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

The COVID-19 pandemic has posed tremendous stress on the health care system. Its effects on pediatric/congenital catheterization program practice and performance have not been described.The purpose of this study was to evaluate how case volumes, risk-profile, and outcomes of pediatric/congenital catheterization procedures changed in response to the first wave of COVID-19 and after that wave.A multicenter retrospective observational study was performed using Congenital Cardiac Catheterization Project on Outcomes Registry (C3PO) data to study changes in volume, case mix, and outcomes (high-severity adverse events [HSAEs]) during the first wave of COVID (March 1, 2020, to May 31, 2020) in comparison to the period prior to (January 1, 2019, to February 28, 2020) and after (June 1, 2020, to December 31, 2020) the first wave. Multivariable analyses adjusting for case type, hemodynamic vulnerability, and age group were performed. Hospital responses to the first wave were captured with an electronic study instrument.During the study period, 12,557 cases were performed at 14 C3PO hospitals (with 8% performed during the first wave of COVID and 32% in the postperiod). Center case volumes decreased from a median 32.1 cases/month (IQR: 20.7-49.0 cases/month) before COVID to 22 cases/month (IQR: 13-31 cases/month) during the first wave (Despite increased case-mix complexity, C3PO programs maintained, if not improved, their performance in terms of HSAE. Exploratory analyses of practice changes may inform future harm-reduction efforts.

Background:Recurrent laryngeal nerve injury leading to vocal cord paralysis is a known complication of cardiothoracic surgery. Its occurrence during interventional catheterisation procedures has been documented in case reports, but there have been no studies to determine an incidence.Objective:To establish the incidence of left recurrent laryngeal nerve injury leading to vocal cord paralysis after left pulmonary artery stenting, patent ductus arteriosus device closure and the combination of the procedures either consecutively or simultaneously.Methods:Members of the Congenital Cardiovascular Interventional Study Consortium were asked to perform a retrospective analysis to identify cases of recurrent laryngeal nerve injury after the aforementioned procedures. Twelve institutions participated in the analysis. They also contributed the total number of each procedure performed at their respective institutions for statistical purposes.Results:Of the 1337 patients who underwent left pulmonary artery stent placement, six patients (0.45%) had confirmed vocal cord paralysis. 4001 patients underwent patent ductus arteriosus device closure, and two patients (0.05%) developed left vocal cord paralysis. Patients who underwent both left pulmonary artery stent placement and patent ductus arteriosus device closure had the highest incidence of vocal cord paralysis which occurred in 4 of the 26 patients (15.4%). Overall, 92% of affected patients in our study population had resolution of symptoms.Conclusion:Recurrent laryngeal nerve injury is a rare complication of left pulmonary artery stent placement or patent ductus arteriosus device closure. However, the incidence is highest in patients undergoing both procedures either consecutively or simultaneously. Additional research is necessary to determine contributing factors that might reduce the risk of recurrent laryngeal nerve injury.

In children with single ventricle physiology, the Glenn procedure is performed to redirect venous return from the superior vena cava directly to the pulmonary arteries and route venous return from the inferior vena cava exclusively to the systemic circulation. Although this surgery successfully palliates the hemodynamic stress experienced by the single ventricle, patients frequently develop pulmonary arteriovenous malformations (PAVMs). Interestingly, PAVMs may regress upon rerouting of hepatic venous effluent to the pulmonary vasculature, suggesting the presence of a circulating “hepatic factor” that is required to prevent PAVMs. Here, we test the hypothesis that hepatic factor is bone morphogenetic protein 9 (BMP9) and/or BMP10. These circulating ligands are produced by the liver and activate endothelial endoglin (ENG)/ALK1 signaling, and mutations in ENG and ALK1 cause hereditary hemorrhagic telangiectasia, a genetic disease associated with AVM development. However, we found no within-subject variation in BMP9, BMP10, or BMP9/10 plasma concentrations when sampled from five cardiovascular sites, failing to support the idea that the Glenn would limit access of these ligands to the lung vasculature. Unexpectedly, however, we found a significant decrease in all three ligand concentrations in Glenn cases versus controls. Our findings suggest that BMP9/BMP10/ENG/ALK1 signaling may be decreased in the Glenn vasculature but fail to implicate these ligands as hepatic factor.

Background: The C3PO-QI (Congenital Cardiac Catheterization Project on Outcomes – Quality Improvement), a multicenter registry launched in 2015, instituted quality improvement (QI) initiatives to reduce patient radiation exposure. Through regular collaboration, this initiative would allow for harmony among active participants, maximizing efforts and efficiency at achieving radiation best practices. This study sought to report these efforts with a detailed methodology for which institutions can target initiatives, reducing radiation exposure, and increasing patient safety. Methods: Data were collected prospectively by 8 C3PO-QI institutions between January 1, 2015 and December 31, 2017. Radiation exposure was measured in dose area product per body weight (dose area product/kg; µGy*m 2 /kg) and reported by expected radiation exposure categories (REC) and institution for 40 published unique procedure types. Targeted interventions addressing selected strategic domains for radiation reduction were implemented in the pediatric catheterization labs of the C3PO-QI institutions. Results: The study consisted of 15 257 unique cases. Median exposure (dose area product/kg) was decreased by 30% for all procedures. Dose area product/kg was reduced in all 3 REC, with the greatest improvement observed in REC I (REC I, −37%; REC II, −23%; REC III, −27%). Although the baseline radiation exposures and exact percent decrease varied across all C3PO-QI sites, each institution demonstrated improvements in radiation dose over time. These improvements occurred with the implementation of institution-specific QI interventions accelerated by participation in the C3PO-QI multicenter collaborative. Conclusions: Substantial radiation dose reductions can be achieved using targeted QI methodology and interventions. Participation in a multicenter QI collaborative may accelerate improvement across all centers due to enhanced engagement and shared learning between sites.

Objectives To describe acute and mid-term results of hybrid perventricular device closure of muscular ventricular septal defects (mVSDs). Background Perventricular device closure of mVSDs can mitigate technical limitations of percutaneous closure and need for cardiopulmonary bypass or ventriculotomy with a surgical approach. Methods This is a multicenter retrospective cohort study of patients undergoing hybrid perventricular mVSD device closure from 1/2004 to 1/2014. Procedural details, adverse events, outcomes, and follow-up data were collected. Patients were divided into two groups: (1) simple (mVSD closure alone) and (2) complex (mVSD closure with concomitant cardiac surgery). Results Forty-seven patients (60% female) underwent perventricular mVSD device closure at a median age of 5.2 months (IQR 1.8–8.9) and weight of 5.1 kg (IQR 4.0–6.9). Procedural success was 91% [100% (n = 22) simple and 84% (n = 21/25) complex]. Adverse events occurred in 19% (9/47) [9% (2/22) simple and 28% (7/25) complex]. Hospital length of stay (LOS) was shorter in the simple vs. complex group (4 vs. 14 days, P

Right ventricular to pulmonary artery (RV-PA) conduits are utilized in the surgical palliation of many forms of congenital heart disease. Catheter-based intervention can prevent the need for certain surgical procedures, however comes at the risk of conduit injury. There are multiple methods for managing conduit tears percutaneously; however, the size of the conduit, the location of the tear, and the size of the patient all determine what catheter-based procedures can be attempted. We present two cases of confined conduit tears formed during RV-PA conduit intervention successfully treated with coil embolization. To our knowledge, these are the first reports of coil embolization to treat confined conduit tears, demonstrating an alternative catheter-based treatment option.

Objectives This study sought to update benchmark values to use a quality measure prospectively. Background Congenital Cardiac Catheterization Outcomes Project – Quality Improvement (C3PO-QI), a multi-center registry, defined initial radiation dose benchmarks retrospectively across common interventional procedures. These data facilitated a dose metric endorsed by the American College of Cardiology in 2014. Methods Data was collected prospectively by 9 C3PO-QI institutions with complete case capture between 1/1/2014 and 6/30/2015. Radiation was measured in total air kerma (mGy), dose area product (DAP) (µGy*M2), DAP per body weight, and fluoroscopy time (min), and reported by age group as median, 75th and 95th%ile for the following six interventional procedures: (1) atrial septal defect closure; (2) aortic valvuloplasty; (3) treatment of coarctation of the aorta; (4) patent ductus arteriosus closure; (5) pulmonary valvuloplasty; and (6) transcatheter pulmonary valve implantation. Results The study was comprised of 1,680 unique cases meeting inclusion criteria. Radiation doses were lowest for pulmonary valvuloplasty (age 15 yrs, median mGy: 1835, DAP: 17990). DAP/kg standardized outcome measures across weights within an age group and procedure type significantly more than DAP alone. Radiation doses decreased for all procedures compared to those reported previously by both median and median weight-based percentile curves. These differences in radiation exposure were observed without changes in median fluoroscopy time. Conclusions This study updates previously established benchmarks to reflect QI efforts over time. These thresholds can be applied for quality measurement and comparison. © 2017 Wiley Periodicals, Inc.

To stratify diverse procedure types into categories with similar radiation exposure in cardiac catheterization for congenital heart disease. Radiation exposures for a comprehensive list of specific procedure types and stratification of outcomes based on radiation risk are not currently available. Data between January 2014 and December 2015 were collected on all cases performed at sites participating in C3PO-QI (Congenital Cardiac Catheterization Outcomes Project-Quality Improvement Initiative) and 9 centers were included. Using expert consensus, 40 unique procedure types were defined by diagnostic characteristics or the intervention(s) performed, and dose area product (DAP) per kilogram of body weight (µGy × m2/kg) was summarized. Using empiric and consensus methods, three radiation risk categories were created. A total of 11,735 cases were included for analysis. Thirteen (n = 7918) procedure types with median DAP/kg

Objective The objective of this study was to investigate variation in practice surrounding elective cardiac catheterization in patients with single-ventricle (SV) congenital heart disease. Design Patient and procedural characteristics and outcomes during SV catheterization were collected prospectively from eight centers using a web-based registry (Congenital Cardiac Catheterization Project on Outcomes). We attempted to identify a population of elective procedures by limiting the cohort in case type and timing. Cases were then stratified by stage of SV palliation (pre-bidirectional cavopulmonary anastomosis [pre-BCPA], pre-Fontan and post-Fontan) and limited by age. Subcohort analysis was performed by mode of airway management (assisted vs. spontaneous ventilation). Institutional variation was assessed. Results Between 2/2007 and 6/2010, 1459 (10.1%) of 14 467 cases in the registry met the inclusion and exclusion criteria, including 326 pre-BCPA, 571 pre-Fontan and 562 post-Fontan procedures. Median patient age was 0.4 (interquartile range 0.3, 0.5), 2.6 (1.0, 3.4) and 9.6 (5.2, 15.4) years and weight was 5.6 (4.8, 6.4), 12.2 (10.5, 14), and 26.3 (16.6, 51.8) kg in the pre-BCPA, pre-Fontan and post-Fontan cohorts, respectively. Cases were more commonly diagnostic in the pre-BCPA cohort (57%) whereas they were more commonly interventional in the pre-Fontan (69%) and post-Fontan (77%) cohorts. At least one adverse event (AE) occurred in 210 cases (14.4%) overall, including 20% of pre-BCPA, 11% of pre-Fontan and 14% of post-Fontan catheterizations. Mode of airway management was associated with statistically significant, but clinically small differences in hemodynamic measures in the pre- and post-Fontan cohorts, but not in the pre-BCPA group. Considerable practice variation exists across centers with variability in airway management, AE rate, case type, interventions performed and fluoroscopy time, in all SV cohorts. Conclusions Elective catheterization in SV patients, frequently performed with intervention, is common among US congenital cardiac centers. However, important variation in practice exists during these procedures in SV patients, at all stages of palliation.

Objective To describe contemporary outcomes of balloon aortic valvuloplasty (BAVP) performed in 22 US centers. Background: BAVP constitutes first-line therapy for congenital aortic stenosis (cAS) in many centers. Methods We used prospectively-collected data from two active, multi-institutional, pediatric cardiac catheterization registries. Acute procedural success was defined, for purposes of this review, as a residual peak systolic gradient ≤ 35 mm Hg and no more than mild aortic regurgitation (AR) for patients with isolated cAS. For patients with mixed aortic valve disease, a residual peak systolic gradient ≤ 35 mm Hg without worsening of AR was considered successful outcome. Results In 373 patients with a median age of 8 months (1 day to 40 years of age) peak systolic gradient had a median of 59 [50, 71] mm Hg pre-BAVP and 22 [15, 30] mm Hg post-BAVP (P

Background: Venoarterial extracorporeal membrane oxygenation (VA-ECMO) provides respiratory and hemodynamic support to pediatric patients in severe cardiac failure. We aim to identify risk factors associated with poorer outcomes in this population. Methods: A retrospective chart review was conducted of pediatric patients requiring VA-ECMO support for cardiac indications at our institution from 2004 to 2015. Data were collected on demographics, indication, markers of cardiac output, ventricular assist device (VAD) insertion, heart transplantation, or left atrial (LA) decompression. Univariate Cox proportional hazards models were used to calculate hazard ratios (HRs) for variables associated with the composite primary outcome of transplant-free survival (TFS). Results: Of the 68 reviewed patients, 65% were male, 84% were white, 38% had a prior surgery, 13% had a prior transplant, 10% had a prior ECMO support, and 87.5% required vasoactive support within six hours of cannulation. The ECMO indications included congenital heart disease repaired >30 days prior (12%), cardiomyopathy (41%), posttransplant rejection (7%), and cardiorespiratory failure (40%). The TFS was 54.5% at discharge and 47.7% at one year. Predictors of transplant and/or death include epinephrine use (hazard ratio [HR] = 2.269, P = .041), elevated lactate (HR = 1.081, P = 0005), and elevated creatinine (HR = 1.081, P = .005) within six hours prior to cannulation. Sixteen (23.6%) patients underwent LA decompression. Placement of VAD occurred in 16 (23.5%) patients, for which nonwhite race (HR = 2.94, P = .034) and prior ECMO (HR = 3.42, P = .053) were the only identified risk factors. Conclusions: Need for VA-ECMO for cardiac support carries high inpatient morbidity and mortality. Epinephrine use and elevated lactate and creatinine were associated with especially poor outcomes. Patients who survived to discharge had good short-term follow-up results.

Background Hypoplastic left heart syndrome and other cardiac lesions with ductal-dependent systemic circulation continue to be challenging to manage, especially in high-risk (HR) populations (those with prematurity, multiple congenital anomalies, moderate to severe tricuspid regurgitation, hemodynamic instability, intact atrial septum). Methods A retrospective study on our institution's experience implementing a hybrid strategy as initial palliation in HR patients with ductal-dependent systemic circulation in HR patients undergoing Norwood versus hybrid procedure. From July 2004 to May 2008, 16 HR patients underwent stage I Norwood procedure. After implementation of a hybrid strategy in 2008, 24 HR patients underwent hybrid procedure from May 2008 to November 2015. Results There was no difference in gestational age, age at procedure, or hospital length of stay. The HR Hybrid group had lower mean weight (2.6 kg vs 3.1 kg, p = 0.026). Thirty-day mortality was lower in the HR Hybrid group (4% vs 31%, p = 0.019), although there was no difference in interstage mortality (17% vs 9%, p = 0.396). Catheter-based reintervention was more prevalent in the HR Hybrid group, but did not have a negative impact on survival. One-year transplant-free survival was similar ( p = 0.416). HR Hybrid patients weighing less than 2.6 kg had higher overall survival (83% vs 25%, p = 0.013), as did patients who were premature (70% vs 0%, p = 0.003). Conclusions In high-risk patients, the hybrid procedure appears to have lower 30-day mortality and may have a survival benefit in premature patients and those less than 2.6 kg. Long-term attrition in this high-risk population is ongoing regardless of early strategy.

Background: Secundum atrial septal defect (ASD) closure devices were granted approval based on industry-sponsored, prospective, nonrandomized, single device studies, demonstrating acceptable efficacy and safety in selected patients. We sought to report community practice and outcomes. Methods and Results: Procedure specific data was collected on cases considered for ASD closure in the congenital cardiac catheterization project on outcomes (C3PO) between February 1, 2007 and June 31, 2010. Eight centers contributed data during this time period. All adverse events (AE) were independently reviewed and classified by a five level severity scale. In 40 months (2/07–6/10), 653 of 688 ASDs were occluded with a single device using an AMPLATZER® Septal Occluder (ASO) in 566 (87%), GORE® HELEX® Septal Occluder (HSO) in 33 (5%), and a CardioSEAL® or STARFlex™ device (CSD) in 54 (8%). Most patients had an isolated ASD (93%). 85% were >2 years of age. The ASD median diameter was 12 mm [8,16] for ASO, with smaller diameters in HSO 8 mm [7,10] and CSD 8 mm [5,10] (P

To describe acute and mid-term results of hybrid perventricular device closure of muscular ventricular septal defects (mVSDs).Perventricular device closure of mVSDs can mitigate technical limitations of percutaneous closure and need for cardiopulmonary bypass or ventriculotomy with a surgical approach.This is a multicenter retrospective cohort study of patients undergoing hybrid perventricular mVSD device closure from 1/2004 to 1/2014. Procedural details, adverse events, outcomes, and follow-up data were collected. Patients were divided into two groups: (1) simple (mVSD closure alone) and (2) complex (mVSD closure with concomitant cardiac surgery).Forty-seven patients (60% female) underwent perventricular mVSD device closure at a median age of 5.2 months (IQR 1.8-8.9) and weight of 5.1 kg (IQR 4.0-6.9). Procedural success was 91% [100% (n = 22) simple and 84% (n = 21/25) complex]. Adverse events occurred in 19% (9/47) [9% (2/22) simple and 28% (7/25) complex]. Hospital length of stay (LOS) was shorter in the simple vs. complex group (4 vs. 14 days, P 0.01). At mid-term follow-up of 19.2 months (IQR 2.3-43) 90% of pts had complete mVSD closure; none developed late heart block, increased atrioventricular (AV) valve insufficiency or ventricular dysfunction.Perventricular device closure of simple mVSD was associated with a high rate of procedural success, few adverse events, and short hospital LOS. Procedural adverse events were associated with the presence of concomitant complex surgery. Residual mVSD, AV valve insufficiency, or ventricular dysfunction were uncommon at mid-term follow-up. © 2017 Wiley Periodicals, Inc.

This study sought to update benchmark values to use a quality measure prospectively.Congenital Cardiac Catheterization Outcomes Project - Quality Improvement (C3PO-QI), a multi-center registry, defined initial radiation dose benchmarks retrospectively across common interventional procedures. These data facilitated a dose metric endorsed by the American College of Cardiology in 2014.Data was collected prospectively by 9 C3PO-QI institutions with complete case capture between 1/1/2014 and 6/30/2015. Radiation was measured in total air kerma (mGy), dose area product (DAP) (µGy*MThe study was comprised of 1,680 unique cases meeting inclusion criteria. Radiation doses were lowest for pulmonary valvuloplasty (age1 yrs, median mGy: 59, DAP: 249) and highest in transcatheter pulmonary valve implantation (age15 yrs, median mGy: 1835, DAP: 17990). DAP/kg standardized outcome measures across weights within an age group and procedure type significantly more than DAP alone. Radiation doses decreased for all procedures compared to those reported previously by both median and median weight-based percentile curves. These differences in radiation exposure were observed without changes in median fluoroscopy time.This study updates previously established benchmarks to reflect QI efforts over time. These thresholds can be applied for quality measurement and comparison. © 2017 Wiley Periodicals, Inc.

Background Balloon pulmonary valvuloplasty (BPV) is the treatment of choice for patients with pulmonary valve stenosis (PS); however, safety and efficacy outcomes are lacking in the current era. Methods Demographic, procedural, and adverse event (AE) data were prospectively collected using a multicenter registry (C3PO) and cases performed between 02/07 and 06/10 at eight institutions. The registry was queried for cases of isolated BPV. Multivariable models were built to determine risk factors for procedure failure and adverse outcomes. Results 211 cases were included (45%

Background: Controversy exists regarding the safety of cardiac catheterizations, particularly interventions, in the early post-operative period following congenital heart surgery. We sought to determine if adverse events are more frequent in patients undergoing early post-operative catheterizations

Objectives This study sought to evaluate the outcome of maternal autoantibody-mediated fetal cardiomyopathy/endocardial fibroelastosis following intravenous gamma globulin (IVIG) and corticosteroid therapy. Background We have previously shown that 85% of fetuses and infants with maternal autoantibody-mediated fetal cardiomyopathy/endocardial fibroelastosis suffer demise or need for transplant. In an attempt to improve this outcome, in 1998, we began to empirically treat affected patients with IVIG and corticosteroids. Methods We reviewed the clinical records and echocardiograms of 20 affected patients encountered in our institutions and treated with IVIG and corticosteroids from 1998 to 2009. Results All 20 were initially referred at a median gestational age of 23 weeks (range 18 to 38 weeks). Nineteen mothers were anti-Ro antibody positive, 8 anti-La antibody positive, and 7 had clinical autoimmune disease. Endocardial fibroelastosis was seen in 16 and was not obvious in 4 others with reduced ventricular function, and 16 (80%) had reduced or borderline ventricular shortening fraction (≤30%) before or after birth. Eighteen had atrioventricular block at referral (16 in 3°). During pregnancy, maternal IVIG was given in 9 and dexamethasone in 17. After birth, 17 infants received IVIG (n = 14) and/or corticosteroids (n = 15). Twelve underwent pacemaker implantation. Four with hydrops at presentation died perinatally, despite initial improvement in function in 3. At a median follow-up of 2.9 years (1.1 to 9.8 years), 16 (80%) patients are currently alive with normal systolic ventricular function and 6 are not paced. Conclusions Treatment of maternal autoantibody-mediated fetal cardiomyopathy/endocardial fibroelastosis with IVIG and corticosteroids potentially improves the outcome of affected fetuses. Further studies are needed to determine the optimal dose and timing of IVIG administration.

Since the early 90s hybrid therapies have been used to treat pulmonary artery stenosis. Overtime expertise has grown and now hybrid pulmonary artery interventions are relatively common in pediatric cardiology, as they offer particular advantages over standard either catheter or surgical techniques alone. A literature review demonstrates that most reports include a relatively small number of patients, in whom procedures are performed either under direct vision and cardiopulmonary bypass or via open chest with direct puncture into the pulmonary artery or right ventricular outflow tract. Feasibly and safety is demonstrated, with a relatively low incidence of serious complication rates. The published experience enhances the importance of hybrid therapies as a therapeutic alternative for pulmonary artery stenosis.

The objective of this study was to investigate variation in practice surrounding elective cardiac catheterization in patients with single-ventricle (SV) congenital heart disease.Patient and procedural characteristics and outcomes during SV catheterization were collected prospectively from eight centers using a web-based registry (Congenital Cardiac Catheterization Project on Outcomes). We attempted to identify a population of elective procedures by limiting the cohort in case type and timing. Cases were then stratified by stage of SV palliation (pre-bidirectional cavopulmonary anastomosis [pre-BCPA], pre-Fontan and post-Fontan) and limited by age. Subcohort analysis was performed by mode of airway management (assisted vs. spontaneous ventilation). Institutional variation was assessed.Between 2/2007 and 6/2010, 1459 (10.1%) of 14 467 cases in the registry met the inclusion and exclusion criteria, including 326 pre-BCPA, 571 pre-Fontan and 562 post-Fontan procedures. Median patient age was 0.4 (interquartile range 0.3, 0.5), 2.6 (1.0, 3.4) and 9.6 (5.2, 15.4) years and weight was 5.6 (4.8, 6.4), 12.2 (10.5, 14), and 26.3 (16.6, 51.8) kg in the pre-BCPA, pre-Fontan and post-Fontan cohorts, respectively. Cases were more commonly diagnostic in the pre-BCPA cohort (57%) whereas they were more commonly interventional in the pre-Fontan (69%) and post-Fontan (77%) cohorts. At least one adverse event (AE) occurred in 210 cases (14.4%) overall, including 20% of pre-BCPA, 11% of pre-Fontan and 14% of post-Fontan catheterizations. Mode of airway management was associated with statistically significant, but clinically small differences in hemodynamic measures in the pre- and post-Fontan cohorts, but not in the pre-BCPA group. Considerable practice variation exists across centers with variability in airway management, AE rate, case type, interventions performed and fluoroscopy time, in all SV cohorts.Elective catheterization in SV patients, frequently performed with intervention, is common among US congenital cardiac centers. However, important variation in practice exists during these procedures in SV patients, at all stages of palliation.

When patients cannot provide responses to health-related quality-of-life (HRQOL) measures in clinical trials, family or friends may be asked to respond. We present a simple, comprehensive method for assessing agreement between patients with head injury and their proxy responders. In contrast to more traditional approaches, this method defines agreement separately for each patient-proxy pair, and compares HRQOL profiles along three dimensions— level , or the average of the ratings; scatter, or the variability in the ratings; and s hape, or the ranks of the ratings. We demonstrate this method in the context of a clinical trial of a treatment for traumatic head injury and compare the results to those obtained using traditional analyses. Options for incorporating proxy responses into clinical trial analyses are discussed.

To describe contemporary outcomes of balloon aortic valvuloplasty (BAVP) performed in 22 US centers.BAVP constitutes first-line therapy for congenital aortic stenosis (cAS) in many centers.We used prospectively-collected data from two active, multi-institutional, pediatric cardiac catheterization registries. Acute procedural success was defined, for purposes of this review, as a residual peak systolic gradient≤35 mm Hg and no more than mild aortic regurgitation (AR) for patients with isolated cAS. For patients with mixed aortic valve disease, a residual peak systolic gradient≤35 mm Hg without worsening of AR was considered successful outcome.In 373 patients with a median age of 8 months (1 day to 40 years of age) peak systolic gradient had a median of 59 [50, 71] mm Hg pre-BAVP and 22 [15, 30] mm Hg post-BAVP (P0.001). Procedural success was achieved in 160 patients (71%). The factors independently associated with procedural success were: first time intervention (OR=2.0 (1.0, 4.0) P=0.04), not-prostaglandin dependent, (OR=3.5 (1.5, 8.1); P=0.003), and isolated cAS (absence of AR) (OR=2.1 (1.1-3.9); P=0.03). Twenty percent of patients experienced adverse events, half of which were of high severity. There was no procedural mortality. Neonatal status was the only factor associated with increased risk of high severity adverse events (OR 3.7; 95% CI 1.5-9.0).In the current era, BAVP results in procedural success (gradient reduction with minimal increase in AR) in 71% of patients treated at US centers where BAVP is considered first-line therapy relative to surgery.

The aim of the study is to explore the indications for cardiac catheterization while on extracorporeal membrane oxygenation (ECMO) and the various catheter interventions performed as well as assess the safety profile and determine the short- and intermediate-term survival. ECMO is a lifesaving intervention for pediatric patients with respiratory and/or cardiovascular failure. There is limited recent literature discussing the survival and outcomes of patients undergoing cardiac catheterization while on ECMO. A retrospective review of consecutive patients undergoing catheterization while on ECMO from 2004 to 2013 was performed. Thirty-six patients who underwent 40 cardiac catheterizations were identified. Indications for catheterization included hemodynamic/anatomic assessment of postoperative (16) and non-operative patients (7), planned catheter interventions (CI) (12), and cardiomyopathy assessment (5). CI were performed during 18 (45 %) catheterizations, including stenting of vessels/surgical shunts (9), balloon atrial septostomy (4), device closure of septal defects/vessels (3), thrombolysis of vessels (2), endomyocardial biopsy (2), and temporary pacer wire placement (1). Unexpected diagnostic information was found in 21 (52 %), and 13 patients were referred for surgical intervention. Successful decannulation was achieved in 86 % of patients. Survival to discharge was 72 % and intermediate survival was 69 % (median = 29 months). Survival was 88 % (15/17) among patients who underwent CI. There were six procedural complications (15 %); five vascular and one non-vascular. There were no complications related to patient transport. Cardiac catheterization and interventions while on ECMO are safe, with a survival to discharge of 72 %. Diagnostic information obtained from catheterization leads to management decisions which may impact survival.

Secundum atrial septal defect (ASD) closure devices were granted approval based on industry-sponsored, prospective, nonrandomized, single device studies, demonstrating acceptable efficacy and safety in selected patients. We sought to report community practice and outcomes.Procedure specific data was collected on cases considered for ASD closure in the congenital cardiac catheterization project on outcomes (C3PO) between February 1, 2007 and June 31, 2010. Eight centers contributed data during this time period. All adverse events (AE) were independently reviewed and classified by a five level severity scale. In 40 months (2/07-6/10), 653 of 688 ASDs were occluded with a single device using an AMPLATZER(®) Septal Occluder (ASO) in 566 (87%), GORE(®) HELEX(®) Septal Occluder (HSO) in 33 (5%), and a CardioSEAL(®) or STARFlex™ device (CSD) in 54 (8%). Most patients had an isolated ASD (93%). 85% were2 years of age. The ASD median diameter was 12 mm [8,16] for ASO, with smaller diameters in HSO 8 mm [7,10] and CSD 8 mm [5,10] (P0.001). AE (n = 82) were recorded in 76 cases, 11.5% (95% CI 9.2%, 14.1%) and classified as high severity in 4.7% (95% CI 3.2%, 6.5%), with no mortality. A new conduction abnormality was detected during 15 cases and did not resolve in one. Transcatheter device retrieval was possible in 7 of 10 device embolizations. Device erosion occurred in 3 of 566, 0.5% (95% CI 0.1%, 1.5%), ASO implants.Although device closure of ASDs is associated with low morbidity and rare mortality, ongoing assessment of device safety profiles are warranted, and registries offer opportunities to facilitate the required surveillance.

ObjectivesIn developing countries, congenital heart disease is often unrecognised, leading to serious morbidity and mortality. Guatemala is one of the few developing countries where expert paediatric cardiac treatment is available and affordable, and therefore early detection could significantly improve outcome. We assessed regional congenital heart disease detection rates in Guatemala, and determined whether they correlated with the regional human development index.MethodsWe retrospectively reviewed all new cardiac referrals made in 2006 to the Unidad de Cirugia Cardiovascular Pediatrica, the only paediatric cardiac centre in Guatemala. We calculated regional detection rates by comparing the number of congenital heart disease referrals with the expected incidence using the National Ministry of Health birth data. We then compared the regional detection rates with the human development index data published in the United Nations 2006 Development Program Report using Spearman’s rank correlation.ResultsAn estimated 3935 infants with cardiac defects were born in Guatemala in 2006, an expected 1380 (35%) of whom had severe forms. Overall, only 533 children (14%) with cardiac defects were referred. Of these, 62% had simple shunt lesions, 13% had cyanotic lesions, and 10% had left-sided obstructive lesions. Only 11.5% of referred patients were neonates. Regional detection rates, ranged 3.2–34%, correlated with the regional human development index (r = 0.75, p < 0.0001).ConclusionsCurrent detection of congenital heart disease in Guatemala is low and correlates with the regional human development index. Those detected are older and have less severe forms, suggesting a high mortality rate among Guatemalan neonates with complex cardiac defects.

When patients cannot provide responses to health-related quality-of-life (HRQOL) measures in clinical trials, family or friends may be asked to respond. We present a simple, comprehensive method for assessing agreement between patients with head injury and their proxy responders. In contrast to more traditional approaches, this method defines agreement separately for each patient-proxy pair, and compares HRQOL profiles along three dimensions-level, or the average of the ratings; scatter, or the variability in the ratings; and shape, or the ranks of the ratings. We demonstrate this method in the context of a clinical trial of a treatment for traumatic head injury and compare the results to those obtained using traditional analyses. Options for incorporating proxy responses into clinical trial analyses are discussed.

Un paciente de 7 anos de edad, con antecedente de ventriculo unico caracterizado por doble entrada ventricular izquierda, estenosis mitral, comunicacion interauricular tipo ostium secundum y colocacion de banda en la arteria pulmonar en la infancia, fue referido para una cirugia de Fontan. Una angiografia de la vena innominada demostro la presencia de vena cava superior izquierda (VCSI) en continuacion con el seno coronario (SC). El drenaje de la vena innominada en la vena cava superior derecha era normal (fig. 1). Una angiografia de la VCSI demostro atresia del orificio del SC y ausencia de comunicacion entre el SC y la auricula izquierda. De esta manera, el SC drenaba anormalmente en la vena innominada a traves de la VCSI, y esta via era el unico drenaje del SC (fig. 2). Este hallazgo no tiene consecuencia clinica cuando la anatomia cardiaca es normal. Sin embargo, es de relevancia en pacientes con ventriculo unico, que requieren una cirugia de anastomosis cavopulmonar o de Fontan. En estos casos, el cierre de la VCSI, comunmente realizado mediante cateterismo o durante la cirugia para evitar un shunt de derecha a izquierda, resulta en isquemia de miocardio al ocluirse totalmente el drenaje del SC. La anastomosis cavopulmonar puede realizarse sin intervenir el SC dejando su drenaje en conexion con la circulacion pulmonar y a traves de la VCSI. La opcion quirurgica propuesta en este paciente, en conjuncion con la cirugia de Fontan, fue el destechamiento del SC para permitir su drenaje en la auricula izquierda y ligadura de la VCSI. Figura 1. Figura 2.

Objectives The aim of this study was to define age-stratified, procedure-specific benchmark radiation dose levels during interventional catheterization for congenital heart disease. Background There is a paucity of published literature with regard to radiation dose levels during catheterization for congenital heart disease. Obtaining benchmark radiation data is essential for assessing the impact of quality improvement initiatives for radiation safety. Methods Data were obtained retrospectively from 7 laboratories participating in the Congenital Cardiac Catheterization Project on Outcomes collaborative. Total air kerma, dose area product, and total fluoroscopy time were obtained for the following procedures: 1) patent ductus arteriosus closure; 2) atrial septal defect closure; 3) pulmonary valvuloplasty; 4) aortic valvuloplasty; 5) treatment of coarctation of aorta; and 6) transcatheter pulmonary valve placement. Results Between January 2009 and July 2013, 2,713 cases were identified. Radiation dose benchmarks are presented including median, 75th percentile, and 95th percentile. Radiation doses varied widely between age groups and procedure types. Radiation exposure was lowest in patent ductus arteriosus closure and highest in transcatheter pulmonary valve placement. Total fluoroscopy time was a poor marker of radiation exposure and did not correlate well with total air kerma and dose area product. Conclusions This study presents age-stratified radiation dose values for 6 common congenital heart interventional catheterization procedures. Fluoroscopy time alone is not an adequate measure for monitoring radiation exposure. These values will be used as baseline for measuring the effectiveness of future quality improvement activities by the Congenital Cardiac Catheterization Project on Outcomes collaborative.