Kathy J Jenkins

Harvard University, Cambridge, Massachusetts, United States

Are you Kathy J Jenkins?

Claim your profile

Publications (164)1051.33 Total impact

  • [Show abstract] [Hide abstract]
    ABSTRACT: Objective: The purpose of this study was to evaluate the utility of radionuclide perfusion lung scanning in the evaluation of primary pulmonary vein stenosis (PVS) in pediatric patients by comparing it with angiography. Materials and methods: We retrospectively identified pediatric patients with primary PVS who underwent both lung scanning and angiography. A cohort of 34 patients was evaluated. The presence of PVS in the right upper, right lower, left upper, and left lower pulmonary veins on angiograms was recorded. Two nuclear medicine physicians evaluated the lung scans for perfusion defects. Agreement between lung scan and angiographic findings was assessed with contingency tables. Sensitivity and specificity of lung scanning for accurate detection of PVS with angiographic findings as the reference standard were assessed by ROC analysis. Results: Angiography depicted PVS in 90 of the total 136 pulmonary veins (66%). Lung scans correctly depicted 65 (72%) of the cases of PVS diagnosed with angiography. The sensitivity and specificity of lung scans were 76.0% and 88.9% for the right upper pulmonary vein, 70.6% and 94.1% for the right lower pulmonary vein, 77.3% and 58.3% for the left upper pulmonary vein, and 65.4% and 87.5% for the left lower pulmonary vein. Conclusion: Lung scan findings correlate with angiographic findings in the detection of primary PVS in pediatric patients. Perfusion lung scanning may have a role in angiographically diagnosed PVS by noninvasively showing relative perfusion at the tissue level.
    American Journal of Roentgenology 09/2015; 205(4):873-877. DOI:10.2214/AJR.14.13581 · 2.73 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: We pursued a multimodality approach to the treatment of patients with pulmonary vein stenosis, incorporating sutureless surgical repair, catheter interventions, and adjunctive chemotherapy. We report our outcomes after surgery. Between January 2007 and August 2013, 49 patients with multivessel pulmonary vein stenosis underwent operations at our institution. We retrospectively reviewed data from a pulmonary vein stenosis registry and the medical records. At the time of the index operation, the median patient age was 6 months (range, 32 days-48 months) and weight was 4.9 kg (range, 2.1-13.4 kg). Fourteen patients (28%) died during the follow-up period (median follow-up was 0.5 years [range, 0.04-4.9 years]). There were 2 deaths (4%) within 30 days. Age at repair <6 months, weight at repair <3 kg, and a preoperative right ventricular systolic pressure < ¾ systemic were found to be associated with mortality. One patient required repeat operation for recurrent stenosis. Thirty-nine patients (80%) underwent postoperative catheterizations. The median number of catheterizations per patient was 2 (range, 0-14). Twenty-nine patients (59%) underwent catheterizations with pulmonary vein intervention. The median number of catheterizations with intervention per patient was 1 (range, 0-14). There were no identifiable associations with need for or number of catheterizations with intervention. Ten patients were listed for lung transplantation: 4 patients were de-listed, 3 patients died waiting, and 3 patients underwent transplant. Using a multimodality approach, we observed acceptable early survival after operation in patients with pulmonary vein stenosis, despite the need for catheter reinterventions. Lung transplantation remains a viable option. Copyright © 2015 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.
    The Journal of thoracic and cardiovascular surgery 06/2015; DOI:10.1016/j.jtcvs.2015.06.050 · 4.17 Impact Factor
  • Circulation Cardiovascular Quality and Outcomes 06/2015; 8(4). DOI:10.1161/CIRCOUTCOMES.115.001956 · 5.66 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: We developed and implemented a patient safety (PS) curriculum targeted at clinicians and nonclinical office practice staff within a large primary care pediatric network. Curricular content was informed by medical literature, local PS experts, and malpractice claims data. Sessions were centered on illustrative closed malpractice cases or informed by identified safety events. Participants provided subjective responses to the postsession evaluations. Invited participants from 12 practices included both clinical and nonclinical practice staff (up to 24 attendees per session). Participants reported that they were confident in their knowledge and skills. Several participants engaged in improvement projects that included active surveillance of high-risk patients, improvements in referral and test result management processes, and the distribution of patient educational materials. We successfully developed and implemented a multifaceted PS curriculum for pediatric providers. Participants enjoyed the sessions and several engaged in new PS projects as a result of the program. © The Author(s) 2015.
    Clinical Pediatrics 05/2015; 54(11). DOI:10.1177/0009922815584929 · 1.15 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Pulmonary vein stenosis (PVS) carries significant morbidity and mortality for affected children, and its management often requires multiple angioplasty procedures. PVS angioplasty can be complicated by systemic embolic events such as stroke, but incidence and risk factors are poorly understood. We reviewed pediatric catheterizations involving PVS angioplasty and/or stent placement performed at Boston Children's Hospital between July 2005 and February 2014. A total of 406 cases were performed in 144 distinct patients. Patients underwent a median of two catheterizations, at median age 1 year and weight 6.9 kg. Eleven (2.7 %) catheterizations were complicated by clinically apparent systemic embolic events, comprising 10 strokes (one with associated hepatic embolism) and 1 renal infarct. Prevalence of clinically evident stroke among this cohort was 7.6 %. Using a prior (uncomplicated) catheterization to allow each patient to serve as their own control, we sought to identify potentially modifiable risk factors for systemic embolic events. Although this analysis was limited by low power, complicated and uncomplicated angioplasties did not appear to differ in case time, contrast dose, anticoagulation management, use of cutting balloons, number of catheter exchanges, or size of long sheath used. Significant non-embolic adverse events were common, occurring in 25 % of catheterizations. Systemic embolism appears to complicate PVS angioplasty at a rate much higher than that described for other congenital catheterizations. This risk may be inherent to the procedure rather than related to any modifiable or operator-dependent factors.
    Pediatric Cardiology 04/2015; 36(7). DOI:10.1007/s00246-015-1165-x · 1.31 Impact Factor
  • Journal of the American College of Cardiology 03/2015; 65(10):A483. DOI:10.1016/S0735-1097(15)60483-1 · 16.50 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: To report procedural characteristics and adverse events on data collected in the registry. The IMPACT - IMproving Paediatric and Adult Congenital Treatment - Registry is a catheterisation registry of paediatric and adult patients with CHD undergoing diagnostic and interventional cardiac catheterisation. We are reporting the procedural characteristics and adverse events of patients undergoing diagnostic and interventional catheterisation procedures from January, 2011 to March, 2013. Demographic, clinical, procedural, and institutional data elements were collected at the participating centres and entered via either a web-based platform or software provided by American College of Cardiology-certified vendors, and were collected in a secure, centralised database. Centre participation was voluntary. During the time frame of data collection, 19,797 procedures were entered into the IMPACT Registry. Procedures were classified as diagnostic only (35.4%); one of six specific interventions (23.8%); other or multiple interventions (40.7%); and were further broken down into four age groups. Anaesthesia was used in 84.1% of diagnostic procedures and 87.8% of interventional ones. Adverse events occurred in 10.0% of diagnostic and 11.1% of interventional procedures. The IMPACT Registry is gathering data to set national benchmarks for diagnostic and certain specific interventional procedures. We are seeing little differences in procedural characteristics or adverse events in diagnostic procedures compared with interventional procedures overall, but there is significant variation in adverse events amongst age categories. Risk stratification and patient acuity scores will be required for further analysis of these differences.
    Cardiology in the Young 02/2015; -1:1-9. DOI:10.1017/S1047951114002637 · 0.84 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: The National Cardiovascular Data Registry (NCDR) launched the IMPACT (Improving Pediatric and Adult Congenital Treatment) Registry in 2010. By 2013, its patient enrollment exceeded that of other current and historical congenital catheterization registries. This study sought to describe procedural results and safety of 6 common congenital interventions performed in patients enrolled during the IMPACT Registry's initial periods. With specified exclusions, we compiled registry data from patients enrolled in the IMPACT Registry from January 2011 through March 2013 who underwent 1 of the following isolated procedures: device closure of atrial septal defect (ASD); device closure of patent ductus arteriosus (PDA); pulmonary valvuloplasty; aortic valvuloplasty; coarctation of the aorta angioplasty and stenting; and pulmonary artery stenting. Patient data, procedural data and results, and adverse events (AEs) were reviewed and described. In 4,152 catheterizations, 1 isolated procedure was reported. There were 1,286 single-ASD procedures, 1,375 PDA procedures, 270 "typical" pulmonary valve procedures, 305 aortic valve procedures, 671 aortic procedures, and 245 pulmonary artery procedures. The reported procedure was performed in >95% of catheterizations. Stated outcomes were accomplished in >98% of ASD and PDA procedures, but less commonly in the others, with coarctation angioplasty procedures being the least successful (51%). Reported major AE rates ranged from 0% to 3.3%; total AE rates ranged from 5.3% to 24.3%. Contemporary community practice, procedural outcomes, and safety for 6 common congenital interventional procedures are reported. These benchmarks may be compared with individual center results and historical single-center and multicenter results. Copyright © 2014 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.
    Journal of the American College of Cardiology 12/2014; 64(23):2439-51. DOI:10.1016/j.jacc.2014.09.045 · 16.50 Impact Factor
  • [Show abstract] [Hide abstract]
    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; 9(6). DOI:10.1111/chd.12232 · 1.08 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Background: 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. Methods: Twenty-eight sites in 17 developing world countries submitted congenital heart surgery data to a registry, received annual benchmarking reports, and created quality improvement teams. Webinars targeted 3 key drivers: safe perioperative practice, infection reduction, and team-based practice. Registry data were audited annually; only verified data were included in analyses. Risk-adjusted standardized mortality ratios (SMRs) and standardized infection ratios among participating sites were calculated. Results: Twenty-seven sites had verified data in at least 1 year, and 1 site withdrew. Among 15,049 cases of pediatric congenital heart surgery, unadjusted mortality was 6.3% and any major infection was 7.0%. SMRs for the overall International Quality Improvement Collaborative for Congenital Heart Surgery in Developing World Countries were 0.71 (95% confidence interval [CI] 0.62-0.81) in 2011 and 0.76 (95% CI 0.69-0.83) in 2012, compared with 2010 baseline. SMRs among 7 sites participating in all 3 years were 0.85 (95% CI 0.71-1.00) in 2011 and 0.80 (95% CI 0.66-0.96) in 2012; among 14 sites participating in 2011 and 2012, the SMR was 0.80 (95% CI 0.70-0.91) in 2012. Standardized infection ratios were similarly reduced. Conclusions: Congenital heart surgery risk-adjusted mortality and infections were reduced in developing world programs participating in the collaborative quality improvement project and registry. Similar strategies might allow rapid reduction in global health care disparities.
    Pediatrics 10/2014; 134(5). DOI:10.1542/peds.2014-0356 · 5.47 Impact Factor
  • [Show abstract] [Hide abstract]
    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; 36(2). DOI:10.1007/s00246-014-1005-4 · 1.31 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    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. DOI:10.1016/j.jacc.2014.06.1150 · 16.50 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Objectives: 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. Methods: A 34-question survey was developed to assess adult congenital heart disease clinicians' awareness, attitudes, and behavior relative to patient satisfaction and administered in-person or online to clinicians from the largely U.S.-based Adult Congenital Heart Association's database of adult congenital heart disease health care providers. Results: Overall, 267 questionnaires were filled out: 108 were collected in person (79% response rate) and 159 online (17.5% response rate). Responses were received from physicians (161); nurses (73); physician assistants (20); and others (13). Although 85% of clinicians believed it was important to inquire about patient satisfaction, only 28% reported routinely inquiring about this dimension of care. Only 34% claimed they had adequate training to cope with varying levels of patient satisfaction, 44% stated that their department utilized patient satisfaction surveys, and 37% received feedback from the hospital management in the preceding 12 months. In multivariate analyses, clinicians that received feedback from the hospital management and had adequate training were more likely to inquire about patient satisfaction. Conclusion: Although patient satisfaction is perceived as an important dimension of quality care by adult congenital heart disease clinicians, most of them reported insufficient institutional support to achieve this. Our findings suggest that clinicians would benefit from health care organizations engaging them in the delivery of this dimension of health care quality.
    Congenital Heart Disease 06/2014; 10(2). DOI:10.1111/chd.12190 · 1.08 Impact Factor
  • [Show abstract] [Hide abstract]
    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; 53(13). DOI:10.1177/0009922814538701 · 1.15 Impact Factor
  • Source
    Journal of the American College of Cardiology 04/2014; 63(12):A524. DOI:10.1016/S0735-1097(14)60524-6 · 16.50 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Context: 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. Objective: We developed a tool, the Complexity Ruler, to quantify the amount of data (as bits) in the patient’s medical record. We designated the amount of data in the medical record as the cognitive complexity of the medical record (CCMR). We hypothesized that CCMR is a useful surrogate for true patient complexity and that higher CCMR correlates with risk of major adverse events. Design: The Complexity Ruler was validated by comparing the measured CCMR with physician rankings of patient complexity on specific inpatient services. It was tested in a case-control model of all patients with major adverse events at a tertiary care pediatric hospital from 2005 to 2006. Main outcome measures: The main outcome measure was an externally reported major adverse event. We measured CCMR for 24 hours before the event, and we estimated lifetime CCMR. Results: Above empirically derived cutoffs, 24-hour and lifetime CCMR were risk factors for major adverse events (odds ratios, 5.3 and 6.5, respectively). In a multivariate analysis, CCMR alone was essentially as predictive of risk as a model that started with 30-plus clinical factors. Conclusions: CCMR correlates with physician assessment of complexity and risk of adverse events. We hypothesize that increased CCMR increases the risk of physician cognitive overload. An automated version of the Complexity Ruler could allow identification of at-risk patients in real time.
    The Permanente journal 03/2014; 18(1):4-8. DOI:10.7812/TPP/12-142
  • Source
    [Show abstract] [Hide abstract]
    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; 35(3). DOI:10.1007/s00246-013-0797-y · 1.31 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    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; 62(23). DOI:10.1016/j.jacc.2013.07.099 · 16.50 Impact Factor
  • [Show abstract] [Hide abstract]
    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; 62(23). DOI:10.1016/j.jacc.2013.07.100 · 16.50 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Unlabelled: 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; 9(2). DOI:10.1111/chd.12105 · 1.08 Impact Factor

Publication Stats

4k Citations
1,051.33 Total Impact Points


  • 2003–2015
    • Harvard University
      Cambridge, Massachusetts, United States
  • 1993–2015
    • Boston Children's Hospital
      • • Department of Cardiac Surgery
      • • Department of Pediatrics
      Boston, Massachusetts, United States
    • Children's National Medical Center
      • Division of Cardiology
      Washington, Washington, D.C., United States
    • University of Massachusetts Boston
      Boston, Massachusetts, United States
  • 1996–2010
    • Harvard Medical School
      • Department of Pediatrics
      Boston, Massachusetts, United States
  • 2005
    • Oregon Health and Science University
      • Division of Cardiothoracic Surgery
      Los Angeles, CA, United States
  • 1998
    • Beth Israel Deaconess Medical Center
      • Department of Medicine
      Boston, Massachusetts, United States