Search

Congenital tracheal stenosis (CTS) is a rare condition characterised by different patterns of tracheal narrowing. The pathological hallmark is the presence of complete tracheal rings, with or without associated anomalies. We present a case of asymptomatic CTS in a patient with unilateral cleft lip. Tracheal stenosis was discovered incidentally immediately prior to surgery for correction of the cleft lip, when several attempts at intubation proved unsuccessful. A CT scan of the neck and proximal chest demonstrated a localised region of airway narrowing of the distal trachea, in addition to a right sided cleft lip without cleft palate. Management of CTS depends on several factors, with surgery being the definitive form of treatment. We review the pathology of CTS, its relationship with craniofacial defects, the role of radiology and management of this disease entity.

This article revisits the relationship between corruption and Foreign Direct Investment inflows in a panel of 42 countries from 1984 to 2012 using pooled mean group estimator in a dynamic heterogeneous panel setting using Westerlund and ARDL panel cointegration tests where the estimations are carried out by three different estimators: the pooled mean group (PMG), mean group (MG), and the dynamic fixed effect (DFE) estimators in order to examine both the long- and short-term effects of corruption on FDI inflows. The results suggest that corruption has a positive impact on FDI inflows in the case of Asia and Africa; and a negative impact in the case of Latin America.

Background: Pulmonary hypertension in children often progresses despite optimal therapy. This document provides an evidence-based clinical practice guideline for the management of children with progressive pulmonary hypertension despite optimal therapy., Methods: A multidisciplinary panel identified pertinent questions regarding the management of children with pulmonary hypertension that has progressed despite optimal therapy, conducted systematic reviews of the relevant literature, and applied the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) approach to develop clinical recommendations., Results: After reviewing the research evidence, the panel considered the balance of desirable (benefits) and undesirable (harms and burdens) effects of the interventions in each proposed question. Valuation of our main outcomes was also considered, along with resources required, equity, acceptability, and feasibility. Recommendations were developed for or against interventional strategies specific to children with pulmonary hypertension that has progressed despite optimal therapy., Conclusions: Although there is a growing population of children with pulmonary hypertension, there is a striking lack of empirical evidence regarding management of those whose disease has progressed despite optimal pharmacotherapy. The panel formulated and provided the rationale for clinical recommendations for or against interventional strategies based on this limited empirical evidence, coupled with expert opinion, to aid clinicians in the management of these complex pediatric patients. In addition, we identified important areas for future research.

Vein of Galen malformations are the most common congenital neurovascular malformation and are a type of choroidal arteriovenous fistula involving the midline primitive choroidal venous circulation. The arteriovenous shunt zone of a VOGM may directly involve the embryonic precursor of the vein of Galen and/or its tributaries within the 3rd ventricle tela choroidea. Dural sinus malformations are characterized by dilated intracranial dural venous sinuses, some of which acquire multifocal arteriovenous shunts within the dural walls of these overgrown venous sinuses.Pial arteriovenous fistulae are high-flow shunts representing direct arterial to venous communication of pial blood vessels, with no definable nidus., Competing Interests: Disclosure The authors have no conflicts of interest to declare., (Crown Copyright © 2024. Published by Elsevier Inc. All rights reserved.)

Background: Pediatric pulmonary vein stenosis (PVS) is often progressive and treatment-refractory, requiring multiple interventions. Hybrid pulmonary vein interventions (HPVIs), involving intraoperative balloon angioplasty or stent placement, leverage surgical access and customization to optimize patency while facilitating future transcatheter procedures. We review our experience with HPVI and explore potential applications of this collaborative approach. Methods: Retrospective chart review of all HPVI cases between 2009 to 2023. Results: Ten patients with primary (n = 5) or post-repair (n = 5) PVS underwent HPVI at median age of 12.7 months (range 6.6 months-9.5 years). Concurrent surgical PVS repair was performed in 7/10 cases. Hybrid pulmonary vein intervention was performed on 17 veins, 13 (76%) with prior surgical or transcatheter intervention(s). One patient underwent intraoperative balloon angioplasty of an existing stent. In total, 18 stents (9 bare metal [5-10 mm diameter], 9 drug eluting [3.5-5 mm diameter]) were placed in 16 veins. At first angiography (median 48 days [range 7 days-2.8 years] postoperatively), 8 of 16 (50%) HPVI-stented veins developed in-stent stenosis. Two patients died from progressive PVS early in the study, one prior to planned reintervention. Median time to first pulmonary vein reintervention was 86 days (10 days-2.8 years; 8/10 patients, 13/17 veins). At median survivor follow-up of 2.2 years (2.3 months-13.1 years), 1 of 11 surviving HPVI veins were completely occluded. Conclusions: Hybrid pulmonary vein intervention represents a viable adjunct to existing PVS therapies, with promising flexibility to address limitations of surgical and transcatheter modalities. Reintervention is anticipated, necessitating evaluation of long-term benefits and durability as utilization increases., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: Author AMQ serves as a consultant and proctor for W.L. Gore and Associates, Medtronic Inc., and B. Braun.

Objective: To assess the association between primary and staged repair of neonatal symptomatic tetralogy of Fallot (sTOF) and neurodevelopmental outcomes in preschool through school-age children., Study Design: Multicenter cohort (n = 9 sites) study of patients with sTOF who underwent neonatal intervention between 2005 and 2017. The neurodevelopmental outcomes measures included caregivers' ratings of executive function with the Behavior Rating Inventory of Executive Function, and psychosocial functioning with the Behavior Assessment System for Children - third Edition (BASC-3). Results were compared with normative data and by treatment strategy (primary repair vs staged repair). A parent survey assessed history of disabilities and access to services related to neurodevelopment., Results: Although the majority of patients (median age 8.3 years, IQR 5.7-11.2) had median Behavior Rating Inventory of Executive Function and BASC-3 scores within the normal range, a proportion had clinically elevated (abnormal) scores, especially in the school-age patient subgroup (Behavior Rating Inventory of Executive Function 24%-30% and BASC 20%-37%). There were no statistically significant differences based on treatment strategy for either the Behavior Rating Inventory of Executive Function or BASC-3. However, lower birth weight, genetic syndrome, and medical complexity were significantly associated with worse executive function, and lower maternal education was associated in school-age children with lower executive and psychosocial functioning. Ongoing disabilities were relatively common (learning disability 35%, speech delay 33%, developmental delay 31%), although up to 50% of children were not receiving educational or developmental services., Conclusions: Elevated executive and psychosocial concerns are present in the patient population with sTOF. Although initial treatment strategy appears unrelated to neurodevelopmental outcomes, lower birth weight, genetic syndrome, and medical complexity and lower maternal education are risk factors. Early recognition of neurodevelopmental concerns can facilitate access to appropriate neurodevelopmental services in this high-risk group., Competing Interests: Declaration of Competing Interest Dr Jeffrey Zampi is a consultant for Medtronic, Inc and WL Gore and Associates. He also serves on the Data Safety Monitoring Board for Encore Medical. Dr Athar Qureshi is a consultant for Medtronic, Inc, B Braun, and WL Gore and Associates. Dr Bryan Goldstein is a consultant for Medtronic, Inc, WL Gore and Associates, PECA labs, and Mezzion Pharma. This study was funded in part by the generous support from the member institutions of the CCRC as well as the Kennedy Hammill Pediatric Cardiac Research Fund, The Liam Sexton Foundation, A Heart Like Ava, and a research grant from Project Heart. The study sponsors did not have any role in the study design; the collection, analysis, and interpretation of data; the writing of the report; or the decision to submit the manuscript for publication., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: Chemotherapy for colorectal cancer (CRC) urgently needs low-toxicity and highly effective phytomedicine. Cepharanthine (Cep) shown to have multiple anti-tumor effects, including colorectal cancer, whose pivotal mechanisms are not fully understood. Herein, the present work aims to reveal the impact of Cep on the mitochondrial and anti-injury functions of CRC cells., Methods: The TOM70/20 expression was screened by bioinformatic databases. SW480 cells were utilized as the colorectal cancer cell model. The expression of TOM70/20 and the downstream molecules were measured by western blots (WB). The ferroptosis was analyzed using Transmission electron microscopy (TEM), C11-BODIPY, PGSK, and DCFH-DA probes, wherein the detection was performed by flow cytometry and laser confocal microscopy. The anti-cancer efficacy was conducted by CCK-8 and Annexin-V/PI assay. The rescue experiments were carried out using Fer-1 and TOM70 plasmid transfection., Results: Bioinformatic data identified TOM20 and TOM70 were highly expressed in colorectal cancer, which could be down-regulated by Cep. Further findings disclosed that Cep treatment destroyed the mitochondria and inactivated the NRF2 signaling pathway, an essential pathway for resistance to ferroptosis, thereby promoting reactive oxygen species (ROS) generation in CRC cells. As a result, prominent ferroptosis could be observed in CRC cells in response to Cep, which thereby led to the reduced cell viability of cancer cells. On the contrary, recovery of TOM70 dampened the Cep-elicited mitochondria damage, ferroptosis, and anti-cancer efficacy., Conclusion: In summary, Cep-mediated TOM inhibition inactivates the NRF2 signaling pathway, thereby triggering ferroptosis and achieving an anti-colorectal cancer effect. The current study provides an innovative chemotherapeutic approach for colorectal cancer with phytomedicine., (© 2024. The Author(s).)

Background: The GORE CARDIOFORM ASD Occluder (GCA, W. L. Gore & Associates) was approved in 2019 for ostium secundum atrial septal defect (ASD) closure., Objectives: This study sought to report the combined pivotal and continued access cohorts of the ASSURED (Safety and Efficacy Study of Transcatheter Closure of Ostium Secundum ASDs) trial results through 36 months., Methods: This prospective, multicenter, single-arm trial evaluated procedural and clinical outcomes of ASD closure with the GCA. The primary endpoints were 6-month closure success following device implantation and composite clinical success (deployment/retention of device, safety, and closure). Technical and procedure success, safety, clinically significant new arrhythmia (CSNA) secondary endpoints, and wire frame fracture (WFF, with fluoroscopy) at 6 and 36 months were evaluated., Results: Of 569 patients (median age of 10.4 years and median weight of 35.0 kg) who underwent attempted secundum ASD closure, 526 were technical successes. The mean stop-flow ASD diameter was 17.6 ± 5.3 mm. All 478 patients with 6-month imaging achieved closure success. Composite clinical success at 6 and 36 months was achieved in 87.6% (468/534) and 84.0% (351/418) of patients, respectively. Technical failure occurred in 8.1% (43/548), 30-day device- or procedure-related serious adverse event in 3.9% (21/534), and 6-month device events in 2.8% (15/534) of patients. At 30 days, 21 of 569 patients (3.7%) had CSNA. At 6 months, 138 of 436 (31.7%) patients had WFFs and 105 of 185 (56.8%) at 36 months (without sequelae)., Conclusions: In this large congenital ASD device trial, the GCA had acceptable results. WFFs, although common, did not result in any clinical sequelae. The unique features, size range, and safety profile expand the options for secundum ASD closure. (Safety and Efficacy Study of Transcatheter Closure of Ostium Secundum ASDs [ASSURED]; NCT02985684)., Competing Interests: Funding Support and Author Disclosures This study was sponsored by W. L. Gore & Associates. Dr Qureshi is a consultant for W. L. Gore & Associates, Medtronic, and B. Braun. Dr Sommer has received institutional funding from W. L. Gore & Associates for working on their national PFO and ASD trials; and serves on the Advisory Board for Conformal Medical. Dr Morgan is a consultant/proctor for W. L. Gore & Associates. Dr Paolillo is a consultant, proctor, and preceptor for W. L. Gore & Associates. Dr Goldstein is a consultant for Medtronic, W. L. Gore & Associates, Edwards Lifesciences, and Mezzion Pharmaceuticals. Dr Sugeng serves on the Speakers Bureau for Philips Healthcare; and is a consultant for Siemens Healthineers and Yale Echo Corelab YCRG. Dr Gillespie is a consultant for W. L. Gore & Associates and Medtronic. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Objective: To describe clinical, functional, surgical, and outcomes data in pediatric patients with a myocardial bridge (MB) evaluated and managed following a standardized approach., Methods: Prospective observational study included patients evaluated in the Coronary Artery Anomalies Program. Anatomy was determined by computed tomography angiography, myocardial perfusion by stress perfusion imaging, and coronary hemodynamic assessment by cardiac catheterization., Results: In total, 39 of 42 patients with a complete evaluation for MB were included (December 2012 to June 2022) at a median age of 14.1 years (interquartile range, 12.2-16.4). Sudden cardiac arrest occurred in 3 of 39 (8%), exertional symptoms in 14 (36%), and no/nonspecific symptoms in 7 (18%) patients. Exercise stress test was abnormal in 3 of 34 (9%), stress perfusion imaging in 8 of 34 (24%), and resting instantaneous wave-free ratio ≤0.89 or diastolic dobutamine fractional flow reserve ≤0.80 in 11 of 21 (52%) patients. As a result, 15 of 39 (38%) patients were determined to have hemodynamically significant MB, 1 of 15 patients started beta-blocker, and 14 of 15 were referred for surgery. Myotomy (n = 11) and coronary bypass (n = 1) were performed successfully, resulting in improved symptoms and stress testing results. One patient required pericardiocentesis postoperatively, and all were discharged without other complications. At median follow-up time of 2.9 (1.8-5.8) years, all (except 2 pending surgery) were doing well without exercise restriction., Conclusions: Pediatric patients with MB can present with myocardial ischemia and sudden cardiac arrest. Provocative stress test and intracoronary hemodynamic tests helped risk-stratify symptomatic patients with MB and concern for ischemia. Surgical repair was safe and effective in mitigating exertional symptoms and stress test results, allowing patients to return to exercise without restriction., Competing Interests: Conflict of Interest Statement The authors reported no conflicts of interest. The Journal policy requires editors and reviewers to disclose conflicts of interest and to decline handling or reviewing manuscripts for which they may have a conflict of interest. The editors and reviewers of this article have no conflicts of interest., (Copyright © 2024 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Single ventricle patients eligible for Fontan completion undergo pre-Fontan catheterization for hemodynamic and anatomic assessment prior to surgery. Cardiac magnetic resonance imaging may be used to evaluate pre-Fontan anatomy, physiology, and collateral burden. We describe our center's outcomes in patients undergoing pre-Fontan catheterization combined with cardiac magnetic resonance imaging. A retrospective review of patients undergoing pre-Fontan catheterization from 10/2018 to 04/2022 at Texas Children's Hospital was performed. Patients were divided into 2 groups: combined cardiac magnetic resonance imaging and catheterization (combined group) and those who underwent catheterization only (catheterization only group). There were 37 patients in the combined group and 40 in the catheterization only group. Both groups were similar in age and weight. Patients undergoing combined procedures received less contrast, and experienced less in-lab time, fluoroscopy time and catheterization procedure time. Median radiation exposure was lower in the combined procedure group but was not statistically significant. Intubation and total anesthesia times were higher in the combined procedure group. Patients undergoing a combined procedure were less likely to have collateral occlusion performed than in the catheterization only group. Bypass time, intensive care unit length of stay, and chest tube duration were similar in both groups at the time of Fontan completion. Combined pre-Fontan assessment decreases catheterization procedure and fluoroscopy time associated with cardiac catheterization at the expense of longer anesthetic times, and results in similar Fontan outcomes compared to when cardiac catheterization alone is utilized., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

To evaluate the association between initial management strategy of neonatal symptomatic Tetralogy of Fallot (sTOF) and later health-related quality of life (HRQOL) outcomes. We performed a multicenter, cross-sectional evaluation of a previously assembled cohort of infants with sTOF who underwent initial intervention at ≤ 30 days of age, between 2005 and 2017. Eligible patients' parents/guardians completed an age-appropriate Pediatric Quality of Life Inventory, a Pediatric Quality of Life Inventory Cardiac Module Heart Disease Symptoms Scale, and a parental survey. The association between treatment strategy and HRQOL was evaluated, and the entire sTOF cohort was compared to published values for the healthy pediatric population and to children with complex congenital heart disease and other chronic illness. The study cohort included 143 sTOF subjects, of which 59 underwent a primary repair, and 84 had a staged repair approach. There was no association between initial management strategy and lower HRQOL. For the entire cohort, in general, individual domain scores decreased as age sequentially increased. Across domain measurements, mean scores for the sTOF cohort were significantly lower than the healthy pediatric population and comparable to those with other forms of complex CHD and other chronic health conditions. The presence of a genetic syndrome was significantly associated with a poor HRQOL (p = 0.003). Initial treatment strategy for sTOF was not associated with differences in late HRQOL outcomes, though the overall HRQOL in this sTOF cohort was significantly lower than the general population, and comparable to others with chronic illness., (© 2024. The Author(s).)

Background: Hepatic and splenic venous access are specialised techniques used to perform diagnostic and interventional procedures in the cardiac catheterisation laboratory. Bleeding events are the most commonly reported complication following hepatic or splenic venous access. The VASCADE Vascular Closure System (Cardiva Medical Inc. Santa Barbara, CA) is an approved device for closure of femoral vascular access tracts in patients ≥18 years of age. We report our experience using VASCADE to close the hepatic or splenic venous access site in the cardiac catheterisation laboratory., Methods: This is a single centre retrospective review of all patients who had percutaneous hepatic or splenic venous access obtained in the cardiac catheterisation laboratory from March 1, 2022 through October 30, 2023 and underwent tract closure with VASCADE., Results: Ten patients (six male) underwent 16 procedures (median age and weight 3.5 years and 12.5 kg) with 15 hepatic and two splenic veins accessed. Successful closure of the access tracts with VASCADE was performed in all patients. There were no major adverse events related to closure of the access sites with VASCADE., Conclusion: VASCADE can be used following transhepatic and trans-splenic venous access in the cardiac catheterisation laboratory to safely close the access tract and potentially reduce the risk of post-procedural bleeding complications. Further evaluation in a larger cohort of patients is needed to ensure VASCADE is safe for use and provides adequate haemostasis following hepatic or splenic venous access, particularly in children.

Background: Neonates with tetralogy of Fallot and symptomatic cyanosis (sTOF) require early intervention, utilizing either a staged repair (SR) or primary repair (PR) approach. They are exposed to several sources of low-dose ionizing radiation, which may contribute to increased cancer risk., Objectives: The purpose of this study was to compare cumulative radiation exposure and associated lifetime attributable risk (LAR) of cancer between treatment strategies in sTOF., Methods: Neonates with sTOF who underwent SR or PR from 2012 to 2017 were retrospectively reviewed from the Congenital Cardiac Research Collaborative. Radiation exposure from all radiologic studies prior to 18 months of age was converted to organ-equivalent doses and projected LAR of cancer incidence using the National Cancer Institute dosimetry tools., Results: There were 242 neonates from 8 centers, including patients with 146 SR and 96 PR. Cumulative total effective dose was significantly higher for SR (median 8.3 mSv, IQR: 3.0-17.4 mSv) than PR (2.1 mSv, IQR: 0.8-8.5 mSv; P  < 0.001). Cumulative organ-level doses were significantly higher in SR compared to PR. Regardless of treatment strategy, LARs were higher in females compared to males. Among organs with median exposure >1 mGy in females, the LAR was highest for breast in SR (mean 1.9/1,000 patients). The highest proportion of cancers attributable to radiation exposure was projected for thyroid cancer in females undergoing SR (7.3%)., Conclusions: Cumulative radiation exposure and LARs were higher among those undergoing SR compared to PR. This will be an important factor to consider in determining the preferred neonatal treatment strategy and should substantiate efforts to reduce radiation exposure in this vulnerable population., Competing Interests: Financial support for this research was derived, in part, from the Kennedy Hammill Pediatric Cardiac Research Fund, the Liam Sexton Foundation, and A Heart Like Ava. Dr Goldstein has reported consulting relationships with Medtronic, W.L. Gore & Associates, and Edwards Lifesciences; and is an advisory board member for PECA Labs. Dr Qureshi has reported consulting relationships with W.L. Gore & Associates, Edwards Lifesciences, and Abiomed. Dr Zampi has reported consulting relationships with Medtronic and W.L. Gore & Associates. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (© 2024 The Authors.)

Background: Transcatheter tricuspid valve-in-valve (ViV) replacement has yielded good hemodynamic outcomes in the treatment of dysfunctional bioprosthetic valves (BPVs). Intentional fracture of certain rigid BPV frames, if feasible, allows a larger implanted valve when compared with implant into an unfractured BPV. There remains limited data on the feasibility of tricuspid valve frame fracture., Aims: Evaluate the feasibility of transcatheter tricuspid ViV replacement with fracture of the underlying BPV ring., Methods: An international multicenter registry of tricuspid ViV replacement with intentional tricuspid valve frame fracture was created. Demographic data along with procedural characteristics, outcomes, and follow-up data were collected. Comparison was made to the pre- and post-ViV replacement with fracture of the tricuspid valve frame conditions., Results: Ten patients from six centers were included with a median age and weight of 29 years and 67.3 kg respectively. Tricuspid valve frame fracture was performed using a median balloon diameter 3 mm (IQR 3-5) larger than the true inner diameter (ID). The final ID was a mean of 1.5 mm (95% CI: 0.35, 2.64: p < 0.05), and median 1.1 mm (0.5, 2.1) larger than the reported true ID of the surgical BPV after ViV replacement. The mean tricuspid inflow gradient by echocardiogram decreased by 6.65 mmHg (95% CI: 4.14, 9.15: p < 0.001). All procedures were without complication, specifically there was no heart block, pericardial effusion, or right coronary disruption., Conclusion: Intentional tricuspid valve frame fracture with tricuspid ViV replacement is feasible and can increase the valve orifice potentially reducing the risk of ViV patient prosthesis mismatch and is not associated with significant complications., (© 2024 The Author(s). Catheterization and Cardiovascular Interventions published by Wiley Periodicals LLC.)

Background: We describe outcomes and management strategies for single-ventricle and bilaterally discontinuous pulmonary arteries (PAs) originating from bilateral ductus arteriosus., Methods: We reviewed 22 patients with aforementioned anatomy and PA centralization from 1995 to 2023, excluding those with biventricular repair., Results: Median age at centralization was 9 days (minimum-maximum, 0 days-2 years). Centralization was performed with systemic-to-pulmonary shunt (n =20 [91%]; 2 after bilateral ductal stents) or bidirectional cavopulmonary connection (n = 2 [9%]) using pericardial roll (n = 14 [64%]), patch-augmented direct anastomosis (n = 7 [32%]), and interposition graft (n = 1 [5%]) techniques. Concurrent total anomalous pulmonary venous connection (TAPVC, n = 11 [50%]) was associated with significantly inferior survival (P = .01). Five patients (23%) died at a median of 59 days (minimum-maximum, 6-257 days) after centralization, all with noncardiac TAPVC. At the latest follow-up for 17 survivors (median, 13.5 years; minimum-maximum, 0.5-25.1 years after centralization), 12 completed Fontan, 4 completed second-stage palliation, and 1 received a transplant before second-stage palliation. PA reintervention was required in 14 patients (64%), including 3 with reoperations independent of staged palliation. Echocardiography from baseline to before the second stage demonstrated branch PA growth with significantly increased diameters (left, P = .0006; right, P = .0002); z-scores significantly increased for right (P = .004) but not left (P = .11)., Conclusions: Successful single-ventricle palliation is possible, although high risk, for patients with bilateral discontinuous ductal PAs. Early postcentralization mortality remains substantial, particularly with associated noncardiac TAPVC. Many require reintervention to maintain PA growth, typically concurrently with staged palliation., Competing Interests: Disclosures Athar M. Qureshi reports a relationship with W. L. Gore & Associates, Inc. that includes: consulting or advisory; with Medtronic PLC that includes: consulting or advisory; and with B. Braun Medical Inc that includes: consulting or advisory. The other authors have no conflicts of interest to disclose., (Copyright © 2024 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objective: To test the hypothesis that neonates with symptomatic tetralogy of Fallot (TOF) and absent ductus arteriosus (ADA) have worse clinical outcomes compared with those with a ductus arteriosus (DA), and that this difference is driven by those born with ADA and with critically deficient pulmonary blood flow (CDPBF)., Study Design: We performed a retrospective, multicenter cohort study of neonates who underwent intervention for symptomatic TOF comparing death and reintervention between subjects with and without a DA identified on fetal echocardiogram or on echocardiogram performed in the first postnatal day. Exclusion criteria were as follows: inability to define DA status, collaterals supplying pulmonary blood flow, atrioventricular septal defect, and absent pulmonary valve. We defined CDPBF as undergoing a procedure to augment pulmonary blood flow on the date of birth or extracorporeal membrane oxygenation prior to such a procedure., Results: The study cohort included 519 patients, among whom 11% had ADA. Patients with ADA were more likely to have a genetic syndrome and had smaller branch pulmonary artery size. In analyses adjusting for center, interventional treatment strategy, genetic syndrome, and minimum branch pulmonary artery size, ADA was associated with higher mortality risk (adjusted hazard ratio of 2.37 (95% CI: 1.07,5.27; P = .034). Seven patients had CDPBF (1.3% of the entire cohort and 12% of patients with ADA)., Conclusions: A minority of symptomatic TOF neonates have ADA, which is associated with higher adjusted mortality risk compared with those with a DA. CDPBF appears to be a rare but important entity in this population., Competing Interests: Declaration of Competing Interest This research is supported, in part, by donations to the Congenital Cardiac Research Collaborative from the Kennedy Hammill Pediatric Cardiac Research Fund, Liam Sexton Foundation, and A Heart Like Ava. The authors declare no conflicts of interest., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: The Alterra adaptive prestent is a novel self-expanding device designed to provide a landing zone for the 29 mm SAPIEN 3 valve to treat pulmonary regurgitation in patients with a right ventricular outflow tract that is too large for a balloon expandable valve alone. The mechanism of fixation for the Alterra prestent is radial force from the self-expanding stent frame, combined with a unique set of flared "tines" that protrude from both ends of the stent., Aims, Methods, and Results: In this report, we describe 6 patients who underwent uncomplicated transcatheter pulmonary valve replacement with an Alterra adaptive prestent and SAPIEN 3 valve and had surveillance chest computed tomography (CT) scans performed 1 day to 21 months after implant. In each patient, the CT scan demonstrated extravascular extension of a portion of the Alterra prestent, without clinical sequelae, but with extension into the ascending aorta in 1 patient and contact with the ascending aorta, left pulmonary vein, or left atrial appendage in 3 others., Conclusions: Surveillance CT imaging shows that the Alterra prestent can perforate the pulmonary artery and/or right ventricle. Although no sequelae were seen in these patients, prestent perforation has the potential to be clinically important. Implanters should be aware of this finding and its potential implications. As experience with the Alterra prestent grows, it will be important to further define the risk factors, incidence, and implications of this phenomenon., (© 2024 Wiley Periodicals LLC.)

With the improvement in the detection of congenital heart disease in fetal life, fetal cardiac interventions are pushing the envelope in hopes of either altering the natural history of disease or improving survival in certain high-risk lesions. These interventions include fetal aortic valvuloplasty for evolving hypoplastic left heart syndrome, fetal atrial septoplasty with or without atrial septal stenting for hypoplastic left heart syndrome and variants with intact or severely restrictive atrial septum, and fetal pulmonary valvuloplasty for severe pulmonary stenosis or pulmonary atresia with intact ventricular septum. This review discusses their indications, technical aspects, and outcomes based on available literature., Competing Interests: Disclosure A.M. Qureshi: Consultant for W.L. Gore and Associates, Edwards Lifesciences, Medtronic Inc, B. Braun and Abiomed Inc, (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: A bioprosthetic valve is recommended for women of childbearing age who require cardiac valve replacement in order to minimize the risk of blood clot formation. However, it should be noted that compared to mechanical valves, bioprosthetic valves have a shorter lifespan and a higher likelihood of requiring reoperation during follow-up. To assess the long-term postoperative results, including the incidence of structural valve deterioration (SVD) and other clinical outcomes, in female patients aged 50 years and younger who underwent BalMedic bovine pericardial bioprosthetic valve replacement, a multicenter retrospective study was implemented in China., Methods: Between 2004 and 2015, a cohort of 86 female patients across three medical centers underwent the implantation of 97 bioprosthetic valves. The primary outcome measure was overall survival (OS), while the secondary outcome measures were preliminary evidence of reoperation, SVD incidence, and bioprosthetic valve-related complications., Results: In this cohort study, 21 patients (24.4%, 21/86) died, while 37 patients (43.0%, 37/86) underwent a second valve replacement. The OS rates at 5 and 10 years were 97.56% and 71.93%, respectively. Additionally, the reoperation-free rates at 5 and 10 years were 92.83% and 80.68%, respectively. Similarly, the rates of freedom from SVD at 5 and 10 years were 95.65% and 51.82%, respectively, and the average duration of bioprosthetic valve replacement in our study was 9.34±3.31 years., Conclusions: Despite the recruitment of younger female patients of child-bearing age in our cohort, the OS, reoperation-free survival, and SVD-free rates of the BalMedic bovine pericardial bioprosthetic valve were not inferior to those of the other age groups in the study or those reported in the literature., Competing Interests: Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://jtd.amegroups.com/article/view/10.21037/jtd-24-441/coif). A.M.Q. is a consultant for W. L. Gore and Associates, Medtronic Inc. and B. Braun, and has received consulting fees and support for attending meetings from them, not related to the submitted article. The other authors have no conflicts of interest to declare., (2024 Journal of Thoracic Disease. All rights reserved.)

New 2-thioxopyrimidinone derivatives ( A1 - A10 ) were synthesized in 87-96% yields via a simple three-component condensation reaction. These compounds were screened extensively through in vitro assays for antioxidant and antibacterial investigations. The DPPH assays resulted in the excellent potency of A6 - A10 as antioxidants with IC 50 values of 0.83 ± 0.125, 0.90 ± 0.77, 0.36 ± 0.063, 1.4 ± 0.07, and 1.18 ± 0.06 mg/mL, which were much better than 1.79 ± 0.045 mg/mL for the reference ascorbic acid. These compounds exhibited better antibacterial potency against Klebsiella with IC 50 values of 2 ± 7, 1.32 ± 8.9, 1.19 ± 11, 1.1 ± 12, and 1.16 ± 11 mg/mL for A6 - A10 . High-throughput screenings (HTS) of these motifs were carried out including investigation of drug-like behaviors, physiochemical property evaluation, and structure-related studies involving DFT and metabolic transformation trends. The radical scavenging ability of the synthesized motifs was validated through molecular docking studies through ligand-protein binding against human inducible nitric oxide synthase (HINOS) PDB ID: 4NOS, and the results were promising. Furthermore, the antiviral capability of the compounds was examined by in silico studies using two viral proteins PDB ID: 6Y84 and PDB ID: 6LU7. Binding poses of ligands were discussed, and amino acids in the protein binding pockets were investigated, where the tested compounds showed much better binding affinities than the standard inhibitors, proving to be suitable leads for antiviral drug discovery. The stabilities of the molecular docked complexes in real systems were validated by molecular dynamics simulations., Competing Interests: The authors declare no competing financial interest., (© 2024 The Authors. Published by American Chemical Society.)

Left ventricular non-compaction cardiomyopathy (LVNC), or non-compaction cardiomyopathy (NCCM), is defined by pronounced left ventricular trabeculations and deep intertrabecular recesses connecting with the ventricular cavity. Patients with NCCM can be asymptomatic or have severe complications, including heart failure, arrhythmias, thromboembolism, and sudden cardiac death. Our case discusses a patient with shortness of breath who was found to have a newly decreased ejection fraction. The workup revealed non-ischemic cardiomyopathy and cardiac MRI showed hyper-trabeculations consistent with NCCM. The patient was started on oral anticoagulation and guideline-directed medical therapy (GDMT) and discharged with an event monitor. NCCM stands as a relatively rare and enigmatic condition, often veiled in ambiguity. The absence of standardized diagnostic and management protocols further complicates its clinical landscape. While echocardiography is the primary diagnostic tool, its tendency for under-diagnosis poses a significant challenge. Conversely, advanced imaging modalities like cardiac MRI may lead to instances of overdiagnosis. Treatment approaches are non-specific, incorporating GDMT, anticoagulation, implantable cardioverter-defibrillator placement, and genetic testing paired with counseling. Prioritizing genetic research is crucial to uncover tailored therapeutic interventions. Establishing consensus guidelines and refining diagnostic accuracy are pivotal steps toward mitigating the risks associated with under and over-diagnosis., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, Bilal et al.)

Background: Percutaneous pulmonary valve implantation (PPVI) is a non-surgical treatment for right ventricular outflow tract (RVOT) dysfunction. During PPVI, a stented valve, delivered via catheter, replaces the dysfunctional pulmonary valve. Stent oversizing allows valve anchoring within the RVOT, but overexpansion can intrude on the surrounding structures. Potentially dangerous outcomes include aortic valve insufficiency (AVI) from aortic root (AR) distortion and myocardial ischemia from coronary artery (CA) compression. Currently, risks are evaluated via balloon angioplasty/sizing before stent deployment. Patient-specific finite element (FE) analysis frameworks can improve pre-procedural risk assessment, but current methods require hundreds of hours of high-performance computation., Methods: We created a simplified method to simulate the procedure using patient-specific FE models for accurate, efficient pre-procedural PPVI (using balloon expandable valves) risk assessment. The methodology was tested by retrospectively evaluating the clinical outcome of 12 PPVI candidates., Results: Of 12 patients (median age 14.5 years) with dysfunctional RVOT, 7 had native RVOT and 5 had RV-PA conduits. Seven patients had undergone successful RVOT stent/valve placement, three had significant AVI on balloon testing, one had left CA compression, and one had both AVI and left CA compression. A model-calculated change of more than 20% in lumen diameter of the AR or coronary arteries correctly predicted aortic valve sufficiency and/or CA compression in all the patients., Conclusion: Agreement between FE results and clinical outcomes is excellent. Additionally, these models run in 2-6 min on a desktop computer, demonstrating potential use of FE analysis for pre-procedural risk assessment of PPVI in a clinically relevant timeframe., (© 2024 The Authors. Catheterization and Cardiovascular Interventions published by Wiley Periodicals LLC.)

Background: Anatomic selection for Harmony valve implant is determined with the aid of a screening report and perimeter plot (PP) that depicts the perimeter-derived radius along the right ventricular outflow tract (RVOT) and projects device oversizing. The PP provides an estimation of suitability for implant, but its sensitivity as a screening method is unknown. This study was performed to describe anatomic features and outcomes in patients who underwent Harmony TPV25 implant despite a PP that predicted inadequate oversizing., Methods: We reviewed RVOT anatomic features and measurements in patients who underwent transcatheter pulmonary valve replacement with the Harmony TPV25 device despite a PP that predicted inadequate oversizing., Results: This study included 22 patients. There were no unsuccessful implants or adverse valve-related events. Anatomic features varied, but all patients fit into 1 of 3 anatomic types characterized by differences in RVOT dimensions. Type 1 patients (n=9) had a long RVOT with a choke point and a wide main pulmonary artery. Type 2 patients (n=6) had a short RVOT that was pyramidal in shape, with no choke point, and extensive main pulmonary artery lengthening/expansion during systole. Type 3 patients (n=7) had a short, bulbous main pulmonary artery with a choke point and an open pulmonary artery bifurcation., Conclusions: Transcatheter pulmonary valve replacement with the Harmony valve is feasible in some patients whose PP fit analysis predicts inadequate oversizing. All cases in this series fit into 1 of 3 anatomic patterns, which are not identified in the screening report. Implanters must review cases individually to assess the feasibility of the implant., Competing Interests: Disclosures Drs McElhinney and Aboulhosn worked as Proctor and consultant for Medtronic and Edwards. Dr Gillespie is a consultant and proctor for Medtronic and W.L. Gore and Associates. Dr Cabalka is a Consultant for Medtronic, Edwards, and B. Braun. Dr Morray worked as Proctor and consultant for Medtronic and Abbott. Dr Balzer worked as Proctor and consultant for Medtronic, Edwards, and Abbott. Dr Qureshi worked as proctor and consultant for Medtronic, W.L. Gore and Associates, and B. Braun. Dr Goldstein worked as consultant and Proctor for Medtronic and W.L. Gore & Associates, Consultant for Edwards, Consultant and Advisory Board Member for PECA Labs and Mezzion Pharmaceuticals.

Competing Interests: Funding Support and Author Disclosures Dr Qureshi is a consultant for Medtronic Inc, W.L. Gore and Associates, and B. Braun. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Background: Transcatheter pulmonary valve replacement (TPVR) with the self-expanding Harmony valve (Medtronic) is an emerging treatment for patients with native or surgically repaired right ventricular outflow tract (RVOT) pulmonary regurgitation (PR). Limited data are available since U.S. Food and Drug Administration approval in 2021., Objectives: In this study, the authors sought to evaluate the safety and short-term effectiveness of self-expanding TPVR in a real-world experience., Methods: This was a multicenter registry study of consecutive patients with native RVOT PR who underwent TPVR through April 30, 2022, at 11 U.S., Centers: The primary outcome was a composite of hemodynamic dysfunction (PR greater than mild and RVOT mean gradient >30 mm Hg) and RVOT reintervention., Results: A total of 243 patients underwent TPVR at a median age of 31 years (Q1-Q3: 19-45 years). Cardiac diagnoses were tetralogy of Fallot (71%), valvular pulmonary stenosis (21%), and other (8%). Acute technical success was achieved in all but 1 case. Procedural serious adverse events occurred in 4% of cases, with no device embolization or death. Hospital length of stay was 1 day in 86% of patients. Ventricular arrhythmia prompting treatment occurred in 19% of cases. At a median follow-up of 13 months (Q1-Q3: 8-19 months), 98% of patients had acceptable hemodynamic function. Estimated freedom from the composite clinical outcome was 99% at 1 year and 96% at 2 years. Freedom from TPVR-related endocarditis was 98% at 1 year. Five patients died from COVID-19 (n = 1), unknown causes (n = 2), and bloodstream infection (n = 2)., Conclusions: In this large multicenter real-world experience, short-term clinical and hemodynamic outcomes of self-expanding TPVR therapy were excellent. Ongoing follow-up of this cohort will provide important insights into long-term outcomes., Competing Interests: Funding Support and Author Disclosures Dr Goldstein is a consultant, proctor, and investigator for Medtronic; is a consultant and investigator for Edwards Lifesciences; is a consultant and proctor for W.L. Gore and Associates; is a consultant and advisory board member for Mezzion Pharmaceuticals; and is a consultant for PECA Labs. Dr McElhinney is a consultant and proctor for Medtronic and Edwards Lifesciences. Dr Gillespie is a consultant and proctor for Medtronic and W.L. Gore and Associates. Dr Aboulhosn is a consultant, advisory board member, and proctor for Medtronic; and is a consultant and proctor for Edwards Lifesciences. Dr Levi is a consultant for Medtronic and Edwards Lifesciences. Dr Cabalka is a consultant and investigator for Medtronic and Edwards Lifesciences; and is a consultant for B. Braun. Dr Zampi is a consultant and proctor for Medtronic and W.L. Gore and Associates; and is on the data safety monitoring board for Encore Medical. Dr Balzer is a consultant and proctor for Medtronic, Edwards Lifesciences, and Abbott. Dr Law is a proctor for Medtronic, Edwards Lifesciences, and W.L. Gore and Associates. Dr Qureshi is a consultant and proctor for Medtronic, W.L. Gore and Associates, and B. Braun. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2024 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Pulmonary embolism is increasing in prevalence among pediatric patients; although still rare, it can create a significant risk for morbidity and death within the pediatric patient population. Pulmonary embolism presents in various ways depending on the patient, the size of the embolism, and the comorbidities. Treatment decisions are often driven by the severity of the presentation and hemodynamic effects; severe presentations require more invasive and aggressive treatment. We describe the development and implementation of a pediatric pulmonary embolism response team designed to facilitate rapid, multidisciplinary, data-driven treatment decisions and management., Competing Interests: Financial/Nonfinancial Disclosures None declared., (Copyright © 2023 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.)

Background: The modified Blalock-Taussig-Thomas shunt is the gold standard palliation for securing pulmonary blood flow in infants with ductal-dependent pulmonary blood flow. Recently, the ductus arteriosus stent (DAS) has become a viable alternative., Methods and Results: This was a retrospective multicenter study of neonates ≤30 days undergoing DAS or Blalock-Taussig-Thomas shunt placement between January 1, 2017 and December 31, 2020 at hospitals reporting to the Pediatric Health Information Systems database. We performed generalized linear mixed-effects modeling to evaluate trends in intervention and intercenter variation, propensity score adjustment and inverse probability weighting with linear mixed-effects modeling to analyze length of stay and cost of hospitalization, and generalized linear mixed modeling to analyze differences in 30-day outcomes. There were 1874 subjects (58% male, 61% White) from 45 centers (29% DAS). Odds of DAS increased with time (odds ratio [OR] 1.23, annually, P <0.01 [95% CI, 1.10-1.38]) with significant intercenter variation (median OR, 3.81 [95% CI, 2.74-5.91]). DAS was associated with shorter hospital length of stay (ratio of geometric means, 0.76 [95% CI, 0.63-0.91]), shorter intensive care unit length of stay (ratio of geometric means, 0.77 [95% CI, 0.61-0.97]), and less expensive hospitalization (ratio of geometric means, 0.70 [95% CI, 0.56-0.87]). Intervention was not significantly associated with odds of 30-day transplant-free survival (OR,1.18 [95% CI, 0.70-1.99]) or freedom from catheter reintervention (OR, 1.02 [95% CI, 0.65-1.58]), but DAS was associated with 30-day freedom from composite adverse outcome (OR, 1.51 [95% CI, 1.11-2.05])., Conclusions: Use of DAS is increasing, but there is variability across centers. Though odds of transplant-free survival and reintervention were not significantly different after DAS, and DAS was associated with shorter length of stay and lower in-hospital costs.

Background: Fontan baffle punctures and creation of Fontan fenestration for cardiac catheterisation procedures remain challenging especially due to the heavy calcification of prosthetic material and complex anatomy., Objectives: We sought to evaluate our experience using radiofrequency current via surgical electrocautery needle for Fontan baffle puncture to facilitate diagnostic, electrophysiology, and interventional procedures., Methods: A retrospective chart review of all Fontan patients (pts) who underwent Fontan baffle puncture using radiofrequency energy via surgical electrocautery from three centres were performed from January 2011 to July 2021., Results: A total of 19 pts underwent 22 successful Fontan baffle puncture. The median age and weight were 17 (3-36 years) and 55 (14-88) kg, respectively. The procedural indications for Fontan fenestration creation included: diagnostic study (n = 1), atrial septostomy and stenting (n = 1), electrophysiology study and ablation procedures (n = 8), Fontan baffle stenting for Fontan failure including protein-losing enteropathy (n = 7), and occlusion of veno-venous collaterals (n = 2) for cyanosis. The type of Fontan baffles included: extra-cardiac conduits (n = 12), lateral tunnel (n = 5), classic atrio-pulmonary connection (n = 1), and intra-cardiac baffle (n = 1). A Fontan baffle puncture was initially attempted using traditional method in 6 pts and Baylis radiofrequency trans-septal system in 2 pts unsuccessfully. In all pts, Fontan baffle puncture using radiofrequency energy via electrocautery needle was successful. The radiofrequency energy utilised was (10-50 W) and required 1-5 attempts for 2-5 seconds. There were no vascular or neurological complications., Conclusions: Radiofrequency current delivery using surgical electrocautery facilitates Fontan baffle puncture in patients with complex and calcified Fontan baffles for diagnostic, interventional, and electrophysiology procedures.

Objective: We report the largest pediatric single-center experience with an Impella (Abiomed Inc) catheter-based axial pump support., Methods: We conducted a retrospective cohort study of all patients with acute decompensated heart failure or cardiogenic shock requiring catheter-based axial pump support between October 2014 and February 2022. The primary outcome per individual encounter (hospital admission) was defined as bridge-to-recovery, bridge-to-durable ventricular assist device support, bridge-to-cardiac transplantation, or death at 6 months after catheter-based axial pump explantation. Adverse events were defined according to the Pediatric Interagency Registry for Mechanical Circulatory Support criteria., Results: Our final study cohort included 37 encounters with 43 catheter-based axial pump implantations. A single catheter-based axial pump device was used for support in 33 encounters (89%), with 2 catheter-based axial pump devices used in 3 (8%) separate encounters and 3 catheter-based axial pump devices used in 1 (3%) encounter. The median [range] age, weight, and body surface area at implantation were 16.8 [6.9-42.8] years, 61.1 [23.1-123.8] kg, and 1.7 [0.8-2.5] m 2 , respectively. The predominant causes of circulatory failure were graft failure/rejection in 16 patients (43%), followed by cardiomyopathy in 7 patients (19%), arrhythmia refractory to medical therapies in 6 patients (16%), myocarditis/endocarditis in 4 patients (11%), and heart failure due to congenital heart disease in 4 patients (11%). Competing outcomes analysis showed a positive outcome with bridge-to-recovery in 58%, bridge-to-durable VAD support in 14%, and bridge-to-cardiac transplantation in 14% at 6 months. Fourteen percent of encounters resulted in death at 6 months., Conclusions: We demonstrate that catheter-based axial pump support in children results in excellent 1- and 6-month survival with an acceptable adverse event profile., (Copyright © 2022. Published by Elsevier Inc.)

Background: Congenital heart disease (CHD) is one of the most common birth defects and consumes a substantial amount of health care resources. CHD leads to heavy economic burdens for families. However, there are limited data regarding the utilization of healthcare resources for CHD. The objectives of this study were to evaluate the composition, changing trends, and factors affecting hospitalization costs for patients with CHD in the western highlands area of China over a 10-year period., Methods: We conducted a study using the International Quality Improvement Collaborative for Congenital Heart Surgery (IQIC) database and information management system of The First Hospital of Lanzhou University between January 2010 and December 2019., Results: Among 3,087 patients hospitalized for CHD surgery, annual CHD hospitalization costs saw an increasing trend over the 10-year period, with an average growth rate of 4.6% per year. The major contributors to the hospitalization costs were surgery, surgical material, and drug costs. Length of stay (β=0.203; 0.379; 0.474, P<0.01), age at hospitalization (β=0.293, P<0.01), proportion of surgery (β=0.090; -0.102; -0.122; -0.110, P<0.01) and drug costs (β=-0.114; -0.147; -0.069, P<0.01), and use of traditional Chinese medicine (β=0.141, P<0.01) were independent factors affecting average hospitalization costs., Conclusions: The financial burden of patients with CHD in the Chinese western highland region is high. Independent of inflation, CHD hospitalization costs are increasing. Measures taken by medical institutions to control the increase in drug costs, and to shorten the length of stay may be expected to have positive effects on reducing the financial burden of individuals with CHD and their families., Competing Interests: Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://jtd.amegroups.com/article/view/10.21037/jtd-23-1246/coif). A.M.Q. is a proctor and consultant for W.L. Gore and Associates and Medtronic Inc., outside the submitted work. The other authors have no conflicts of interest to declare., (2023 Journal of Thoracic Disease. All rights reserved.)

Competing Interests: Funding Support and Author Disclosures Dr Stapleton is a proctor for Edwards Lifesciences; and is a consultant for Medtronic. Dr Qureshi is a consultant for W.L. Gore, Abiomed Inc. and Associates, Edwards Lifesciences, and Medtronic. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Background: Anomalous aortic origin of the left coronary artery with intraseptal course is a rare coronary anomaly associated with an increased risk of myocardial ischaemia. The role and techniques for surgical intervention are evolving, with numerous novel surgical techniques for this challenging anatomy reported in the last 5 years. We report our single-centre experience with surgical repair of intraseptal anomalous left coronary artery in the paediatric population, including clinical presentation, evaluation, and short- to mid-term outcomes., Methods: All patients with coronary anomalies presenting to our institution undergo standardised clinical evaluation. Five patients aged 4 to 17 years underwent surgical intervention for intraseptal anomalous aortic origin of the left coronary artery between 2012 and 2022. Surgical techniques included coronary artery bypass grafting (n = 1), direct reimplantation with limited supra-arterial myotomy via right ventriculotomy (n = 1), and transconal supra-arterial myotomy with right ventricular outflow tract patch reconstruction (n = 3)., Results: All patients had evidence of haemodynamically significant coronary compression, and three had evidence of inducible myocardial ischaemia pre-operatively. There were no deaths or major complications. Median follow-up was 6.1 months (range 3.1-33.4 months). Patients who underwent supra-arterial myotomy (with or without reimplantation) had improved coronary flow and perfusion based on stress imaging and catheterisation data., Conclusions: Surgical approaches to intraseptal anomalous left coronary artery with evidence of myocardial ischaemia continue to evolve, with new techniques demonstrating promising improvement in coronary perfusion. Further studies are warranted to determine long-term outcomes and refine indications for repair.

Objectives: To determine the safety and feasibility of over-expansion of right ventricle to pulmonary artery conduits during transcatheter pulmonary valve placement., Background: Transcatheter pulmonary valve placement is an alternative to surgical pulmonary valve replacement. Traditionally, it was thought to be unsafe to expand a conduit to >110% of its original size., Methods: This retrospective cohort study from two centers includes patients with right ventricle to pulmonary artery conduits with attempted transcatheter pulmonary valve placement from 2010 to 2017. Demographic, procedural, echocardiographic and follow-up data, and complications were evaluated in control and overdilation (to >110% original conduit size) groups., Results: One hundred and seventy-two patients (51 overdilation and 121 control) had attempted transcatheter pulmonary valve placement (98% successful). The overdilation group was younger (11.2 versus 16.7 years, p < 0.001) with smaller conduits (15 versus 22 mm, p < 0.001); however, the final valve size was not significantly different (19.7 versus 20.2 mm, p = 0.2). Baseline peak echocardiographic gradient was no different (51.8 versus 55.6 mmHg, p = 0.3). Procedural complications were more frequent in overdilation (18%) than control (7%) groups (most successfully addressed during the procedure). One patient from each group required urgent surgical intervention, with no procedural mortality. Follow-up echocardiographic peak gradients were similar (24.1 versus 26 mmHg, p = 0.5)., Conclusions: Over-expansion of right ventricle to pulmonary artery conduits during transcatheter pulmonary valve placement can be performed successfully. Procedural complications are more frequent with conduit overdilation, but there was no difference in the rate of life-threatening complications. There was no difference in valve function at most recent follow-up, and no difference in rate of reintervention. The long-term outcomes of transcatheter pulmonary valve placement with conduit over-expansion requires further study.

A previously healthy nine-year-old boy with anomalous aortic origin of the left coronary artery (AAOLCA) with high-risk anatomy demonstrated negative stress on magnetic resonance imaging. Invasive cardiac catheterization for intracoronary flow measurement was performed and demonstrated compromised coronary flow during pharmacologic stress and significant stenosis on angiography. The patient underwent surgical intervention with normalization of coronary flow upon postoperative evaluation. Invasive intracoronary flow determination with angiography under provocative stress is emerging as a critical data point for risk stratification and management decision-making in high-risk AAOLCA patients with negative noninvasive perfusion studies., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: The data on the use of Gore Cardioform Septal Occluder (GCA; W. L. Gore and Associates, Inc.) for atrial septal defect (ASD) with deficient rims is limited., Methods: All patients evaluated by transesophageal echocardiogram (TEE) for ASD occlusion were included. TEE planes at 35°, 0°, and 90° were assessed for anterior-superior (AS) and posterior (P), anterior-inferior (AI) and posterior-superior (PS), as well as superior (S) and inferior (I) rims. ASD size >20 mm, and rims less than 5 mm were defined as large and deficient, respectively. We included patients who had a procedural failure along with the patients in whom the procedure was not attempted after echocardiogram in the unsuccessful group., Results: In 148 patients, the median weight, age, and ASD size were 36 kg (range, 8-60 kg), 11.8 years (range, 1-60 years), and 14.2 ± 8.28 mm, respectively. One or more deficient rims were noted in 112 of 148 (75.7%): 99 (67%) AS, 36 (24%) P, 17 (11%) AI, 30 (20%) PS, 26 (18%) S, and 33 (22%) I. ASD closure was performed in 115 (78%) patients. The procedure was successful in 111 (96.5%) patients with procedural failure in 4 (3.4%) patients. Multiple deficient rims were associated with reduced procedural success (OR 0.36, 95% CI, 0.25-0.56). On multivariate analysis deficient P, PS, and I rims were associated with an unsuccessful group (P = .001, .046, and .005, respectively). Complications included 1 device embolization, 1 vascular injury, and 5 arrhythmias., Conclusions: Transcatheter closure of ASDs with deficient rims is feasible using GCA. Large ASDs with deficient P, PS, and I rims were associated with unsuccessful closure. Risk stratification and comprehensive evaluation of ASD rims is vital for the use of GCA.