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.

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.

Radiation dose benchmarks during cardiac catheterization for congenital heart disease in the United States.

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

Transcatheter elimination of left-to-right shunts in infants with bronchopulmonary dysplasia is feasible and safe.

OBJECTIVE: To test the hypothesis that transcatheter elimination of left-to-right (L-R) cardiac shunts in former premature infants with bronchopulmonary dysplasia (BPD) is feasible, safe, and is associated with an improvement in respiratory status. DESIGN: Retrospective case review. PATIENTS: Twelve patients with BPD who underwent an attempt at transcatheter closure of an L-R shunt lesion within the first year of life at a single center. Median weight was 5.4 kg and median age was 6 months. Fifteen L-R shunt lesions included patent ductus arteriosus (n = 1), atrial septal defect (ASD) (n = 9), and aortopulmonary collaterals (n = 5). OUTCOME MEASURES: Echocardiographic and clinical markers were collected before and after intervention as well as procedural variables including successful elimination of the shunt and procedural complications. RESULTS: The L-R shunts were successfully occluded in 11/12 (91.6%) patients without any significant procedural adverse event. The ASD closure group demonstrated a decrease in right heart size after the procedure. All patients required respiratory support prior to, and 1 month after, the procedure while only 5/10 (50%) required respiratory support at 12 months of age (P = .0129). There was no change in the median weight percentile over time. CONCLUSION: Transcatheter occlusion of L-R shunts can be performed safely and effectively in children with BPD. Further studies may clarify the role of such therapy in improvement in respiratory physiology over time.

Use of a dose-dependent follow-up protocol and mechanisms to reduce patients and staff radiation exposure in congenital and structural interventions.

BACKGROUND: Increasingly complex structural/congenital cardiac interventions require efforts at reducing patient/staff radiation exposure. Standard follow-up protocols are often inadequate in detecting all patients that may have sustained radiation burns. METHODS: Single-center retrospective chart review divided into four intervals. Phase 1 (07/07-06/08, 413 procedures (proc)): follow-up based on fluoroscopy time only; frame rate for digital acquisition (DA) 30 fps, and fluoroscopy (FL) 30 fps. Dose-based follow-up was used for phase 2-4. Phase 2 (07/08-08/09, 458 proc): DA: 30 fps, FL: 15 fps. Phase 3 (09/09-06/10, 350 proc): DA: 15-30 fps, FL: 15 fps, use of added radiation protection drape. Phase 4 (07/10-10/10, 89 proc): DA: 15-30 fps, FL: 15 fps, superior noise reduction filter (SNRF) with high-quality fluoro-record capabilities. RESULTS: There was a significant reduction in the median cumulative air kerma between the four study periods (710 mGy vs. 566 mGy vs. 498 mGy vs. 241 mGy, P < 0.001), even though the overall fluoroscopy times remained very similar (25 min vs. 26 min vs. 26 min vs. 23 min, P = 0.957). There was a trend towards lower physician radiation exposure over the four study periods (137 mrem vs. 126 mrem vs. 108 mrem vs. 59 mrem, P = 0.15). Fifteen patients with radiation burns were identified during the study period. When changing to a dose-based follow-up protocol (phase 1 vs. phase 2), there was a significant increase in the incidence of detected radiation burns (0.5% vs. 2%, P = 0.04). CONCLUSIONS: Dose-based follow-up protocols are superior in detecting radiation burns when compared to fluoroscopy time-based protocols. Frame rate reduction of fluoroscopy and cine acquisition and use of modified imaging equipment can achieve a significant reduction to patient/staff exposure.

An institutional approach to interventional strategies for complete vascular occlusions.

Complete vascular occlusions are rare but potentially lethal. Reports on transcatheter therapy are limited to solitary case reports. The study was conducted as a single-center retrospective chart review. Between January 2003 and December 2009, 26 patients underwent cardiac catheterizations for either a known complete thoracic vascular occlusion or for incidental complete vascular occlusion that was noted during cardiac catheterization. Procedural technique, risk factors, and outcomes are reviewed. Median age at the time of diagnosis was 13.3 years (range 2 months to 54 years). In 6 of 26 (23%) patients, the vascular occlusion was of acute onset. Ninety-two percent of patients had at least 1 known risk factor for vascular occlusion, whereas 54% of patients had ≥ 2 risk factors. Successful rehabilitation was achieved in 19 patients, with direct catheter and/or wire manipulation being used to cross the occluded vessel in 15 (75%) patients, radiofrequency (RF) perforation in 3 patients, and perforation with Brockenbrough needle in 1 patient. Subsequent techniques included AngioJet (n = 2), balloon angioplasty and/or stent implantation (n = 18), and adjuvant local administration of recombinant tissue-plasminogen activator (n = 3). Reinterventions were required in 5 patients, and 8 patients died during the study period from causes unrelated to the catheterization procedure(s). Median follow-up of patients after successful recanalization was 12.6 months (range 1 day to 64 months), and the median duration of survival free from reintervention was 11.8 months (range 1 day to 64 months). In most patients, risk factors can be identified that are associated with the occurrence of a complete vascular occlusion. Different treatment strategies are used in patients having chronic compared with acute occlusions. Residual vascular lesions (hypoplasia or stenosis) are often associated with occurrence of reobstructions and should therefore be treated to prevent such an occurrence. Postprocedural anticoagulation is important in maintaining vascular patency.

Predictors of retrograde aortic arch obstruction after hybrid palliation of hypoplastic left heart syndrome.

A potential complication after hybrid stage 1 palliation for hypoplastic left heart syndrome (HLHS) is retrograde aortic arch obstruction (RAAO). This can lead to increased morbidity and unplanned surgical or interventional procedures in the interstage period. This study aimed to identify potential predictors of RAAO by analyzing initial echocardiograms and angiograms before hybrid stage 1 palliation. For this study, 96 patients who underwent hybrid stage 1 palliation between July 2002 and July 2009 were reviewed, 68 of which had standard HLHS and met the inclusion criteria. The initial echocardiogram, hybrid stage 1 angiograms, and follow-up echocardiograms were reviewed. Anatomic and hemodynamic measurements were obtained by both modalities, and comparisons were made between those who developed RAAO and those who did not. Of the 68 patients, 20 (29%) had RAAO. The mean aortic root size was smaller for the patients who had RAAO (3.6 vs 4.4 mm; p = 0.036). The angiographic angle between the aortic isthmus and the patent ductus arteriosus (PDA) was significantly larger in the RAAO group (86° vs 63°; p = 0.008). The retrograde aortic arch velocities were higher in the RAAO group. Patients with RAAO have a smaller aortic root and higher retrograde velocities on initial echocardiogram. Patients with RAAO show a larger angle between the retrograde arch and PDA on angiogram. Because RAAO is an important potential complication after hybrid stage 1 palliation for HLHS, identification of predictors of RAAO may lead to improved care and outcome for patients with RAAO.

Comparing balloon angioplasty, stenting and surgery in the treatment of aortic coarctation.

This article summarizes the currently available data regarding the management of coarctation, comparing balloon angioplasty, surgery and transcatheter stent therapy. The vast majority of reported studies are retrospective institutional series and comparative prospective studies are scarce, making it difficult to clearly recommend a treatment strategy. Long-term data are lacking, with most studies evaluating transcatheter approaches having a median follow-up of 5 years or less. Surgical repair is recommended for most patients with duct-dependent neonatal coarctation, while stent implantation for older children and adults has been shown to have excellent short-term results. Balloon angioplasty for (native) coarctation is effective in leading to an acute gradient reduction, but has been found to have a higher incidence of recoarctation or aortic wall complications. Long-term data from prospective registries, such as the Comprehensive, Continuous, Integrated System of Care (CCISC), are required before being able to make more firm recommendations upon the most appropriate treatment algorithm.

Radiation protection during hybrid procedures: innovation creates new challenges.

BACKGROUND: The cooperation between interventional cardiologists and cardiothoracic surgeons has expanded the spectrum of treatment modalities for patients with congenital heart disease. These hybrid techniques have created new challenges, one of which being the provision of adequate but practical radiation protection. This study evaluates the use of a lightweight radiation protection drape (RADPAD) that may be suitable for shielding during hybrid procedures. METHODS: To simulate a pediatric patient, an 8.7 liter water-filled tub was placed on an X-ray table and exposed to 10-second cine acquisition runs. Radiation exposure was measured at twelve specified locations around the table using a model with three different levels of radiation protection: no shielding, shielding using a traditional 0.35 mm lead-equivalent apron, and shielding using the 0.25 mm lead-equivalent RADPAD. RESULTS: The traditional lead apron and the RADPAD significantly reduced the amount of radiation dose when compared with no shielding. The standard lead apron provided slightly greater radiation protection than the RADPAD (0.000064 radiation absorbed dose [rad] vs. 0.000091 rad; p = 0.012). The measured rad was significantly higher on the right side of the table, and the measured radiation dose decreased significantly with increasing distance from the table. CONCLUSIONS: The RADPAD has been shown to function as an efficient shielding device, even though it does not quite match the protection that can be expected from a standard lead apron. It complies with regulatory radiation protection requirements and its lightweight and sterile use make it particularly useful during hybrid procedures in the operating room.

Completion angiography after cardiac surgery for congenital heart disease: complementing the intraoperative imaging modalities.

Residual structural pathology after cardiac surgery for congenital heart disease may complicate postoperative recovery. Completion angiograms obtained in the operating room may facilitate early detection and therapy of residual structural abnormalities. Our objective here is to report our institutional experience performing completion angiograms after cardiopulmonary bypass surgery. Between October 2007 and August 2008, 31 patients underwent completion angiograms after 32 cardiac surgical procedures. The median age was 7.5 months (range, 50 days to 31.2 years) and the median weight was 6.5 kg (range, 3.1-153 kg). Type of procedure, angiographic findings, and therapeutic decision were retrospectively reviewed. Procedures (proc) evaluated through completion angiography included comprehensive stage II or Glenn (n = 13), aortic arch reconstruction/conduit (n = 3), repair/palliation of tetralogy of Fallot or pulmonary atresia with ventricular septal defect (n = 4), PVR or conduit replacement (n = 5), and others (n = 7). Unexpected pathology was identified in 18 of 32 (56.3%) proc, which included left pulmonary artery (LPA) stenoses (n = 15), right pulmonary artery (RPA) stenoses (n = 11), and stenosis impairing coronary blood flow (after DKS; n = 1). In 9 of 32 (28.1%) proc, findings may have led to a change in therapeutic management. This included surgical revision (n = 1), 'Hybrid' therapy in the same setting (n = 2: LPA stent, 1; RPA balloon, 1), early catheterization within 3 months (n = 4), and change in medical management (n = 2: change in anticoagulation, 1; early CT, 1). Complications related to completion angiography were seen in only a single procedure (LPA staining). In conclusion, completion angiograms using a dedicated Hybrid cardiac operating suite may aid in early diagnosis and therapy of postsurgical abnormalities. They complement other methods of intraoperative imaging and may reduce the potential need for early surgical or transcatheter reintervention.

An animal model for hybrid stage I palliation of hypoplastic left heart syndrome.

Hybrid palliation has evolved as an important alternative treatment strategy for patients with hypoplastic left heart syndrome (HLHS). The procedural cooperation between cardiothoracic surgeon and interventional cardiologist is not intuitive and there is an increasing demand to avoid mistakes of the 'early learning curve,' through in vivo teaching using an animal model. We describe a unique experience of developing a piglet model that can be used to teach the procedural technique of hybrid Stage I palliation. The model has been used in about 140 piglets and consists of percutaneous patent ductus arteriosus (PDA) balloon angioplasty using a 6-mm balloon in piglets with a weight of 2-2.5 kg, followed within 4-7 days by bilateral PA banding as well as PDA stent placement in a hybrid procedure through a midline sternotomy.

Atrial septal interventions in patients with hypoplastic left heart syndrome.

OBJECTIVES: To report an institutional experience performing percutaneous atrial septal interventions in patients with hypoplastic left heart syndrome (HLHS). BACKGROUND: The success of the Hybrid approach in palliating patients with HLHS is crucially dependant on relieving any significant interatrial restriction. Data on transcatheter interventions to relieve atrial septal restrictions in patients with HLHS are limited. METHODS: We retrospectively reviewed 67 transcatheter atrial septal interventions that were performed between July 2002 and September 2007 in 56 patients with HLHS. The median weight was 3.35 kg. About 10.7% of patients had an intact atrial septum. Balloon atrial septostomy (BAS) was used in 77.6% of procedures, additional techniques in 35.8% of procedures. Patients were divided into those with standard atrial septal anatomy (group A, n = 33) and those with complex atrial septal anatomy (group B, n = 23). RESULTS: The mean trans-septal gradient was reduced significantly from 7 mm Hg to 1 mm Hg with the median time to discharge being 3.5 days. Major adverse events were seen in 8.9% of procedures, whereas minor adverse events occurred in 26.8% of procedures. Adverse events were significantly less common in patients with standard atrial septal anatomy, compared to those with complex atrial septal anatomy (25.6% versus 50.0%). About 19.6% patients required repeated atrial septal interventions. Survival up to and including Comprehensive stage II palliation was 73% group A, and 57% in group B. CONCLUSIONS: With utilization of appropriate techniques and equipment, atrial septal interventions in HLHS can be performed successfully in virtually all patients. Complex atrial septal anatomy is technically challenging and has a higher incidence of procedural adverse events. In the majority of patients, standard BAS can be performed safely, and is usually the only intervention required to achieve adequate relief of atrial septal restriction until Comprehensive stage II palliation.