Steven J Kindel

Cook Children's Health Care System, Fort Worth, Texas, United States

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Publications (11)47.32 Total impact

  • [Show abstract] [Hide abstract]
    ABSTRACT: Recent guidelines recommend assessment of systolic function and filling pressures to augment angiographic grading of cardiac allograft vasculopathy (CAV); however, no data exist on the utility of these guidelines. The aims of this study were to evaluate whether the assessment of systolic and diastolic graft function, in addition to angiography, improves recognition of patients at high risk of graft loss and to assess the ability of adult filling-pressure thresholds to discriminate graft dysfunction in pediatric patients. This study reviewed Pediatric Heart Transplant Study data from 1993 to 2009. Graft dysfunction was defined as significant systolic dysfunction (ejection fraction [EF] <45%) or the presence of restrictive hemodynamic features. Additional pediatric hemodynamic cutpoints of right atrial pressure (RAP) >12 mm Hg or pulmonary capillary wedge pressure (PCWP) >15 mm Hg were analyzed. In the study, 8,122 angiograms were performed in 3,120 patients, and 70% of patients had at least 1 angiogram. Angiographic incidence of CAV was 5%, 15%, and 28% at 2, 5, and 10 years, respectively, and most disease was mild. The presence of graft dysfunction identified patients at greater risk for graft loss even in children with mild angiographic vasculopathy (p < 0.0001). An RAP >12 mm Hg or a PCWP >15 mm Hg was sufficient to detect patients at high risk of graft loss even with mild angiographic disease. Patients with only mild angiographic CAV have significantly better outcomes than do patients with moderate or severe disease. The presence of an EF <45%, an RAP >12 mm Hg, or a PCWP >15 mm Hg identifies children at increased risk of graft loss even in the presence of only mild angiographic vasculopathy. Copyright © 2015 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.
    Journal of the American College of Cardiology 08/2015; 66(5):547-557. DOI:10.1016/j.jacc.2015.05.063 · 16.50 Impact Factor
  • Steven J Kindel · Brian F Joy · Elfriede Pahl · Eric L Wald
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    ABSTRACT: Although cardiac transplantation is life-saving, morbidities from immunosuppression are significant. EoE is a complication of calcineurin inhibitors following liver transplant causing feeding intolerance, weight loss, vomiting, and dysphagia. There are limited reports of EoE following heart transplantation. We performed a retrospective single-center review of pediatric cardiac transplant patients from 2000 to 2010. A case-control analysis of patients with and without EoE was performed evaluating heart transplantation outcomes such as rates of rejection, CAV, PTLD, and graft loss. Eighty-six transplants were performed in 84 patients; 34 (40%) underwent diagnostic endoscopy, and 10 (12%) had EoE. Median time to diagnosis of EoE was 3.7 yr (IQR: 2.0-5.2). There were no differences in demographics or use of induction medications between patients with or without EoE. Patients with EoE had fewer episodes of treated rejection (1.0 vs. 2.5; p = 0.04). Four of 10 (40%) EoE patients had PTLD compared with only 2/24 (8%) of those without EoE (p = 0.048; OR 7.33 [95% CI: 1.1-50.2]). There were no differences in CAV or graft loss between groups. EoE should be considered as a cause of GI symptoms in children after cardiac transplantation and may be associated with fewer rejection episodes and increased rates of PTLD, thus representing a marker of over-immunosuppression.
    Pediatric Transplantation 06/2014; 18(5). DOI:10.1111/petr.12302 · 1.44 Impact Factor
  • The Journal of Heart and Lung Transplantation 04/2014; 33(4):S306. DOI:10.1016/j.healun.2014.01.825 · 6.65 Impact Factor
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    ABSTRACT: Background: Patients with failing Fontan circulation are at high risk for complications after heart transplantation (HTx) because of multiple prior operations, elevated panel reactive antibody, hepatic dysfunction, coagulopathy, protein-losing enteropathy (PLE), and poor nutrition. The purpose of this review was to evaluate the outcome of HTx for these patients, including those who are status post-Fontan conversion. Methods: Of 206 heart transplants at Ann & Robert H. Lurie Children's Hospital of Chicago from 1990 to 2012, 22 patients had a failing Fontan. Median age at HTx was 12.2 years, median interval from initial Fontan to HTx was 7.1 years. Potential preoperative risk factors included PLE (n = 15), mechanical ventilation (n = 8), prior Fontan conversion (n = 7), renal failure (n = 3), and plastic bronchitis (n = 2) Median number of prior operations was 3. Donor branch pulmonary arteries were used in 17 patients. Results: There were 5 early deaths (23%), due to graft failure (1), pulmonary hypertension (1), and infection (3). There were 3 late deaths (13%) at 1, 5, and 8 years. Two of 3 patients with preoperative renal failure died. Survivors who had preoperative PLE (n = 11) and preoperative plastic bronchitis (n = 2) experienced complete resolution of these pathological conditions after heart transplantation. Median length of stay was 30 days. Five of 7 Fontan conversion patients survived, and 6 of 8 preoperative ventilator-dependent patients survived. One-, 5-, and 10-year survival was 77%, 66%, and 45%, respectively. Conclusions: The operative mortality of HTx for patients with a failing Fontan is high. Using the donor branch pulmonary arteries greatly facilitated the transplant. Because infection caused the majority of early deaths, lower intensity initial immunosuppression may be warranted. Transplantation was successful in treating PLE in all survivors. Prior Fontan conversion was not a risk factor. Preoperative mechanical ventilation was not a risk factor. Preoperative renal failure may be a relative contraindication. Earlier referral of failing Fontan patients may improve results.
    The Annals of Thoracic Surgery 10/2013; 96(4):1413-1419. DOI:10.1016/j.athoracsur.2013.05.087 · 3.85 Impact Factor
  • Steven J Kindel · Elfriede Pahl
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    ABSTRACT: Heart transplantation is an accepted therapy for end-stage heart disease in children and adults. Over the past 25 years, the perioperative and 1-year mortality has steadily improved, leading to an increased focus on midterm and late-term complications. Cardiac allograft vasculopathy (CAV) is the leading cause of late graft loss in children. The prevalence of disease increases steadily after transplantation from 5% at 2 years to 35% by 10 years according to multiple database analyses. Allograft vasculopathy is the end point of a complex interaction of stimuli including chronic rejection, endothelial dysfunction, infection, and traditional cardiac risk factors. While an increased understanding of risks associated to CAV has led to more aggressive surveillance approaches, the rates of CAV remain high and outcomes after diagnosis of CAV are very poor with up to 50% of children suffering graft loss or death within 2 years of diagnosis. In an attempt to combat the development and progression of CAV, multiple medical and interventional strategies have been utilized. Pharmacologic approaches have focused on the use of various immunosuppressants and adjuvant medications to combat inflammation and immune mediated graft injury. While randomized controlled trials are rare in pediatric heart transplant cohorts, sufficient adult data have been developed in both controlled and observational trials to provide a framework for the prevention and management of patients with CAV. However, none of these interventions have been shown to be effective in significantly prolonging graft survival and retransplantation remains the only reliable therapy for severe CAV.
    Congenital Heart Disease 05/2012; 7(4):324-35. DOI:10.1111/j.1747-0803.2012.00666.x · 1.08 Impact Factor
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    ABSTRACT: Cardiomyopathy is a heterogeneous disease with a strong genetic component. A research-based pediatric cardiomyopathy registry identified familial, syndromic, or metabolic causes in 30% of children. However, these results predated clinical genetic testing. We determined the prevalence of familial, syndromic, or metabolic causes in 83 consecutive unrelated patients referred for genetic evaluation of cardiomyopathy from 2006 to 2009. Seventy-six percent of probands (n = 63) were categorized as familial, syndromic, or metabolic. Forty-three percent (n = 18) of hypertrophic cardiomyopathy (HCM) patients had mutations in sarcomeric genes, with MYH7 and MYBPC3 mutations predominating. Syndromic (17%; n = 7) and metabolic (26%; n = 11) causes were frequently identified in HCM patients. The metabolic subgroup was differentiated by decreased endocardial shortening fraction on echocardiography. Dilated cardiomyopathy (DCM) patients had similar rates of syndromic (20%; n = 5) and metabolic (16%; n = 4) causes, but fewer familial cases (24%; n = 6) compared with HCM patients. The cause of cardiomyopathy is identifiable in a majority of affected children. An underlying metabolic or syndromic cause is identified in >35% of children with HCM or DCM. Identification of etiology is important for management, family-based risk assessment, and screening.
    Journal of cardiac failure 05/2012; 18(5):396-403. DOI:10.1016/j.cardfail.2012.01.017 · 3.05 Impact Factor
  • The Journal of Heart and Lung Transplantation 04/2012; 31(4):S53-S54. DOI:10.1016/j.healun.2012.01.137 · 6.65 Impact Factor
  • S.J. Kindel · B.F. Joy · E. Pahl · E.L. Wald
    The Journal of Heart and Lung Transplantation 04/2012; 31(4):S211. DOI:10.1016/j.healun.2012.01.625 · 6.65 Impact Factor
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    ABSTRACT: Pediatric cardiomyopathy is a genetically het-erogeneous disease associated with significant morbidity. Although identification of underlying etiology is important for management, therapy, and screening of at risk family members, molec-ular diagnosis is difficult due to the large num-ber of causative genes, the high rate of private mutations, and cost. In this study, we aimed to define the genetic basis of pediatric cardiomy-opathy and test a novel diagnostic tool using a custom targeted microarray coupled to massive-ly parallel sequencing. Three patients with car-diomyopathy were screened using a custom NimbleGen sequence capture array containing 110 genes and providing 99.9% coverage of the exons of interest. The sensitivity and specificity was over 99% as determined by comparison to long-range polymerase chain reaction (PCR)-based massively parallel sequencing, Sanger sequencing of missense variants, and single nucleotide polymorphisms genotyping using the Illumina Infinium Omni1 array. Overall, 99.73% of the targeted regions were captured and sequenced at over 10x coverage, allowing reli-able mutation calling in all patients. Analysis identified a total of 165 non-synonymous coding single nucleotide polymorphisms (cSNPs) of which 89 were unique and 14 were novel. On average, each patient had 4 cSNPs predicted to be pathogenic. In conclusion, we report a car-diomyopathy sequencing array that allows simul-taneous assessment of 110 genes. Comparison of targeted sequence capture versus PCR-based enrichment methods demonstrates that the for-mer is more sensitive and efficient. Array-based sequence capture technology followed by mas-sively parallel sequencing is promising as a robust and comprehensive tool for genetic screening of cardiomyopathy. These results pro-vide important information about genetic archi-tecture and indicate that improved annotation of variants and interpretation of clinical signifi-cance, particularly in cases with multiple rare variants, are important for clinical practice.
    01/2012; 2(1). DOI:10.4081/cardiogenetics.2012.e7
  • Steven J. Kindel · Elfriede Pahl
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    ABSTRACT: Cardiomyopathy in children accounts for greater than 50% of the cases of end-stage cardiac disease leading to heart transplantation in children. While early survival is excellent, late survival is limited with an average graft half-life of approximately 15years in children. Cardiac allograft vasculopathy is a not uncommon complication of transplantation and is the leading cause of late graft loss and retransplantation in pediatric populations. Studies of the United Network of Organ Sharing database and the Pediatric Heart Transplant Study group report rates of coronary vasculopathy that increase from 5% at 2years to 35% at 10years. Coronary artery vasculopathy is a complex process caused by both immune mediated endothelial dysfunction and vascular changes as well as typical cardiovascular risk factors. Unfortunately, despite vigilant surveillance protocols, new onset graft dysfunction and sudden cardiac death can be the presenting symptoms of new disease. In recent years multiple medical and adjuvant therapies have been studied in relation to potential management to minimize this disease process. Further research and collaborative multi-center trials will be the most effective means of developing strategies for the prevention and treatment of coronary vasculopathy in pediatric heart transplant patients.
    Progress in Pediatric Cardiology 08/2011; 32(1):37-42. DOI:10.1016/j.ppedcard.2011.06.008
  • The Journal of Heart and Lung Transplantation 04/2011; 30(4). DOI:10.1016/j.healun.2011.01.507 · 6.65 Impact Factor

Publication Stats

21 Citations
47.32 Total Impact Points


  • 2014
    • Cook Children's Health Care System
      Fort Worth, Texas, United States
  • 2013
    • Ann & Robert H. Lurie Children's Hospital of Chicago
      Chicago, Illinois, United States
  • 2012
    • Northwestern Memorial Hospital
      Chicago, Illinois, United States
    • Cincinnati Children's Hospital Medical Center
      • Department of Pediatrics
      Cincinnati, Ohio, United States
  • 2011–2012
    • Children's Memorial Hospital
      Chicago, Illinois, United States