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Background: Current metrics used to adjust for case mix complexity in congenital cardiac catheterization are becoming outdated due to the introduction of novel procedures, innovative technologies, and expanding patient subgroups. This study aims to develop a risk adjustment methodology introducing a novel, clinically meaningful adverse event outcome and incorporating a modern understanding of risk., Methods: Data from diagnostic only and interventional cases with defined case types were collected for patients ≤18 years of age and ≥2.5 kg at all Congenital Cardiac Catheterization Project on Outcomes participating centers. The derivation data set consisted of cases performed from 2014 to 2017, and the validation data set consisted of cases performed from 2019 to 2020. Severity level 3 adverse events were stratified into 3 tiers by clinical impact (3a/b/c); the study outcome was clinically meaningful adverse events, severity level ≥3b (3bc/4/5)., Results: The derivation data set contained 15 224 cases, and the validation data set included 9462 cases. Clinically meaningful adverse event rates were 4.5% and 4.2% in the derivation and validation cohorts, respectively. The final risk adjustment model included age <30 days, Procedural Risk in Congenital Cardiac Catheterization risk category, and hemodynamic vulnerability score (C statistic, 0.70; Hosmer-Lemeshow P value, 0.83; Brier score, 0.042)., Conclusions: CHARM II (Congenital Heart Disease Adjustment for Risk Method II) risk adjustment methodology allows for equitable comparison of clinically meaningful adverse events among institutions and operators with varying patient populations and case mix complexity performing pediatric cardiac catheterization., Competing Interests: None.

Background: Early postoperative catheterizations (EPOCs) within 6 weeks after a congenital heart surgical procedure can treat residual lesions and provide important clinical information. However, EPOCs are often assumed to impose additional risk on a vulnerable patient population. This study aimed to describe the EPOC population, evaluate procedural safety, compare EPOC patients with procedure-matched non-EPOC patients, and determine risk factors for poor outcomes using data from the Congenital Cardiac Catheterization Project on Outcomes registry., Methods: In a retrospective cohort, demographic, clinical, and procedural characteristics were analyzed for diagnostic and interventional catheterizations performed in 13 participating institutions from January 2014 to December 2017, excluding patients after heart transplant. The primary outcome was a high-severity adverse event (AE). Three distinct analyses included (1) describing the full cohort and EPOC patients, (2) comparing EPOC patients with and without a high-severity AE, and (3) comparing EPOC patients with controls matched on case type., Results: This study included 17,776 catheterizations, with 1399 EPOCs. The high-severity AE rate was 6.4% overall, 8.9% in the EPOC cohort, and 8.4% in matched controls (P = .74). The association between EPOC status and high-severity AE was not significant in a multivariable model (P = .17). In EPOCs with a high-severity AE, median procedure duration was 30 minutes longer (P < .001), and median time from surgical procedure to catheterization was 3 days longer (P = .05)., Conclusions: EPOC was not associated with additional risk. Individual patient characteristics of size and hemodynamic vulnerability may serve as informative predictors. Timely catheterization may preempt further clinical deterioration, and intraprocedure duration optimization may correlate with improved outcomes., (Copyright © 2023 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.

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.

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: 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.)

Objective: The objective is to quantify radiation dose in children undergoing cardiac catheterization and determine the impact of increased reporting transparency on total radiation exposure., Background: Cardiac catheterization (cath) can result in significant radiation exposure in children. There has been growing interest in quantifying and reducing radiation exposure in pediatric cath procedures. Our center underwent a slight change in practice recently that resulted in direct physician reporting of radiation dose following every case., Methods: We reviewed cath procedures across three different eras in four cath categories: post-heart transplant annual cath, unilateral pulmonary artery (PA) stent placement, pre-Fontan cath, and pre-Glenn cath. The eras were defined as: Era 1, 1/2009-1/2011; Era 2, 1/2011-9/2013; and Era 3, 9/2013-5/2014. In Era 3, the physician performing the cath was responsible for reporting the radiation data., Results: Across the three eras, there were significant decreases in cumulative air KERMA (mGy) among all four cath categories. From Era 2 to Era 3, the greatest decreases in radiation were noted, particularly in dose area product (cGy·cm2) in the transplant annual evaluation and pre-Glenn cases. In Era 1, 2 cases (1.2%) had a frame rate reduction, while in Era 2, 22 cases (12.0%), and in Era 3, 83 cases (21.6%) had frame rate changes (P<0.01)., Conclusions: Increased physician awareness of radiation exposure is associated with a significant reduction in radiation dose across a variety of cath procedures. This is seen not only by the overall reduction in radiation across case types, but also as the frame rate was more frequently changed during individual cases, indicating an important change in physician behavior and practice., (© 2015 Wiley Periodicals, Inc.)

Objectives: The purpose of this study was to examine the outcomes of catheter dilation interventions in general upon surgical anastomotic sites in the immediate postoperative period., Background: Early postoperative cardiac catheter intervention is regarded as high-risk, particularly when a dilation intervention across a fresh suture line is performed., Methods: All catheter dilation interventions performed upon a fresh suture line within 30 days of congenital heart surgery between August 2005 and December 2013 were reviewed. Values are reported as median and interquartile range (IQR). Primary endpoint was procedural success, which was defined as an increase in vessel diameter of >75% of the adjacent normal vessel or 50% increase over pre-dilation diameter. Secondary endpoints included procedural complications and survival to hospital discharge., Results: Forty-five patients, median age 0.15 years (0.03-0.51 years), weight 4.1 kg (3.1-6.4 kg), underwent 62 interventional procedures on median postoperative day 7 (3-13 days). Among the 62 dilation interventions at surgical anastomotic areas, were 30 stent and 32 balloon angioplasty procedures. There were two major complications including: arterial dissection during stent placement and a procedural mortality due to pulmonary artery avulsion during angioplasty. There were 12 deaths (27%) prior to hospital discharge. The median ratio of maximum stent diameter to stenosis diameter was 2.62 (2.27-3.73). The median ratio of maximum balloon diameter to stenosis diameter was 2.27 (1.84-2.94). Stent placement was more likely to result in procedural success (OR 2.1; 95% confidence interval 1.6-2.8)., Conclusion: Though caution is paramount, early postoperative catheter dilation intervention across fresh suture lines can be performed safely in small, critically ill children., (© 2015 Wiley Periodicals, Inc.)

Objectives: This study sought to demonstrate that early cardiac catheterization, whether used solely as a diagnostic modality or for the use of transcatheter interventional techniques, can be used effectively and with an acceptable risk in the post-operative period., Background: Cardiac catheterization offers important treatment for patients with congenital heart disease. Early post-operative cardiac catheterization is often necessary to diagnose and treat residual anatomic defects. Experience with interventional catheterization to address post-operative concerns is limited., Methods: This was a retrospective cohort study. The medical and catheterization data of pediatric patients who underwent a cardiac catheterization ≤30 days after congenital heart surgery between November 2004 and July 2013 were reviewed. Patients who underwent right heart catheterization and endomyocardial biopsy after heart transplantation were excluded., Results: A total of 219 catheterizations (91 interventional procedures, 128 noninterventional catheterizations) were performed on 193 patients. Sixty-five interventions (71.43%) were dilations, either balloon angioplasty or stent implantation. There was no difference in survival to hospital discharge between those who underwent an interventional versus noninterventional catheterization (p = 0.93). One-year post-operative survival was comparable between those who underwent an intervention (66%) versus diagnostic (71%) catheterization (p = 0.58). There was no difference in the incidence of major or minor complications between the interventional and diagnostic catheterization cohorts (p = 0.21)., Conclusions: Cardiac catheterization, including transcatheter interventions, can be performed safely in the immediate post-operative period after congenital heart surgery., (Copyright © 2014 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objective: Fluid restriction is often employed immediately following cardiac surgery in children. The goal of this approach is to achieve an early negative fluid balance, which theoretically should lead to less interstitial edema and earlier extubation. The purpose of this study was to determine whether time to negative fluid balance in infants after undergoing systemic-to-pulmonary artery shunt palliation impacts duration of mechanical ventilation and hospital length of stay., Design: This is a retrospective study of neonates who underwent a modified systemic-to-pulmonary artery shunt at a single institution., Setting: University hospital pediatric cardiac intensive care unit (CICU)., Patients: Neonates who underwent a modified systemic-to-pulmonary artery shunt between January 1, 2009 and June 1, 2011., Outcome Measures: Information collected included time to negative fluid balance (in hours), CICU and hospital length of stay (in days), and the number of patients who had delayed sternal closure and/or underwent cardiopulmonary bypass., Results: Data were available for 65 subjects. Median fluid administration in the 24 hours postoperatively was 43.9 cc/kg/day (interquartile range: 32.9-61.0). Mean time to negative fluid balance was 25.0 ± 12.8 hours. Time to negative fluid balance was not associated with time to extubation, CICU and hospital length of stay, or change in weight-for-age z-score at intensive care unit discharge., Conclusion: Time to negative fluid balance is not associated with duration of mechanical ventilation, CICU, and hospital length of stay in patients after undergoing systemic-to-pulmonary artery shunt palliation. The utility of a restricted fluid strategy immediately following infant heart surgery is questionable., (© 2014 Wiley Periodicals, Inc.)

Infants with congenital heart disease have impaired weight gain during the first several months of life. Efforts have focused on improving weight gain and nutritional status during the first months of life. Close examination of the data suggests that the immediate postoperative period is problematic. Etiology of this early growth failure should be identified to develop effective interventions. This is a retrospective study of neonates who underwent modified systemic-to-pulmonary artery shunt, including Norwood palliation, at Children's Healthcare of Atlanta between January 2009 and July 2011. We analyzed growth from time of surgical intervention to hospital discharge. Measures of calculated weight-for-age Z-score (WAZ score) were performed using the World Health Organization's Anthro Software (version 3.2.2, January 2011; WHO, Geneva, Switzerland). Seventy-three patients were identified. Eight patients did not meet inclusion criteria. Complete data were collected on the remaining 65 patients. Median caloric intake patients received was 50.4 [interquartile range (IQR) 41.6 to 63.6] calories/kg/day while exclusively on parental nutrition. At hospital discharge, the median WAZ score was -2.0 (IQR -2.7 to -1.2) representing an overall median WAZ score decrease of -1.3 (IQR -1.7 to -0.7) from time of shunt palliation to hospital discharge. Despite studies showing poor weight gain in infants with congenital heart disease after neonatal palliation, this study reports the impact of hospital-based nutritional practices on weight gain in infants during the immediate postoperative period. Our data demonstrate that actual caloric intake during the cardiac intensive care unit stay is substantially below what is recommended.