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The objective of this study was to evaluate the impact of the regular introduction of new technologies into interventional cardiac catheterization procedures, in this case new atrial septal defect (ASD) closure devices, while conducting a multi-center collaborative initiative to reduce radiation usage during all procedures. Data were collected prospectively by 8 C3PO institutions between January 1, 2014 and December 31, 2017 for ASD device closure procedures in the cardiac catheterization lab during a quality improvement (QI) initiative aimed at reducing patient radiation exposure. Radiation exposure was measured in dose area product per body weight (µGy*m2/kg). Use of proposed practice change strategies at the beginning and end of the QI intervention period was assessed. Radiation exposure was summarized by institution and by initial type of device used for closure. This study included 602 ASD device closures. Without changes in patient characteristics, total fluoroscopy duration, or number of digital acquisitions, median radiation exposure decreased from 37 DAP/kg to 14 DAP/kg from 2014 to 2017. While all individual centers decreased overall median DAP/kg, the use of novel devices for ASD closure correlated with a temporary period of worsening institutional radiation exposure and increased fluoroscopy time. The introduction of new ASD closure devices resulted in increased radiation exposure during a QI project designed to reduce radiation exposure. Therefore, outcome assessment must be contextualized in QI projects, hospital evaluation, and public reporting, to acknowledge the expected variation during innovation and introduction of novel therapies.

Graphical abstract, Highlights • LMCA ostial atresia has variable clinical presentation. • Diagnosis of LMCA ostial atresia is challenging and often requires multiple modalities. • Coronary revascularization is possible for LMCA ostial atresia by varying surgical techniques.

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.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.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.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 (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.

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

We present a patient with a supported Ross procedure and severe pulmonary homograft stenosis who developed cardiac arrest while undergoing transcatheter pulmonary valve replacement and was found to have a large iatrogenic aortopulmonary window. Cardiopulmonary resuscitation was initiated followed by covered stent placement, extracorporeal membrane oxygenation support, and ultimately emergent surgery with a good outcome. (Level of Difficulty: Advanced.), Graphical abstract, We present a patient with a supported Ross procedure and severe pulmonary homograft stenosis who developed cardiac arrest while undergoing…

Graphical abstract, Highlights • Kawasaki disease can present with incomplete clinical signs particularly in infants • Coronary complications are more common in infants with incomplete Kawasaki disease • Coronary anatomy must be carefully assessed in infants with cardiovascular collapse

The objective of this study was to evaluate the impact of the regular introduction of new technologies into interventional cardiac catheterization procedures, in this case new atrial septal defect (ASD) closure devices, while conducting a multi-center collaborative initiative to reduce radiation usage during all procedures. Data were collected prospectively by 8 C3PO institutions between January 1, 2014 and December 31, 2017 for ASD device closure procedures in the cardiac catheterization lab during a quality improvement (QI) initiative aimed at reducing patient radiation exposure. Radiation exposure was measured in dose area product per body weight (µGy*m

Introduction In patients with tetralogy of Fallot (TOF), use of transannular patch (TAP) may be required in order to relieve significant right ventricular outflow tract obstruction, subsequently resulting in pulmonary insufficiency (PI). The monocusp valve has been used to temporarily reduce insufficiency in hopes to improve short and midterm outcomes. The purpose of this study was to assess for potential benefits of the monocusp valve in this subset of patients. Design Between 2005 and 2016, 119 patients with TOF with pulmonary stenosis who underwent repair with TAP were analyzed, 43 (36.1%) had a monocusp valve placed. Immediate outcomes were assessed by postoperative echocardiograms, ICU data including time to extubation, chest tube duration, reintervention, length of stay, and mortality. Results Median age of repair was similar for monocusp group at 143.5 days and nonmonocusp at 137.0 days (P = .93). Peak preoperative right ventricular outflow tract obstruction was higher in the monocusp group (80 mm Hg vs. 70 mm Hg, P ≤ .01). Patients who had monocusp placed had longer bypass time. There was less PI for monocusp group immediately after repair and at discharge (P ≤ .01). There was no difference in days of intubation, chest tube duration, length of hospitalization, reintervention rates, or mortality. Conclusion Decreasing the degree of PI with a monocusp valve in patients undergoing repair for TOF repair with TAP does not improve clinical outcomes in the immediate postoperative period.

Transcatheter valve-in-valve and valve-in-ring implantation has become a common approach to treating patients with failed bioprosthetic tricuspid valves as well as failed surgical repairs of the tricuspid valve where an annuloplasty ring has been utilized. We describe a case where a Melody valve was percutaneously implanted in a native tricuspid valve with severe stenosis following surgical repair without a supporting annuloplasty ring.

Background There remains limited options for end stage pulmonary arterial hypertension that is refractory to medical therapy. The reprisal of the Potts shunt (pulmonary artery to descending aorta anastomosis) has been used to decrease right ventricle (RV) afterload by creating a right to left shunt in effort to promote lung transplant free survival. Methods Retrospective review of three patients ages 16, 19, and 27 years old who underwent the creation of a “reverse” Potts shunt at our institution in 2016. Results The patients were WHO functional class III or IV and on three classes of pulmonary hypertension specific drug therapy including IV prostacyclin. All patients had RV dysfunction and suprasystemic RV pressures. Two patients had a catheterization for dilation and stenting of a tiny patent ductus arteriosus (PDA) and one had emergent surgical placement of a pulmonary artery to descending aorta conduit. The catheterization procedures were well tolerated without complications. The surgical procedure was complicated with heavy bleeding and respiratory failure. All patients recovered from their procedure and experienced improved functional class with decrease in RV pressures to systemic levels but no improvement in RV function. One catheterization patient required restenting due to stent fracture at 7 months. Our surgical patient died from massive hemoptysis 13 months after the procedure. Conclusions Reverse Potts shunt physiology may be an option for end-stage PAH patients with suprasystemic RV pressures. Optimal timing of this procedure remains unclear, but if feasible, PDA stenting, even in adult patients may be accomplished with low morbidity.

OBJECTIVE: To improve our understanding of pulmonary arteriovenous malformations in univentricular congenital heart disease, our objective was to identify the effects of hepatic vein and superior vena cava constituents on lung microvascular endothelial cells independent of blood flow. METHODS: Paired blood samples were collected from the hepatic vein and superior vena cava in children 0-10 years-old undergoing cardiac catheterization. Isolated serum was subsequently used for in vitro endothelial cell assays. Angiogenic activity was assessed using tube formation and scratch migration. Endothelial cell survival was assessed using proliferation (BrdU incorporation, cell cycle analysis) and apoptosis (caspase 3/7 activity, Annexin-V labeling). Data were analyzed using Wilcoxon signed-rank test and repeated measures analysis. RESULTS: Upon incubating lung microvascular endothelial cells with 10% patient serum, hepatic vein serum increases angiogenic activity (tube formation, p=0.04, n=24; migration, p

A previously healthy, preadolescent female suffered an unwitnessed cardiac arrest with prompt return of circulation following bystander initiated resuscitation. Workup demonstrated the cause of her cardiac arrest to be distal left anterior descending coronary artery occlusion with small apical left ventricular transmural myocardial infarction, from a paradoxical embolus traversing a previously undiagnosed large sinus venous defect. This case demonstrates the value of cardiac magnetic resonance imaging may bring to the diagnosis of the pathophysiology leading to cardiac arrest.

Background Transcatheter pulmonary valve replacement (TPVR) is associated with a risk of procedural serious adverse events (SAE) and exposure to ionizing radiation. Objectives The purpose of this study was to define the risk of, and associations with, SAE and high-dose radiation exposure using large-scale registry data. Methods The analysis of the multicenter C3PO-QI registry was limited to patients who underwent TPVR from January 1, 2014, to December 31, 2016. SAE were defined as the occurrence of ≥1 moderate, major, or catastrophic events. Radiation dose was reported as dose area product adjusted for weight. Associations with outcome measures were explored in univariate and multivariable analyses. Results A total of 530 patients (59% male) underwent TPVR at a median age of 18.3 years (interquartile range [IQR]: 12.9 to 27.3 years) and weight of 58 kg (IQR: 43 to 77 kg) at 14 centers. Implant substrate included homograft (41%), bioprosthesis (30%), native right ventricular outflow tract (RVOT) (27%) and other (2%). TPVR indications were pulmonary insufficiency (28%), stenosis (23%), and mixed (49%). AE and SAE occurred in 26% and 13% of cases, respectively, including 1 mortality. SAE were more frequent in homograft conduit than other RVOT substrates, although SAE type and severity differed between implant substrates. Median radiation dose was 198 μGy·m2/kg (IQR: 94 to 350 μGy·m2/kg). Higher radiation dose was associated with older age, greater RVOT obstruction, and concomitant interventions (p Conclusions The incidence of SAE during TPVR in the C3PO-QI registry is high, but mortality is uncommon. Radiation dose is greater than for other congenital interventions and is associated with patient and procedural factors. Reintervention is common during early follow-up.

Objectives This study sought to update benchmark values to use a quality measure prospectively. Background Congenital Cardiac Catheterization Outcomes Project – Quality Improvement (C3PO-QI), a multi-center registry, defined initial radiation dose benchmarks retrospectively across common interventional procedures. These data facilitated a dose metric endorsed by the American College of Cardiology in 2014. Methods Data was collected prospectively by 9 C3PO-QI institutions with complete case capture between 1/1/2014 and 6/30/2015. Radiation was measured in total air kerma (mGy), dose area product (DAP) (µGy*M2), DAP per body weight, and fluoroscopy time (min), and reported by age group as median, 75th and 95th%ile for the following six interventional procedures: (1) atrial septal defect closure; (2) aortic valvuloplasty; (3) treatment of coarctation of the aorta; (4) patent ductus arteriosus closure; (5) pulmonary valvuloplasty; and (6) transcatheter pulmonary valve implantation. Results The study was comprised of 1,680 unique cases meeting inclusion criteria. Radiation doses were lowest for pulmonary valvuloplasty (age 15 yrs, median mGy: 1835, DAP: 17990). DAP/kg standardized outcome measures across weights within an age group and procedure type significantly more than DAP alone. Radiation doses decreased for all procedures compared to those reported previously by both median and median weight-based percentile curves. These differences in radiation exposure were observed without changes in median fluoroscopy time. Conclusions This study updates previously established benchmarks to reflect QI efforts over time. These thresholds can be applied for quality measurement and comparison. © 2017 Wiley Periodicals, Inc.

The Melody® transcatheter pulmonary valve system was developed for placement within right ventricle-to-pulmonary artery conduits in patients with CHD for treatment of stenosis or regurgitation, providing an alternative to open-heart surgery. Abnormal systemic venous connections altering the catheter course to the right ventricle-to-pulmonary artery conduit may present a challenge to Melody® valve implantation. We present two such cases, in which the Melody® valve was successfully implanted in teenage patients with congenitally corrected transposition of the great arteries after Senning atrial switch operation. Despite the abnormal catheter course, the right ventricle-to-pulmonary artery was approachable via the right femoral vein allowing for deployment of the Melody® valve in the appropriate position. This suggests that systemic vein-to-left atrium baffles are not prohibitive of Melody® valve implantation. This is an important implication considering the substantial population of ageing patients with CHD who have undergone atrial switch. Melody® valve implantation can be considered as a viable option for treatment of these patients if they develop right ventricle-to-pulmonary artery conduit failure.

To stratify diverse procedure types into categories with similar radiation exposure in cardiac catheterization for congenital heart disease. Radiation exposures for a comprehensive list of specific procedure types and stratification of outcomes based on radiation risk are not currently available. Data between January 2014 and December 2015 were collected on all cases performed at sites participating in C3PO-QI (Congenital Cardiac Catheterization Outcomes Project-Quality Improvement Initiative) and 9 centers were included. Using expert consensus, 40 unique procedure types were defined by diagnostic characteristics or the intervention(s) performed, and dose area product (DAP) per kilogram of body weight (µGy × m2/kg) was summarized. Using empiric and consensus methods, three radiation risk categories were created. A total of 11,735 cases were included for analysis. Thirteen (n = 7918) procedure types with median DAP/kg

Congenital coronary artery anomalies are a well-known cause of life-threatening syncope, but usually present with either cardiac arrest, or after resolution of the episode. We discuss a young man with exertional syncope, who upon evaluation in the field, had electrocardiographic evidence of severe but transient myocardial ischemia. This case provides rare insight into the pathophysiology of sudden cardiac death in patients with anomalous left coronary artery from the right aortic sinus.

Neonatal tetralogy of Fallot (TOF) repair carries an increased risk of low birthweight or premature infants. Studies are investigating stents in the right ventricular outflow tract (RVOT) as an alternative to aortopulmonary shunts. The authors review their institutional experience with RVOT stenting in the high-risk infant with TOF. Data on sequential patients who received RVOT stents were reviewed, with collection of their surgical, echocardiographic, and catheterization data. Size-matched control subjects were identified and outcomes compared. Six infants went to the catheterization lab for RVOT stenting from 2008 to 2010. Five of these patients had placement of an RVOT stent after balloon dilation. The median saturations were 71% on 48% fraction of inspired oxygen (FiO2), with improvement to 94% (p < 0.001) on 39% FiO2 24 h after stent placement. As shown by echocardiography, the diameter of the median right pulmonary artery (RPA) was 2.6 mm (z-score, -3.3), and the diameter of the left pulmonary artery (LPA) was 2.0 mm (z-score, -4.5). Repeat echocardiography before surgery showed a statistically significant increase in RPA and LPA size as well as a modified McGoon ratio (p < 0.05). Four of the five patients subsequently underwent TOF repair. No stent fractures occurred. One patient had repair 10 days after stent placement secondary to stent malposition and tricuspid valve injury. The authors' experience with stents in the RVOT of TOF patients has yielded good results, with significant improvement in oxygen saturations. Patients had successful elective surgical repair and stent removal without longer cardiopulmonary bypass times or recognizable complications compared with shunted patients.

This study sought to update benchmark values to use a quality measure prospectively.Congenital Cardiac Catheterization Outcomes Project - Quality Improvement (C3PO-QI), a multi-center registry, defined initial radiation dose benchmarks retrospectively across common interventional procedures. These data facilitated a dose metric endorsed by the American College of Cardiology in 2014.Data was collected prospectively by 9 C3PO-QI institutions with complete case capture between 1/1/2014 and 6/30/2015. Radiation was measured in total air kerma (mGy), dose area product (DAP) (µGy*MThe study was comprised of 1,680 unique cases meeting inclusion criteria. Radiation doses were lowest for pulmonary valvuloplasty (age1 yrs, median mGy: 59, DAP: 249) and highest in transcatheter pulmonary valve implantation (age15 yrs, median mGy: 1835, DAP: 17990). DAP/kg standardized outcome measures across weights within an age group and procedure type significantly more than DAP alone. Radiation doses decreased for all procedures compared to those reported previously by both median and median weight-based percentile curves. These differences in radiation exposure were observed without changes in median fluoroscopy time.This study updates previously established benchmarks to reflect QI efforts over time. These thresholds can be applied for quality measurement and comparison. © 2017 Wiley Periodicals, Inc.

We report the case of a child with Abernethy malformation with an anomalous connection between the portal vein and the coronary sinus. After 30 months of close follow-up, the patient developed hepatoblastoma, a previously documented complication of the Abernethy malformation. This case reports a unique variant of Abernethy malformation and documents the first reported case of hepatoblastoma in a patient with type 2 Abernethy malformation.

Background: Controversy exists regarding the safety of cardiac catheterizations, particularly interventions, in the early post-operative period following congenital heart surgery. We sought to determine if adverse events are more frequent in patients undergoing early post-operative catheterizations

Objectives: The present study evaluates two transcatheter closure strategies utilized at a single center and makes recommendations for device selection when occluding the patent ductus arteriosus. Background: A variety of devices are available for transcatheter closure of the patent ductus arteriosus (PDA) but no guidelines exist to guide operator device choice. Methods: A total of 132 patients underwent attempted transcatheter PDA closure utilizing one of two consecutive closure strategies between January 2000 and June 2005. Strategy A (n = 64; January 2000–May 2003) utilized Gianturco coils only. Strategy B (n = 68; June 2003–June 2005) utilized a single Gianturco coil for the PDA with a minimal diameter ≤1 mm (n = 28) or an Amplatzer Duct Occluder (ADO) if the PDA diameter exceeded 1 mm (n = 40). Success was defined as complete occlusion on a follow up echocardiogram. Results: 58 of 64 (90.6%) patients treated utilizing strategy A had successful coil implantation. 68 of 68 (100%) patients treated utilizing strategy B had successful coil/device implantation. At follow up echocardiography, 32 of 44 (72.7%) strategy A patients had complete ductal closure, as compared with 57 of 58 (98.3%) strategy B patients (P < 0.0001). Stepwise logistic regression analysis identified closure strategy as the most powerful predictor of procedural success (OR = 85.9; CI 5.6–9.99). Conclusions: A transcatheter PDA closure strategy consisting of a single Gianturco coil for PDA ≤ 1 mm or an ADO for larger sized PDA (strategy B) achieves superior outcomes compared to the use of coils alone. © 2008 Wiley-Liss, Inc.

We describe an open-chest epicardial approach to transcatheter pulmonary artery stenting in a critically ill infant following heart transplantation. Technical considerations, indications, and feasibility are discussed. This case provides another example of the value of a "hybrid" approach (combining surgery and interventional cardiology) to complex congenital heart disease.

Early treatment with intravenous immunoglobulin (IVIG) is necessary to help reduce the risk of coronary artery abnormalities, such as coronary artery aneurysms and to help alleviate symptoms, in Kawasaki disease. Some patients, however, do not respond to an initial dose of IVIG and require additional doses. Prediction of these IVIG nonresponders may be of assistance in altering initial therapy to make it more effective. The Egami score has been validated in the Japanese population to predict IVIG nonresponders but has shown to be ineffective in US populations. This study evaluates the Egami score in a Midwest US population, subdividing patients by race and the diagnosis of typical or atypical type of Kawasaki disease. Patients were included in the study if they met criteria for Kawasaki disease and received IVIG in the inpatient setting. A total of 182 patients were studied, and in all studied groups, the Egami score had poor sensitivity at predicting IVIG nonresponders. Sensitivity of the score differed between races and differed between typical and atypical Kawasaki disease. The Egami score, as well as other systems, have been validated to predict IVIG nonresponders. These, however, lack sensitivity in the US population. Other scores developed in the United States have also lacked sensitivity, likely due to the absence of race or Kawasaki disease classification as variables. The development of a sensitive scoring system to predict IVIG nonresponders in US populations will require the incorporation of race and Kawasaki disease classification, factors that seem to alter IVIG response.

Antiepileptic medications have been reported to cause disturbances in cardiac conduction. Lacosamide decreases seizure burden by modulating sodium channels. Although it has been demonstrated to have few side effects, there have been reports of clinically significant cardiac conduction disturbances. We report the case of a child with hypoplastic left-heart syndrome and well-controlled multifocal atrial tachycardia who developed haemodynamically significant atrial tachycardia after receiving two doses of lacosamide.

Percutaneous closure of atrial septal defects (ASD) in adults has emerged as an alternative to surgery. We report a rare complication of an atrial septal occluder device embolization into the pulmonary artery which was detected by fluoroscopy and echocardiography. The potential usefulness of live/real time three-dimensional transthoracic echocardiography in the management of patients undergoing percutaneous ASD occlusion is described.

Annual surveillance coronary angiograpyhy to screen for graft coronary vasculopathy is routine practice after orthotopic heart transplantation. Traditionally, this is performed with direct coronary angiography using static single-plane or biplane angiography. Recently, technological advances have made it possible to perform dual-axis rotational coronary angiography (RA). This technique differs from standard static single-plane or biplane angiography in that a single detector is preprogrammed to swing through a complex 80° arc during a single injection. It has the advantage of providing a perspective of the vessels from a full arc of images rather than from one or two static images per contrast injection. The current study evaluated two coronary angiography techniques used consecutively at a single center to evaluate pediatric heart transplant recipients for graft coronary vasculopathy. A total of 23 patients underwent routine coronary angiography using both biplane static coronary angiography (BiP) and RA techniques at the Children’s Hospital of Wisconsin from February 2009 to September 2010. Demographic and procedure data were collected from each procedure and analyzed for significance utilizing a Wilcoxon rank sum test. No significant demographic or procedural differences between the BiP and the RA procedures were noted. Specific measures of radiation dose including fluoroscopy time and dose area product were similar among the imaging techniques. The findings show that RA can be performed safely and reproducibly in pediatric heart transplant recipients. Compared with standard BiP, RA does not increase radiation exposure or contrast use and in our experience has provided superior angiographic imaging for the evaluation of graft coronary vasculopathy.

dilation of the pulmonary valve with a balloonhas become widely accepted as the treatmentof choice for individuals with congenital pulmonaryvalvar stenosis. This technique is safe and effective inpatients of all ages. Currently, balloon valvoplasty isused for a wide spectrum of obstructive pulmonary val-var pathology, including valvar stenosis, dysplasia andatresia. In this review, we will address the history,indications, technique, immediate and long-term out-comes, as well as future outlook, of percutaneous bal-loon valvoplasty for the wide spectrum of congenitalpulmonary valvar disease.

This study examined the histological response of the bladder wall serosa to the implantation of wireless microstimulators secured with a single polypropylene suture. Two to three microstimulators were implanted in each of three casts for an eight week implantation period to allow sufficient time for a bladder-wall injury-response to develop. Gross observation revealed encapsulation of the microstimulators with no perforation to the bladder lumen or migration from the bladder wall. Histological evaluation confirmed that all the microstimutators were encapsulated with a thin connective tissue sheath and a thickened subserosal layer. There was no remarkable difference in tissue morphology compared with normal bladder wall sections for five of seven stimulators. Two microstimulators in one cat revealed a moderate to severe inflammatory response confined to a small area around the stimulator. In a second cat, a suture extended through the bladder wall. The microstimulators were observed with a scanning electron microscope after explantation. The electrode surfaces, bonding interface between silicon and glass and insulating films that were exposed to biological fluids were carefully inspected. All these observations indicate that the glass capsule reliably protected the sealed cavity of the microstimulators from moisture. These results indicate the microstimulator should be considered for further studies such as effects of stimulation and long-term implantation.

Objective: Kawasaki Disease (KD) is one of the most common vasculitides of childhood. Its precise pathophysiology, however, is not very well understood. KD occurs most frequently in southeastern Asian, particularly Japanese, populations and, thus, has been described extensively in this population. The American population is markedly different, having a significantly greater degree of racial hetero geneity. There is a paucity of data regarding differences in KD among different races in the American population. This study characterizes and compares clinical findings, laboratory values, echocardiogra phy findings, treatment efficacy, and long-term sequelae in a diverse population.