Safety and efficacy of combined dilation/stenting of venous abnormalities, including complete obstructions, during lead extractions in patients with congenital heart disease.

INTRODUCTION: Management of transvenous leads in patients with congenital heart disease (CHD) can be complicated by venous obstructions and residual shunts. We present our experience performing concurrent lead extraction and dilation/stenting of venous pathways, including patients with complete venous obstruction. METHODS: All cases of concurrent lead extraction and recanalization of vena cavae/baffles between 2017 and 2021 at Boston Children's Hospital were retrospectively included and reviewed for safety and efficacy. RESULTS: Eight patients, 4 female, median 38.5 years of age (range 16.7-49 years) and 81.6 kg weight (range 41.3-97.8 kg) at time of procedure were included. All patients had CHD, a majority (n = 7) having transposition of the great arteries palliated via atrial switch. All leads were removed in their entirety, with most patients having two leads extracted (n = 7). Median lead dwell time was 13.8 years (range 3.6-35.3 years). Three patients had complete obstructions, three required stenting of their innominate veins and three required recanalization of their femoral vessels. Median procedure time was 9.8 h (range 5.4-12.8 h). Complications included blood transfusion (n = 2), arrhythmia (n = 3), pleural effusion (n = 1), and pressure ulcer (n = 1). There were no cardiac perforations, venous tears, or deaths. CONCLUSION: Lead extraction along with dilation and stenting of venous anomalies, though long in duration, proved effective with minimal complications. This combined procedure can safely and effectively resolve complete obstructions secondary to transvenous leads.

Transcatheter pulmonary valve replacement in surgically-created double-barrel right ventricular outflow tracts: A single center case-series.

This manuscript describes the feasibility and approach to the assessment and performance of transcatheter pulmonary valve replacement (tPVR) in patients with surgically-created "double-barrel" right ventricular outflow tracts (RVOT). Patients with tetralogy of Fallot may have coronary anomalies which prohibit the performance of traditional tetralogy of Fallot repair. In certain cases, this may necessitate the placement of a right ventricle to pulmonary artery conduit in addition to the native RVOT, which is left in situ, creating so-called "double-barrel" RVOTs. When these patients develop RVOT dysfunction later in life, they would typically be referred for reoperation due to concerns for risk of coronary compression associated with a transcatheter approach. However, whether a transcatheter approach with valve replacement in the native RVOT is feasible or safe is unknown. This was a retrospective review of patients with a surgically created "double-barrel" RVOTs who underwent cardiac catheterization for assessment of tPVR at Boston Children's Hospital. From July 2012 to July 2022, there were four patients with "double-barrel" RVOTs who underwent assessment for tPVR. The age at catheterization ranged between 22 and 39 years. In three out of four patients, coronary compression testing was negative. These three patients had successful tPVR in the native RVOT. At follow up, all three patients were free of greater than mild regurgitation by echocardiogram and had a maximum instantaneous gradient across the RVOT ranging between 20 and 33 mmHg. Performance of tPVR in patients with surgically created "double-barrel" RVOTs is feasible. The safety of this procedure depends crucially on coronary artery assessment at all stages.

Car T Cells in Solid Tumors: Overcoming Obstacles.

Chimeric antigen receptor T cell (CAR T cell) therapy has emerged as a prominent adoptive cell therapy and a therapeutic approach of great interest in the fight against cancer. This approach has shown notorious efficacy in refractory hematological neoplasm, which has bolstered its exploration in the field of solid cancers. However, successfully managing solid tumors presents considerable intrinsic challenges, which include the necessity of guiding the modified cells toward the tumoral region, assuring their penetration and survival in adverse microenvironments, and addressing the complexity of identifying the specific antigens for each type of cancer. This review focuses on outlining the challenges faced by CAR T cell therapy when used in the treatment of solid tumors, as well as presenting optimizations and emergent approaches directed at improving its efficacy in this particular context. From precise localization to the modulation of the tumoral microenvironment and the adaptation of antigen recognition strategies, diverse pathways will be examined to overcome the current limitations and buttress the therapeutic potential of CAR T cells in the fight against solid tumors.

Quantum Approximate Optimization Algorithm Pseudo-Boltzmann States.

In this Letter, we provide analytical and numerical evidence that the single-layer quantum approximate optimization algorithm on universal Ising spin models produces thermal-like states. We find that these pseudo-Boltzmann states can not be efficiently simulated on classical computers according to the general state-of-the-art condition that ensures rapid mixing for Ising models. Moreover, we observe that the temperature depends on a hidden universal correlation between the energy of a state and the covariance of other energy levels and the Hamming distances of the state to those energies.

Predictors of Serious Adverse Events and High-Level Cardiorespiratory Support in Patients Undergoing Transcatheter Pulmonary Vein Interventions.

Patients with pulmonary vein stenosis (PVS) often require frequent transcatheter pulmonary vein (PV) interventions for management of restenosis. Predictors of serious adverse events (AEs) and need for high-level cardiorespiratory support (mechanical ventilation, vasoactive support, and/or extracorporeal membrane oxygenation) 48 h after transcatheter PV interventions have not been reported. This is a single-center retrospective cohort analysis of patients with PVS who underwent transcatheter PV interventions from 3/1/2014 to 12/31/2021. Univariate and multivariable analyses were performed using generalized estimating equations to account for within-patient correlation. 240 patients underwent 841 catheterizations involving PV interventions (median 2 catheterizations per patient [1,3]). At least one serious AE was reported in 100 (12%) cases, the most common of which were pulmonary hemorrhage (n = 20) and arrhythmia (n = 17). There were 14 severe/catastrophic AEs (1.7% of cases) including three strokes and one patient death. On multivariable analysis, age less than 6 months, low systemic arterial saturation (< 95% in patients with biventricular [BiV] physiology, < 78% in single ventricle [SV] physiology), and severely elevated mean PA pressure (≥ 45 mmHg in BiV, ≥ 17 mmHg in SV) were associated with SAEs. Age less than 1 year, hospitalization prior to catheterization, and moderate-severe RV dysfunction were associated with high-level support after catheterization. Serious AEs during transcatheter PV interventions in patients with PVS are common, although major events such as stroke or death are uncommon. Younger patients and those with abnormal hemodynamics are more likely to experience serious AEs and require high-level cardiorespiratory support after catheterization.

Pre-Glenn aorto-pulmonary collaterals in single-ventricle patients.

BACKGROUND: In single-ventricle patients undergoing staged-bidirectional Glenn, 36-59% have aorto-pulmonary collateral flow, but risk factors and clinical outcomes are unknown. We hypothesise that shunt type and catheter haemodynamics may predict pre-bidirectional Glenn aorto-pulmonary collateral burden, which may predict death/transplantation, pulmonary artery or aorto-pulmonary collateral intervention. METHODS: Retrospective cohort study of patients undergoing a Norwood procedure for single-ventricle anatomy. Covariates included clinical and haemodynamic characteristics up to/including pre-bidirectional Glenn catheterisation and aorto-pulmonary collateral burden at pre-bidirectional Glenn catheterisation. Multivariable models used to evaluate relationships between risk factors and outcomes. RESULTS: From January 2011 to March 2016, 104 patients underwent Norwood intervention. Male sex (odds ratio 3.36, 95% confidence interval 1.17-11.4), age at pre-bidirectional Glenn assessment (2.12, 1.33-3.39 per month), and pulmonary to systemic flow ratio (1.23, 1.08-1.41 per 0.1 unit) were associated with aorto-pulmonary collateral burden. Aorto-pulmonary collateral burden was not associated with death/transplantation (hazard ratio 1.19, 95% confidence interval 0.37-3.85), pulmonary artery (sub-hazard ratio 1.38, 0.32-2.61), or aorto-pulmonary collateral interventions (sub-hazard ratio 1.11, 0.21-5.76). Longer post-Norwood length of stay was associated with greater risk of death/transplantation (hazard ratio 1.22 per week, 95% confidence interval 1.08-1.38), but lower risk of aorto-pulmonary collateral intervention (sub-hazard ratio 0.86 per week, 95% confidence interval 0.75-0.98). Time to pre-bidirectional Glenn catheterisation was associated with lower risk of pulmonary artery (sub-hazard ratio 0.80 per month, 95% confidence interval 0.65-0.98) and aorto-pulmonary collateral intervention (sub-hazard ratio 0.79, 0.63-0.99). Probability of moderate/severe aorto-pulmonary collateral burden increased with left-to-right shunt (22.5% at <1.0, 57.6% at >1.4) and the age at pre-bidirectional Glenn catheterisation (10.6% at <2 months, 56.9% at >5 months). CONCLUSIONS: Aorto-pulmonary collateral burden is common after Norwood procedure and increases as age at bidirectional Glenn increases. As expected, higher pulmonary to systemic flow ratio is a marker for greater aorto-pulmonary collateral burden pre-bi-directional Glenn; aorto-pulmonary collateral burden does not confer risk of death/transplantation or pulmonary artery intervention.

Hemodynamic and anatomic changes after fetal aortic valvuloplasty are associated with procedural success and postnatal biventricular circulation.

BACKGROUND: There are minimal data characterizing the trajectory of left heart growth and hemodynamics following fetal aortic valvuloplasty (FAV). METHODS: This retrospective study included patients who underwent FAV between 2000 and 2019, with echocardiograms performed pre-FAV, immediately post-FAV, and in late gestation. RESULTS: Of 118 fetuses undergoing FAV, 106 (90%) underwent technically successful FAV, of which 55 (52%) had biventricular circulation. Technically successful FAV was associated with improved aortic valve growth (p < 0.001), sustained antegrade aortic arch (AoA) flow (p = 0.02), improved mitral valve (MV) inflow pattern (p = 0.002), and favorable patent foramen ovale (PFO) flow pattern (p = 0.004) from pre-FAV to late gestation. Compared to patients with univentricular outcome, patients with biventricular outcome had less decrement in size of the left ventricle (LV) (p < 0.001) and aortic valve (p = 0.005), as well as more physiologic PFO flow (p < 0.001) and antegrade AoA flow (p < 0.001) from pre-FAV to late gestation. In multivariable analysis, echocardiographic predictors of biventricular outcome were less decline in LV end diastolic dimension (p < 0.001), improved PFO flow (p = 0.004), and sustained antegrade AoA flow (p = 0.002) from pre-FAV to late gestation. CONCLUSION: Stabilization of left heart growth and improved hemodynamics following successful FAV through late gestation are associated with postnatal biventricular circulation.

ICU Admission Tool for Congenital Heart Catheterization (iCATCH): A Predictive Model for High Level Post-Catheterization Care and Patient Management.

OBJECTIVES: Currently, there are no prediction tools available to identify patients at risk of needing high-complexity care following cardiac catheterization for congenital heart disease. We sought to develop a method to predict the likelihood a patient will require intensive care level resources following elective cardiac catheterization. DESIGN: Prospective single-center study capturing important patient and procedural characteristics for predicting discharge to the ICU. Characteristics significant at the 0.10 level in the derivation dataset (July 1, 2017 to December 31, 2019) were considered for inclusion in the final multivariable logistic regression model. The model was validated in the testing dataset (January 1, 2020 to December 31, 2020). The novel pre-procedure cardiac status (PCS) feature, collection started in January 2019, was assessed separately in the final model using the 2019 through 2020 dataset. SETTING: Tertiary pediatric heart center. PATIENTS: All elective cases coming from home or non-ICU who underwent a cardiac catheterization from July 2017 to December 2020. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: A total of 2,192 cases were recorded in the derivation dataset, of which 11% of patients ( n = 245) were admitted to the ICU, while 64% ( n = 1,413) were admitted to a medical unit and 24% ( n = 534) were discharged home. In multivariable analysis, the following predictors were identified: 1) weight less than 5 kg and 5-9.9 kg, 2) presence of systemic illness, 3) recent cardiac intervention less than 90 days, and 4) ICU Admission Tool for Congenital Heart Catheterization case type risk categories (1-5), with C -statistics of 0.79 and 0.76 in the derivation and testing cohorts, respectively. The addition of the PCS feature fit into the final model resulted in a C -statistic of 0.79. CONCLUSIONS: The creation of a validated pre-procedural risk prediction model for ICU admission following congenital cardiac catheterization using a large volume, single-center, academic institution will improve resource allocation and prediction of capacity needs for this complex patient population.

Life-Threatening Hemoptysis in a Pediatric Referral Center.

OBJECTIVES: Hemoptysis is uncommon in children, even among the critically ill, with a paucity of epidemiological data to inform clinical decision-making. We describe hemoptysis-associated ICU admissions, including those who were critically ill at hemoptysis onset or who became critically ill as a result of hemoptysis, and identify predictors of mortality. DESIGN: Retrospective cohort study. Demographics, hemoptysis location, and management were collected. Pediatric Logistic Organ Dysfunction-2 score within 24 hours of hemoptysis described illness severity. Primary outcome was inhospital mortality. SETTING: Quaternary pediatric referral center between July 1, 2010, and June 30, 2017. PATIENTS: Medical/surgical (PICU), cardiac ICU, and term neonatal ICU admissions with hemoptysis during or within 24 hours of ICU admission. INTERVENTIONS: No intervention. MEASUREMENTS AND MAIN RESULTS: There were 326 hemoptysis-associated ICU admissions in 300 patients. Most common diagnoses were cardiac (46%), infection (15%), bronchiectasis (10%), and neoplasm (7%). Demographics, interventions, and outcomes differed by diagnostic category. Overall, 79 patients (26%) died inhospital and 109 (36%) had died during follow-up (survivor mean 2.8 ± 1.9 yr). Neoplasm, bronchiectasis, renal dysfunction, inhospital hemoptysis onset, and higher Pediatric Logistic Organ Dysfunction-2 score were independent risk factors for inhospital mortality (p < 0.02). Pharmacotherapy (32%), blood products (29%), computerized tomography angiography (26%), bronchoscopy (44%), and cardiac catheterization (36%) were common. Targeted surgical interventions were rare. Of survivors, 15% were discharged with new respiratory support. Of the deaths, 93 (85%) occurred within 12 months of admission. For patients surviving 12 months, 5-year survival was 87% (95% CI, 78-92) and mortality risk remained only for those with neoplasm (log-rank p = 0.001). CONCLUSIONS: We observed high inhospital mortality from hemoptysis-associated ICU admissions. Mortality was independently associated with hemoptysis onset location, underlying diagnosis, and severity of critical illness at event. Additional mortality was observed in the 12-month posthospital discharge. Future directions include further characterization of this vulnerable population and management recommendations for life-threatening pediatric hemoptysis incorporating underlying disease pathophysiology.

Surgical management of pediatric renovascular hypertension and midaortic syndrome at a single-center multidisciplinary program.

OBJECTIVE: To evaluate the outcomes of various surgical approaches in the treatment of renovascular hypertension and midaortic syndrome (MAS) in children. METHODS: We performed a retrospective medical record review of patients who had undergone surgery for renovascular hypertension from 2010 to 2018 at our center under the care of a multidisciplinary team. The operative interventions included mesenteric artery growth improves circulation (MAGIC), tissue expander-stimulated lengthening of arteries (TESLA), aortic bypass using polytetrafluorethylene, renal artery reimplantation, and autotransplantation. The MAGIC procedure uses the meandering mesenteric artery as a free conduit for aortic bypass. The TESLA procedure is based on lengthening the normal distal aorta and iliac arteries by gradual filling of a retroaortic tissue expander for several weeks, followed by resection of the stenotic aorta and subsequent primary reconstruction. RESULTS: A total of 39 patients were identified, 10 with isolated renal artery stenosis, 26 with MAS, and 3 with systemic inflammatory vasculitis. The median age at presentation and surgery was 6.4 years (range, 0-16.3 years) and 9.3 years (range, 0-9.2 years), respectively. The MAS-associated syndromes included neurofibromatosis type 1 (15.4%) and Williams syndrome (5.1%), although most cases were idiopathic. At surgery, 33.3% had had stage 1 hypertension (HTN), 53.8% stage 2 HTN, and 12.8% normal blood pressure with a median of three antihypertensive medications. Follow-up of 37 patients at a median of 2.5 years demonstrated normal blood pressure in 86.1%, stage 1 HTN in 8.3%, and stage 2 HTN in 5.6%, with a median of one antihypertensive medication for the entire cohort. CONCLUSIONS: The patterns of vascular involvement leading to renovascular hypertension in children are variable and complex, requiring thoughtful multidisciplinary planning and surgical decision-making. The MAGIC and TESLA procedures provide feasible approaches for aortic bypass and reconstruction using autologous tissues and will result in normalization of blood pressure in 85% of children 2.5 years after surgery.

Preliminary findings on the use of intravascular ultrasound in the assessment of pediatric pulmonary vein stenosis.

OBJECTIVE: Determine the feasibility of performing intravascular ultrasound (IVUS) in pediatric pulmonary vein stenosis (PVS) and investigate whether IVUS can delineate the mechanism of PVS. BACKGROUND: The use of IVUS in pediatric patients with PVS has not been reported. METHODS: Retrospective, single center, cohort analysis of all patients who underwent IVUS of pulmonary veins from August 2016 to December 2019. RESULTS: IVUS was performed on 81 pulmonary veins in 50 pediatric patients (median age = 1.7 years [0.9, 3.1], median weight = 8.6 kg [7.3, 11.8]). All veins accepted the IVUS catheter (.014 or .018), with adequate visualization in 88% (71/81) of imaged veins, and improvement in visualization in the more recent period (23/24; 96%). Veins were categorized as having presumed intimal thickening (PIT) with luminal narrowing (n = 36), ostial narrowing without PIT (n = 14), distortion/compression (n = 6), normal (n = 2), and stent with (n = 9) or without in-stent stenosis (n = 4). In veins with at least 6 months of follow up, (re)intervention occurred more commonly in veins with PIT (14/19; 74%) versus veins without PIT (3/13; 23%; p = 0.01). There were no IVUS related adverse events. CONCLUSION: IVUS can be used safely in pediatric patients and can reliably demonstrate vein lumen and wall architecture. With further refinement, IVUS has the potential to differentiate intimal neo-proliferation from other mechanisms of obstruction. The exact role of IVUS in the assessment of pediatric PVS is yet to be determined.

Percutaneous intraluminal downsizing of systemic-to-pulmonary artery shunts: a novel application of the Diabolo stent technique-Case series and description of the technique.

OBJECTIVE: This article aims to describe the feasibility of a novel application of the Diabolo stent technique for percutaneous downsizing of systemic-to-pulmonary artery shunts in patients with pulmonary overcirculation. BACKGROUND: Pulmonary overcirculation can lead to systemic hypoperfusion, impaired systemic oxygen delivery, and hemodynamic instability especially in patients with single-ventricle physiology. Different medical and surgical strategies to treat pulmonary overcirculation have been reported so far, but no percutaneous approach amenable to further adjustment over time has been described. METHODS: Retrospective review of patients who underwent percutaneous intraluminal reduction of a systemic-to-pulmonary artery shunt with a covered stent in a Diabolo "hour-glass" configuration. RESULTS: From July 2018 to February 4, 2019, consecutive patients underwent trans-catheter downsizing of a systemic-to-pulmonary artery shunt. The median age was 3.3 years (range 0.7-5.7 years). All patients had symptomatic pulmonary overflow despite maximal medical therapy. All patients underwent successful implantation of a covered stent in a Diabolo "hour-glass" configuration without any complications. Successful reduction in pulmonary overcirculation was noted in all patients with a decrease of mean pulmonary artery pressure from a median of 29 mmHg (range 25-32 mmHg) to a median of 21 mmHg (range 14-27 mmHg). CONCLUSIONS: Percutaneous intraluminal downsizing of systemic-to-pulmonary artery shunts with a covered stent in a Diabolo configuration is safe and feasible. With this technique, the pulmonary blood flow can be adjusted in a physiologic closed-chest condition and further regulated over time.

The snared wire technique for Sapien valve implantation in the pulmonary position.

OBJECTIVES: Description of the snared wire technique (SWT) to facilitate the delivery of the Sapien valve in pulmonary position, and comparison with standard delivery technique. BACKGROUND: Transcatheter pulmonary valve replacement (TPVR) with the Sapien delivery system has proven to be challenging. Therefore, alternative strategies for facilitating its delivery in this position are needed. METHODS: Retrospective analysis of patients who underwent TPVR with or without the new SWT. The SWT was chosen as an elective strategy when the anatomy was judged to be challenging for TPVR (planned SWT) or as a rescue strategy when a standard delivery failed (rescue SWT). RESULTS: From February 2018 to January 2020, 84 patients underwent TPVR with a Sapien S3 valve using either a standard delivery (n = 63, 75%) or a SWT (n = 21, 25%). Fifteen patients underwent a planned SWT, six patients underwent a rescue SWT after failure of a standard delivery. All planned SWT cases were successful and, compared to the standard delivery group, no significant differences were found in terms of time to valve-deployment, fluoroscopy time, procedure time, or frequency of complications. Rescue SWT cases had longer fluoroscopy time (p = .05), longer time to valve-deployment (p = .0001), and higher frequency of complications (p = .002) including tricuspid valve injury (p = .0004), but allowed the operator to successfully implant the valve into the desired location. CONCLUSIONS: Even in the most challenging anatomies, the SWT represents a feasible and effective alternative strategy for TPVR with the Sapien valve that should be considered when other techniques have failed.

Topological Amplification in Photonic Lattices.

We characterize topological phases in photonic lattices by unveiling a formal equivalence between the singular value decomposition of the non-Hermitian coupling matrix and the diagonalization of an effective Hamiltonian. Our theory reveals a relation between topological insulators and directional amplifiers. We exemplify our ideas with an array of photonic cavities which can be mapped into an AIII topological insulator. We investigate stability properties and prove the existence of stable topologically nontrivial steady-state phases. Finally, we show numerically that the topological amplification process is robust against disorder in the lattice parameters.

Floquet-Engineered Vibrational Dynamics in a Two-Dimensional Array of Trapped Ions.

We demonstrate Floquet engineering in a basic yet scalable 2D architecture of individually trapped and controlled ions. Local parametric modulations of detuned trapping potentials steer the strength of long-range interion couplings and the related Peierls phase of the motional state. In our proof of principle, we initialize large coherent states and tune modulation parameters to control trajectories, directions, and interferences of the phonon flow. Our findings open a new pathway for future Floquet-based trapped-ion quantum simulators targeting correlated topological phenomena and dynamical gauge fields.

Relationship Between Time to Left Atrial Decompression and Outcomes in Patients Receiving Venoarterial Extracorporeal Membrane Oxygenation Support: A Multicenter Pediatric Interventional Cardiology Early-Career Society Study.

OBJECTIVES: To assess the variation in timing of left atrial decompression and its association with clinical outcomes in pediatric patients supported with venoarterial extracorporeal membrane oxygenation across a multicenter cohort. DESIGN: Multicenter retrospective study. SETTING: Eleven pediatric hospitals within the United States. PATIENTS: Patients less than 18 years on venoarterial extracorporeal membrane oxygenation who underwent left atrial decompression from 2004 to 2016. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: A total of 137 patients (median age, 4.7 yr) were included. Cardiomyopathy was the most common diagnosis (47%). Cardiac arrest (39%) and low cardiac output (50%) were the most common extracorporeal membrane oxygenation indications. Median time to left atrial decompression was 6.2 hours (interquartile range, 3.8-17.2 hr) with the optimal cut-point of greater than or equal to 18 hours for late decompression determined by receiver operating characteristic curve. In univariate analysis, late decompression was associated with longer extracorporeal membrane oxygenation duration (median 8.5 vs 5 d; p = 0.02). In multivariable analysis taking into account clinical confounder and center effects, late decompression remained significantly associated with prolonged extracorporeal membrane oxygenation duration (adjusted odds ratio, 4.4; p = 0.002). Late decompression was also associated with longer duration of mechanical ventilation (adjusted odds ratio, 4.8; p = 0.002). Timing of decompression was not associated with in-hospital survival (p = 0.36) or overall survival (p = 0.42) with median follow-up of 3.2 years. CONCLUSIONS: In this multicenter study of pediatric patients receiving venoarterial extracorporeal membrane oxygenation, late left atrial decompression (≥ 18 hr) was associated with longer duration of extracorporeal membrane oxygenation support and mechanical ventilation. Although no survival benefit was demonstrated, the known morbidities associated with prolonged extracorporeal membrane oxygenation use may justify a recommendation for early left atrial decompression.

Three-dimensional transesophageal echocardiography-guided transcatheter closure of multiple mitral paravalvular leaks demonstrating real time avoidance of device-induced valve malfunction.

Transcatheter closure of mitral paravalvular leaks has become an accepted alternative to surgical repair, especially in patients at high risk for reoperation. Device placement using three-dimensional (3D) transesophageal echocardiography (TEE) guidance allows en-face views of the valve and provides detailed information for device sizing and placement. We present a case demonstrating hybrid transapical transcatheter 3D TEE-guided closure of two mitral paravalvular leaks. We demonstrate real time 3D TEE guidance for device sizing and placement, and early real time recognition of mechanical mitral valve leaflet impairment during device deployment.

Elective Extracorporeal Membrane Oxygenation Support for High-Risk Pediatric Cardiac Catheterization.

OBJECTIVE: The utility of extracorporeal membrane oxygenation (ECMO) as an elective support modality for high-risk cardiac procedures is extensively described in adults, but its use in children is limited to isolated reports. The objective of this study was to analyze the outcomes of patients who underwent elective cannulation to ECMO for this purpose. DESIGN: Single-center, retrospective chart review. SETTING: Free-standing pediatric tertiary care center. PARTICIPANTS: Patients who underwent elective cannulation to ECMO for cardiorespiratory support during a high-risk cardiac catheterization procedure. INTERVENTIONS: Elective ECMO cannulation for high-risk percutaneous cardiac interventions or electrophysiology procedures. MEASUREMENTS AND MAIN RESULTS: Survival to discharge was 71.4% compared with 30% for patients who required extracorporeal cardiopulmonary resuscitation in the cardiac catheterization laboratory. The mean duration of cannulation was 137.43 hours (range 27-615 h, median 55 h). There were no major neurologic sequelae, but ECMO circuit thrombosis (57%) was relatively common. CONCLUSION: The use of elective ECMO support for high-risk pediatric cardiac catheterizations can be accomplished safely and may allow for an improved rate of survival with lower rates of severe adverse events compared with extracorporeal cardiopulmonary resuscitation as rescue therapy.

Tissue expander-stimulated lengthening of arteries for the treatment of midaortic syndrome in children.

BACKGROUND: Midaortic syndrome (MAS) is a rare condition characterized by stenosis of the abdominal aorta. Patients with disease refractory to medical management will usually require either endovascular therapy or surgery with use of prosthetic graft material for bypass or patch angioplasty. We report our early experience with a novel approach using a tissue expander (TE) to lengthen the normal native arteries in children with MAS, allowing primary aortic repair without the need for prosthetic graft material. METHODS: We conducted a retrospective review of patients with MAS undergoing the TE-stimulated lengthening of arteries (TESLA) procedure at our institution from 2010 to 2014. Data are presented as mean (range). RESULTS: Five patients aged 4.8 years (3-8 years) underwent the TESLA procedure. Stages of this procedure include the following: stage I, insertion of retroaortic TE; stage II, serial TE injections; and stage III, final repair with excision of aortic stenosis and primary end-to-end aortic anastomosis. Stage II was completed in 4 months (1-9 months) with 12 (7-20) TE injections. Goal lengthening was achieved in all patients. Stage III could not be completed in one patient because of extreme aortic inflammation, which precluded safe excision of the aortic stenosis and required use of a prosthetic bypass graft. The other four patients completed stage III with two (one to three) additional vessels also requiring reconstruction (renal or mesenteric arteries). At 3.2 years (1-6 years) of follow-up, all patients are doing well. CONCLUSIONS: The TESLA procedure allows surgical correction of MAS without the need for prosthetic grafts in young children who are still growing.

Contrast volume to estimated glomerular filtration rate ratio for prediction of contrast-induced acute kidney injury after cardiac catheterization in adults with congenital heart disease.

BACKGROUND: Adults with congenital heart disease (ACHD) are vulnerable to contrast-induced acute kidney injury (CI-AKI) after cardiac catheterization. The aim of this study was to identify risk factors for clinically significant CI-AKI and evaluate the predictive value of contrast volume to estimated glomerular filtration rate ratio (V/eGFR) for the risk of CI-AKI following catheterization in the ACHD population. METHODS: ACHD patients who underwent catheterization at Boston Children's hospital between 1/2011 and 1/2017 were retrospectively analyzed. CI-AKI was defined as an increase in serum creatinine ≥0.3 mg/dL within 48 hr or ≥1.5 times baseline within 7 days of procedure. Controls without CI-AKI were matched for calendar year of catheterization with cases using a 3:1 ratio. RESULTS: Of 453 catheterizations meeting inclusion criteria, 27 catheterizations (5.9%) were complicated by CI-AKI, with dialysis being used to manage renal dysfunction in five of these events. Older age, male gender, admission prior to catheterization, and V/eGFR ratio were found to be related to risk of CI-AKI. Patients with a V/eGFR ≥ 2.6 had a significantly higher risk of CI-AKI (OR = 6.4; 95% CI = 2.0-20.4; P = 0.002). Survival at 3 years post-catheterization, was significantly shorter for CI-AKI cases compared to controls (49% versus 97%; P < 0.001) even in those with return to baseline renal function prior to discharge (60% versus 97%, P < 0.001). CONCLUSION: In ACHD patients undergoing cardiac catheterization, a higher V/eGFR ratio is a strong predictor of clinically significant CI-AKI. Development of CI-AKI is a poor prognostic indicator and is associated with decreased survival in this population.