Catheterization for Congenital Heart Disease Adjustment for Risk Method II.

BACKGROUND: Current metrics used to adjust for case mix complexity in congenital cardiac catheterization are becoming outdated due to the introduction of novel procedures, innovative technologies, and expanding patient subgroups.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.

Coronary artery dilation in non-hospitalised children with asymptomatic or mild COVID-19.

INTRODUCTION: Infection with Sars-CoV-2 is known to cause cardiac injury and coronary artery changes in moderate to severe acute COVID-19 and post-acute multisystem inflammatory syndrome in children (MIS-C). However, little is known about the potential for cardiac involvement, in particular coronary artery dilation, in asymptomatic or mild cases of COVID-19. METHODS: A retrospective review of children ≤ 18 years of age with a history of asymptomatic or mild COVID-19 disease who underwent echocardiography after Sars-CoV-2 infection is conducted. Patients were excluded if they had been hospitalised for COVID-19/MIS-C or had a history of cardiac disease that could affect coronary artery dimension. Coronary artery dilation was defined as the Boston Z-score greater than 2.0. RESULTS: One hundred and fifty-seven patients met inclusion criteria with a mean age of 9.4 years (+/- 5.4 years). Eighty-four (54%) patients were identified as having COVID-19 through positive antibody testing. All patients underwent electrocardiogram and echocardiogram as part of their cardiology evaluation. One hundred and thirty-five (86%) patients had a normal evaluation or only a minor variant on electrocardiogram, while 22 patients had abnormalities on echocardiogram, 4 of which demonstrated coronary artery dilation based on the Boston Z-score. CONCLUSIONS: Much of the literature for post-infectious screening and follow-up focuses on patients with a history of moderate to severe COVID-19 disease, emphasising the need for surveillance for the potential development of myocarditis. In this study, 4 out of 157 (2.5%) children with a history of asymptomatic or mild COVID-19 disease without MIS-C were found to have some degree of coronary artery dilation. The significance of this finding currently remains unknown.

Safety and Short-Term Outcomes for Infants < 2.5 kg Undergoing PDA Device Closure: A C3PO Registry Study.

To evaluate short-term procedural outcomes and safety for infants < 2.5 kg who underwent catheterization with intended patent ductus arteriosus (PDA) device closure in a multi-center registry, as performance of this procedure becomes widespread. A multi-center retrospective review was performed using data from the Congenital Cardiac Catheterization Project on Outcomes (C3PO) registry. Data were collected for all intended cases of PDA closure in infants < 2.5 kg from April 2019 to December 2020 at 13 participating sites. Successful device closure was defined as device placement at the conclusion of the catheterization. Procedural outcomes and adverse events (AE) were described, and associations between patient characteristics, procedural outcomes and AEs were analyzed. During the study period, 300 cases were performed with a median weight of 1.0 kg (range 0.7-2.4). Successful device closure was achieved in 98.7% of cases with a 1.7% incidence of level 4/5 AEs, including one periprocedural mortality. Neither failed device placement nor adverse events were significantly associated with patient age, weight or institutional volume. Higher incidence of adverse events associated with patients who had non-cardiac problems (p = 0.017) and cases with multiple devices attempted (p = 0.064). Transcatheter PDA closure in small infants can be performed with excellent short-term outcomes and safety across institutions with variable case volume.

The Need for Surgery After Vascular or Cardiac Trauma, or Technical Adverse Events in the Congenital Cardiac Catheterization Laboratory.

Data on the frequency and outcome of surgical interventions as a result of adverse events (AE) encountered in the pediatric and congenital cardiac catheterization laboratory are limited. This study analyzes the outcomes of specific types of AE that are most likely to require immediate surgical intervention. Data from the C3PO registry were analyzed to identify specific types of significant vascular/cardiac trauma or technical adverse events (stent/device/coil embolization/migration). The relationship between these AE and an "adverse outcome" (defined as either surgery, ECMO, or death) were analyzed. Between 01/2014 and 12/2017, 25,731 cases were entered into the C3PO registry. Vascular or cardiac trauma were observed in 92 cases (0.36% cases in C3PO), and technical adverse events were observed in 176 cases (0.68% cases in C3PO). The two highest procedure type risk categories (PREDIC3T) accounted for 61% of the cases in the cardiac/vascular trauma cohort, and 34% in the technical AE cohort. For vascular/cardiac trauma, 24 (26%) had an adverse outcome, with ECMO in 8 (9%), surgery in 19 (20%), and death in 9 (10%). For technical AE 25 (14%) had an adverse outcome, with ECMO in 3 (2%), surgery in 23 (13%), and death in 3 (2%). Survival after cardiac surgery secondary to an AE was 68% for cardiac/vascular trauma, and 96% for technical adverse events. RF perforation of the pulmonary valve was the procedure most likely to result in cardiac/vascular trauma (10%), with 57% of those having an adverse outcome. Atrial septal interventions accounted for 29% of all adverse outcomes in the cardiac/vascular trauma cohort. Non-elective or emergent cases were associated with a significantly higher incidence of an adverse outcome for both, cardiac/vascular trauma (OR 7.1) and technical adverse events (OR 2.7). Surgery within the last 30 days was associated with a significantly higher incidence of an adverse outcome for cardiac/vascular trauma only (OR 4.2). Significant cardiac/vascular trauma or stent/device/coil embolization/migration are rare, but high consequence AE. With appropriate surgical and ECMO backup, a high survival can be achieved. The potential need for and impact of immediate surgical backup seems to be higher for cardiac/vascular trauma (in particular after specific case types), than for device/coil migration/embolization, and as such case specific backup arrangements are required.

Percutaneous closure of giant aneurysmal coronary artery-to-coronary sinus fistulae with guidance from three-dimensional printed models: a case series.

Background: Coronary artery fistulae are abnormal communications of coronary arteries with systemic vasculature, pulmonary vasculature, or cardiac chambers. Use of multimodality imaging can be paramount to understanding anatomical and functional features of these complex vascular lesions, therefore optimizing success of potential curative interventions. Case summary: We present two patients with incidentally discovered giant aneurysmal coronary arteries with distal fistulous connections to the coronary sinus, which were successfully closed percutaneously with Amplatzer Septal Occluders using the assistance of three-dimensional (3D) printed heart models. Conclusion: Computed tomography-guided reconstruction with 3D multiplanar, multicolour printed models can help augment visuospatial understanding of the size, origin, course, and drainage of giant aneurysmal coronary artery-to-coronary sinus fistulae, and with manual bench testing can assist with choosing accurately sized and shaped devices for closure.

Chylous pericardial effusion with cardiac tamponade in a child treated with imatinib.

Chylous pericardial effusions are extremely rare outside of thoracic and cardiac surgery patients. We report the case of an 8-year-old girl with history of recurrent benign giant cell granulomas who developed a large chylous pericardial effusion with cardiac tamponade soon after beginning therapy with imatinib. In this article, we discuss the presentation, diagnosis, and management and review the published literature of this rarely reported side effect of this medication.

Changes in Practice/Outcomes of Pediatric/Congenital Catheterization in Response to the First Wave of COVID.

Background: 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. Objectives: 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. Methods: 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. Results: 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 (P = 0.001). The proportion of cases with risk factors for HSAE increased during the first wave, specifically proportions of infants and neonates (P < 0.001) and subjects with renal insufficiency (P = 0.02), recent cardiac surgery (P < 0.001), and a higher hemodynamic vulnerability score (P = 0.02). The observed HSAE risk did not change significantly (P = 0.13). In multivariable analyses, odds of HSAE during the first wave of COVID (odds ratio: 0.75) appeared to be lower than that before COVID, but the difference was not significant (P = 0.09). Conclusions: 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.

IMPACTing the Future: Are we on the Right Track?

More than ten years have passed since data collection was initiated in the IMPACT registry. Over the last decade, difficulties in data access and a lack of autonomy have led to a disengagement of the congenital community, with the usefulness of IMPACT to patients and providers being questioned. This article outlines some of problems identified using an online survey among interventional congenital cardiologists, and provides possible solutions for the future.

Procedural Risk in Congenital Cardiac Catheterization (PREDIC3T).

Background Advancements in the field, including novel procedures and multiple interventions, require an updated approach to accurately assess patient risk. This study aims to modernize patient hemodynamic and procedural risk classification through the creation of risk assessment tools to be used in congenital cardiac catheterization. Methods and Results Data were collected for all cases performed at sites participating in the C3PO (Congenital Cardiac Catheterization Project on Outcomes) multicenter registry. Between January 2014 and December 2017, 23 119 cases were recorded in 13 participating institutions, of which 88% of patients were <18 years of age and 25% <1 year of age; a high-severity adverse event occurred in 1193 (5.2%). Case types were defined by procedure(s) performed and grouped on the basis of association with the outcome, high-severity adverse event. Thirty-four unique case types were determined and stratified into 6 risk categories. Six hemodynamic indicator variables were empirically assessed, and a novel hemodynamic vulnerability score was determined by the frequency of high-severity adverse events. In a multivariable model, case-type risk category (odds ratios for category: 0=0.46, 1=1.00, 2=1.40, 3=2.68, 4=3.64, and 5=5.25; all P≤0.005) and hemodynamic vulnerability score (odds ratio for score: 0=1.00, 1=1.27, 2=1.89, and ≥3=2.03; all P≤0.006) remained independent predictors of patient risk. Conclusions These case-type risk categories and the weighted hemodynamic vulnerability score both serve as independent predictors of patient risk for high-severity adverse events. This contemporary procedure-type risk metric and weighted hemodynamic vulnerability score will improve our understanding of patient and procedural outcomes.

Long-Term Outcomes of the Coarctation of the Aorta Stent Trials.

BACKGROUND: Long-term outcome data of stent-implantation for coarctation of the aorta are limited. We report up to 5 years of postimplant follow-up in patients enrolled into the COAST (Coarctation of the Aorta Stent Trial) and the COAST II trial (Covered Cheatham-Platinum Stents for Prevention or Treatment of Aortic Wall Injury Associated With Coarctation of the Aorta), evaluating the bare and Covered Cheatham-Platinum Stents for the treatment of coarctation of the aorta and associated aortic wall injury. METHODS: Data was prospectively collected during the 2 multi-center studies, enrolling 248 patients (COAST: n=121, COAST II: n=127). Late follow-up data (48­60 month) was compared with immediate (1 month) and early (12 months) follow-up. RESULTS: There was a notable decrease in the use of antihypertensive medication, from 53% at immediate, to 42% at early, and 29% at late follow-up. The cumulative incidence of stent fractures was 0% immediately, 2.9% at early, and 24.4% at late follow-up. Independent predictors for stent fractures at late follow-up were age <18 years, male sex, minimum stent diameter ≥12 mm, and use of bare metal stent. The cumulative incidence of reintervention was 1.6% at immediate, 5.1% at early, and 21.3% at late follow-up. Independent predictors for reinterventions at late follow-up were age <18 years, post implantation systolic arm-leg blood pressure gradient ≥10 mm Hg, minimum stent diameter at implantation <12 mm, and initial coarctation minimum diameter <6 mm. There were 13 patients with aortic aneurysms, with a cumulative incidence of 6.3% at late follow-up. CONCLUSIONS: Coarctation stenting is effective at maintaining obstruction relief up to 60 months postimplant with reduction in the number of patients requiring antihypertensive medication. However, an increase in-stent fractures and reinterventions were observed between medium and long-term follow-up. Covered stents appear to confer some protection from the development of stent fractures but do not provide complete protection from late aneurysm formation. REGISTRATION: URL: https://www.clinicaltrials.gov; Unique identifiers: NCT00552812 and NCT01278303.

Cyanosis in a patient after Fontan palliation due to unrecognised hepatic vein to coronary sinus communication.

A 6-year-old male with heterotaxia, abnormal systemic and pulmonary venous drainage, and a history of Fontan completion presented with desaturations and was found by cardiac catheterisation to have a hepatic vein to coronary sinus connection. This was successfully occluded using an Amplatzer Muscular Ventricular Septal Defect Occluder.

Percutaneous Implantation of Adult Sized Stents for Coarctation of the Aorta in Children ≤20 kg: A 12-Year Experience.

BACKGROUND: Stent implantation (SI) is more effective than balloon angioplasty for the treatment of coarctation of the aorta (CoA). Due to technical factors, balloon angioplasty is more commonly performed in small patients. We sought to evaluate outcomes of percutaneous adult sized SI for the treatment of CoA in small patients. METHODS: A single-center retrospective review of all patients ≤20 kg who underwent percutaneous adult sized SI for native or recurrent CoA from 2004 to 2015 was performed. RESULTS: Thirty-nine patients (20 patients ≤10 kg) were identified, with 28 (71.8%) having recurrent CoA and 22 (56.4%) previously failed balloon angioplasty. At the time of SI, the median (range) patient age and weight were 1.1 (0.3-7.9) years and 10 (5.5-20.4) kg, respectively. SI resulted in significant improvements in the median gradient (26 mm Hg [interquartile range (IQR), 18-42] to 0 mm Hg [IQR, 0-2]; P< 0.05) and median minimum diameter (3.6 mm [IQR, 2.4-4.8] to 7.7 mm [IQR, 6.5-9.4]; P<0.05). Seven patients (18%) had procedural adverse events. Twenty-seven (69%) patients underwent elective reintervention at a median time of 49.3 (IQR, 26.5-63.2) months from SI, with 8 (21%) stents requiring repeat SI for stent fracture. Over a median follow-up of 67.2 (IQR, 33.8-116.1) months, 25 patients (69%) were without hypertension or blood pressure gradient. Three (11%) patients developed femoral arterial occlusion. CONCLUSIONS: Adult sized SI is an alternative to surgical intervention for small patients with CoA. SI carries a risk of access-related complications, which may improve with the development of lower profile stents with adult sized maximum diameters.

Coronary Artery Dilation in an Asymptomatic Pediatric Patient with COVID19 Antibodies.

We describe a 16-year-old asymptomatic male who presented with coronary artery dilation (z score + 2.3) identified on echo performed solely for presence of COVID-19 antibodies. This case raises the question of whether cardiac screening should be considered for all patients with a history of COVID-19.

The Deconditioning Effect of the COVID-19 Pandemic on Unaffected Healthy Children.

The COVID-19 pandemic has had devastating direct consequences on the health of affected patients. It has also had a significant impact on the ability of unaffected children to be physically active. We evaluated the effect of deconditioning from social distancing and school shutdowns implemented during the COVID-19 pandemic on the cardiovascular fitness of healthy unaffected children. This is a single-center, retrospective case-control study performed in an urban tertiary referral center. A cohort of 10 healthy children that underwent cardiopulmonary exercise testing after COVID-19 hospital restrictions were lifted was compared to a matched cohort before COVID-19-related shutdowns on school and after-school activities. Comparisons of oxygen uptake (VO2) max and VO2 at anaerobic threshold between the pre- and post-COVID-19 cohorts were done. The VO2 max in the post-COVID cohort was significantly lower than in the pre-COVID cohort (39.1 vs. 44.7, p = 0.031). Only one out of ten patients had a higher VO2 max when compared to their matched pre-COVID control and was also the only patient with a documented history of participation in varsity-type athletics. The percentile of predicted VO2 was significantly lower in the post-COVID cohort (95% vs. 105%, p = 0.042). This study for the first time documented a significant measurable decline in physical fitness of healthy children as a result of the COVID-19 pandemic and its associated restrictions. Measures need to be identified that encourage and facilitate regular exercise in children in a way that are not solely dependent on school and organized after-school activities.

Registries, Risk Calculators, and Risk-Adjusted Outcomes: Current Usage, Limitations, and Future Prospects.

Small study sizes are a limiting factor in assessing outcome measures in pediatric cardiology. It is even more difficult to assess the outcomes of congenital catheterizations where the sample sizes are even smaller, particularly on a individual institutional level. The creation of multicenter registries is a method by which investigators can pool data to better assess quality and outcome measures of these procedures. No registry is perfect with several being available today, each with its own strengths and weaknesses. In addition, there are a multitude of methods currently used to assess quality and outcomes from the data contained in these registries, each having its own limitations as well. Nonetheless, multicenter registrities remain one of the best available options to improve the quality of care for pediatric interventional cardiac catheterization. Below, we provide an overview of the current state of quality assessment/improvement in pediatric interventional cardiology including a review of the available registrities and the metrics used to measure quality of care and outcomes.

Predictors of Late Mortality in D-Transposition of the Great Arteries After Atrial Switch Repair: Systematic Review and Meta-Analysis.

Background Existing data on predictors of late mortality and prevention of sudden cardiac death after atrial switch repair surgery for D-transposition of the great arteries (D-TGA) are heterogeneous and limited by statistical power. Methods and Results We conducted a systematic review and meta-analysis of 29 observational studies, comprising 5035 patients, that reported mortality after atrial switch repair with a minimum follow-up of 10 years. We also examined 4 additional studies comprising 105 patients who reported rates of implantable cardioverter-defibrillator therapy in this population. Average survival dropped to 65% at 40 years after atrial switch repair, with sudden cardiac death accounting for 45% of all reported deaths. Mortality was significantly lower in cohorts that were more recent and operated on younger patients. Patient-level risk factors for late mortality were history of supraventricular tachycardia (odds ratio [OR] 3.8, 95% CI 1.4-10.7), Mustard procedure compared with Senning (OR 2.9, 95% CI 1.9-4.5) and complex D-TGA compared with simple D-TGA (OR 4.4, 95% CI 2.2-8.8). Significant risk factors for sudden cardiac death were history of supraventricular tachycardia (OR 4.7, 95% CI 2.2-9.8), Mustard procedure (OR 2.2, 95% CI 1.1-4.1), and complex D-TGA (OR 5.7, 95% CI 1.8-18.0). Out of a total 124 implantable cardioverter-defibrillator discharges over 330 patient-years in patients with implantable cardioverter-defibrillators for primary prevention, only 8% were appropriate. Conclusions Patient-level risk of both mortality and sudden cardiac death after atrial switch repair are significantly increased by history of supraventricular tachycardia, Mustard procedure, and complex D-TGA. This knowledge may help refine current selection practices for primary prevention implantable cardioverter-defibrillator implantation, given disproportionately high rates of inappropriate discharges.

Systolic/diastolic ratio correlates with end diastolic pressures in pediatric patients with single right ventricles.

BACKGROUND: Increased ventricular end-diastolic pressure (VEDP) is a known risk factor for morbidity and mortality in patients with single right ventricle (RV) physiology. Previous studies have shown mixed results correlating echocardiographic measurements with catheter-derived VEDP in this population. Goal of this study was to evaluate if echocardiographic systolic/diastolic ratio (S/D) correlated with VEDP. METHODS: Patients with single RV physiology who underwent simultaneous echocardiography and catheterization were evaluated. Systolic and diastolic durations were measured using tricuspid inflow durations from Doppler analysis to calculate the S/D ratio. VEDP was obtained from the catheterization report. RESULTS: Twenty-seven studies were performed on patients with single RV physiology. Median age at time of catheterization was 11.4 months (range, 0-132 months). Mean VEDP was 9.9 ± 4.5 mm Hg. S/D ratio was 1.8 ± 0.5. S/D ratio significantly correlated with VEDP (r = 0.63, P < .01). Optimum value of S/D ratio for discriminating between patients with high (>10 mm Hg) vs low EDP was found to be 1.9. High S/D ratio had an area under the curve of 0.82 (0.65, 1.0), with 75% sensitivity and 89% specificity for predicting elevated VEDP. CONCLUSION: In patients with single RV physiology, S/D significantly correlated with VEDP. S/D ratio is a simple technique that may be useful in both estimating and discriminating between high and low VEDP in this complex patient population.