Intervention for critical aortic stenosis in Hutchinson-Gilford progeria syndrome.

Hutchinson-Gilford Progeria Syndrome (HGPS) is an ultra-rare genetic premature aging disease that is historically fatal in teenage years, secondary to severe accelerated atherosclerosis. The only approved treatment is the farnesyltransferase inhibitor lonafarnib, which improves vascular structure and function, extending average untreated lifespan of 14.5 years by 4.3 years (30%). With this longer lifespan, calcific aortic stenosis (AS) was identified as an emerging critical risk factor for cardiac death in older patients. Intervention to relieve critical AS has the potential for immediate improvement in healthspan and lifespan. However, HGPS patient-device size mismatch, pervasive peripheral arterial disease, skin and bone abnormalities, and lifelong failure to thrive present unique challenges to intervention. An international group of experts in HGPS, pediatric and adult cardiology, cardiac surgery, and pediatric critical care convened to identify strategies for successful treatment. Candidate procedures were evaluated by in-depth examination of 4 cases that typify HGPS clinical pathology. Modified transcatheter aortic valve replacement (TAVR) and left ventricular Apico-Aortic Conduit (AAC) placement were deemed high risk but viable options. Two cases received TAVR and 2 received AAC post-summit. Three were successful and 1 patient died perioperatively due to cardiovascular disease severity, highlighting the importance of intervention timing and comparative risk stratification. These breakthrough interventions for treating critical aortic stenosis in HGPS patients could rewrite the current clinical perspective on disease course by greatly improving late-stage quality of life and increasing lifespan. Expanding worldwide medical and surgical competency for this ultra-rare disease through expert information-sharing could have high impact on treatment success.

From Surgical to Total Transcatheter Stage I Palliation: Exploring Evidence and Perspectives.

Neonates with single ventricle physiology and ductal-dependent systemic circulation, such as those with hypoplastic left heart syndrome, undergo palliation in the first days of life. Over the past decades, variations on the traditional Stage 1 palliation, also known as Norwood operation, have emerged. These include the hybrid palliation and the total transcatheter approach. Here, we review the current evidence and data on different Stage 1 approaches, with a focus on their advantages, challenges, and future perspectives. Overall, although controversy remains regarding the superiority or inferiority of one approach to another, outcomes after the Norwood and the hybrid palliation have improved over time. However, both procedures still represent high-risk approaches that entail exposure to sternotomy, surgery, and potential cardiopulmonary bypass. The total transcatheter Stage 1 palliation spares patients the surgical and cardiopulmonary bypass insults and has proven to be an effective strategy to bridge even high-risk infants to a later palliative surgery, complete repair, or transplant. As the most recently proposed approach, data are still limited but promising. Future studies will be needed to better define the advantages, challenges, outcomes, and overall potential of this novel approach.

Growth of the right ventricular outflow tract in repaired tetralogy of Fallot: A longitudinal CMR study.

BACKGROUND: Many patients with repaired tetralogy of Fallot require pulmonary valve replacement (PVR) due to significant pulmonary regurgitation (PR). Transcatheter PVR (TPVR) is an equally effective and less invasive alternative to surgical PVR but many native right ventricular outflow tracts (RVOTs) are too large for TPVR at time of referral. Understanding the rate of growth of the RVOT may help optimize timing of referral. This study aims to examine the longitudinal growth of the native RVOT over time in repaired tetralogy of Fallot (TOF). METHODS: A retrospective review of serial cardiac MRI cardiovascular magnetic resonance (CMR) data from 121 patients with repaired TOF and a native RVOT (median age at first CMR 14.7 years, average interval between the first and last CMR of 8.1 years) was performed to measure serial changes in RVOT diameter, cross-sectional area, perimeter-derived diameter, and length. RESULTS: All parameters of RVOT size continued to grow with increasing age but growth was more rapid in the decade after TOF repair (for minimum systolic diameter, mean increase of 5.7 mm per 10 years up to year 12, subsequently 2.3 mm per 10 years). The RVOT was larger with a transannular patch and in patients without pulmonary stenosis (p < 0.001 for both), but this was not associated with rate of growth. More rapid RVOT enlargement was noted in patients with larger right ventricular end-diastolic volume (RVEDV), higher PR fraction, and greater rates of increases in RVEDV and PR (p < 0.001 for all) CONCLUSIONS: in patients with repaired TOF, using serial CMR data, we found that RVOT size increased progressively at all ages, but the rate was more rapid in the first decade after repair. More rapid RVOT enlargement was noted in patients with a larger RV, more PR, and greater rates of increases in RV size and PR severity. These results may be important in considering timing of referral for transcatheter pulmonary valves, in planning transcatheter and surgical valve replacement, and in designing future valves for the native RVOT.

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.

Transcatheter Palliation With Pulmonary Artery Flow Restrictors in Neonates With Congenital Heart Disease: Feasibility, Outcomes, and Comparison With a Historical Hybrid Stage 1 Cohort.

BACKGROUND: Neonates with complex congenital heart disease and pulmonary overcirculation have been historically treated surgically. However, subcohorts may benefit from less invasive procedures. Data on transcatheter palliation are limited. METHODS: We present our experience with pulmonary flow restrictors (PFRs) for palliation of neonates with congenital heart disease, including procedural feasibility, technical details, and outcomes. We then compared our subcohort of high-risk single ventricle neonates palliated with PFRs with a similar historical cohort who underwent a hybrid Stage 1. Cox regression was used to evaluate the association between palliation strategy and 6-month mortality. RESULTS: From 2021 to 2023, 17 patients (median age, 4 days; interquartile range [IQR], 2-8; median weight, 2.5 kilograms [IQR, 2.1-3.3]) underwent a PFR procedure; 15 (88%) had single ventricle physiology; 15 (88%) were high-risk surgical candidates. All procedures were technically successful. At a median follow-up of 6.2 months (IQR, 4.0-10.8), 13 patients (76%) were successfully bridged to surgery (median time since PFR procedure, 2.6 months [IQR, 1.1-4.4]; median weight, 4.9 kilograms [IQR, 3.4-5.8]). Pulmonary arteries grew adequately for age, and devices were easily removed without complications. The all-cause mortality rate before target surgery was 24% (n=4). Compared with the historical hybrid stage 1 cohort (n=23), after adjustment for main confounding (age, weight, intact/severely restrictive atrial septum or left ventricle to coronary fistulae), the PFR procedure was associated with a significantly lower all-cause 6-month mortality risk (adjusted hazard ratio, 0.26 [95% CI, 0.08-0.82]). CONCLUSIONS: Transcatheter palliation with PFR is feasible, safe, and represents an effective strategy for bridging high-risk neonates with congenital heart disease to surgical palliation, complete repair, or transplant while allowing for clinical stabilization and somatic growth.

Transcatheter Ductal Stents Versus Surgical Systemic-Pulmonary Artery Shunts in Neonates With Congenital Heart Disease With Ductal-Dependent Pulmonary Blood Flow: Trends and Associated Outcomes From the Pediatric Health Information System Database.

Background Surgical systemic-to-pulmonary artery shunts have been the standard approach to establish stable pulmonary blood flow in neonates with congenital heart disease with ductal-dependent pulmonary blood flow. More recently, transcatheter ductal stents have been performed as an alternative, less invasive intervention. We aimed to characterize trends in the utilization of surgical shunts versus ductal stents and compare associated outcomes. Methods and Results Using data from the Pediatric Health Information System, we retrospectively analyzed neonates with congenital heart disease with ductal-dependent pulmonary blood flow who underwent surgical shunt or ductal stent placement between January 2016 and December 2021. Patients were identified by International Classification of Diseases, Tenth Revision (ICD-10) diagnosis and procedure codes. The primary outcome was length of hospital stay. Secondary outcomes were reintervention risk and adjusted hospital costs. Of 936 patients included, 65.2% underwent a surgical shunt over the 6-year period. The proportion who underwent ductal stenting increased from 19% to 53.4% from 2016 to 2021. The median adjusted difference in postintervention length of hospital stay was 11 days greater for the surgical shunt cohort (95% CI, 7.2-14.8; P<0.001). The adjusted reintervention risks within 3 (odds ratio [OR], 3.37 [95% CI, 1.91-5.95], P<0.001) and 6 months (OR, 2.43 [95% CI, 1.62-3.64], P<0.001) were significantly greater in the ductal stent group. Median adjusted index hospital costs were $198 300 ($11 6400-$340 000) versus $120 400 ($81 800-$192 400) for the surgical shunt and ductal stent cohorts, respectively (P<0.001). Conclusions Ductal stenting has become an increasingly utilized palliative approach to secure pulmonary blood flow in neonates with congenital heart disease with ductal-dependent pulmonary blood flow in the United States. Ductal stenting is associated with decreased length of hospital stay and reduced overall cost for the index hospitalization but with a greater reintervention risk than surgical shunting.

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

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

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.

Left Atrial Decompression in Pediatric Patients Supported With Extracorporeal Membrane Oxygenation for Failure to Wean From Cardiopulmonary Bypass: A Propensity-Weighted Analysis.

Background Left atrial (LA) decompression on extracorporeal membrane oxygenation (ECMO) can reduce left ventricular distension, allowing myocardial rest and recovery, and protect from lung injury secondary to cardiogenic pulmonary edema. However, clinical benefits remain unknown. We sought to evaluate the association between LA decompression and in-hospital adverse outcome (mortality, transplant on ECMO, or conversion to ventricular assist device) in patients who failed to wean from cardiopulmonary bypass using a propensity score to adjust for baseline differences. Methods and Results Children (aged <18 years) with biventricular physiology supported with ECMO for failure to wean from cardiopulmonary bypass after cardiac surgery from 2000 through 2016, reported to the ELSO (Extracorporeal Life Support Organization) Registry, were included. Inverse probability of treatment weighted logistic regression was used to test the association between LA decompression and in-hospital adverse outcomes. Of the 2915 patients supported with venoarterial ECMO for failure to wean from cardiopulmonary bypass, 1508 had biventricular physiology and 279 (18%) underwent LA decompression (LA+). Genetic and congenital abnormalities (P=0.001) and pulmonary hypertension (P=0.010) were less frequent and baseline arrhythmias (P=0.022) were more frequent in LA+ patients. LA+ patients had longer pre-ECMO mechanical ventilation and CBP time (P<0.001), and used aortic cross-clamp (P=0.001) more frequently. Covariates were well balanced between the propensity-weighted cohorts. In-hospital adverse outcomes occurred in 47% of LA+ patients and 51% of the others. Weighted multivariate logistic regression showed LA decompression to be protective for in-hospital adverse outcomes (adjusted odds ratio, 0.775 [95% CI, 0.644-0.932]). Conclusions LA decompression independently decreased the risk of in-hospital adverse outcome in pediatric venoarterial ECMO patients who failed to wean from cardiopulmonary bypass, suggesting that these patients may benefit from LA decompression.

Interpreting Quality Improvement When Introducing New Technology: A Collaborative Experience in ASD Device Closures.

The objective of this study was to evaluate the impact of the regular introduction of new technologies into interventional cardiac catheterization procedures, in this case new atrial septal defect (ASD) closure devices, while conducting a multi-center collaborative initiative to reduce radiation usage during all procedures. Data were collected prospectively by 8 C3PO institutions between January 1, 2014 and December 31, 2017 for ASD device closure procedures in the cardiac catheterization lab during a quality improvement (QI) initiative aimed at reducing patient radiation exposure. Radiation exposure was measured in dose area product per body weight (µGy*m2/kg). Use of proposed practice change strategies at the beginning and end of the QI intervention period was assessed. Radiation exposure was summarized by institution and by initial type of device used for closure. This study included 602 ASD device closures. Without changes in patient characteristics, total fluoroscopy duration, or number of digital acquisitions, median radiation exposure decreased from 37 DAP/kg to 14 DAP/kg from 2014 to 2017. While all individual centers decreased overall median DAP/kg, the use of novel devices for ASD closure correlated with a temporary period of worsening institutional radiation exposure and increased fluoroscopy time. The introduction of new ASD closure devices resulted in increased radiation exposure during a QI project designed to reduce radiation exposure. Therefore, outcome assessment must be contextualized in QI projects, hospital evaluation, and public reporting, to acknowledge the expected variation during innovation and introduction of novel therapies.

Trend and Outcomes for Surgical Versus Transcatheter Patent Ductus Arteriosus Closure in Neonates and Infants at US Children's Hospitals.

Background Pharmacologic therapy for patent ductus arteriosus closure is not consistently successful. Surgical ligation (SL) or transcatheter closure (TC) may be needed. Large multicenter analyses comparing outcomes and resource use between SL and TC are lacking. We hypothesized that patients undergoing TC have improved outcomes compared with SL, including mortality, hospital and intensive care unit length of stay, and mechanical ventilation. Methods and Results Using the 2016 to 2020 Pediatric Health Information System database, characteristics, outcomes, and charges of patients aged <1 year who underwent TC or SL were analyzed. A total of 678 inpatients undergoing TC (n=503) or SL (n=175) were identified. Surgical patients were younger (0.1 versus 0.53 years; P<0.001) and more premature (60% versus 20.3%; P<0.001). Surgical patients had higher mortality (1.7% versus 0%; P=0.02). Using inverse probability of treatment weighting by the propensity score, multivariable-adjusted analyses demonstrated favorable outcomes in TC: intensive care unit admission rates (adjusted odds ratio [OR], 0.2; 95% CI, 0.11-0.32; P<0.001); mechanical ventilation rates (adjusted OR, 0.3; 95% CI, 0.19-0.56; P<0.001); and shorter hospital (adjusted coefficient, 2 days shorter; 95% CI, 1.3-2.7; P<0.001) and postoperative (adjusted coefficient, 1.2 days shorter; 95% CI, 0.1-2.3; P=0.039) stays. Overall charges and readmission rates were similar. Among premature neonates and infants, hospital (adjusted difference in medians, 4 days; 95% CI, 1.7-6.3 days; P<0.001) and postoperative stays (adjusted difference in medians, 3 days; 95% CI, 1.1-4.9 days; P=0.002) were longer for SL. Conclusions TC is associated with lower mortality and reduced length of stay compared with SL. Rates of TC continue to increase compared with SL.

The Burden of Radiation Exposure During Transcatheter Closure of Atrial Septal Defect.

Radiation reduction in the pediatric cardiac catheterization laboratory is well-suited for targeted quality improvement (QI) interventions. Transcatheter atrial septal defect (ASD) closure was chosen for this QI project based on a homogenous procedural population and inter-operator variability in radiation usage, with the aim to reduce radiation exposure during ASD device closure by 50% over 1 year. The aim for this project was defined and a Key Driver Diagram (KDD) was created with three domains for change: modification of procedural practice, reporting and monitoring/feedback, and team engagement. All patients undergoing attempted transcatheter ASD closure were considered for inclusion. The primary outcome, % reduction in median radiation dose (DAP/Kg), was determined through comparison with a historical cohort. Additional radiation metrics, procedural characteristics, and adverse events (AE) were compared to the historical cohort. Radiation exposure (DAP/kg) was reduced by 55% with a median dose reduction from 26 (15, 61) in a historical cohort to 12 (6, 22) in the intervention population (p <0.001). Fluoroscopy time and cine acquisition utilization significantly decreased. Procedure time, procedural success (defined as successful delivery of the device) and AE did not increase in the QI cohort. Successful practice changes included standardized procedural strategies to limit fluoroscopy and cine acquisition, improved fluoroscopic practice, engagement of the multidisciplinary team, and feedback with data reporting by electronic and in-person reminders. In conclusion, application of QI methodologies such as KDD with engagement of a multidisciplinary team can effectively reduce radiation in the pediatric catheterization laboratory.

Acute and Short-Term Outcomes of Percutaneous Transcatheter Mitral Valve Replacement in Children.

[Figure: see text].

Elective Non-Urgent Balloon-Atrial Septostomy in Infants with d-Transposition of the Great Arteries Does Not Eliminate the Need for PGE1 Therapy at the Time of Arterial Switch Operation.

Once a mainstay in the treatment of neonates with d-transposition of the great arteries (d-TGA), the application of balloon atrial septostomy (BAS) in the d-TGA population has become more selective. Currently, there is no clear evidence for or against a selective BAS strategy. The aims of this single-center retrospective study were to determine the incidence of BAS in the neonatal d-TGA population in the current era, to measure the rate of procedural success, and to compare the outcomes and complication rates of patients who underwent BAS to those who underwent neonatal ASO alone. Between 2012 and 2018, 147 patients with d-TGA underwent initial medical management and ASO, 73 of which underwent BAS. The percentage of patients that underwent BAS decreased from 73 to 33% over the study time period. In patients with d-TGA with intact ventricular septum, 33% of patients remained off of PGE1 at the time of surgery regardless of BAS. In d-TGA with ventricular septal defect, 85.7% of those that underwent BAS and 54.1% of those who did not remained off of PGE1 at the time of surgery, however, this difference did not reach statistical significance. In this single institution retrospective cohort of patients with d-TGA, the performance of a technically successful balloon atrial septostomy did not eliminate the need for PGE1 therapy at the time of definitive ASO. This was true regardless of the presence or absence of a ventricular septal defect.

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.

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.

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.