PDA Stent Placement *6: High-Impact Limitations of Angiography to Delineate a Complex PDA.

This is a case of an infant with duct-dependent pulmonary circulation, who required 6 stents delivered over three procedures to fully stent the arterial duct, which originated in a very unusual fashion. The attainable angiographic projections were unable to profile its origin, and only a CT scan was ultimately able to delineate the (stenotic) ductal origin from the aorta.

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.

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.

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.

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.

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 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.

Transcatheter pulmonary valve replacement: State of the art.

BACKGROUND: The number of patients undergoing transcatheter pulmonary valve replacement has been steadily increasing, resulting in many research projects focusing on this patient population. This has highlighted the need to summarize the currently available data. METHODS: A review of the literature was conducted and the results summarized. RESULTS: At present, the two commercially available valves approved for transcatheter pulmonary valve replacement include the Edwards Sapien valve and the Medtronic Melody valve. More data has been published relating to the Melody valve; this included the incidence of coronary anatomy prohibiting valve implantation of 5%, an incidence of conduit disruption of 6%, and freedom from stent fracture of the Melody valve stent of 60% at 30 months. Short and medium term outcomes seem to be comparable if not superior to surgical valve replacement, but long-term data is still lacking. There may also be a cost benefit with transcatheter valve implantation when compared to surgical pulmonary valve replacement. CONCLUSIONS: Transcatheter pulmonary valve replacement is here to stay. The main challenge of treating patients with a native right ventricular outflow tract still needs to be addressed and device development efforts centered in this area. © 2015 Wiley Periodicals, Inc.

Quality metrics in cardiac catheterization for congenital heart disease: utility of 30-day mortality.

OBJECTIVES: To characterize the frequency and attributability of death among patients who died within 30 days of their cardiac catheterization (30-day mortality). BACKGROUND: 30-day postprocedure mortality is commonly used as a quality outcome metric in national cardiac catheterization registries. It is unclear if this parameter is sufficiently specific to meaningfully capture mortality attributable to cardiac catheterization in patients with congenital heart disease (CHD). METHODS: Multicenter cohort study with 3 participating centers. Records were retrospectively reviewed for patients who died within 30 days of catheterization (06/2007-06/2012). Attributability of death was assigned to each case. RESULTS: A total of 14,707 cardiac catheterization procedures were performed during the study period. Death occurred within 30 days in 279/14,707 (1.9%) of cases. Among the patients who died, 53% of cases were emergent or urgent cases. The median age was 4 mos (1 day-45 years). Death was attributable to the catheterization procedure in 29/279 (10%) of cases. Death was attributable to cardiac surgery in 14%, precatheterization clinical status in 34%, postcatheterization clinical status in 22%, and noncardiac comorbidity in 19%. In 1%, death attributability could not be established. CONCLUSIONS: While valuable in adult settings, 30-day mortality is inadequate as a quality metric among patients with CHD undergoing cardiac catheterization. To derive the optimal benefit from catheterization registry data, more robust methodologies to capture procedure-related mortality are needed. © 2014 Wiley Periodicals, Inc.

Catheterization diastolic pressures correlate with diastolic dyssynchrony in patients with single right ventricles.

INTRODUCTION: Diastolic dyssynchrony has correlated with pulmonary capillary wedge pressures in patients with normal cardiac anatomy. No data exist in single right ventricle (sRV) patients. Goal of this study was to determine if diastolic dyssynchrony in sRV patients correlates with ventricular end-diastolic pressures (VEDP). METHODS: Tissue Doppler imaging (TDI) and strain rate (SR) analysis of sRV patients undergoing catheterization were performed. Time interval from onset of QRS to peak TDI e'-wave was obtained. Differences in intervals were calculated: QRS (RV) - QRS (IVS) and QRS (RV) - QRS (LV). Time interval from onset of QRS to peak strain rate early diastolic wave (SRe) was obtained for the 6 segment model sRV. Standard deviation of the 6 SRe time intervals was calculated. Correlation of VEDP with timing intervals was analyzed. RESULTS: Forty sRV patients were evaluated. Age was 2.8 ± 3.5 years. Catheterization VEDP of the sRV was 9.3 ± 3.9 mmHg (median 8 mmHg range 4-24 mmHg). QRS (RV) - QRS (IVS) was 22.3 ± 18.1 msec and QRS (RV) - QRS (LV) was 23.7 ± 19.0 msec. SRe standard deviation of the sRV was 61.6 ± 23.9 msec. There was no significant correlation with VEDP and QRS (RV) - QRS (IVS) (r = 0.1, P = NS) or with QRS (RV) - QRS (LV) (r = 0.2, P = NS). There was a significant correlation of VEDP with the SRe standard deviation value (r = 0.4, P < 0.05). CONCLUSION: Diastolic dyssynchrony correlated with VEDP in patients with sRV physiology. Future studies are needed to determine the significance of these findings.

Device closure of secundum atrial septal defects in infants weighing less than 8 kg.

This study aimed to assess the technical aspects of atrial septal defect (ASD) closure using the Amplatzer septal occluder (ASO) and the Gore Helex septal occluder (GHSO) for infants weighing less than 8 kg and to determine the safety, effectiveness, and near-to-intermediate-term outcome of the closure. The Mid-Atlantic Group of Interventional Cardiology Registry of percutaneous, transcatheter ASD closure procedures was reviewed for this analysis. Patients from 10 hospitals in the United States were included. The cohort for this report consisted of 68 patients weighing less than 8 kg (range, 2.3-7.8 kg; mean, 5.5 ± 1.6 kg) and ranging in age from 1 to 24 months (mean, 8.6 ± 4.7 months). The indications for ASD closure were failure to thrive, significant right heart enlargement, shunts otherwise thought to be hemodynamically significant, and poor overall clinical status. Devices were successfully implanted in 66 of the 68 infants (97.1 % procedural success rate). Five minor procedure-related complications occurred. At follow-up assessment, clinical status had improved significantly as measured by improved weight gain and decreased ventilator or oxygen dependence. All residual shunts spontaneously closed during the follow-up period. Six late deaths occurred, none of which were clearly device related. The ASO and GHSO can be safely and effectively implanted for ASD closure in infants weighing less than 8 kg. These procedures usually are successful and seldom complicated, resulting in significant clinical improvement.

Comparing echocardiographic assessment of systolic function with catheterization data in patients with single right ventricles.

PURPOSE: Echocardiographic evaluation of systolic function in patients with single right ventricles (SRV) is important but remains challenging. Minimal data exist correlating echocardiographic indices with catheterization data in this population. The goal of this study was to evaluate which echocardiographic measurement correlated best with dP/dt (max) obtained by cardiac catheterization in SRV patients. METHODS: Patients with SRV physiology who underwent simultaneous echocardiography and cardiac catheterization were evaluated. Echocardiographic data included fractional area change % (FAC), displacement, TDI s'wave, myocardial performance index (MPI), global systolic strain, and global SR s wave. Maximum positive rate of ventricular pressure change measured as dP/dt (max) was obtained from the cardiac catheterization report. Correlations of echocardiographic and catheterization variables were examined using the Pearson correlation. RESULTS: Twenty-seven SRV patients were studied. Median age at the time of the catheterization was 11.4 months (range 0 - 132 months). dP/dt (max) values ranged from 337-1860 mmHg/s with a median of 994 mmHg/s. Mean FAC was 27.15 +/- 7.13%, displacement was 7.35 +/- 2.88 mm, TDI s' was 4.98 +/- 1.93 cm/sec, MPI was 0.41 +/- 0.17, global strain was-14.85 +/- 4.32%, and global SR s wave was -1.03 +/- 0.34 sec(-1). There were no significant correlations between dP/dt (max) and any of the echocardiographic measurements of systolic function in SRV patients. CONCLUSION: In patients with SRV physiology, catheterization-derived dP/dt (max) did not correlate with echocardiographic measurements of systolic function. Larger studies are needed to determine which non-invasive parameter best describes systolic function in patients with SRV.

The Medtronic Melody® transcatheter pulmonary valve implanted at 24-mm diameter--it works.

OBJECTIVES: We report the Melody valve implanted and/or expanded to 24-mm diameter. BACKGROUND: The Medtronic Melody valve has been implanted up to 22 mm in the pulmonary position for over a decade. METHODS: A retrospective chart review was performed on 82 patients who underwent Melody valve implant. Technical implant method, pre- and postimplant echocardiographic findings, and initial follow-up were reviewed. RESULTS: Between 04/2008 and 12/2011, 13 Melody valves were successfully implanted in 11 patients, median age 35 years (range 16-61 years), in the pulmonary (bioprosthetic valve, right ventricle to pulmonary artery conduit, native valve) position (n = 9), tricuspid position (bioprosthetic valve n = 3), and aortic position (bioprosthetic valve n = 1). Ten valves were delivered on a 24-mm balloon in balloon catheter and three were implanted using a 22-mm Ensemble balloon delivery system, followed by postdilation using a 24-mm × 2-cm Atlas balloon catheter. Postimplant, the median peak systolic gradient across the pulmonary valve was 7 mm Hg and median gradient across the tricuspid valve was 3 mm Hg. There was no change in gradient across the Melody valve in the aortic position where valve prosthesis-patient mismatch was present. Postimplant intracardiac echocardiography demonstrated none or mild valve regurgitation. No more than mild regurgitation was noted at a median follow-up of 9.5 months. CONCLUSIONS: The Melody valve can be implanted at 24 mm in the stenotic/regurgitant bioprosthetic pulmonary, tricuspid, and aortic valve, dysfunctional right ventricle to pulmonary artery conduit, and the native right ventricular outflow tract, whereas the valve remains competent with only mild regurgitation.

Relationship between procedural adverse events associated with cardiac catheterization for congenital heart disease and operator factors: results of a multi-institutional registry (C3PO).

BACKGROUND: Data examining the effect of operator years in practice and volume on adverse events (AE) after cardiac catheterization in patients with congenital heart disease is limited. METHODS AND RESULTS: Data were prospectively collected using a multi-center registry (C3PO). 10,885 catheterizations performed between 02/07 and 06/10 at eight institutions were included. AE rates were risk-adjusted for hemodynamic vulnerability, procedure type risk group, and age and compared between operators with different years in practice (YIP) and volume. AE occurred in 13% of procedures. Operators with less than five YIP had higher adjusted odds of any AE (OR 1.42, 95% CI 1.14-1.77) or a high severity AE (OR 1.35, 95% CI 1.04-1.75), when compared with operators with 5 to less than 25 YIP (5 < 25), while operators with ≥25 YIP had higher odds of a high severity (but not any) AE (OR 1.39, 95% CI 1.08-1.80). Operators with <5 YIP had a higher percentage of preventable AE (out of all AE, 16% vs. 8%, P < 0.001) as well as higher odds of vascular or cardiac trauma (OR 1.81, 95% CI 1.11-2.97), or technical AE (OR 1.98, 95% CI 1.31-2.99) when compared with operators with 5 < 25 YIP. There was no consistent relationship between operator volume, and incidence of AE. CONCLUSIONS: Operators with less than 5 years in practice have higher risk-adjusted AE rates. While an important consideration in guiding and mentoring operators with fewer years in practice, it is important to emphasize that reporting adverse events does not take into account procedural efficacy.

Low weight as an independent risk factor for adverse events during cardiac catheterization of infants.

BACKGROUND: Studies have documented the importance of procedure type and hemodynamic variables on the incidence of procedure related adverse events (AE) after cardiac catheterization. However, little is known about the impact of low weight on the incidence and severity of AE. METHODS: Data were prospectively collected using a multicenter registry (C3PO). Infants <1 year were divided into four weight categories: <2 kg, 2-3 kg, 3-5 kg, ≥5 kg. AE severity was classified as level 1-5 (none, minor, moderate, major, death). RESULTS: Eight centers submitted details on 3,679 cases (34% diagnostic) performed in infants <1 year from 2/07 to 6/10: <2 kg: 57 (1.5%), 2-3 kg: 403 (11%), 3-5 kg: 1,527 (41.5%), ≥5 kg: 1,692 (46%). AE occurred in 20% of cases (<2 kg: 28%, 2-3 kg: 25%, 3-5 kg: 23%, ≥5 kg: 16%) with 41% of all AE being level 3-5 AE. Death occurred more frequently in the <2 kg group (12%), 71% of which were interventional cases. The case-related mortality in all other weight groups was <1%. By multivariable analysis, weight <2 kg, 2-3 kg, and 3-5 kg were independent risk factors for high severity (level 3-5) AE (<2 kg: OR 2, 95%CI 1.1-3.6; 2-3 kg: OR 1.4, 95%CI 1-1.8; 3-5 kg: OR 1.3, 95%CI 1.1-1.5), with similar findings for all AE. Blood transfusions were more common in lower weight categories (<2 kg: 42%, 2-3 kg: 29%, 3-5 kg: 25%, ≥5 kg: 15%, p<0.001). CONCLUSIONS: The risk of AE during cardiac catheterization of infants increases with lower weight. Infants who weigh less than 2 kg have a significantly higher risk of adverse events (most notably death) even after correcting for hemodynamic vulnerability and procedure type risk group.

Adverse events rates and risk factors in adults undergoing cardiac catheterization at pediatric hospitals--results from the C3PO.

OBJECTIVE: Determine the frequency and risk factors for adverse events (AE) for adults undergoing cardiac catheterization at pediatric hospitals. BACKGROUND: Adult catheterization AE rates at pediatric hospitals are not well understood. The Congenital Cardiac Catheterization Project on Outcomes (C3PO) collects data on all catheterizations at eight pediatric institutions. METHODS: Adult (≥ 18 years) case characteristics and AE were reviewed and compared with those of pediatric (<18 years) cases. Cases were classified into procedure risk categories from 1 to 4 based on highest risk procedure/intervention performed. AE were categorized by level of severity. Using a multivariate model for high severity AE (HSAE), standardized AE rates (SAER) were calculated by dividing the observed rates of HSAE by the expected rates. RESULTS: 2,061 cases (15% of total) were performed on adults and 11,422 cases (85%) were performed on children. Adults less frequently underwent high-risk procedure category cases than children (19% vs. 30%). AE occurred in 10% of adult cases and 13% of pediatric cases (P < 0.001). HSAE occurred in 4% of adult and 5% of pediatric cases (P = 0.006). Procedure-type risk category (Category 2, 3, 4 OR = 4.8, 6.0, 12.9) and systemic ventricle end diastolic pressure ≥ 18 mm Hg (OR 3.1) were associated with HSAE, c statistic 0.751. There were no statistically significant differences in SAER among institutions. CONCLUSIONS: Adults undergoing catheterization at pediatric hospitals encountered AE less frequently than children did. The congenital heart disease adjustment for risk method for adults with congenital heart disease is a new tool for assessing procedural risk in adult patients.

Safety and efficacy of balloon pulmonary valvuloplasty: a multicenter experience.

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%, <1 month). Procedural success was achieved in 91% procedures, being defined as one or more of the following: post-BPV peak systolic valvar gradient to < 25 mm Hg (88%), decrease in gradient by 50% (79%), or reduction of RV/systemic pressure ratio by 50% (45%). Procedural success was more common in neonates, when compared to older patients (96% vs. 87%, P = 0.03). Risk factors for procedural failure included moderate or severe pulmonary valve thickening (OR 2.9, CI 1-8.3), and presence of supravalve PS (OR 9.6, CI 2.7-33.8). Low severity AEs (levels 1-2) occurred in 9% of patients and higher severity AEs (levels 3-5) occurred in 3% of patient; there were no deaths. Risk factors for any AE (levels 1-5) were age below 1 month (OR 3.5, CI 1.3-8.9), as well as operator experience of less than 10 years (OR 3.8, CI 1.5-9.9). CONCLUSIONS: Procedural success is common and AEs, especially higher severity AEs, are rare for BPV in patients with isolated PS. Results have improved considerably when compared to historical data.

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.