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.

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.

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.

Transcatheter Occlusion of the Patent Ductus Arteriosus in 747 Infants <6 kg: Insights From the NCDR IMPACT Registry.

OBJECTIVES: The authors sought to identify risk factors associated with major adverse events (MAEs) in infants <6 kg undergoing transcatheter patent ductus arteriosus (PDA) occlusion. BACKGROUND: Transcatheter PDA occlusion is among the safest of interventional cardiac procedures in adults and older children, but use among infants <6 kg has not been characterized adequately. METHODS: Using the IMPACT (IMproving Pediatric and Adult Congenital Treatments) registry, we identified infants <6 kg undergoing transcatheter PDA occlusion (January 1, 2011, to March 1, 2015). Using mixed-effects multivariate regression, the authors assessed characteristics predictive of MAE or composite failure (procedural failure or MAE). Individual safety metrics (e.g., embolization, malposition) were also examined for differences across weight thresholds: extremely low weight (LW) (<2 kg), very LW (2 to <4 kg), and LW (4 to <6 kg). RESULTS: Transcatheter PDA occlusion was attempted in 747 infants <6 kg at 73 hospitals. Rate of procedural success was 94.3%. MAEs were observed in 12.6% of cases; the most common events were acute arterial injury and device embolization in 3.5% and 2.4% of cases, respectively. Younger age (<30 days) was associated with greater risk of a MAE (risk ratio: 3.3; 95% confidence interval: 1.5 to 7.6) and composite failure (risk ratio: 3.0; 95% confidence interval: 1.4 to 6.7). Risk of embolization was higher among extremely LW (10.5%) than very LW or LW infants (1.6% and 2.5%, respectively; p = 0.050). CONCLUSIONS: Among infants <6 kg, transcatheter PDA occlusion is technically feasible, but risks of MAE are noteworthy. These findings may help inform patient selection and procedural approach for transcatheter PDA occlusion and direct targeted research efforts to support the practice of evidence-based medicine.

Transcatheter pulmonary valve implantation: valve technology and procedural outcome.

PURPOSE OF REVIEW: Procedural technique and short-term outcomes of transcatheter pulmonary valve implantation (TPVI) have been widely described. The purpose of this article is to provide an update on current valve technology, and to focus on recent data surrounding TPVI in the dilated right ventricular outflow tract (RVOT), hybrid interventions, significant outcomes, and procedural costs. RECENT FINDINGS: Transcatheter valve technology has expanded with current trials evaluating self-expandable valves that can be implanted in dilated RVOTs. Until those valves are widely available, hybrid techniques have been shown to offer a potential alternative in these patients, as well as in patients of small size. Although medium-term results of TPVI have shown 5-year freedom from reintervention or replacement of 76%, new data have underlined some concerns relating to bacterial endocarditis after the procedure. Procedural costs remain a concern, but vary greatly between institutions and healthcare systems. SUMMARY: TPVI has emerged as one of the most innovative procedures in the treatment of patients with dysfunctional RVOT and pulmonary valves. Further device development is likely to expand the procedure to patients of smaller size and with complex, dilated RVOTs.

Percutaneous Patent Ductus Arteriosus (PDA) Closure During Infancy: A Meta-analysis.

CONTEXT: Patent ductus arteriosus (PDA) is a precursor to morbidity and mortality. Percutaneous (catheter-based) closure is the procedure of choice for adults and older children with a PDA, but use during infancy (<1 year) is not well characterized. OBJECTIVE: Investigate the technical success and safety of percutaneous PDA closure during infancy. DATA SOURCES: Scopus, Web of Science, Embase, PubMed, and Ovid (Medline) were searched through December 2015 with no language restrictions. STUDY SELECTION: Publications needed to clearly define the intervention as percutaneous PDA closure during infancy (<1 year of age at intervention) and must have reported adverse events (AEs). DATA EXTRACTION: The study was performed according to the Systematic Reviews and Meta-Analysis checklist and registered prospectively. The quality of the selected studies was critically examined. Data extraction and assignment of AE attributability and severity were independently performed by multiple observers. Outcomes were agreed on a priori. Data were pooled by using a random-effects model. RESULTS: Thirty-eight studies were included; no randomized controlled trials were found. Technical success of percutaneous PDA closure was 92.2% (95% confidence interval [CI] 88.8-95.0). Overall AE and clinically significant AE incidence was 23.3% (95% CI 16.5-30.8) and 10.1% (95% CI 7.8-12.5), respectively. Significant heterogeneity and publication bias were observed. LIMITATIONS: Limitations include lack of comparative studies, lack of standardized AE reporting strategy, and significant heterogeneity in reporting. CONCLUSIONS: Percutaneous PDA closure during infancy is feasible and associated with few catastrophic AEs; however, the limitations constrain the interpretability and generalizability of the current findings.

Pulmonary Arteriovenous Malformations and Risk of Stroke.

Pulmonary arteriovenous malformations (PAVMs) are rare, with an estimated incidence of 2 to 3 cases per 100,000, with most PAVMs (50%-80%) occurring in patients with hereditary hemorrhagic telangiectasia. Hypoxemia and orthodeoxia are some of the more common clinical presentations. The estimated risk of stroke secondary to PAVMs is as high as 2.6% to 25.0%. A combination of chest radiograph and contrast echocardiography is a good screening evaluation in patients with suspected PAVMs. Transcatheter therapy is the most suitable therapeutic option in most patients with PAVMs, and a variety of devices can be used to occlude the PAVMs.

Surgical strategies and novel alternatives for the closure of ventricular septal defects.

A variety of therapies are available to close ventricular septal defects (VSDs). These include surgical closure on bypass, percutaneous device closure, as well as perventricular hybrid closure. Due to the incidence of heart block (1-5%) associated with percutaneous device closure of perimembranous VSDs, surgical closure presently remains the gold standard and preferred therapy for these defects. Therapeutic options are more varied for muscular VSDs. Beyond infancy, transcatheter closure offers excellent results with low morbidity and mortality, without the need for cardiopulmonary bypass. Infants however have a higher incidence of adverse events using a percutaneous approach. Large mid-muscular VSDs in infants can be treated successfully using a hybrid approach, surgical closure on bypass or a percutaneous approach. However, VSDs located apically or anteriorly are difficult to identify surgically and for these infants, perventricular hybrid closure should be considered as the preferred therapeutic modality. However, some VSD's also can be closed percutaneously.

Percutaneous Patent Ductus Arteriosus (PDA) Closure in Very Preterm Infants: Feasibility and Complications.

BACKGROUND: Percutaneous closure of patent ductus arteriosus (PDA) in term neonates is established, but data regarding outcomes in infants born very preterm (<32 weeks of gestation) are minimal, and no published criteria exist establishing a minimal weight of 4 kg as a suitable cutoff. We sought to analyze outcomes of percutaneous PDA occlusion in infants born very preterm and referred for PDA closure at weights <4 kg. METHODS AND RESULTS: Retrospective analysis (January 2005-January 2014) was done at a single pediatric center. Procedural successes and adverse events were recorded. Markers of respiratory status (need for mechanical ventilation) were determined, with comparisons made before and after catheterization. A total of 52 very preterm infants with a median procedural weight of 2.9 kg (range 1.2-3.9 kg) underwent attempted PDA closure. Twenty-five percent (13/52) of infants were <2.5 kg. Successful device placement was achieved in 46/52 (88%) of infants. An adverse event occurred in 33% of cases, with an acute arterial injury the most common complication. We observed no association between weight at time of procedure and the risk of an adverse event. No deaths were attributable to the PDA closure. Compared to precatheterization trends, percutaneous PDA closure resulted in improved respiratory status, including less exposure to mechanical ventilation (mixed effects logistic model, P<0.01). CONCLUSIONS: Among infants born very preterm, percutaneous PDA closure at weights <4 kg is generally safe and may improve respiratory health, but risk of arterial injury is noteworthy. Randomized clinical trials are needed to assess clinically relevant differences in outcomes following percutaneous PDA closure versus alternative (surgical ligation) management strategies.

Practice Variation in Single-Ventricle Patients Undergoing Elective Cardiac Catheterization: A Report from the Congenital Cardiac Catheterization Project on Outcomes (C3PO).

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.