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

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

Heart failure (HF) is common in adult congenital heart disease (ACHD) patients; however, use of continuous-flow ventricular assist devices (CF-VADs) remains rare. We reviewed outcomes of patients with congenital heart disease greater than or equal to 18 years of age at the time of CF-VAD implant at the affiliated pediatric and adult institutions between 2006 and 2020. In total, 18 ACHD patients (15 with great anatomical complexity) received 21 CF-VADs. Six patients (median age 34 years) received seven percutaneous CF-VADs with a median duration of support of 20 days (3-44 days) with all patients survived to hospital discharge and two patients were bridged to durable CF-VADs. Fourteen patients (median age 38 years) received durable CF-VADs. Thirteen patients (93%) survived to hospital discharge and the median duration of support was 25.8 months (6.4-52.1 months). Estimated survival on durable CF-VAD at 1, 3, and 5 years was 84%, 72%, and 36%, respectively. Three patients were successfully bridged to transplantation. Device-related complications include cerebrovascular accident (n = 5), driveline infection (n = 3), device infection requiring chronic antibiotic therapy (n = 4), gastrointestinal bleeding (n = 6), and presumed pump thrombosis (n = 5). These results show percutaneous and durable CF-VADs can support ACHD patients with advanced HF., Competing Interests: Disclosure: C.R.B. reports financial relationship with Medtronic Inc. H.T. reports relationship with Abbott. O.H.F. reports financial relationships with Syncardia, Medtronic Inc., and Abbott. A.Q. reports financial relationships with Medtronic and Abiomed. I.A. serves as a consultant and proctor for Berlin Heart, Inc., Medtronic Inc., Jarvik Inc., BiVACOR Inc., and Sony-Olympus Medical Solutions Inc. A.C. reports financial relationships with Medtronic Inc. and Abbott. The remaining authors have no conflicts of interest to report., (Copyright © ASAIO 2022.)

This article reviews the physiology of the ductus arteriosus, the pathophysiology of the patent ductus arteriosus (PDA), and the role advanced imaging such as computed tomography (CT) and magnetic resonance imaging (MRI) can play in guiding diagnosis and percutaneous or surgical intervention. A PDA can have variable clinical and radiologic presentations and can be important to characterize in patients with vascular rings, aortic maldevelopment and congenital heart disease. An understanding of the PDA and the application of CT and MRI can allow the radiologist to provide key information to physicians who plan to close a PDA or maintain PDA patency in the setting of ductal-dependent congenital heart disease., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Pulmonary vein atresia (PVA) may lead to pulmonary hypertension, cardiac failure, and death. Transcatheter or surgical treatments have rarely been offered to this population because of perceived poor outcomes. We describe single center outcomes of transcatheter management of PVA. Retrospective chart review of PVA patients who underwent cardiac catheterization at a single tertiary center. Sixty patients underwent catheterization for evaluation of PVA from 1995 to 2019. The age at the initial catheterization was 1.6 (0.7, 5.97) years. Two thirds of PVA patients had associated congenital heart disease (n=40). PVA recanalization was attempted in 34 patients, successful in 23/34 (68%) of the initial attempts. 3/23 (13%) underwent balloon angioplasty alone, and 20/23 (87%) received drug-eluting stents, with no procedural mortalities. 22/23 patients had transcatheter reinterventions during an interval of 2.1 (0.3, 5.1) years. Right ventricular systolic to aortic systolic pressure ratio (in biventricular patients) at the index catheterization was 0.45 (0.34, 0.68) in survivors versus 0.69 (0.54, 0.83) in those who died; P = 0.012 (n=45). The baseline right ventricular or pulmonary artery systolic to aortic systolic pressure ratio of ≥0.54 at the initial catheterization was predictive of mortality. We hereby demonstrate that transcatheter recanalization of PVA with placement of drug-eluting stents can be performed safely with acceptable success rate. With appropriate use of re-interventions for restenosis as indicated, PVA can be successfully palliated with good long-term patency and distal growth of the affected veins. Pulmonary hypertension is a risk factor for mortality in patients with PVA and biventricular circulation. Percutaneous recanalization of PVA is safe and feasible, and with placement of drug-eluting stents, carries a high acute success rate and results in growth of the distal pulmonary veins. However, close vigilance and reinterventions are required due to restenosis., (Copyright © 2022. Published by Elsevier Inc.)

Background: The modified Blalock-Taussig-Thomas shunt is a critically important palliation for patients with insufficient pulmonary blood flow associated with congenital heart disease. After creating a modified Blalock-Taussig-Thomas shunt patients experience high rates of early postoperative morbidity and mortality., Methods: This is a single-institution retrospective cohort study. A query of The Society of Thoracic Surgeons database identified relevant patients whose health records were manually queried for echocardiography and operative reports. Patients with ductal-dependent systemic circulation were excluded. Primary outcomes were early serious adverse events and in-hospital mortality. Secondary outcomes were time to primary outcomes and postoperative lengths of stay. We investigated the correlation of demographics, presence of competitive pulmonary blood flow, and surgical and anatomic factors on outcomes., Results: After exclusions our cohort comprised 155 patients. Thirty-three patients (21.3%) experienced an early serious adverse event, 10 (6.5%) early shunt malfunction, and 11 (7.1%) in-hospital mortality. Smaller shunt size, smaller shunted pulmonary artery size, surgical approach, and site of proximal shunt anastomosis were independently associated with morbidity and mortality., Conclusions: Anatomic elements imparting increased resistance along the modified Blalock-Taussig-Thomas shunt predispose to increased morbidity and mortality, particularly in the early postoperative period. Despite the significant heterogeneity of patients receiving such shunts, similar risk profiles are observed regardless of lesion or presence of competitive flow. A surgical approach using thoracotomy with shunt anastomosis to the subclavian artery, where feasible, results in the subclavian artery as the point of natural resistance, allowing for placement of larger shunts and yielding lower morbidity and mortality., (Copyright © 2022 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Isolated pulmonary artery (PA) of ductal origin (IPADO) is a rare cardiac defect which requires surgical repair, with or without preceding palliation. We sought to determine the impact of treatment strategy on outcomes. Retrospective study of consecutive patients with IPADO that underwent staged or primary repair from 1/05 to 9/16 at 6 Congenital Cardiac Research Collaborative centers. Patients with single ventricle physiology, major aortopulmonary collaterals, or bilateral IPADO were excluded. Primary outcome was isolated PA z-score at late follow-up. Secondary outcomes included PA symmetry index (isolated:confluent PA diameter) and reintervention burden. Propensity score adjustment was used to account for baseline differences. Of 60 patients in the study cohort, 26 (43%) underwent staged and 34 (57%) primary repair. The staged and primary repair groups differed in weight at diagnosis and presence of other heart disease but not in baseline PA dimensions. Staged patients underwent ductal stent (n = 16) or surgical shunt (n = 10) placement followed by repair at 210 vs. 21 days in the primary repair group (p < 0.001). At median follow-up of 4.5 years post-repair, after adjustment, isolated PA z-score (- 0.74 [- 1.75, - 0.26] vs. - 1.95 [- 2.91, - 1.59], p = 0.012) and PA symmetry index (0.81 [0.49, 1.0] vs. 0.55 [0.48, 0.69], p = 0.042) significantly favored the staged repair group. Freedom from PA reintervention was not different between groups (adjusted HR 0.78 [0.41, 1.48]; p = 0.445). A staged approach to repair of IPADO is associated with superior isolated PA size and symmetry at late follow-up. Consideration should be given to initial palliation in IPADO patients, when feasible.

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: Pulmonary atresia with intact ventricular septum (PAIVS) with right ventricle-dependent coronary circulation (RVDCC) carries suboptimal outcomes primarily due to cardiac ischemia. We hypothesize clinical outcomes are affected by the level of coronary obstruction, a surrogate for vulnerable myocardium., Methods: We conducted a single-institution retrospective analysis of all neonates with PAIVS with RVDCC from 1995 to 2017. RVDCC was defined as the presence of any coronary-cameral fistula with coronary obstruction proximal to the fistula and angiographic evidence of RV perfusion of the myocardium through the fistulous communication. Location of coronary obstruction was categorized as either proximal or distal segments, using the SYNTAX score criteria. Transplant-free survival was compared between patients with proximal and distal obstruction, then these groups were compared with patients without RVDCC., Results: Of 103 neonates with PAIVS, 28 (27%) had RVDCC: 18 proximal (64%), 10 distal (36%). Median age at last follow-up for patients with RVDCC was 1.8 years (interquartile range, 0.3-8.1 years). All deaths (10 of 28, 36%) occurred at 6 months old or earlier. Proximal coronary artery obstruction was associated with decreased transplant-free survival relative to distal obstruction (hazard ratio = 3.63; 95% confidence interval, 1.01-13.00; P = .048). Transplant-free survival at 1 year was 33% and 70% in the proximal and distal obstruction groups, respectively. Compared with patients without RVDCC, patients with proximal obstruction had significantly lower transplant-free survival (P < .001), whereas patients with distal obstruction did not (P = .217)., Conclusions: The location of coronary artery obstruction affects clinical outcome and may represent a potential branch point in the management for PAIVS with RVDCC., (Copyright © 2020 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

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.

We present a case of temporary guidewire pacing in a patient with Fontan anatomy during transcatheter aortic valve implantation. Temporary pacing was successfully achieved utilizing this method without complications. There is an increasing population of patients with complex congenital heart disease and expanding variety of transcatheter interventions. Due to limitations in vascular access and surgical anatomies, guidewire pacing may have a wide array of potential applications in pediatrics and the congenital heart disease population., (© 2019 Wiley Periodicals, Inc.)

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.)

Background: We reviewed our single institutional experience with pediatric ventricular assist device (VAD) support over the last 2 decades, with an aim to improve our current management and gain an insight into the future direction., Methods: A retrospective review was conducted on all patients that had undergone VAD support between 1996 and 2017. Outcomes were analyzed based on the type of VADs, whether temporary or durable devices. Primary end points were positive outcomes, including bridge-to-transplantation, bridge-to-recovery, alive on device, and bridge-to-bridge to another VAD, or negative outcomes, including death during VAD support or in-hospital death after bridge-to-recovery. The Pediatric Interagency Registry for Mechanical Circulatory Support definition was used to classify adverse events., Results: Overall, 201 VADs were implanted in 159 patients, with 82 (41%) and 119 (59%) being temporary and durable support, respectively. There has been a trend toward an increasing annual implant volume both with temporary and durable VADs. Positive outcomes were achieved in 80% (66 of 82) of those with temporary support, with bridge-to-recovery (53% [35 of 66]) and bridge-to-bridge to another VAD (38% [25 of 66]) being the predominant outcomes. Of those on durable support, 84% (100 of 119) achieved positive outcomes, with bridge-to-transplant (66% [78 of 119]) being the leading destination. The most notable change during the study period was the introduction of implantable continuous-flow VADs, resulting in outpatient management becoming a routine practice. No patients were discharged on VAD support before 2004, but 85% were discharged on VADs with discharge capability after 2013., Conclusions: The present study has demonstrated the evolutional changes of pediatric VAD support and their effect on clinical outcomes over the last 2 decades., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

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: Transcatheter right ventricle decompression in neonates with pulmonary atresia and intact ventricular septum is technically challenging, with risk of cardiac perforation and death. Further, despite successful right ventricle decompression, re-intervention on the pulmonary valve is common. The association between technical factors during right ventricle decompression and the risks of complications and re-intervention are not well described., Methods: This is a multicentre retrospective study among the participating centres of the Congenital Catheterization Research Collaborative. Between 2005 and 2015, all neonates with pulmonary atresia and intact ventricular septum and attempted transcatheter right ventricle decompression were included. Technical factors evaluated included the use and characteristics of radiofrequency energy, maximal balloon-to-pulmonary valve annulus ratio, infundibular diameter, and right ventricle systolic pressure pre- and post-valvuloplasty (BPV). The primary end point was cardiac perforation or death; the secondary end point was re-intervention., Results: A total of 99 neonates underwent transcatheter right ventricle decompression at a median of 3 days (IQR 2-5) of age, including 63 patients by radiofrequency and 32 by wire perforation of the pulmonary valve. There were 32 complications including 10 (10.5%) cardiac perforations, of which two resulted in death. Cardiac perforation was associated with the use of radiofrequency (p=0.047), longer radiofrequency duration (3.5 versus 2.0 seconds, p=0.02), and higher maximal radiofrequency energy (7.5 versus 5.0 J, p<0.01) but not with patient weight (p=0.09), pulmonary valve diameter (p=0.23), or infundibular diameter (p=0.57). Re-intervention was performed in 36 patients and was associated with higher post-intervention right ventricle pressure (median 60 versus 50 mmHg, p=0.041) and residual valve gradient (median 15 versus 10 mmHg, p=0.046), but not with balloon-to-pulmonary valve annulus ratio, atmospheric pressure used during BPV, or the presence of a residual balloon waist during BPV. Re-intervention was not associated with any right ventricle anatomic characteristics, including pulmonary valve diameter., Conclusion: Technical factors surrounding transcatheter right ventricle decompression in pulmonary atresia and intact ventricular septum influence the risk of procedural complications but not the risk of future re-intervention. Cardiac perforation is associated with the use of radiofrequency energy, as well as radiofrequency application characteristics. Re-intervention after right ventricle decompression for pulmonary atresia and intact ventricular septum is common and relates to haemodynamic measures surrounding initial BPV.

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.)

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., (© 2017 Wiley Periodicals, Inc.)

Background: Outcomes after right ventricle (RV) decompression in infants with pulmonary atresia with intact ventricular septum vary widely. Descriptions of outcomes are limited to small single-center studies., Methods and Results: Neonates undergoing RV decompression for pulmonary atresia with intact ventricular septum were included from 4 pediatric centers. Primary end point was reintervention post-RV decompression; secondary end points included circulation type at latest follow-up. Ninety-nine patients (71 with pulmonary atresia with intact ventricular septum and 28 with virtual atresia) underwent RV decompression at median 3 (25th-75th, 2-5) days of age. Seventy-one patients (72%) underwent at least 1 reintervention after decompression. Median duration of follow-up was 3 years (range, 1-10). Freedom from reintervention was 51% at 1 month and 23% at 3 years. In multivariable analysis, reintervention was associated with virtual atresia (hazard ratio [HR], 0.51; 95% confidence interval [CI], 0.28-091; P =0.027), smaller RV length (HR, 0.94; 95% CI, 0.89-0.99; P =0.027), and ≤mild tricuspid regurgitation (TR; HR, 3.58; 95% CI, 2.04-6.30; P <0.001). Patients undergoing surgical shunt or ductal stent were less likely to have virtual atresia (HR, 0.36; 95% CI, 0.15-0.85; P =0.02) and more likely to have higher RV end-diastolic pressure (HR, 1.07; 95% CI, 1.00-1.15; P =0.057) and ≤mild TR (HR, 3.50; 95% CI, 1.75-7.0; P <0.001). Number of reinterventions was associated with ≤mild TR (rate ratio, 1.87; 95% CI, 1.23-2.87; P =0.0037). Multivariable analysis indicated that <2-ventricle circulation status was associated with ≤mild TR (odds ratio, 18.6; 95% CI, 5.3-65.2; P <0.001) and lower RV area (odds ratio, 0.81; 95% CI, 0.72-0.91; P <0.001)., Conclusions: Patients with pulmonary atresia with intact ventricular septum deemed suitable for RV decompression have a high reintervention burden although most achieve 2-ventricle circulation. TR ≤mild at baseline is strongly associated with reintervention and <2-ventricle circulation at medium-term follow-up. Degree of baseline TR may be an important marker of long-term outcomes in this population., (© 2017 American Heart Association, Inc.)

Objectives: To describe the early multi-center, clinical experience with the Medtronic Micro Vascular Plug™ (MVP) in children with congenital heart disease (CHD) undergoing vascular embolization., Background: The MVP is a large diameter vascular occlusion device that can be delivered through a microcatheter for embolization of abnormal blood vessels., Methods: A retrospective review of embolization procedures using the MVP in children with CHD was performed in 3-centers. Occlusion of patent ductus arteriosus using the MVP was not included., Results: Ten children underwent attempted occlusion using the MVP. The most common indication to use the MVP was failed attempted occlusion using other embolic devices. Five, single ventricle patients (median age 3-years, median weight 14.9 kg) underwent occlusion of veno-venous collaterals following bidirectional Glenn operation. Three patients (Median age 8 years) underwent occlusion of coronary artery fistulae (CAF). Two patients (age 7 months and 1 year) underwent occlusion of large aorto-pulmonary collaterals. A 7-day-old child with a large CAF required 2 MVPs and an Amplatzer Vascular Plug (AVP-II) for complete occlusion. Occlusion of all other blood vessels was achieved using a single MVP. One MVP embolized distally in an 8-years-old child with a large CAF. There were no other procedural complications or during follow-up (median 9 months)., Conclusions: The MVP is a new, large-diameter vascular embolization device that can be delivered through a microcatheter. It may play an important role in providing highly effective occlusion of abnormal vessels in children., (© 2017, Wiley Periodicals, Inc.)

Background: Percutaneous pulmonary valve implantation (PPVI) is utilized for dysfunctional right ventricular outflow tracts (RVOT) to relieve obstruction, regurgitation, and RV dysfunction. PPVI has not been reported to induce arrhythmias. This study is the first to report the incidence of ventricular tachycardia (VT) after PPVI., Methods: This was a retrospective study of all patients who had PPVI at a single institution. All patients were admitted after PPVI for overnight telemetry monitoring. Patients with no prior history of VT and newly detected VT within 24 hours post-PPVI were considered to have VT related to PPVI., Results: In total, 79 patients had PPVI (age 17 ± 9 years, 66% tetralogy of Fallot/pulmonary atresia). PPVI-related VT was detected in 6 patients (7.5%). These patients had a lower BMI (17.5 ± 2.0 vs. 23.1 ± 6.6, P = 0.04). There was no difference in age, native conduit or percutaneous valve size, or change in the minimum diameter of the RVOT from pre- to post-PPVI. In the 6 patients, VT was non-sustained and monomorphic at rates between 120 and 170. Five started on β-blockers. No patient required surgical explantation of the valve. Over a median follow-up of 2 years, 4 have weaned off medications and all are free of recurrence of VT with normal Holters., Conclusions: PPVI may be associated with transient VT in the acute peri-procedural period. Patients of smaller size may be more susceptible. All patients were managed conservatively and none of the patients had a recurrence, which is suggestive of a transient phenomenon., (© 2016, 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.)

Objective: Critically ill children with cardiac disease often require prolonged central venous access. Thrombosis of systemic veins or the need to preserve vessels for future cardiac procedures limits sites for placement of central venous catheters in these patients. This study evaluates the use of Broviac placement via the transhepatic approach for this patient population., Design: A retrospective review., Setting: A tertiary care center., Patients: All children with complex congenital heart disease who underwent transhepatic Broviac placement between May 2000 and April 2004., Interventions: Transhepatic Broviac placement., Measurements and Main Results: Thirty-two children with a median age of 5 months (20 days-5.3 yrs) and a median weight of 4.2 kg (2.2-24.9 kg) underwent 40 transhepatic Broviac placements. There were three (8.8%) procedural-related complications. One patient suffered an intra-abdominal bleed requiring an urgent laparotomy and removal of the Broviac, one patient required transfusion because of a mild self-contained intra-abdominal bleed, and one patient developed temporary complete heart block. There was one catheter infection. Thrombus was noted by echocardiography on the tip of two Broviacs; however, no intracardiac vegetations or embolic events occurred. There was no mortality related to the procedure. Broviacs remained in place for a median of 36 days (1 day-6 months). Five Broviacs were dislodged inadvertently (two during cardiac massage and three resulting from patient manipulation). The remaining Broviacs were electively removed safely without coil embolization. At a median follow-up of 3.5 months (10 days-3 yrs), there have been no long-term complications related to the Broviacs., Conclusions: Transhepatic Broviac catheters can be used safely in critically ill children with cardiac disease and remain indwelling for adequate periods of time. This modality of prolonged vascular access should be considered for children whose veins are occluded or need to be preserved for future procedures.

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.