Long-Term Results of the Atrial Septal Defect Occluder ASSURED Trial for Combined Pivotal/Continued Access Cohorts.

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).

Health-Related Quality of Life After Neonatal Treatment of Symptomatic Tetralogy of Fallot: Insights from the Congenital Cardiac Research Collaborative.

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.

Cumulative Radiation Exposure and Lifetime Cancer Risk in Patients With Tetralogy of Fallot Requiring Early Intervention.

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.

Hepatic and splenic venous access tract closure using the VASCADE vascular closure system following percutaneous intervention in patients with CHD.

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.

Feasibility of intentional bioprosthetic valve fracture in the tricuspid position.

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 replatement 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.

Long-term outcomes of the BalMedic bovine pericardial bioprosthetic valve in female patients ≤50 years: a multicenter retrospective study.

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.

Extravascular protrusion of the Alterra adaptive prestent identified on surveillance computed tomography imaging.

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.

Current Understanding of Indications, Technical Aspects and Outcomes of Fetal Cardiac Interventions.

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.

Managing Bilateral Discontinuous Pulmonary Arteries of Ductal Origin in Single-Ventricle Anatomy.

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.

Outcomes of Neonates Born with Symptomatic Tetralogy of Fallot and Absent Ductus Arteriosus.

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.

Hybrid Interventions for Pulmonary Vein Stenosis: Leveraging Intraoperative Endovascular Adjuncts in Challenging Clinical Scenarios.

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.

Transcatheter Pulmonary Valve Replacement With the Harmony Valve in Patients Who Do Not Meet Recommended Oversizing Criteria on the Screening Perimeter Plot.

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.

Finite element modeling with patient-specific geometry to assess clinical risks of percutaneous pulmonary valve implantation.

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.

Early Outcomes From a Multicenter Transcatheter Self-Expanding Pulmonary Valve Replacement Registry.

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.

Design, Synthesis, and Evaluation of Novel Magnetic Nanoparticles Combined with Thiophene Derivatives for the Removal of Cr(VI) from an Aqueous Solution.

Most heavy metals are harmful to human health and the environment, even at extremely low concentrations. In natural waters, they are usually found only in trace amounts. Researchers are paying great attention to nanotechnology and nanomaterials as viable solutions to the problem of water pollution. This research focuses on the synthesis of organic thiophene derivatives that can be used as grafted ligands on the surface of silica-coated iron oxide nanoparticles to remove Cr(VI) chromium ions from water. The Vilsmeier-Haack reaction allows the formation of aldehyde groups in thiophene derivatives, and the resulting products were characterized by the FT-IR, NMR, and GC-MS. Schiff base is used as a binder between organic compounds and nanoparticles by the reaction of aldehyde groups in thiophene derivatives and amine groups on the surface of coated iron oxide nanoparticles. Schiff base functionalized Fe3O4 composites (MNPs@SiO2-SB-THCA) and (MNPs@SiO2-SB-THCTA) were successfully synthesized by homogeneous and heterogeneous methods and characterized by a combination of FT-IR, transmission electron microscopy, X-ray photoelectron spectroscopy, and thermogravimetric analysis. The adsorption studies, kinetic modeling, adsorption isotherms, and thermodynamics of the two materials, MNPs@SiO2-SB-THCA and MNPs@SiO2-SB-THCTA, were investigated for the removal of Cr(VI) from water at room temperature and at 50 mg/L. The high adsorption capacity at pH 6 for MNPs@SiO2-SB-THCTA was 15.53 mg/g, and for MNPs@SiO2-SB-THCA, it was 14.31 mg/g.

Myocardial bridges in a pediatric population: Outcomes following a standardized approach.

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.

Implementing a Pediatric Pulmonary Embolism Response Team Model: An Institutional Experience.

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.

Ceftriaxone-induced Kounis syndrome: A case report and review of the literature.

Kounis syndrome is defined as cardiovascular symptoms that occur secondary to allergic or hypersensitivity insults, and is also called allergic angina and allergic myocardial infarction. We report a case of pre-operative ceftriaxone-induced Kounis syndrome with no evident dermatological manifestation, and describe our diagnostic dilemma. The patient was symptomatically managed and discharged in stable condition with a warning against future use of ceftriaxone.