Kathy J Jenkins

Harvard Medical School, Boston, Massachusetts, United States

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Publications (138)609.87 Total impact

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    ABSTRACT: Background Growth failure is common in infants with single ventricle. This study evaluated the use of a learning network, the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC), to spread optimized nutritional practices and improve infant growth.MethodsA previously identified Nutritional Bundle was spread among NPC-QIC sites. Primary outcome: interstage weight-for-age z-score change (ΔWAZ) between discharge from stage 1 palliation (S1) and stage 2 surgical palliation (S2). Variation among sites in interstage ΔWAZ was evaluated before (Period 1) and after (Period 2) spread of Nutritional Bundle. We performed an analysis of NPC-QIC registry infants presenting for S2 at sites previously shown to have significant variation in interstage patient growth.ResultsFour hundred seven infants from 15 sites underwent S2 between 2008 and 2013: 158 in Period 1 (December 2008–December 2010) and 249 in Period 2 (December 2010–April 2013). Median age at S2 was 4.9 months (2.6–12.8) with no difference between periods. There was significant variation in interstage ΔWAZ among sites in Period 1 (P = .01) but not in Period 2 (P = .39). More patients had an interstage ΔWAZ <0 in Period 1 (43%) than Period 2 (32%) (P = .03). In Period 1, the median interstage ΔWAZ was <0 in six sites while in Period 2 no site had median interstage ΔWAZ <0. Sites with the worst patient growth in Period 1 had marked improvement in Period 2 (P = .02, .06, and .06, respectively).Conclusions Spread of optimal nutritional practices led to decreased variation in interstage growth with most improvement observed at sites with the worst baseline growth outcomes.
    Congenital Heart Disease 11/2014; · 1.01 Impact Factor
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    ABSTRACT: There is little information about congenital heart surgery outcomes in developing countries. The International Quality Improvement Collaborative for Congenital Heart Surgery in Developing World Countries uses a registry and quality improvement strategies with nongovernmental organization reinforcement to reduce mortality. Registry data were used to evaluate impact.
    Pediatrics. 10/2014;
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    ABSTRACT: Among infants with single ventricle congenital heart disease (SVD) requiring Stage I palliation (S1P), the impact of prenatal diagnosis (PD) on outcomes has been variably characterized. We investigated the impact of PD in a large multi-center cohort of survivors of S1P in the National Pediatric Cardiology Quality Improvement Collaborative (NPCQIC) registry. Retrospective analysis of demographic and outcomes data among infants enrolled in the NPCQIC database; eligibility includes SVD requiring S1P and survival to discharge. From 43 contributing surgical centers, 591 infants had data available through time of BDG (519) or interstage death (55). Median gestational age was 39 weeks (31-46), and 66 % had variants of hypoplastic left heart syndrome. PD was made in 445 (75 %), with significant variation by center (p = 0.004). While infants with PD had slightly lower gestational age at birth (p < 0.001), there were no differences in birth weight, the presence of major syndromes or other organ system anomalies. Those without PD were more likely to have atrioventricular valve regurgitation (p = .002), ventricular dysfunction (p = 0.06), and pre-operative risk factors including acidosis (p < 0.001), renal insufficiency (p = 0.007), and shock (p = 0.05). Post-operative ventilation was shorter in the PD group (9 vs. 12 d, p = 0.002). Other early post-operative outcomes, interstage course, and outcomes at BDG were similar between groups. In a large cohort of infants with SVD surviving to hospital discharge after S1P, PD showed significant inter-site variation and was associated with improved pre-operative status and shorter duration of mechanical ventilation. The significance of such associations merits further study.
    Pediatric cardiology. 08/2014;
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    ABSTRACT: This paper aims to update clinicians on “hot topics” in the management of patients with D-loop transposition of the great arteries (D-TGA) in the current surgical era. The arterial switch operation (ASO) has replaced atrial switch procedures for D-TGA, and 90% of patients now reach adulthood. The Adult Congenital and Pediatric Cardiology Council of the American College of Cardiology assembled a team of experts to summarize current knowledge on genetics, pre-natal diagnosis, surgical timing, balloon atrial septostomy, prostaglandin E1 therapy, intraoperative techniques, imaging, coronary obstruction, arrhythmias, sudden death, neoaortic regurgitation and dilation, neurodevelopmental (ND) issues, and lifelong care of D-TGA patients. In simple D-TGA: 1) familial recurrence risk is low; 2) children diagnosed pre-natally have improved cognitive skills compared with those diagnosed post-natally; 3) echocardiography helps to identify risk factors; 4) routine use of BAS and prostaglandin E1 may not be indicated in all cases; 5) early ASO improves outcomes and reduces costs with a low mortality; 6) single or intramural coronary arteries remain risk factors; 7) post-ASO arrhythmias and cardiac dysfunction should raise suspicion of coronary insufficiency; 8) coronary insufficiency and arrhythmias are rare but are associated with sudden death; 9) early- and late-onset ND abnormalities are common; 10) aortic regurgitation and aortic root dilation are well tolerated; and 11) the aging ASO patient may benefit from “exercise-prescription” rather than restriction. Significant strides have been made in understanding risk factors for cardiac, ND, and other important clinical outcomes after ASO.
    Journal of the American College of Cardiology 08/2014; 64(5):498–511. · 14.09 Impact Factor
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    ABSTRACT: Patient-centered care and patient satisfaction represent key dimensions of health care quality. This is relevant for the growing number of patients with life-long conditions. In the present study, our goal was to examine clinicians' attitudes and behavior with respect to patient satisfaction in adult congenital heart disease outpatient clinics.
    Congenital Heart Disease 06/2014; · 1.01 Impact Factor
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    ABSTRACT: Objective. To assess the current state of quality improvement and patient safety (QIPS) education at a large teaching hospital. Methods. We surveyed 429 trainees (138 residents, 291 clinical fellows) and 38 program directors (PDs; 2 were PDs of >1 program) from 39 Accreditation Council for Graduate Medical Education-accredited training programs. Results. Twenty-nine PDs (76.3%) and 259 trainees (60.3%) responded. Most trainees (68.8%) reported participation in projects culminating in scholarly products (39.9%) or clinical innovations (44%). Most PDs reported that teaching (88.9%) and project supervision (83.3%) are performed by expert faculty. Nearly half of the PDs (45.8%) and trainees (49.6%) perceived project-based learning to be of equal value to formal curricula. Compared with trainees, a greater proportion of PDs reported needs for funding for projects, teaching faculty to provide mentorship, and faculty development (P < .05). Conclusions. Providing additional financial, administrative, and operational support could enhance the value of curricula and projects. Developing expert teaching faculty is paramount.
    Clinical pediatrics. 06/2014;
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    ABSTRACT: Patients in tertiary care hospitals are more complex than in the past, but the implications of this are poorly understood as "patient complexity" has been difficult to quantify. A "Complexity Ruler" was validated and tested in a case-control model of all patients with major adverse events at a tertiary care pediatric hospital from 2005 to 2006. Above empirically derived cutoffs, 24-hour and lifetime cognitive complexity of the medical records were risk factors for major adverse events.
    The Permanente journal 01/2014; 18(1):4-8.
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    ABSTRACT: The objective of this study is to identify predictors of prolonged intensive care unit (ICU) length of stay (LOS) for single ventricle patients following Stage I palliation. We hypothesize that peri-operative factors contribute to prolonged ICU stay among children with hypoplastic left heart syndrome (HLHS) and its variants. In 2008, as a part of the Joint Council on Congenital Heart Disease initiative, the National Pediatric Cardiology-Quality Improvement Collaborative established a data registry for patients with HLHS and its variants undergoing staged palliation. Between July 2008 and August 2011, 33 sites across the United States submitted discharge data essential to this analysis. Data describing the patients, their procedures, and their hospital experience were entered. LOS estimates were generated. Prolonged LOS in the ICU was defined as stay greater than or equal to 26 days (i.e., 75th percentile). Statistical analyses were carried out to identify pre-operative, operative, and post-operative predictors of prolonged LOS in the ICU. The number of patients with complete discharge data was 303, and these subjects were included in the analysis. Univariate and multivariate analyses were performed. Multivariate analysis revealed that lower number of enrolled participants (e.g., 1-10) per site, the presence of pre-operative acidosis, increased circulatory arrest time, the occurrence of a central line infection, and the development of respiratory insufficiency requiring re-intubation were associated with prolonged LOS in the ICU. Prolonged LOS in the ICU following Stage I palliation in patients with HLHS and HLHS variant anatomy is associated with site enrollment, circulatory arrest time, pre-operative acidosis, and some post-operative complications, including central line infection and re-intubation. Further study of these associations may reveal strategies for reducing LOS in the ICU following the Norwood and Norwood-variant surgeries.
    Pediatric Cardiology 10/2013; · 1.20 Impact Factor
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    ABSTRACT: Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect. We explore "Hot Topics" to highlight areas of emerging science for clinicians and scientists in moving towards a better understanding of long-term management in patients with repaired TOF. From a genetic perspective, the etiology of TOF is multifactorial, with a familial recurrence risk of 3%. Cardiac Magnetic Resonance (CMR) is the gold-standard assessment tool based on its superior imaging of the right ventricular (RV) outflow tract, pulmonary arteries, aorta and AP collaterals, and its ability to quantify biventricular size and function, pulmonary regurgitation (PR) and myocardial viability. Atrial reentrant tachycardia will develop in more than 30% of patients, and high-grade ventricular arrhythmias will be seen in about 10% of patients. The overall incidence of sudden cardiac death is estimated at 0.2 % per year. Risk stratification even with electrophysiologic (EP) testing and CMR remains imperfect. Drug therapy has largely been abandoned, and defibrillator placement, despite high complications and inappropriate discharges, is often recommended for patients at higher risk. Definitive information about optimal surgical strategies for primary repair to preserve RV function, reduce arrhythmia and optimize functional status is lacking. Post-operative lesions are often amenable to transcatheter intervention. In selected cases, pulmonary regurgitation (PR) may be treated by transcatheter valve insertion. Ongoing surveillance of RV function is a critical component of clinical assessment. Once RV dysfunction occurs, there are no medical therapies that have been shown to be effective, except for resynchronization with biventricular pacing. For patients with significant PR with RV dilatation, optimal timing of pulmonary valve replacement remains uncertain, although accepted criteria are emerging.
    Journal of the American College of Cardiology 09/2013; · 14.09 Impact Factor
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    ABSTRACT: We sought to develop quality indicators (QIs) for outpatient management of adult congenital heart disease (ACHD) patients. There are no published QIs to promote quality measurement and improvement for ACHD patients. Working groups of ACHD experts reviewed published literature and US, Canadian and European guidelines to identify candidate QIs. For each QI we specified a numerator, denominator, period of assessment and data source. We submitted the QIs to a 9-member panel of international ACHD experts. The panel rated the QIs for validity and feasibility in 2 rounds, on a scale of 1-9, using the University of California Los Angeles (UCLA)/RAND modified-Delphi method and final QI selection was based on median scores. Sixty-two QIs were identified regarding appropriateness and timing of clinical management, testing and test interpretation. Each QI was ascertainable from health records. After the first round of rating, 29 QIs were accepted, none were rejected and 33 were equivocal; on the second round, 55 QIs were accepted. Final QIs included: 8 for atrial septal defects; 9 for aortic coarctation; 12 for Eisenmenger; 9 for Fontan; 9 for D-transposition of the great arteries; and 8 for tetralogy of Fallot. This project resulted in development of the first set of QIs for ACHD care based on literature, guidelines and a modified Delphi process. These QIs provide a quality of care assessment tool for six ACHD conditions. This rigorously designed set of QIs should facilitate measuring and improving quality of care for this growing group of patients.
    Journal of the American College of Cardiology 09/2013; · 14.09 Impact Factor
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    ABSTRACT: Early recognition of pulmonary vein stenosis (PVS) is crucial for optimizing clinical outcomes. Our goal was to characterize radiographic patterns specific to pediatric patients with PVS to facilitate early detection. PATIENTS AND METHODS: Pediatric patients with multivessel (≥2) intraluminal PVS were identified from a single-center registry. Initial chest radiographs were reviewed. Radiographic findings were summarized using frequencies and percentages for categorical data, and medians and ranges for continuous data. Interrater agreement was assessed using kappa statistics. RESULTS: Chest radiographs of 41 PVS patients were evaluated; median age at presentation 5.2 (0.5-102.6) months. Underlying congenital heart disease was present in 31 (76%), lung disease in four (10%), and neither in six (15%). Common heart diseases were hypoplastic left heart syndrome (five, 12%), totally anomalous pulmonary venous connection (nine, 22%), and heterotaxy (five, 12%). PVS was bilateral in 22 (54%), right-sided in six (14%), and left-sided in 13 (32%). All chest radiographs were abnormal. Increased interstitial opacity was present in all patients, reticular opacity in 35 (85%), and ground-glass opacity in 29 (71%). Consolidation (one, 2%), pleural effusions (four, 10%), and nodular opacities (0) were unusual. Distributional heterogeneity was common (17, 42%). Interrater agreement was generally high (kappa >0.84) except for lobe location. Findings were similar among patients with isolated PVS, PVS with congenital heart disease, and PVS with lung disease. CONCLUSION: Diagnosis of PVS should be considered in infants with increased interstitial opacity, reticular opacity, and ground-glass opacity on chest radiography, especially if findings are heterogeneous.
    Congenital Heart Disease 06/2013; · 1.01 Impact Factor
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    ABSTRACT: This review discusses the current safety issues related to U.S. Food and Drug Administration approved atrial septal defect devices and proposes a potential avenue to gather additional safety data including factors, which may be involved in device erosion.
    JACC Cardiovascular Interventions 05/2013; 6(5):433–442. · 7.42 Impact Factor
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    ABSTRACT: Variability in medical practice in the United States leads to higher costs without achieving better patient outcomes. Clinical practice guidelines, which are intended to reduce variation and improve care, have several drawbacks that limit the extent of buy-in by clinicians. In contrast, standardized clinical assessment and management plans (SCAMPs) offer a clinician-designed approach to promoting care standardization that accommodates patients' individual differences, respects providers' clinical acumen, and keeps pace with the rapid growth of medical knowledge. Since early 2009 more than 12,000 patients have been enrolled in forty-nine SCAMPs in nine states and Washington, D.C. In one example, a SCAMP was credited with increasing clinicians' rate of compliance with a recommended specialist referral for children from 19.6 percent to 75 percent. In another example, SCAMPs were associated with an 11-51 percent decrease in total medical expenses for six conditions when compared with a historical cohort. Innovative tools such as SCAMPs should be carefully examined by policy makers searching for methods to promote the delivery of high-quality, cost-effective care.
    Health Affairs 05/2013; 32(5):911-20. · 4.64 Impact Factor
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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; · 2.51 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; · 1.57 Impact Factor
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    ABSTRACT: BACKGROUND: <0.0001 for all). Hospital mortality and 2-week readmission rates were unchanged at MSMC between the 2 time periods and were not different from the national rates.Conclusion-Shorter length of stay and cost savings compared with national data were observed after implementation of fast tracking.
    Circulation Cardiovascular Quality and Outcomes 02/2013; · 5.66 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; · 1.01 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. · 1.01 Impact Factor

Publication Stats

3k Citations
609.87 Total Impact Points

Institutions

  • 1996–2014
    • Harvard Medical School
      • • Department of Radiology
      • • Department of Pediatrics
      Boston, Massachusetts, United States
  • 1991–2014
    • Boston Children's Hospital
      • Department of Cardiac Surgery
      Boston, Massachusetts, United States
  • 2012
    • Johns Hopkins Medicine
      Baltimore, Maryland, United States
    • Virginia Commonwealth University
      Richmond, Virginia, United States
  • 2011
    • Riley Hospital for Children
      • Department of Cardiology
      Indianapolis, Indiana, United States
    • University of California, San Francisco
      San Francisco, California, United States
  • 2010
    • McGill University Health Centre
      Montréal, Quebec, Canada
  • 2009
    • Cincinnati Children's Hospital Medical Center
      • Division of Cardiology
      Cincinnati, OH, United States
  • 2007
    • University of Massachusetts Boston
      Boston, Massachusetts, United States
  • 2005
    • Oregon Health and Science University
      • Division of Cardiothoracic Surgery
      Los Angeles, CA, United States
  • 2001
    • The Children's Hospital of Philadelphia
      • Department of Pediatrics
      Philadelphia, PA, United States
  • 1998
    • Beth Israel Deaconess Medical Center
      • Department of Medicine
      Boston, Massachusetts, United States