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ABSTRACT: Objectives: The Coarctation of the Aorta Stent Trial (COAST) is a pivotal trial of the NuMED Cheatham Platinum Stent(®) for treatment of Coarctation of the Aorta (CoA). Background: CoA is a congenital obstruction to flow through the aorta. Stents have been used since the 1990s to treat CoA; none have been approved by the Food and Drug Administration for this indication. Methods: In a prospective, multi-center study 105 patients received stents for treatment of CoA. Data was collected in the catheterization laboratory, on discharge and at one month post procedure. Results: Of 105 patients 69% were male; 57% had native CoA. Noninvasive, baseline systolic blood pressure (SBP) showed Upper Extremity (UE) SBP to be 140 ± 16 mmHg and UE to Lower Extremity (LE) SBP difference 29 ± 17 mmHg. At catheterization baseline ratio of minimum CoA diameter to diameter of descending aorta (CoA:DAo) was 0.46 ± 0.16. 104/105 implants were successful with one stent migration. There were no deaths or serious complications. Paradoxical hypertension occurred in 6%. 4% of patients experienced somewhat serious adverse events related to the procedure. All patients achieved relief of ascending aorta to DAo pressure gradient: mean = 2.0 ± 4.0 mmHg (p<0.001). The CoA:DAo increased to 0.84 ± 0.18. At one month UE SBP was 120 ± 12 mmHg, UE to LE SBP difference = -1 ± 12 mmHg. 99% of patients had UE to LE SBP difference <20 mmHg. Conclusions: Stenting of CoA, using the NuMED Cheatham Platinum Stent is acutely safe and effective for treatment of CoA. © 2013 Wiley Periodicals, Inc.
Catheterization and Cardiovascular Interventions 04/2013; · 2.29 Impact Factor
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ABSTRACT: BACKGROUND: One of the important benefits of using multidetector computed tomography (MDCT) is its capability to generate high-quality two-dimensional (2-D) multiplanar (MPR) and three-dimensional (3-D) images from volumetric and isotropic axial CT data. However, to the best of our knowledge, no results have been published on the potential diagnostic role of multiplanar and 3-D volume-rendered (VR) images in detecting pulmonary vein stenosis, a condition in which MDCT has recently assumed a role as the initial noninvasive imaging modality of choice. OBJECTIVE: The purpose of this study was to compare diagnostic accuracy and interpretation time of axial, multiplanar and 3-D VR images for detection of proximal pulmonary vein stenosis in children, and to assess the potential added diagnostic value of multiplanar and 3-D VR images. MATERIALS AND METHODS: We used our hospital information system to identify all consecutive children (< 18 years of age) with proximal pulmonary vein stenosis who had both a thoracic MDCT angiography study and a catheter-based conventional angiography within 2 months from June 2005 to February 2012. Two experienced pediatric radiologists independently reviewed each MDCT study for the presence of proximal pulmonary vein stenosis defined as ≥ 50% of luminal narrowing on axial, multiplanar and 3-D VR images. Final diagnosis was confirmed by angiographic findings. Diagnostic accuracy was compared using the z-test. Confidence level of diagnosis (scale 1-5, 5 = highest), perceived added diagnostic value (scale 1-5, 5 = highest), and interpretation time of multiplanar or 3-D VR images were compared using paired t-tests. Interobserver agreement was measured using the chance-corrected kappa coefficient. RESULTS: The final study population consisted of 28 children (15 boys and 13 girls; mean age: 5.2 months). Diagnostic accuracy based on 116 individual pulmonary veins for detection of proximal pulmonary vein stenosis was 72.4% (84 of 116) for axial MDCT images, 77.5% (90 of 116 cases) for multiplanar MDCT images, and 93% (108 of 116 cases) for 3-D VR images with significantly higher accuracy with 3-D VR compared to axial (z = 4.17, P < 0.001) and multiplanar (z = 3.34, P < 0.001) images. Confidence levels for detection of proximal pulmonary vein stenosis were significantly higher with 3-D VR images (mean level: 4.6) compared to axial MDCT images (mean level: 1.7) and multiplanar MDCT images (mean level: 2.0) (paired t-tests, P < 0.001). Thus, 3-D VR images (mean added diagnostic value: 4.7) were found to provide added diagnostic value for detecting proximal pulmonary vein stenosis (paired t-test, P < 0.001); however, multiplanar MDCT images did not provide added value (paired t-test, P = 0.89). Interpretation time was significantly longer and interobserver agreement was higher when using 3-D VR images than using axial MDCT images or MPR MDCT images for diagnosing proximal pulmonary vein stenosis (paired t-tests, P < 0.001). CONCLUSIONS: Use of 3-D VR images in the diagnosis of proximal pulmonary vein stenosis in children significantly increases accuracy, confidence level, added diagnostic value and interobserver agreement. Thus, the routine use of this technique should be encouraged despite its increased interpretation time.
Pediatric Radiology 03/2013; · 1.67 Impact Factor
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ABSTRACT: To develop a risk-adjustment method for evaluation of in-hospital mortality after noncardiac neonatal surgery regardless of gestational age.
Infants ≤ 30 days old undergoing noncardiac surgical procedures were identified by using the Kids' Inpatient Database (KID) 2000 + 2003. Neonates were included regardless of gestational age. International Classification of Disease, Ninth Revision, Clinical Modification codes were used to assign procedures to 1 of 4 previously derived risk categories. Prematurity and other clinical variables were assessed in logistic regression analysis. The final multivariable model was validated in 3 independent data sets: KID 2006, Pediatric Health Information System (PHIS) 2001-2003, and PHIS 2006-2008. The model was applied to generate standardized mortality ratios for institutions within PHIS 2006-2008.
Among 18437 eligible cases in KID 2000 + 2003, 15278 (83%) had 1 of 66 procedure codes assigned to a risk category and were eligible for analysis. In-hospital mortality for premature infants was 10.5% compared with 2.0% for full-term neonates. In addition to risk category, the clinical variables improving prediction of in-hospital death were prematurity, serious respiratory conditions, necrotizing enterocolitis, neonatal sepsis, and congenital heart disease. Area under the receiver-operator characteristic curve for the final model was 0.90. The model also showed excellent discrimination in the 3 validation data sets (0.90, 0.89, and 0.89). Within 41 institutions in PHIS, standardized mortality ratios ranged from 0.37 to 1.91.
This validated method provides a tool for risk adjustment of neonates undergoing noncardiac surgery to allow comparative analyses of in-hospital mortality.
PEDIATRICS 08/2012; 130(3):e568-74. · 4.47 Impact Factor
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ABSTRACT: Coarctation of the aorta (CoA) accounts for 4% to 5% of congenital cardiac abnormalities. Stent therapy has become an accepted alternative to surgery for older children and adults, although there are no balloon-expandable stents approved by the Food and Drug Administration for use in the aorta. The Cheatham-Platinum (CP) stent was designed for CoA therapy and is widely used outside the United States. We have designed the first prospective trial of stent therapy for CoA to serve as the pivotal trial for Food and Drug Administration approval of the CP stent.
The COAST study is a prospective, multicenter, single-arm clinical study. The population includes patients with native or recurrent CoA. Four primary outcome variables were defined. For each variable, the stent will be compared to performance guidelines derived from surgical experience. The first efficacy outcome is reduction in arm-leg systolic blood pressure gradient, and the second is reduction in hospital length of stay. Safety outcomes include the following: the occurrence of any serious or somewhat serious adverse event attributed to the stent or implantation procedure and the occurrence of postprocedure paradoxical hypertension. A total of 105 patients treated with the CP stent will be enrolled.
To ascertain the effectiveness and safety of an interventional device, randomized controlled trials have been offered as the criterion standard. However, these trials are not well suited to study rare conditions such as CoA, especially once the therapy in question has received acceptance within the medical community. New clinical trial and statistical approaches are needed to evaluate such therapies. The COAST study is an example of this kind of innovative trial design.
American heart journal 07/2012; 164(1):7-13. · 4.65 Impact Factor
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ABSTRACT: Objective. Mortality among children with congenital and acquired heart disease has decreased significantly over the past decades. We sought to determine whether the underlying problems leading to death in these patients had changed over the past decade. Methods. We reviewed medical records for 100 deaths of cardiac patients in 2004-2005 and 100 deaths in 1995-1996. Demographic, clinical, and procedural data as well as circumstances of death were collected. A consensus committee reviewed each case and sought to identify the condition leading to death. These conditions were classified as predominantly surgical or medical. Results. General patient characteristics (age, gender, cardiac history, comorbidities, proportion of surgical patients) did not change significantly between the two time periods. However, in 1995-1996, 64% of deceased surgical patients had died within 30 days of surgery. This rate was nearly halved to only 38% by 2004-2005 (P= .003). Furthermore, the conditions leading to death changed significantly: 51% of patient deaths in 1995-1996 resulted from a surgical problem, 29% from a medical condition. This ratio was reversed in 2004-2005: Only 31% of patient deaths were due to a surgical problem, while 50% of deaths resulted from a medical condition (P= .005). The most common medical conditions resulting in death were pulmonary vein stenosis, pulmonary arterial hypertension, and primary myocardial failure. Conclusions. The proportion of deaths within 30 days of cardiac surgery decreased significantly over the past decade. While surgical causes accounted for the majority of these deaths in 1995-1996, most patient deaths in 2004-2005 resulted from cardiac medical causes.
Congenital Heart Disease 05/2012; · 0.90 Impact Factor
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Jeffrey P. Jacobs,
Marshall L. Jacobs,
Francois G. Lacour-Gayet, Kathy J. Jenkins,
Kimberlee Gauvreau,
Emile Bacha,
Bohdan Maruszewski,
David R. Clarke,
Christo I. Tchervenkov,
J. William Gaynor,
Thomas L. Spray,
Giovanni Stellin,
Sean M. O’Brien,
Martin J. Elliott,
Constantine Mavroudis
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ABSTRACT: Quality-of-care evaluation must take into account variations in “case mix.” This study reviewed the application of two case-mix
complexity-adjustment tools in the Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database: the Aristotle Basic
Complexity (ABC) score and the Risk Adjustment in Congenital Heart Surgery (RACHS-1) method. The 2006 STS Congenital Heart
Surgery Database Report, the first STS report to incorporate both methods, included 45,635 operations from 47 centers. Each
operation was assigned an ABC score in a range from 1.5 (lowest complexity) to 15 (highest complexity), an ABC level in a
range from 1 (lowest complexity) to 4 (highest complexity), and a RACHS-1 category in a range from 1 (lowest risk) to 6 (highest
risk). The overall discharge mortality was 3.9% (1,222/31,719 eligible cardiac index operations). Of the eligible cardiac
index operations, 85.8% (27,202/31,719) were eligible for analysis by the RACHS-1 method, and 94.0% (29,813/31,719) were eligible
for analysis by the ABC approach. With both RACHS-1 and ABC, as complexity increases, discharge mortality also increases.
The ABC approach allows classification of more operations, whereas the RACHS-1 discriminates better at the higher end of complexity.
Complexity stratification is a useful method for analyzing the impact of case mix on pediatric cardiac surgical outcomes.
Both the RACHS-1 and ABC methods facilitate complexity stratification in the STS database.
Pediatric Cardiology 04/2012; 30(8):1117-1130. · 1.30 Impact Factor
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ABSTRACT: Pulmonary vein stenosis (PVS) is a progressive disease that is frequently lethal. We have previously identified neoproliferation of myofibroblasts as the mechanism for progressive intraluminal PVS. PVS occurs in association with other congenital heart diseases (CHD) and in structurally normal hearts. This study sought to describe the spectrum of CHD seen with PVS and explore risk factors associated with mortality.
All patients diagnosed over a 12-year period with a combination of PVS involving ≥2 vessels and CHD were identified. Cases were categorized according to major anatomic and physiologic categories. Patient and disease characteristics associated with time to death were explored.
Eighty-two cases followed longitudinally at our institution were analyzed. Anatomic diagnoses included nonheterotaxy + anomalous pulmonary venous return (29%), heterotaxy + anomalous veins (20%), two ventricles + normal veins (22%), and single ventricle + normal veins (29%). Median age at diagnosis was 5.3 months (0-24 years). Despite multiple treatments, there were 35 (43%) deaths in the group with an estimated survival of 71%, 64%, and 44% at 1, 2, and 5 years, respectively. Bilateral disease at diagnosis (hazard ratio [HR] 3.9 [1.7, 9.2], P= .002), age <5 months at diagnosis (HR 3.4 [1.6, 7.6], P= .002), and involvement of >2 pulmonary veins at diagnosis (HR 3.7 [1.6, 8.8], P= .003) were associated with shorter time to death in univariate analysis. In multivariable analysis, both bilateral disease (HR 2.9 [1.2, 7.1]P= .02) and age <5 months at diagnosis (HR 2.4 [1.1, 5.6]P= .03) were independently associated with time to death.
Bilateral disease and earlier age at diagnosis are independent predictors of poor survival in patients with CHD and PVS, while patients with unilateral disease presenting at an older age have a better prognosis. These findings are helpful in risk stratification of patients with CHD and multivessel PVS.
Congenital Heart Disease 04/2012; 7(4):378-86. · 0.90 Impact Factor
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ABSTRACT: The Adult Congenital and Pediatric Cardiology (AC/PC) Section was established to develop a clear voice within the American College of Cardiology and address the myriad issues facing the congenital heart disease profession. The Section is governed by the AC/PC Council, which includes pediatric cardiologists, adult congenital cardiologists, a cardiac care associate, and a fellow-in-training member. The Council is responsible for bidirectional communication between the College's Board of Trustees and the AC/PC Section members. Since its founding in 2004, Section objectives have been defined by the College's mission: to advocate for quality cardiovascular care through education, research promotion, and the development and application of standards and guidelines and to influence health care policy. The pillars of the College-advocacy, quality, education, and member engagement-serve as the defining template for the Section's strategy. The Section has developed work groups in advocacy, clinical practice, education and training, quality, and publications. A separate leadership group has been developed for adult congenital heart disease. Work groups are open to all Section members. Recognition of the importance of lifelong care in congenital heart disease led Section leaders to incorporate pediatric cardiology and adult congenital heart disease content into each of the work groups. There are more than 1,200 Section members, with nearly 400 members actively contributing to Section activities. This article outlines Section efforts to date and highlights significant successes to date.
Journal of the American College of Cardiology 01/2012; 59(1):84-7. · 14.16 Impact Factor
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ABSTRACT: To determine the safety and efficacy of the chemotherapeutic agents vinblastine and methotrexate in the treatment of children with progressive multivessel intraluminal pulmonary vein stenosis (PVS).
Children received weekly vinblastine and methotrexate for a period of 1 year. Outcomes (for patients receiving ≥1 month of chemotherapy) were classified separately for patients with isolated PVS and PVS with congenital heart disease (CHD). Primary efficacy outcome was "response to treatment" categorized by echocardiographic criteria of response. Survival to 1 year was also evaluated. All adverse events were classified according to Cancer Therapy Evaluation Program, Common Terminology Criteria version 3.0. Events were further classified as related to chemotherapy, cardiac, or other causes.
Among 29 patients enrolled, 28 received at least one dose of chemotherapy and were evaluable for toxicity, while 23 were evaluable for response (21 CHD, 2 isolated). Both patients in the isolated group had progressive disease and died. Overall, 33% (7/21) of patients with PVS and CHD had stable disease; 1-year survival of 38%; and four patients continue in remission (93, 96, 124, and 125 months after treatment initiation). While both cardiac-related (19%) and chemotherapy-related (53%) toxicities were common, most were asymptomatic laboratory changes. Grade 3 (13%) and grade 4 (4%) toxicities were reversible, and no treatment-related grade 5 toxicities were observed.
We report on the first prospective trial of chemotherapy for infants and children targeting the presence of myofibroblastic cells within the lesions of PVS based on myofibroblastic proliferation associated with desmoid tumors of infancy. The toxicity profile resulted in numerous treatment delays and interruptions that, combined with limited information on the natural history of PVS in this patient population, hampered our ability to determine the true efficacy of this approach. These results will be important as a baseline for clinical trials in this patient population.
Congenital Heart Disease 11/2011; 6(6):608-23. · 0.90 Impact Factor
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Lisa Bergersen,
Kimberlee Gauvreau,
Susan R Foerster,
Audrey C Marshall,
Doff B McElhinney,
Robert H Beekman,
Russel Hirsch,
Jacqueline Kreutzer,
David Balzer,
Julie Vincent,
William E Hellenbrand,
Ralf Holzer,
John P Cheatham,
John W Moore,
Grant Burch,
Laurie Armsby,
James E Lock, Kathy J Jenkins
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ABSTRACT: This study sought to develop a method to adjust for case mix complexity in catheterization for congenital heart disease to allow equitable comparisons of adverse event (AE) rates.
The C3PO (Congenital Cardiac Catheterization Project on Outcomes) has been prospectively collecting data using a Web-based data entry tool on all catheterization cases at 8 pediatric institutions since 2007.
A multivariable logistic regression model with high-severity AE outcome was built using a random sample of 75% of cases in the multicenter cohort; the models were assessed in the remaining 25%. Model discrimination was assessed by the C-statistic and calibration with Hosmer-Lemeshow test. The final models were used to calculate standardized AE ratios.
Between August 2007 and December 2009, 9,362 cases were recorded at 8 pediatric institutions of which high-severity events occurred in 454 cases (5%). Assessment of empirical data yielded 4 independent indicators of hemodynamic vulnerability. Final multivariable models included procedure type risk category (odds ratios [OR] for category: 2 = 2.4, 3 = 4.9, 4 = 7.6, all p < 0.001), number of hemodynamic indicators (OR for 1 indicator = 1.5, ≥2 = 1.8, p = 0.005 and p < 0.001), and age <1 year (OR: 1.3, p = 0.04), C-statistic 0.737, and Hosmer-Lemeshow test p = 0.74. Models performed well in the validation dataset, C-statistic 0.734. Institutional event rates ranged from 1.91% to 7.37% and standardized AE ratios ranged from 0.61 to 1.41.
Using CHARM (Catheterization for Congenital Heart Disease Adjustment for Risk Method) to adjust for case mix complexity should allow comparisons of AE among institutions performing catheterization for congenital heart disease.
09/2011; 4(9):1037-46. · 1.07 Impact Factor
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John M Karamichalis,
Pedro J del Nido,
Ravi R Thiagarajan, Kathy J Jenkins,
Hua Liu,
Kimberlee Gauvreau,
Frank A Pigula,
Francis E Fynn-Thompson,
Sitaram M Emani,
John E Mayer,
Emile A Bacha
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ABSTRACT: We hypothesize that a measure of the immediate postoperative severity of illness after the stage I Norwood operation reflects technical performance or the adequacy of anatomic repair and can serve as a predictor of hospital mortality, reinterventions, and clinical outcomes.
One hundred thirty-five patients undergoing stage I were retrospectively studied (2004 to 2007). The severity of illness on postoperative day 1 (POD1) was measured using the Pediatric Risk of Mortality III (PRISM) scoring system. Technical performance scores (optimal, adequate, inadequate) were calculated before hospital discharge. Hospital mortality, postoperative reinterventions, and complications were recorded. Postoperative reintervention was defined as need for cardiac catheterization laboratory or operating room based procedure that included balloon dilation or repair of arch obstruction, shunt revision, reoperations for bleeding, and extracorporeal membrane oxygenation support.
Hospital mortality was 14.1% (n=19). The rate of complications and reinterventions was, respectively, 28.1% (n=38) and 26.7% (n=36). The POD1 PRISM score was associated with technical performance (p=0.003). Higher POD1 PRISM scores were associated with mortality (p<0.001), complications (p<0.001), and reinterventions (p=0.001). The POD1 PRISM score had high discrimination for mortality, complications, reinterventions, and inadequate technical performance (areas under the receiver operating characteristic curve were 0.835, 0.776, 0.773, and 0.710, respectively; p≤0.001 for all).
The severity of illness as measured by PRISM score on POD1 after the stage I Norwood operation has strong association and discrimination with hospital mortality, postoperative reinterventions, inadequate technical performance, and major postoperative complications. It may be used as an early surrogate of technical performance to initiate a search for and correction of technical deficiencies.
The Annals of thoracic surgery 06/2011; 92(2):660-5. · 3.74 Impact Factor
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ABSTRACT: The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) is the first quality improvement collaborative in pediatric cardiology, and its registry captures information on interstage care and outcomes of infants after the Norwood procedure. The purpose of this study was to evaluate variation in interstage outpatient clinical care practices for infants discharged home after the Norwood procedure.
Data for the first 100 infants enrolled in the NPC-QIC registry were evaluated. The care domains assessed for variation included: (1) discharge communication with outpatient cardiologist and primary care physician (PCP); (2) nutrition plan at hospital discharge; and (3) planned use of home surveillance strategies.
One hundred infants were discharged home between July 2008 and February 2010, from 21 participating US pediatric cardiac programs. Median age at discharge was 29 (11-188) days. Interstage outpatient care was provided at the Norwood center for 62 infants, at other centers for 25, and at a combination of centers for 13. Complete discharge communication (defined as written communication of medication list, nutrition plan, and red flag checklist) was relayed to only 45 outpatient cardiologists and to 26 PCPs. Nutrition route at discharge was exclusively oral in 49, combined oral and nasogastric (NG)/nasojejunal (NJ) in 38, exclusively NG/NJ in six, combined oral and gastrostomy tube (GT) in six, and exclusively GT in one infant. Home surveillance strategies were utilized for 81 infants (oximetry and weight monitoring in 77, oximetry alone in four), with no home surveillance in 19 infants.
Considerable variation exists in interstage outpatient care after the Norwood procedure in the care domains of discharge communication, nutrition, and home surveillance. Standardizing care around evidence-based practices may improve the outcomes for these very high-risk children.
Congenital Heart Disease 03/2011; 6(2):98-107. · 0.90 Impact Factor
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David W Brown,
Jean A Connor,
Frank A Pigula,
Kudret Usmani,
Thomas S Klitzner,
Robert H Beekman,
John D Kugler,
Gerard R Martin,
Steven R Neish,
Geoffrey L Rosenthal,
Carole Lannon, Kathy J Jenkins
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ABSTRACT: As the first multicenter quality improvement collaborative in pediatric cardiology, the Joint Council on Congenital Heart Disease National Pediatric Cardiology Quality Improvement Collaborative registry collects information on the clinical care and outcomes of infants discharged home after first-stage palliation of single-ventricle heart disease, the Norwood operation, and variants. We sought to describe the preoperative and intraoperative characteristics of the first 100 patients enrolled in the National Pediatric Cardiology Quality Improvement Collaborative registry.
From 21 contributing centers, 59% of infants were male, with median birth weight of 3.1 kg (1.9-5.0 kg); the majority had hypoplastic left heart syndrome (71%). A prenatal diagnosis of congenital heart disease was made in 75%; only one had fetal cardiac intervention. Chromosomal anomalies were present in 8%, and major noncardiac organ system anomalies were present in 9%. Preoperative risk factors were common (55%) but less frequent in those with prenatal cardiac diagnosis (P= .001). Four patients underwent a preoperative transcatheter intervention. Substantial variation across participating sites was demonstrated for choice of initial palliation for the 93 patients requiring a full first-stage approach, with 50% of sites performing stage I with right ventricle to pulmonary artery conduit as the preferred operation; 89% of hybrid procedures were performed at a single center. Significant intraoperative variation by site was noted for the 83 patients who underwent traditional surgical stage I palliation, particularly with use of regional perfusion and depth of hypothermia.
In summary, there is substantial variation across surgical centers in the successful initial palliation of infants with single-ventricle heart disease, particularly with regard to choice of palliation strategy, and intraoperative techniques including use of regional perfusion and depth of hypothermia. Further exploration of the relationship of such variables to subsequent outcomes after hospital discharge may help reduce variability and improve long-term outcomes.
Congenital Heart Disease 03/2011; 6(2):108-15. · 0.90 Impact Factor
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ABSTRACT: The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) has established a national data registry for patients with hypoplastic left heart syndrome and its variants undergoing staged palliation. The goal of this collaborative is to better understand current care practices and to improve outcomes in children with these severe and complex forms of congenital heart disease. In this study, we describe the postoperative intensive care course, and its variations, for the first 100 patients enrolled into the registry.
Patients were enrolled from 21 contributing sites and were discharged home after stage I palliation between July 2008 and February 2010. Following stage I palliation, enrolled participants remained in the intensive care unit for a median duration of 11 days (range: 3-68 days). Duration of intensive care unit stay varied and was greatest for those patients with aortic atresia versus aortic hypoplasia (P= 0.04) and for those who underwent a modified Blalock-Taussig shunt as part of their palliation. The duration of intensive care unit stay also varied by contributing site (medians ranged from 8 to 18 days). Participants requiring reoperation had significantly prolonged lengths of stay (P= .0003). Inotropic agent use among univentricular registry participants also varied by site. The majority of recipients received milrinone (87%), dopamine (64%), and epinephrine (62%). Cardiac catheterization following surgery occurred in 20 patients. Fifteen percent of participants underwent an interventional procedure. Complication following stage I palliation was also fairly common.
Considerable variation exists in the postoperative course and management of univentricular patients following stage I palliation. Variation in length of intensive care unit stay, inotropic agent use, need for reoperation or cardiac catheterization, and postoperative complications are described. Further studies to determine etiologies for observed variation may result in improved standards of care and better outcomes during the interstage period.
Congenital Heart Disease 03/2011; 6(2):116-27. · 0.90 Impact Factor
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Circulation 11/2010; 122(19):1957-67. · 14.74 Impact Factor
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ABSTRACT: Imaging of the lymphatic system is critical in preoperative planning of resections of complex lymphatic malformations. However, safe, effective imaging methods with sufficient resolution to identify the lymphatics have been lacking. In this study, we demonstrate the use of gadolinium-labeled dendrimers to image the lymphatics in small and large animal models during magnetic resonance lymphangiography.
Polyamidoamine G6-Gd_1B4M_N-hydroxysuccinimide was synthesized and administered intradermally in the extremities of normal mice and pigs at several doses.
The lymphatics were well demonstrated in both animal models and there was rapid uptake in the deep lymphatic system, including the thoracic duct. A significant dose reduction was achieved (1 µmol Gd/kg) in the 35-kg pig compared with mice, while still producing excellent results. No toxicity was observed and only minor inflammatory changes were observed at the injection site 30 days later.
We demonstrate that a low dose of a macromolecular magnetic resonance contrast agent can provide rapid imaging of the deep lymphatic system in both small and large animals. This data provides a basis to consider a similar agent in clinical trials.
Nanomedicine 10/2010; 5(8):1183-91. · 5.05 Impact Factor
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ABSTRACT: A Standardized Clinical Assessment and Management Plan (SCAMP) is a novel quality improvement initiative that standardizes the assessment and management of all patients who carry a predefined diagnosis. Based on periodic review of systemically collected data the SCAMP is designed to be modified to improve its own algorithm. One of the objectives of a SCAMP is to identify and reduce resource utilization and patient care costs.
We retrospectively reviewed resource utilization in the first 93 arterial switch operation (ASO) SCAMP patients and 186 age-matched control ASO patients. We compared diagnostic and laboratory testing obtained at the initial SCAMP clinic visit and control patient visits. To evaluate the effect of the SCAMP over time, the number of clinic visits per patient year and echocardiograms per patient year in historical control ASO patients were compared to the projected rates for ASO SCAMP participants.
Cardiac magnetic resonance imaging (MRI), stress echocardiogram, and lipid profile utilization were higher in the initial SCAMP clinic visit group than in age-matched control patients. Total echocardiogram and lung scan usage were similar. Chest X-ray and exercise stress testing were obtained less in SCAMP patients. ASO SCAMP patients are projected to have 0.5 clinic visits and 0.5 echocardiograms per year. Historical control patients had more clinic visits (1.2 vs. 0.5 visits/patient year, P<.01) and a higher echocardiogram rate (0.92 vs. 0.5 echocardiograms/patient year, P<.01)
Implementation of a SCAMP may initially lead to increased resource utilization, but over time resource utilization is projected to decrease.
Congenital Heart Disease 07/2010; 5(4):374-81. · 0.90 Impact Factor
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ABSTRACT: Little is known regarding the incidence and financial impact of adverse events associated with the surgical care of children. The purpose of this study was to characterize the epidemiology and resource utilization associated with these events using definitions validated from the adult population.
We conducted a 6-year audit (Jan 2003-Dec 2008) of adverse events associated with the 100 most common general pediatric surgical procedures from the Pediatric Health Information System database. We audited 23 events as defined by the National Surgical Quality Improvement Project and modified Agency for Healthcare Research and Quality Patient Safety Indicators. Excess length of stay and total hospital charges attributable to events were determined for each procedure after adjusting for confounders.
Overall 30-day incidence of any adverse event was 10.3% in our sample of 331,093 patients. The most common events were transfusions (30% of all events), wound complications (15%), and events associated with central access (11%). The cumulative incidence of serious events including cardiac arrest, stroke, deep venous thrombosis and pulmonary embolish was less than 0.3%. Ten procedures accounted for 62% of all events, and all 10 were associated with significant (P < .01) increases in length of stay and total hospital charges when any event occurred. Circumcisions, soft-tissue biopsies, pyloromyotomies, and repair of abdominal wall hernias accounted for only 3% of events despite comprising nearly 25% of operative volume.
A relatively small number of pediatric surgical procedures contribute to a disproportionate share of adverse events. Although the National Surgical Quality Improvement Project and Agency for Healthcare Research and Quality criteria can identify pediatric procedures associated with a significant risk of morbidity, the relatively high 30-day event rates captured for some procedures may be heavily influenced by underlying co-morbidity profiles not related to the surgical disease or intervention. Furthermore, the validity of applying adult-focused "adverse" event definitions for the pediatric population should be further explored. Collaborative efforts will be needed to develop more clinically meaningful outcome measures for the purpose of quality improvement end points.
Journal of Pediatric Surgery 06/2010; 45(6):1126-36. · 1.45 Impact Factor
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ABSTRACT: To develop a risk adjustment method for in-hospital mortality in newborns undergoing noncardiac surgery.
Understanding variation in outcomes is critical to guide quality improvement. Reliable outcome assessments need risk adjustment to allow comparisons.
Infants <or=30-days-old undergoing noncardiac surgical procedures were identified using the Kids' Inpatient Database (KID); year 2000. Premature infants were excluded. Procedures identified by ICD-9-CM codes with >or=20 cases in the data set were placed into 4 risk categories by in-hospital mortality rates. Clinical variables were added to the model to better predict mortality; areas under the receiver-operator characteristic (ROC) curves were compared. The final model was validated in the KID 2003 database.
Among 6103 eligible cases in the KID 2000, 5117 (83.8%) could be assigned to a risk category. Mortality rates were 0.2% in risk category 1, 2.5% in category 2, 6.4% in 3, and 18.4% in 4. The odds of mortality increased in each risk category relative to category 1 (P < 0.001 for each). In multivariable models adjusting for risk category, the clinical variables most predictive of in-hospital death were serious respiratory conditions and necrotizing enterocolitis. The area under the ROC curve for the full model including clinical risk factors was 0.92 in the KID 2000. The model was validated using data for KID 2003 and showed excellent discrimination (ROC = 0.90).
This validated method provides a means of risk adjustment in groups of newborns undergoing noncardiac surgery, and should allow for comparative analyses of in-hospital mortality.
Annals of surgery 03/2010; 251(4):754-8. · 7.90 Impact Factor
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ABSTRACT: Tremendous advances have occurred in catheter-based interventions for congenital heart disease. Multicenter trials of these advances are either out of date or have been limited in scope. As such little is known on the application of these techniques in the current era. The IMPACT Registry (IMproving Pediatric and Adult Congenital Treatments) will allow us to measure variability in the performance and outcomes of both diagnostic and interventional cardiac catheterization procedures in all children and adults with congenital heart disease. The IMPACT Registry will be harmonized with the Society of Thoracic Surgeons Congenital Heart Disease Database, thereby allowing us to compare catheter-based interventions with surgical interventions when appropriate. The initial release of the registry will only include hospital-based outcomes, but ultimately it will transition to a longitudinal registry. The IMPACT Registry will provide the necessary benchmark tools for quality improvement activities for cardiac catheterization procedures in congenital heart disease.
Pediatric Cardiac Surgery Annual of the Seminars in Thoracic and Cardiovascular Surgery 01/2010; 13(1):20-5.
