Search

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

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

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

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

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

Background: Tricuspid valve injury can occur during implantation of a SAPIEN valve in the pulmonary position. We describe our experience using a long Gore DrySeal (GDS) sheath to protect the tricuspid valve during advancement of the Commander delivery system., Methods: Retrospective single center review of all patients who underwent placement of a SAPIEN valve in the right ventricular outflow tract between January 2016 and April 2020. Patients were divided into two groups: delivery of the valve using standard technique (Group I), and with the use of a GDS (Group II), for comparison., Results: There were 48 patients in total: 25 in Group I and 23 in Group II. In Group II, the first 10 patients had a 29 mm S3 placed through a 26 French (Fr), 65 cm GDS. We then performed additional crimping of the S3 onto the balloon after the balloon catheter was withdrawn to position the valve on the balloon outside the body. Subsequently, seven had a 29 mm S3 placed through a 24 Fr GDS, and four had a 26 mm S3 placed through a 22 Fr GDS including one weighing 16 kg. Two had a 23 mm S3 placed through a 22Fr GDS as the 20Fr GDS was not available in our lab. Severe tricuspid valve injury occurred in 2/25 (8%) of Group I patients and 0/23 of Group II patients., Conclusion: Use of a long GDS may protect the tricuspid valve from injury during implantation of the S3 valve in the pulmonary position, and is technically feasible in smaller patients., (© 2020 Wiley Periodicals LLC.)

Iatrogenic aortopulmonary communications (IAPCs) are an uncommon but important complication after percutaneous intervention in postoperative patients. Iatrogenic aortopulmonary communications typically occur after balloon angioplasty or other interventions of the pulmonary outflow tract in certain anatomic configurations in which there is a denuded tissue plane between the pulmonary artery and aorta. They can present with signs and symptoms ranging from subtle clues which are difficult to recognize to near immediate hemodynamic instability. Once recognized, these lesions can become management dilemmas, and intraprocedural interventions can be complicated by complex anatomy and inadequate visualization by standard imaging techniques. We report cases where intracardiac echocardiography (ICE) was integral in the evaluation and management of IAPC as complications of prior transcatheter interventions. We found using ICE safely and effectively identified IAPCs and reduced the technical difficulty of intervention after IAPC discovery.

Background: Patent ductus arteriosus (PDA) stent placement in infants with ductal-dependent pulmonary blood flow is being increasingly used in clinical practice., Objective: To correlate computed tomographic (CT) angiography morphology and length of the PDA with catheter angiography and its relation to eventual PDA stent length., Materials and Methods: We retrospectively identified all pediatric patients who underwent PDA stenting at our institute from 2004 to 2018. We included children who had CT angiography prior to stenting. PDA length was measured by a radiologist blinded to the catheter angiography data, using Syngo-via post-processing software (Siemens, Erlangen, Germany). Vessel centerline technique was used. We measured the actual length of the duct as well as straight length between aortic and pulmonary ends. PDA morphology tortuosity index was classified as straight (Type I), mildly tortuous with 1 turn (Type II) and tortuous with >1 turn (Type III), and the PDA origin was noted. The PDA was also measured and morphology classified on catheter angiography by an interventional cardiologist blinded to the CT angiography findings. We compared the CT angiography and catheter angiography lengths, straight lengths and stent length using scatter plots and intraclass correlation coefficient (ICC)., Results: A total of 83 children who had PDA stenting were identified, of whom 17 had prior CT angiography. Fifteen of these were neonates. There was agreement between CT angiography and catheter angiography regarding the PDA morphology tortuosity index in 94% of cases and PDA origin in 100% of cases. There was moderate agreement between CT angiography and catheter angiography actual and straight PDA lengths, with ICC coefficients of 0.65 and 0.68, respectively. There was moderate agreement between CT angiography actual length, CT angiography straight length, catheter angiography actual length and eventual stented PDA length, with ICCs of 0.57, 0.67 and 0.73, respectively. There was poor agreement between catheter angiography straight length and eventual stented PDA length, with an ICC of 0.39., Conclusion: PDA length and morphology description on CT angiography correlates well with catheter angiography and can be a reliable guide for the interventional cardiologist in decision-making regarding appropriate choice of PDA stent length.

Background: The novel coronavirus (COVID-19) pandemic has placed severe stress on healthcare systems around the world. There is limited information on current practices in pediatric cardiac catheterization laboratories in the United States (US)., Objectives: To describe current practice patterns and make recommendations regarding potential resource allocation for congenital cardiac catheterization during the COVID-19 pandemic., Methods: A web-based survey was distributed regarding case candidacy and catheterization laboratory preparedness. Centers were categorized based on the current degree of disease burden in that community (as of April 1, 2020). Data and consensus opinion were utilized to develop recommendations., Results: Respondents belonged to 56 unique US centers, with 27 (48.2%) located in counties with a high number of COVID-19 cases. All centers have canceled elective procedures. There was relative uniformity (>88% agreement) among centers as to which procedures were considered elective. To date, only three centers have performed a catheterization on a confirmed COVID-19 positive patient. Centers located in areas with a higher number of COVID-9 cases have been more involved in a simulation of donning and doffing personal protective equipment (PPE) than low-prevalence centers (46.7% vs 10.3%, respectively; P<.001). Currently, only a small fraction of operators has been reassigned to provide clinical services outside their scope of practice., Conclusions: At this stage in the COVID-19 pandemic, pediatric/congenital catheterization laboratories have dramatically reduced case volumes. This document serves to define current patterns and provides guidance and recommendations on the preservation and repurposing of resources to help pediatric cardiac programs develop strategies for patient care during this unprecedented crisis.

Background: Carotid artery (CA) and axillary artery (AA) access are increasingly used for transcatheter stenting of the patent ductus arteriosus (PDA), although reports are limited., Methods: The Congenital Catheterization Research Collaborative (CCRC) reviewed multicenter data from infants who underwent PDA stenting via the CA or AA approach from 2008 to 2017, and compared outcomes to those of infants undergoing PDA stenting via the femoral artery (FA) approach. Post-procedure ultrasound (US) imaging was reviewed., Results: Forty-nine infants underwent PDA stenting from the CA (n = 43) or AA (n = 6) approach, compared with 55 infants who underwent PDA stenting from the FA approach. The PDA was the sole pulmonary blood flow (PBF) source in 61% of infants in the CA/AA cohort, compared with 33% of the FA cohort (p < .01). Ductal tortuosity for CA/AA cohort was Type I (straight) in 10 (20%), Type II (one turn) in 17 (35%), and Type III (multiple turns) in 22 (45%) infants and reflected a greater degree of tortuosity when compared to the FA cohort (p < .01). In 17 infants with CA/AA approach, the "flip technique" was used, and was associated with shorter procedure times for highly tortuous PDA (Type III) patients. Rates of procedural complications were similar across access sites. Most common complications were access site injury (thrombus or bleeding) and stent malposition. No complications were specifically related to the "flip technique.", Conclusions: Use of CA and AA approach for PDA stenting was found to be more commonly employed in sole source PBF and highly tortuous PDAs. Procedural modifications such as the "flip technique" may lead to shorter procedure times. CA and AA approaches are associated with a similar burden of procedural or late complications. Post-procedural surveillance of the CA and AA is suggested, given the incidence of vascular findings on US., (© 2019 Wiley Periodicals, Inc.)

Purpose of Review: To review the most recent literature on pediatric transcatheter ductal intervention including ductus arteriosus occlusion and stenting., Recent Findings: With the development and FDA approval of smaller ductal devices, including most recently the Amplatzer Piccolo Occluder (Abbott, Abbott Park, IL), transcatheter ductus arteriosus device closure is now being safely performed in premature infants and patients < 6 kg using a transvenous approach. In patients with ductus-dependent pulmonary blood flow, ductal stenting with pre-mounted coronary artery stents has been shown to be an acceptable alternative to the surgically placed Blalock-Taussig shunt. Centers with experience in ductal stenting have demonstrated success, even with the tortuous ductus. Innovation in transcatheter device technology and procedural practices have allowed for significant advances. Transcatheter ductal device closure is a reasonable alternative to surgical ligation even in premature, low-birthweight infants. Ductal stenting is also an accepted alternative to the modified Blalock-Taussig shunt. We anticipate continued advancement and procedural refinement over the next several years.

Background In infants with ductal-dependent pulmonary blood flow, the impact of palliation strategy on interstage growth and feeding regimen is unknown. Methods and Results This was a retrospective multicenter study of infants with ductal-dependent pulmonary blood flow palliated with patent ductus arteriosus (PDA) stent or Blalock-Taussig shunt (BTS) from 2008 to 2015. Subjects with a defined interstage, the time between initial palliation and subsequent palliation or repair, were included. Primary outcome was change in weight-for-age Z -score. Secondary outcomes included % of patients on: all oral feeds, feeding-related medications, higher calorie feeds, and feeding-related readmission. Propensity score was used to account for baseline differences. Subgroup analysis was performed in 1- (1V) and 2-ventricle (2V) groups. The cohort included 66 PDA stent (43.9% 1V) and 195 BTS (54.4% 1V) subjects. Prematurity was more common in the PDA stent group ( P =0.051). After adjustment, change in weight-for-age Z -score did not differ between groups over the entire interstage. However, change in weight-for-age Z -score favored PDA stent during the inpatient interstage ( P =0.005) and BTS during the outpatient interstage ( P =0.032). At initial hospital discharge, PDA stent treatment was associated with all oral feeds ( P <0.001) and absence of feeding-related medications ( P =0.002). Subgroup analysis revealed that 2V but not 1V patients demonstrated significant increase in weight-for-age Z -score. In the 2V cohort, feeding-related readmissions were more common in the BTS group ( P =0.008). Conclusions In infants with ductal-dependent pulmonary blood flow who underwent palliation with PDA stent or BTS, there was no difference in interstage growth. PDA stent was associated with a simpler feeding regimen and fewer feeding-related readmissions.

Background: Patients with single ventricle anatomy and ductal-dependent pulmonary blood flow may be initially palliated with either modified Blalock-Taussig shunt (BTS) or ductus arteriosus stent (DAS). Comparisons of outcomes during the interstage period and at the time of superior cavopulmonary connection (SCPC) are lacking and may differ between palliation strategies., Methods: Infants with single ventricle anatomy and ductal-dependent pulmonary blood flow palliated with either DAS or BTS from 2008 to 2015 were reviewed across 4 centers. Interstage outcomes, and for those who had SCPC, anatomy, hemodynamics, and perioperative clinical outcomes were compared. Thirty-five patients with DAS and 136 patients with BTS were included., Results: At initial palliation, demographic, clinical variables, and pulmonary artery size were similar. Interstage death, transplant, or unplanned reintervention to treat cyanosis occurred in 25.7% of DAS and 35.8% of BTS, P =0.27. Reintervention was more common with DAS (48.6% versus 2.2%; P <0.001). Twenty-three DAS patients and 111 BTS patients underwent SCPC. Preoperative hemodynamics and overall pulmonary atresia growth were similar, although right pulmonary artery growth was better with DAS (change in z -score: 1.57 versus 0.65, P =0.026). SCPC intraoperative and postoperative courses were similar., Conclusions: In patients with single-ventricle anatomy and ductal-dependent pulmonary blood flow, interstage outcomes, hemodynamics before SCPC, and acute postoperative outcomes were similar. Overall reintervention was more common in the DAS group, driven by more frequent planned reintervention. Unplanned reintervention, death, and transplant were similar. Both groups demonstrated good pulmonary atresia growth. DAS is a reasonable initial palliative alternative to BTS in select patients.

Objectives: We aimed to assess the experience using a percutaneous axillary artery approach for insertion of arterial ductal stents in patients with critical right ventricular outflow tract lesions at two tertiary pediatric cardiology centers., Background: Patent ductus arteriosus stenting is an accepted palliative alternative to BT shunts for neonates with critical right heart lesions. Access to tortuous ductus' may be challenging via the femoral artery, whereas the carotid artery presents a low risk of stroke. Recently, the axillary artery has been utilized for access in these patients., Methods: We performed a retrospective review of neonates who underwent stent placement or angioplasty using percutaneous axillary artery approach at two tertiary care centers from October 2016 to November 2018. Medical records were reviewed to ascertain demographic, clinical, and outcome data., Results: Axillary artery access was performed in 20 patients (16 primary ductal stents and 4 re-interventions) at a median (IQR) procedural weight of 3.4 (3-3.9) kg. Median (IQR) procedural time was 110 (75-150) min. The median (IQR) ICU stay and intubation times were 14 (0-94) hr and 5 (0-40) hr, respectively. There were three access-related vascular complications which were managed conservatively with no long-term effects. Two patients subsequently died due to non-procedure related causes., Conclusions: Ductal stenting via a percutaneous axillary artery approach is a viable option in neonates with critical right ventricular outflow tract lesions. This approach provides an additional access site for PDA stenting which may be utilized in patients with vertical duct morphology., (© 2019 Wiley Periodicals, Inc.)

Background: Traditional approaches to pediatric cardiac catheterization have relied on femoral venous access. Upper- extremity venous access may enable cardiac catheterization procedures to be performed safely for diagnostic and interventional catheterizations. The objective of this multicenter study was to demonstrate the feasibility and safety of upper-extremity venous access in a pediatric cardiac catheterization laboratory., Methods: A retrospective chart review of all patients who underwent cardiac catheterization via upper-extremity vascular access was performed., Results: Eighty-two cardiac catheterizations were attempted via upper-extremity vein on 72 patients. Successful access was obtained in 75 catheterizations (91%) in 67 patients. Median age at catheterization was 18.79 years (interquartile range [IQR], 13.02-32.75 years; n = 75) with a median weight of 59.4 kg (IQR, 43.3-76.5 kg; n = 75). The youngest patient was 4.1 months old, weighing 4.3 kg. Local anesthesia or light sedation was utilized in 46 procedures (61%). Diagnostic right heart catheterization was the most common procedure (n = 65; 87%), with intervention performed via the upper extremity in 8 cases (11%). Median fluoroscopy time was 10.02 min (IQR, 2.87-36.26 min; n = 75), with dose area product/kg of 3.765 μGy•m²/kg (IQR, 0.74-34.12 μGy•m²/kg; n = 64). Median sheath duration time was 48 min (IQR, 19.5-147 min; n = 57) and median total procedure time was 116 min (IQR, 80.5-299 min; n = 65). Median length of stay for outpatient procedures was 5.37 hr (IQR, 4.25-6.92 hr; n = 27). There were no procedural complications., Conclusion: Upper-extremity venous access is a useful, feasible, and safe modality for cardiac catheterization in the pediatric cardiac catheterization laboratory.

Clinical research in the treatment of patients with congenital heart disease (CHD) is limited by the wide variety of CHD manifestations and therapeutic options as well as the generally low incidence of CHD. The availability of comprehensive, contemporary outcomes studies is therefore limited. This inadequacy may result in a lack of data-driven medical decision making. In 2013, clinician scientists at two centers began a research collaboration, the Congenital Catheterization Research Collaborative (CCRC). Over time, the CCRC has grown to include nine cardiac centers from across the United States, with a common data coordinating center. The CCRC seeks to generate high-quality, contemporary, statistically robust, and generalizable outcomes research which can help address important clinical questions in the treatment of CHD. To date, the CCRC has reported on multicenter outcomes in: neonates with congenital aortic stenosis, infants undergoing right ventricular decompression for pulmonary atresia and intact ventricular septum, and infants with ductal-dependent pulmonary blood flow. The CCRC has been successful at leveraging large multicenter cohorts of patients in a contemporary period to perform comparative studies. In the future, the CCRC plans to continue to perform hypothesis-driven retrospective and prospective observational studies of CHD populations where controversy exists or where novel interventions or therapies have emerged. Quality improvement efforts including lesion-specific registry development may be an additional potential future target., (© 2019 Wiley Periodicals, Inc.)

Objectives: To devise a classification scheme for ductal morphology in patients with ductal dependent pulmonary blood flow (PBF) that can be used to assess outcomes., Background: The impact of ductal morphology on outcomes following patent ductus arteriosus (PDA) stenting is not well defined., Methods: Patients <1 year of age who underwent PDA stenting for ductal dependent PBF at the four centers comprising the Congenital Catheterization Research Collaborative (CCRC) were included. A classification scheme for PDA morphology was devised based on a tortuosity index (TI)-Type I (straight), Type II (one turn), and Type III (multiple turns). A subtype classification was used based upon the ductal origin., Results: One hundred and five patients underwent PDA stenting. TI was Type I in 58, Type II in 24, and Type III in 23 PDAs, respectively. There was a significant association between ductal origin and vascular access site (p < 0.001). Procedure times and need for >1 stent did not differ based on TI. Greater TI was associated with pulmonary artery (PA) jailing (p = 0.003). Twelve (11.4%) patients underwent unplanned reintervention, more commonly with greater TI (p = 0.022) and PA jailing (p < 0.001). At the time of subsequent surgical repair/palliative staging, PA arterioplasty was performed in 32 patients, more commonly when a PA was jailed (p = 0.048). PA jailing did not affect PA size at follow up., Conclusions: The proposed qualitative and quantitative PDA morphology classification scheme may be helpful in anticipating outcomes in patients with ductal dependent PBF undergoing PDA stenting., (© 2019 Wiley Periodicals, Inc.)

The use of prostaglandin-E1 immediately after birth and subsequent surgical creation of the modified Blalock-Taussig shunt (BTS) shunt have remarkably improved the prognosis and survival of children with congenital heart disease and ductal-dependent pulmonary blood flow (PBF). Despite the advancement in surgical techniques, bypass strategies, and postoperative management, significant morbidity and mortality after BTS still remain. Patent ductus arteriosus stenting has been shown to be as an acceptable alternative to BTS placement in select infants with ductal-dependent PBF. Newer procedural techniques and equipment, along with operator experience have all contributed to procedural refinement associated with improved outcomes over the recent years. In this article, we review the procedural and periprocedural details, with an emphasis on recent advances of this procedure., (© 2019 Wiley Periodicals, Inc.)

Background: Congenital coronary artery fistula is a rare coronary anomaly. Most commonly, such fistulae drain into the right side of the heart or the pulmonary artery. Children with coronary artery fistulae are generally asymptomatic, although they may have left ventricular enlargement in the setting of a moderate sized left to right shunt. Symptoms of congestive heart failure or ischemia are very rare in neonatal period, and suggest the presence of a very large shunt and/or coronary steal., Methods: Single center retrospective review of transcatheter intervention on coronary artery fistulae presenting with symptoms in the neonatal period from January 2000 to March 2018. Patients with associated congenital heart diseases (except atrial septal defect) were excluded. Patient records, catheterization data, angiograms and noninvasive imaging were reviewed., Results: Two patients underwent transcatheter intervention for symptomatic coronary artery fistula in the first few weeks of life. The first patient had multiple right and left coronary artery to right ventricle fistulae and presented with severe biventricular systolic dysfunction. Transcatheter closure of the fistulae was performed using multiple Gianturco coils. The second patient had a large left main coronary artery to left ventricle fistula (with left anterior descending and circumflex coronary artery atresia) presenting with symptoms of ischemia. This large fistula was closed using one Amplatzer Vascular Plug type-II and two Micro-Vascular Plugs. Both patients had improvement in symptoms post intervention and are doing well at the last follow up at 12 years and 7 months respectively., Conclusions: We hereby describe the rare presentation of symptomatic coronary artery fistulae in the neonatal period and their successful transcatheter management. This is also the first description of left anterior descending and circumflex coronary artery atresia in the setting of a large left main coronary artery to left ventricle fistula., (© 2018 Wiley Periodicals, Inc.)

Objectives: This study sought to determine the safety and feasibility of transcatheter pulmonary valve replacement (TPVR) using the Melody valve in native (nonconduit) right ventricular outflow tracts (nRVOT), and to identify factors associated with successful TPVR., Background: The Melody valve is Food and Drug Administration-approved for TPVR within right ventricle-to-pulmonary artery conduits and bioprosthetic pulmonary valves. However, most patients needing pulmonary valve replacement have nRVOT and TPVR has been adapted for this indication., Methods: In this multicenter retrospective study of all patients presenting for nRVOT TPVR, we collected pre-procedural magnetic resonance imaging, echocardiography, and catheterization data, and evaluated procedural and early outcomes., Results: Of 229 patients (age 21 ± 15 years from 11 centers), 132 (58%) had successful TPVR. In the remaining 97, TPVR was not performed, most often because of prohibitively large nRVOT (n = 67) or compression of the aortic root or coronary arteries (n = 18). There were no deaths and 5 (4%) serious complications, including pre-stent embolization requiring surgery in 4 patients, and arrhythmia in 1. Higher pre-catheterization echocardiographic RVOT gradient was associated with TPVR success (p = 0.001) and larger center volume approached significance (p = 0.08). Magnetic resonance imaging anterior-posterior and lateral RVOT diameters were smaller in implanted versus nonimplanted patients (18.0 ± 3.6 mm vs. 20.1 ± 3.5 mm; p = 0.005; 18.4 ± 4.3 mm vs. 21.5 ± 3.8 mm; p = 0.002)., Conclusions: TPVR in the nRVOT was feasible and safe. However, nearly half the patients presenting for catheterization did not undergo TPV implantation, mainly because of prohibitively large nRVOT size. Improved understanding of magnetic resonance imaging data and availability of larger devices may improve the success rate for nRVOT TPVR., (Copyright © 2018 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Percutaneous transcatheter pulmonary valve replacement (TPVR) has good clinical and hemodynamic outcomes in treating dysfunctional bioprosthetic valves (BPV) in the pulmonary position. Valve-in-valve therapy can further decrease the inner diameter (ID), potentially resulting in patient-prosthesis mismatch in patients with smaller BPVs., Methods and Results: To evaluate feasibility and outcomes of intentional BPV fracture to enlarge the pulmonary valve orifice with TPVR, 37 patients from 13 centers who underwent TPVR with intended BPV fracture were evaluated. A control cohort (n=70) who underwent valve-in-valve TPVR without attempted fracture was evaluated. BPV was successfully fractured in 28 patients and stretched in 5 while fracture was unsuccessful in 4. A Melody valve was implanted in 25 patients with fractured/stretched frame and a Sapien (XT 3) valve in 8. Among patients whose BPV was fractured/stretched, the final ID was a median of 2 mm larger (0-6.5 mm) than the valve's true ID. The narrowest diameter after TPVR in controls was a median of 2 mm smaller ( P<0.001) than true ID. Right ventricular outflow tract gradient decreased from median 40 to 8 mm Hg in the fracture group. Cases with fracture/stretching were matched 1:1 (weight, true ID) to controls. Post-TPVR peak gradient was lower but not significant (8.3±5.2 versus 11.8±9.2 mm Hg; P=0.070). There were no fracture-related adverse events., Conclusions: Preliminary experience shows intentional fracture of BPV frame can be useful for achieving larger ID and better hemodynamics after valve-in-valve TPVR.

Objectives: This study sought to evaluate the safety, feasibility, and outcomes of transcatheter pulmonary valve replacement (TPVR) in conduits ≤16 mm in diameter., Background: The Melody valve (Medtronic, Minneapolis, Minnesota) is approved for the treatment of dysfunctional right ventricular outflow tract (RVOT) conduits ≥16 mm in diameter at the time of implant. Limited data are available regarding the use of this device in smaller conduits., Methods: The study retrospectively evaluated patients from 9 centers who underwent percutaneous TPVR into a conduit that was ≤16 mm in diameter at the time of implant, and reported procedural characteristics and outcomes., Results: A total of 140 patients were included and 117 patients (78%; median age and weight 11 years of age and 35 kg, respectively) underwent successful TPVR. The median original conduit diameter was 15 (range: 9 to 16) mm, and the median narrowest conduit diameter was 11 (range: 4 to 23) mm. Conduits were enlarged to a median diameter of 19 mm (29% larger than the implanted diameter), with no difference between conduits. There was significant hemodynamic improvement post-implant, with a residual peak RVOT pressure gradient of 7 mm Hg (p < 0.001) and no significant pulmonary regurgitation. During a median follow-up of 2.0 years, freedom from RVOT reintervention was 97% and 89% at 2 and 4 years, respectively, and there were no deaths and 5 cases of endocarditis (incidence rate 2.0% per patient-year)., Conclusions: In this preliminary experience, TPVR with the Melody valve into expandable small diameter conduits was feasible and safe, with favorable early and long-term procedural and hemodynamic outcomes., (Copyright © 2018 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objectives: The authors sought to assess the intermediate-term effects of percutaneous placed valves in the branch pulmonary artery (PA) position., Background: Most patients with large right ventricular outflow tracts (RVOTs) are excluded from available percutaneous pulmonary valve options. In some of these patients, percutaneous branch PA valve implantation may be feasible. The longer-term effects of valves in the branch PA position is unknown., Methods: Retrospective data were collected on patients with significant pulmonary regurgitation who had a percutaneous branch PA valve attempted., Results: Percutaneous branch PA valve implantation was attempted in 34 patients (18 bilateral and 16 unilateral). One-half of the patients were in New York Heart Association (NHYA) functional class III or IV pre-implantation. There were 2 failed attempts and 6 procedural complications. At follow-up, only 1 patient had more than mild valvar regurgitation. The right ventricular end-diastolic volume index decreased from 147 (range: 103 to 478) ml/m 2 to 101 (range: 76 to 429) ml/m 2 , p < 0.01 (n = 16), and the right ventricular end-systolic volume index decreased from 88.5 (range: 41 to 387) ml/m 2 to 55.5 (range: 40.2 to 347) ml/m 2 , p < 0.01 (n = 13). There were 5 late deaths. At a median follow-up of 2 years, all other patients were in NYHA functional class I or II., Conclusions: Percutaneous branch PA valve implantation results in a reduction in right ventricular volume with clinical benefit in the intermediate term. Until percutaneous valve technology for large RVOTs is refined and more widely available, branch PA valve implantation remains an option for select patients., (Copyright © 2018 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

The aim of this study was to define characteristics of those patients who are referred for device closure of an Atrial septal defect (ASD), but identified to "crossover" surgery. All patients who underwent surgical and device (Amplatzer or Helex occluder) closures of secundum ASDs from 2001 to 2010 were reviewed and organized into three groups: surgical closure, device closure, and "crossover" group. 369 patients underwent ASD closure (265 device, 104 surgical). 42 of the 265 patients referred for device closure "crossed over" to the surgical group at various stages of the catheterization procedure. The device group had defect size measuring 14.2 mm (mean) and an ASD index (Defect Size (mm)/BSA) of 14.0 compared to the corresponding values in the surgical group (20.1 mm, ASD index 25.9) (P < 0.001) and in the "crossover" group (20.7 mm, 22.6 ASD index) (P < 0.001). 79 patients in the device group had a deficient rim, and 86% were located in the retroaortic region. 33 patients in the "crossover" group had deficient rims with 70% deficiency in the posterior/inferior rim. The device group with deficient rims had an ASD index of 14.7 compared with the crossover group ASD index of 23.8 (P < 0.001). Comparing the device and "crossover" groups, an ASD index greater than 23.7 had a 90% specificity in "crossing over" to surgery. The crossover and surgical groups had statistically larger ASD defect size indexes compared with the device group. Deficient rim in the posterior/inferior rim is associated with a large ASD size index which is a predictive factor for crossing over to surgery. Catheterization did not negatively impact surgical results in the "crossover" group.

Background: Infants with ductal-dependent pulmonary blood flow may undergo palliation with either a patent ductus arteriosus (PDA) stent or a modified Blalock-Taussig (BT) shunt. A balanced multicenter comparison of these 2 approaches is lacking., Methods: Infants with ductal-dependent pulmonary blood flow palliated with either a PDA stent or a BT shunt from January 2008 to November 2015 were reviewed from the 4 member centers of the Congenital Catheterization Research Collaborative. Outcomes were compared by use of propensity score adjustment to account for baseline differences between groups., Results: One hundred six patients with a PDA stent and 251 patients with a BT shunt were included. The groups differed in underlying anatomy (expected 2-ventricle circulation in 60% of PDA stents versus 45% of BT shunts; P =0.001) and presence of antegrade pulmonary blood flow (61% of PDA stents versus 38% of BT shunts; P <0.001). After propensity score adjustment, there was no difference in the hazard of the primary composite outcome of death or unplanned reintervention to treat cyanosis (hazard ratio, 0.8; 95% confidence interval [CI], 0.52-1.23; P =0.31). Other reinterventions were more common in the PDA stent group (hazard ratio, 29.8; 95% CI, 9.8-91.1; P <0.001). However, the PDA stent group had a lower adjusted intensive care unit length of stay (5.3 days [95% CI, 4.2-6.7] versus 9.19 days [95% CI, 7.9-10.6]; P <0.001), a lower risk of diuretic use at discharge (odds ratio, 0.4; 95% CI, 0.25-0.64; P <0.001) and procedural complications (odds ratio, 0.4; 95% CI, 0.2-0.77; P =0.006), and larger (152 mm 2 /m 2 [95% CI, 132-176] versus 125 mm 2 /m 2 [95% CI, 113-138]; P =0.029) and more symmetrical (symmetry index, 0.84 [95% CI, 0.8-0.89] versus 0.77 [95% CI, 0.75-0.8]; P =0.008] pulmonary arteries at the time of subsequent surgical repair or last follow-up., Conclusions: In this multicenter comparison of palliative PDA stent and BT shunt for infants with ductal-dependent pulmonary blood flow adjusted for differences in patient factors, there was no difference in the primary end point, death or unplanned reintervention to treat cyanosis. However, other markers of morbidity and pulmonary artery size favored the PDA stent group, supporting PDA stent as a reasonable alternative to BT shunt in select patients., (© 2017 American Heart Association, Inc.)

Most diagnostic testing in patients with anomalous aortic origins of coronary arteries, myocardial bridges, and coronary artery changes after Kawasaki disease are performed with the use of noninvasive techniques. In some cases, however, further diagnostic information is needed to guide the clinician in treating these patients. In such instances, cardiac catheterization with invasive anatomic and functional testing is an invaluable tool. Moreover, interventional treatment in the cardiac catheterization laboratory may be performed in a small subset of these patients. As the diagnosis of these conditions is now becoming more common, it is important for pediatric interventional cardiologists to be familiar with these techniques. In this article, the role of angiography, intravascular ultrasound, fractional flow reserve, and optical coherence tomography in these patients is reviewed., (© 2017 Wiley Periodicals, Inc.)

Background: Atrial transseptal puncture (TSP) for cardiac catheterization procedures remain challenging in children and adults with complex congenital heart disease (CHD)., Objectives: We sought to evaluate our experience using radiofrequency (RF) current via surgical electrocautery needle for TSP to facilitate diagnostic and interventional procedures., Methods: Retrospective chart review of all patients (pts) who underwent TSP using RF energy (10-25 W) via surgical electrocautery from three centers from January 2011 to January 2017 were evaluated. Echocardiograms were reviewed to define the atrial septum as normal and complex (thin aneurysmal, thick/fibrotic, synthetic patch material, and extra cardiac conduit)., Results: A total of 54 pts underwent 55 successful TSP. Median age was 12.5 years (1 day-54 years) and weight was 52.7 kg (2-162). Indications for TSP included; EP study and ablation procedures in structurally normal hearts (n = 24) and in complex atrial septum/CHD and structural heart disease pts (n = 30): Electrophysiology study and ablation in 4, diagnostic catheterization in 9, and interventional procedures in 17 pts were performed. Atrial TSP was successful in 54/55 (98%). Atrial perforation with tiny-small pericardial effusion not requiring intervention was noted in 2 pts. TSP was unsuccessful in one critically ill neonate with unobstructed TAPVR and restricted atrial septum who experienced cardiac arrest requiring CPR, ECMO, and emergent surgery., Conclusions: RF current delivery using surgical electrocautery for TSP is a feasible and an effective option in patients with complex CHD for diagnostic, interventional, and electrophysiology procedures., (© 2017 Wiley Periodicals, Inc.)

Background: Follow-up of transcatheter pulmonary valve replacement (TPVR) with the Melody valve has demonstrated good short-term and long-term outcomes, but there are no published studies focused on valve performance in the Contegra bovine jugular vein conduit., Methods and Results: This is a retrospective, multicenter study of the short- and intermediate-term outcomes of Melody TPVR within the Contegra conduit in the right ventricle to pulmonary artery position. Data from 13 centers were included in the analysis. During the study period, 136 patients underwent 139 catheterizations for attempted Melody TPVR with a median follow-up of 3 years (1 day to 9.1 years). Of the 136 patients, 117 underwent successful Melody TPVR. Two patients underwent a second Melody TPVR. The majority of patients underwent placement of ≥1 stents before transcatheter pulmonary valve implantation. There was a significant reduction in peak conduit pressure gradient acutely after transcatheter pulmonary valve implantation (39 versus 10 mm Hg; P <0.001). At most recent follow-up, the maximum pulmonary valve gradient by echocardiogram remained significantly reduced relative to prevalve implant measurements (65.9 versus 27.3 mm Hg; P <0.001). The incidence of Melody transcatheter pulmonary valve stent fracture (3.4%) and infectious endocarditis (4.3%) were both low. Serious adverse events occurred in 3 patients., Conclusions: Melody TPVR in Contegra conduits is safe and effective and can be performed in a wide range of conduit sizes with preserved valve function and low incidence of stent fracture and endocarditis., (© 2017 American Heart Association, Inc.)

Objectives: To describe the early multicenter, clinical experience with the Medtronic Micro Vascular Plug™ (MVP) for the occlusion of patent ductus arteriosus (PDA) in premature infants., Background: The MVP is a large diameter plug that can be delivered through a microcatheter for occlusion of abnormal blood vessels., Methods: A Retrospective review of PDA embolization procedures performed in two centers using the MVP was performed., Results: Fifteen premature infants underwent attempted PDA occlusion using the MVP. The gestational age and birth weight were 25.6 ± 2.5 weeks and 735 ± 251 g, respectively. The median weight and age at the time of the procedure were 1,210 g (700-3,500 g) and 4.5 weeks (2-12 weeks), respectively. Median procedure and fluoroscopy times were 45 and 6.5 min, respectively. The median radiation and contrast doses were 19.7 mGy and 2.4 mL/kg, respectively. Antegrade occlusion was successfully achieved in 13 patients <2 kg with only femoral venous access aided by echo guidance. The two patients >2 kg had arterial access and attempted retrograde occlusion; one of which was unsuccessful due to the PDA being short and wide. Complete closure was observed in 13 of 14 successful procedures (93%), with one patient having a small residual shunt that was not seen on follow-up. There were no complications related to the procedure or noted during follow-up (Median 11 months)., Conclusions: The MVP is a new, large-diameter vascular embolization device that may be useful for the occlusion of PDA in extremely small, premature infants. © 2016 Wiley Periodicals, Inc., (© 2016 Wiley Periodicals, Inc.)

The evaluation of the vast majority of children with anomalous aortic origin of a coronary artery (AAOCA) and/or myocardial bridges is performed with non-invasive testing. However, a subset of these patients may benefit from invasive testing for risk stratification. All patients included in the Coronary Anomalies Program (CAP) at Texas Children's Hospital who underwent cardiac catheterization were included. Techniques included selective coronary angiograms (SCA), intravascular ultrasound (IVUS), and fractional flow reserve (FFR) measurements with provocative testing using adenosine and/or dobutamine infusions. Out of the 131 patients followed by the CAP between 12/12-4/16, 8 (6%) patients underwent 9 cath investigations at median age 13.1 (2.6-18.7) years and median weight 49.5 (11.4-142.7) kg. Six patients presented with cardiac signs/symptoms. Four patients had myocardial bridges of the left anterior descending (LAD) coronary artery, 2 patients had isolated AAOCA, and 2 patients had an anomalous left coronary artery (LCA) with an intramyocardial course of the LAD. SCA was performed in all patients. FFR was positive in 4/6 patients: IVUS showed >70% intraluminal narrowing in 3/5 patients. One patient had hemodynamic instability that reversed with catheter removal from the coronary ostium. Based on the catheterization data obtained, findings were reassuring in three patients, surgery was performed in three patients, and two patients are being medically managed/restricted from competitive sports. In our small cohort of patients, we demonstrated that IVUS and FFR can safely be performed in children and may help to risk stratify some patients with AAOCA and myocardial bridges.

Patients with congenital heart disease and pulmonary valve disease need multiple procedures over their lifetimes to replace their pulmonary valves. Chronic pulmonary stenosis, regurgitation, or both have untoward effects on ventricular function and on the clinical status of these patients. To date, all right ventricle-pulmonary artery conduits have had relatively short lifespans. Percutaneous pulmonary valve implantation, although relatively new, will probably reduce the number of operative procedures that these patients will have to undergo over a lifetime. Refinement and further development of this procedure holds promise for the extension of this technology to other patient populations.

Objectives: To describe the effectiveness of the Helex Septal Occluder (HSO) to close multiple atrial septal defects (mASDs). Background : Limited information is available describing closure of mASDs with the HSO., Methods: A total of 28 patients who underwent closure of mASDs with the HSO were identified by retrospective review of our catheterization database between 2001 and 2012. Procedural details and follow up information were collected., Results: Median age was 19.2 years, median weight 48 kg, with 10 (36%) patients weighing <25 kg. Indication for closure was RV enlargement (RVE) in all patients and additionally neurologic events occurred in 3/28 (11%). Median stop-flow diameter for the largest ASD was 14 (4-23) mm. One HSO was implanted in 21/28 (75%), 2 in 6/28 (21%), and 3 in 1/28 (4%). One embolization and one transient arrhythmia occurred with no sequelae. Immediate residual shunt was absent in 5/28 (18%), trivial in 15/28 (54%), small in 6/28 (21%), and moderate in 2/28 (7%). Of the 25 patients with ≥6 months follow-up (median 53 months), residual shunt was absent in 13/25 (52%), trivial in 5/25 (20%), and small in 7/25 (28%). RVE resolved in all but one patient with no other associated lesions and ≥6 months of follow-up. No patient with prior neurological event had recurrence at last follow-up., Conclusions: We conclude that closure of mASDs with ≥1 HSO is effective with a low complication rate. The ability of HSO devices to overlap or sandwich each other may facilitate safe implantation of multiple devices in smaller patients., (© 2013 Wiley Periodicals, Inc.)

Objectives: To identify complications and measures that can be undertaken to prevent complications from transhepatic central venous access., Background: Utility and safety data from a large experience of patients undergoing transhepatic venous access are lacking., Methods: The records of patients who underwent transhepatic venous access between June 2000 and October 2012 at The Center for Pediatric and Congenital Heart Disease at The Cleveland Clinic were reviewed., Results: One hundred twenty-four transhepatic procedures were performed in 81 patients. The median weight of the patients was 6.3 (2.2-94) kg at a median age of 7 months (2 weeks to 35 years). Diagnostic catheterizations were performed in 75, interventions in 39, and central venous line placement in 91 procedures. The median maximum sheath size inserted was 8 (4-14) French. There were 10 (8%) major adverse events in 8 procedures-bleeding related (n = 6 with 1 death as a result) or complete heart block with instability or requiring intervention (n = 4). Heart block persisted after the catheterization in three patients (2 days, 2 weeks, 1 month). Minor adverse events (transient heart block) occurred in 5 (4 %) procedures. Weight, age, and French size of sheath (P = 0.46, 0.84, and 0.18) were not associated with complications. In addition, time to gain access was not different between non-complicated and complicated cases (median 19.5 versus 15.1 min, P = 0.72). Diagnostic and interventional procedures were not associated with more complications compared with procedures involving central venous line placement alone (P = 0.5)., Conclusions: Transhepatic access is an extremely useful modality of access to the heart and vascular structures in some patients with complex heart disease. Prevention of complications should focus on minimizing bleeding risks and heart block., (© 2013 Wiley Periodicals, Inc.)

The purpose of the present study was to assess the results and technical aspects of attempted transcatheter recanalization of occluded pulmonary arteries or major systemic veins from our center. Occluded pulmonary arteries or major systemic veins are often not considered amenable to transcatheter treatment and can be a cause of significant hemodynamic compromise for patients. The records of patients with occluded pulmonary arteries or major systemic veins who underwent cardiac catheterization from April 1997 to February 2011 were reviewed. We identified 18 patients, of whom, 9 had occluded pulmonary arteries and 9 occluded systemic veins. Recanalization was achieved in 17 of the 18 patients (94%). At a median follow-up of 35 months (range 6 months to 12 years), all vessels remained patent. The freedom from reintervention rate in the entire cohort was 61% at 6 months, 35% at 1 year, and 35% at 5 years. Freedom from an unplanned reintervention was 67% at 6 months. Procedural adverse events occurred in 2 patients. One death occurred within 24 hours of the catheterization procedure and 2 late deaths occurred, all unrelated to the procedure. In conclusion, both acute and chronic success at recanalizing the occluded pulmonary arteries and major systemic veins can be achieved using percutaneous techniques. Careful follow-up is needed, because reintervention can be necessary to maintain long-term vessel patency without stenosis., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Background: Pediatric physicians regularly face the problem of uncertain procedural anticoagulation in children, especially in neonates. We sought to evaluate the safety, plasma concentration (pharmacokinetics, PK), pharmacodynamics (PD), and dosing guidelines of bivalirudin when used as a procedural anticoagulant in pediatric percutaneous intravascular procedures., Methods and Results: Pediatric subjects undergoing percutaneous intravascular procedures for congenital heart disease were enrolled and received the current weight-based dose used in percutaneous coronary interventions (0.75 mg/kg bolus, 1.75 mg/kg/hr infusion). Blood samples for PK/PD analyses were drawn, and safety was evaluated by monitoring bleeding and thrombosis events. A total of 110 patients (11 neonates, 33 infants, 32 young children, and 34 older children) were enrolled; 106 patients received the protocol dose. The PK/PD response of bivalirudin was predictable and behaved in a manner similar to that in adults. Weight-normalized bivalirudin clearance rates were more rapid in neonates and decreased with increasing age. Bivalirudin concentrations were slightly lower in neonates, with a trend to an increase with age. Activating clotting time response was consistent with adult studies and prolonged in all age groups, and there was reasonable correlation between activating clotting time and bivalirudin plasma concentrations across all age groups. There were few major bleeding (2 of 110, 1.8%) or thrombotic events (9 of 110, 8.2%) reported., Conclusions: PK/PD response of bivalirudin in the pediatric population is predictable and behaves in a manner similar to that in adults. Using adult dosing, bivalirudin safely provided the expected anticoagulant effect in the pediatric population undergoing intravascular procedures for congenital heart disease., (Copyright © 2010 Wiley-Liss, Inc.)

One or more breaks in the wire frame of the HELEX Septal Occluder (W.L. Gore and Associates, Flaggstaff, Arizona) were identified in 5% of patients in the multicenter pivotal trial leading to FDA approval. Fractures of the perimeter frame have not previously been associated with any apparent deleterious clinical effects. We report a case in which the broken ends of wire at a frame fracture were located in a position that resulted in perforation of the anterior mitral valve leaflet. There was significant mitral regurgitation and the device was removed surgically. The possible etiologies and prevention of wire frame fractures are also discussed., (Copyright 2009 Wiley-Liss, Inc.)

The objective of this study was to evaluate the incidence of pre-existing catheterization left pulmonary artery (LPA) gradients and correlation of these gradients with later LPA stenosis after successful patent ductus arteriosus (PDA) occlusion. We performed a single-center review of 130 patients with PDA closure from October 1993 to February 2005. We analyzed the pre-PDA closure LPA pressure gradients at catheterization to determine if these were predictive of late LPA stenosis. On follow-up, a V (max) >2 m/s by echocardiogram (transthoracic echocardiography; TTE) was considered indicative of possible LPA stenosis. Left lung perfusion of <35% was considered diagnostic of significant LPA stenosis. Post PDA closure, possible LPA stenosis by TTE was seen in 8 of 128 patients (6.25%). Seven of these eight had precatheter LPA gradients >7 mm Hg. Five of these had perfusion scans, three of the five had significant LPA stenosis, and two underwent LPA angioplasty. Patients with LPA catheter gradients >7 mm Hg were more likely to have possible LPA stenosis by TTE, significant LPA stenosis by lung scan, and intervention with LPA angioplasty. In conclusion, a preclosure main pulmonary artery-to-LPA pressure gradient >7 mm Hg was found in all patients who developed significant LPA stenosis on follow-up after transcatheter PDA closure. It appears likely that these patients have LPA abnormality rather than stenosis caused by the PDA occlusion device. Patients with preclosure LPA gradients >7 mm Hg should undergo follow-up evaluations for detection of significant stenosis and may require treatment if an important flow abnormality is documented.

Although transesophageal echocardiography is often used for guidance during transcatheter interventions, few data exist regarding the use of the newer modality of intracardiac echocardiography. This brief report summarizes our single center experience using intracardiac echocardiographic guidance during transcatheter interventional procedures for congenital heart disease.

Pediatric cardiac interventions via percutaneous common carotid artery (CCA) access have been shown to be safe and effective. However, the impact of placement of a sheath in the carotid artery for interventions on cerebral perfusion is unknown. In this study we used cerebral near-infrared spectroscopy (NIRS) to analyze the effects of percutaneous CCA access for cardiac interventions on cerebral perfusion. This study is a retrospective chart review carried out at a tertiary care center on all pediatric patients who underwent percutaneous cardiac catheterization via carotid artery access from January 2010 to January 2020. All patients who had ipsilateral NIRS recorded on the side of carotid artery access were included. Patients with only partial or no ipsilateral NIRS data were excluded. The primary outcome measure was the change in NIRS upon CCA access; the mean NIRS for 15 min before obtaining access was compared to the mean NIRS during the procedure and to the mean NIRS 15 min after removal of the CCA sheath. We hypothesized that there would be a significant drop in NIRS values on the side of CCA access. There were 48 catheterizations in the study period where percutaneous CCA was accessed. Of those, 21 catheterizations had complete data and were included in the study. 13 (62%) were of males. The median age was 23 days (IQR 7–79). The indications for CCA access were patent ductus arteriosus stent implantation (n = 13; 62%), aortic valvuloplasty (n = 5; 24%), balloon angioplasty of coarctation of aorta (n = 2; 10%), and renal artery angioplasty (n = 1; 4%). In 16 patients (72%), the left common carotid artery was accessed. The median weight of the patients was 3.3 kg (IQR 2.8–2.9). The most common sheath size used was 4F, in 16 patients (72%). The mean NIRS prior to the procedure was 67 ± 15%, during the procedure was 68 ± 20%, and after removal of sheath was 68 ± 21%. Paired t test of cerebral NIRS before, during, and after the procedure showed no significant change with CCA access (P = 0.08). No patient in the series had a documented neurologic deficit following the procedure. Percutaneous CCA access was not associated with a decrease in NIRS on the side of the access during the procedure, suggesting there was no significant acute change in cerebral perfusion with CCA access. [ABSTRACT FROM AUTHOR]

Introduction: Pulmonary atresia with the ventricular septal defect is a rare congenital heart defect with high anatomic variability. The most important management question relates to the sources of pulmonary blood flow. The ability to differentiate between ductal dependence and major aortopulmonary collateral arteries is critical to achieving good outcomes and avoiding life-threatening hypoxia in the postneonatal period. Having accurate information about pulmonary arteries, major aortopulmonary collateral arteries, and sources of blood supply to each pulmonary segment is crucial for choosing the optimal surgical strategy. The purpose of this study is to compare computed tomography angiography (CTA) with cardiac catheterization for anatomic delineation of surgically relevant anatomy in pulmonary atresia with ventricular septal defect with major aortopulmonary collateral arteries. Materials and Methods: Retrospective review of all children with pulmonary atresia with ventricular septal defect with major faortopulmonary collateral arteries cared for at a large tertiary children's hospital who underwent cardiac catheterization with angiography and CTA close to each other without interval therapy. All studies were performed between 2007 and 2011. Results: There were 9 patients who met the inclusion criteria. Pulmonary artery anatomy (confluent vs. nonconfluent) was correctly identified in 9 patients by CTA and 8 patients by catheterization. There were no significant differences between CTA and catheterization in the identification of major aortopulmonary collateral arteries (mean = 3.4 collaterals/study via catheterization; mean = 3.1 collaterals/study via CTA; P = 0.67). CTA was superior to catheterization in the delineation of segmental pulmonary blood flow (P = 0.006). Conclusion: CTA and catheterization are equivalent in their ability to delineate pulmonary artery anatomy and major aortopulmonary collateral arteries. [ABSTRACT FROM AUTHOR]

Children with homozygous familial hypercholesterolemia are at risk for early cardiovascular events secondary to coronary artery disease. Current medical therapy does not ameliorate this risk. Liver transplantation offers the most effective option to reduce circulating levels of low-density lipoprotein cholesterol and thereby reduce risk of cardiovascular events. Angiographic evidence of regression of coronary artery disease is presented. • Current therapy for homozygous FH does not result in persistently normal LDL-c levels. • Contrast cCTA may provide a reliable assessment of atherosclerosis. • Study of QOL during apheresis and after liver transplantation in children is needed. • Coronary artery atherosclerosis may regress after liver transplantation. [ABSTRACT FROM AUTHOR]

Background Interventional cardiac catheterization (cath) and electrophysiology (EP) procedures are not routinely performed together. There are several perceived barriers affecting this practice, though there are also advantages for both the patient and practitioner to a combined approach. Methods This was a single-center retrospective study reviewing combined cath and EP procedures with a preprocedural intention to intervene at Texas Children's Hospital from 2001 to 2014. We excluded procedures in which the intended procedure was purely diagnostic in nature. Results A total of 121 patients requiring 125 procedures were identified, of which 61 patients underwent 62 procedures that met our inclusion criteria. Potential subgroups of interest included adult congenital heart disease patients (26% of cohort), single ventricle anatomy (34%), and heterotaxy (19%) and collectively 58% of procedures involved a patient in one of these groups. The combined nature of the procedure did not preclude a cath or EP intervention in any patient. There were no mortalities. There were three adverse events, affecting 4.8% of procedures. Conclusions Combined interventional cardiac cath and EP procedures in pediatric patients and those with congenital heart disease can be performed safely in a high-volume center. These combined procedures save patients the risk and inconvenience of multiple procedures, and further investigation into cost savings is warranted. [ABSTRACT FROM AUTHOR]