative morbidity and hospital resource use.

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

OBJECTIVE:The Autus Size-Adjustable Valve (ASAV) is a novel surgically implanted fully synthetic expandable pulmonary valve designed for pediatric patients. This first-in-human study evaluated initial device feasibility and safety. METHODS:This was a Food and Drug Administration-approved prospective, single-arm, multicenter Investigational Device Exemption Early Feasibility Study to evaluate safety and preliminary effectiveness of the ASAV in pediatric patients aged 18 months to 16 years who require pulmonary valve replacement. All potential subjects underwent review by a screening committee to determine eligibility. Subjects had pre- and postoperative evaluation via transthoracic echocardiography. One-year outcomes are reported. RESULTS:Eleven subjects (9 male) were enrolled at 3 centers. Median age at implant was 8.2 years (range, 2.7-13.9) years and body surface area ranged from 0.6 to 1.3 m2. The ASAV was size-adjusted at implant in all subjects, ranging from 14 mm to 20 mm internal diameter (z score -1 to +1). Median hospital length of stay was 4 (range, 3-5) days. There was no mortality, and 100% of subjects were free from device-related adverse events. No thrombus was observed. No subject developed endocarditis. At 1-year follow-up, 10 (90.9%) subjects had no pulmonary regurgitation (1 subject [8.1%] had trivial pulmonary regurgitation), and the mean right ventricle-pulmonary artery pressure gradient was 16 ± 6 mm Hg. CONCLUSIONS:One-year results of the novel ASAV Early Feasibility Study demonstrated intact valve function and no device-related adverse events. The ASAV is now under evaluation in an Investigational Device Exemption pivotal study.

Objective Aortic valve reconstruction (AVNeo) with neocuspidization, or the Ozaki procedure with complete cusp replacement for aortic valve disease, has excellent immediate-term technical results in children and midterm results in adults. Limited midterm results of AVNeo in younger patients have been reported. We report our midterm outcomes of neocuspidization with Ozaki technique for congenital aortic and truncal valve disease. Methods A retrospective analysis was performed on all patients with congenital aortic and truncal valve disease who had a 3-leaflet AVNeo procedure at a single institution from August 2015 to June 2023. Outcome measures included mortality, surgical or catheter-based reinterventions, and echocardiography. Results In total, 74 patients aged 12.8 ± 7.3 years underwent 3 leaflet AVNeo; 31 patients had aortic regurgitation (AR), 7 had aortic stenosis (AS), and 36 patients had AS/AR. Five patients had truncus arteriosus. The aortic valve was quadricuspid in 2.7%, 46% tricuspid, 34% bicuspid, and 18% unicusp of patients. In total, 57% had previous aortic valve repairs and 9% had replacements. Preoperative echocardiography mean annular diameter was 2.03 ± 0.48 cm, and peak gradient for patients with AS/AR was 60.1 ± 24.4 mm Hg. Autologous pericardium was used in 42% (n = 31) of patients, PhotoFix bovine in 58% (n = 43) of patients, and CardioCel bovine pericardium in 2% (n = 2) of patients. In total, 71 patients had concomitant procedures, including 11 who underwent aortic root enlargement including 34 with aortic sinus enlargement. Median intensive care unit and hospital length of stay were 1.9 and 7.0 days, respectively. There were no hospital mortalities or early conversions to valve replacement. At discharge, 95% of patients had mild or less regurgitation and median peak aortic gradient was 16.9 ± 9.0 mm Hg. Over median follow-up of 3.2 years, 30 patients (40%) required aortic valve reoperation (PhotoFix, 25/43 patients, 58%; and autologous, 5/31 patients, 16%). Freedom from reoperation at 1 and 3 years for autologous was 97 and 86%, and for PhotoFix was 97% and 75% (P = .006). Freedom from a composite outcome of moderate or greater regurgitation and/or reoperation at 1 and 3 years for autologous was 89% and 67% and for PhotoFix was 92% and 54% (P = .13). Failure mode for autologous pericardium was related to calcification and for PhotoFix pericardium was dehiscence at the annular suture line. Conclusions The AVNeo procedure has acceptable short-term results in all patients but especially poor midterm results with PhotoFix bovine pericardium. AVNeo could be considered with caution for valve reconstruction in congenital patients if native autologous pericardium is available.