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Pediatric heart transplantation

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https://www.readbyqxmd.com/read/28546755/pediatric-ventricular-assist-devices-current-challenges-and-future-prospects
#1
REVIEW
Sarah Burki, Iki Adachi
The field of mechanical circulatory support has made great strides in the preceding 2 decades. Although pediatric mechanical circulatory support has lagged behind that of adults, the gap between them is expected to close soon. The only device currently approved by the US Food and Drug Administration for use in children is the Berlin Heart EXCOR ventricular assist device (VAD). The prospective Berlin Heart Investigational Device Exemption Trial demonstrated good outcomes, such as bridge to transplantation or recovery, in ~90% of children supported with this device...
2017: Vascular Health and Risk Management
https://www.readbyqxmd.com/read/28546378/pediatric-lung-transplantation
#2
Stuart C Sweet
Pediatric lung transplant is a viable option for treatment of end-stage lung disease in children, with > 100 pediatric lung transplants reported to the Registry of the International Society of Heart and Lung Transplantation each year. Long-term success is limited by availability of donor organs, debilitation as a result of chronic disease, impaired mucus clearance resulting from both surgical and pharmacologic interventions, increased risk for infection resulting from immunosuppression, and most importantly late complications, such as chronic lung allograft dysfunction...
June 2017: Respiratory Care
https://www.readbyqxmd.com/read/28536746/characteristics-of-clinically-diagnosed-pediatric-myocarditis-in-a-contemporary-multi-center-cohort
#3
Ryan J Butts, Gerard J Boyle, Shriprasad R Deshpande, Katheryn Gambetta, Kenneth R Knecht, Carolina A Prada-Ruiz, Marc E Richmond, Shawn C West, Ashwin K Lal
The objective of this study was to describe a contemporary cohort of pediatric patients hospitalized for clinically suspected myocarditis. A retrospective chart review was performed at seven tertiary pediatric hospitals. Electronic medical records were searched between 2008 and 2012 for patients ≤18 years admitted with an ICD-9 code consistent with myocarditis. Patients were excluded if the admitting or consulting cardiologist did not suspect myocarditis during the admission or an alternative diagnosis was determined...
May 23, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28528723/ventricular-pacing-in-single-ventricles-a-bad-combination
#4
Anica Bulic, Frank J Zimmerman, Scott R Ceresnak, Ira Shetty, Kara S Motonaga, Anne Freter, Anthony V Trela, Deb Hanisch, Lisa Russo, Kishor Avasarala, Anne M Dubin
BACKGROUND: Chronic ventricular pacing (VP) is associated with systolic dysfunction in a subset of pediatric patients with heart block and structurally normal hearts. The effect of chronic VP in congenital heart disease is less well understood, specifically in the single-ventricle (SV) population. OBJECTIVE: To determine the longitudinal effect of VP in SV patients. METHODS: SV patients with heart block and dual-chamber pacemakers requiring >50% VP were compared with nonpaced (controls) SV patients matched for age, sex, and SV morphology...
June 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/28504342/sudden-death-in-a-pediatric-heart-transplant-recipient-with-peripheral-eosinophilia-and-eosinophilic-myocardial-infiltrates
#5
William McEachern, Justin Godown, Debra A Dodd, Anne I Dipchand, Jennifer L Conway, Gregory J Wilson, Robert D Hoffman
Eosinophilia has been rarely reported in pediatric heart transplant recipients and has been suggested to play a role in graft rejection. We report a case of a young female patient with peripheral blood eosinophilia who died suddenly 2 years following ABO-incompatible heart transplantation. She was found at autopsy to have myocardial infiltration of not only T-lymphocytes and macrophages expected in acute cellular rejection but also of eosinophils, B-lymphocytes, and plasma cells indicating myocarditis.
May 14, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28472305/recessive-taf1a-mutations-reveal-ribosomopathy-in-siblings-with-end-stage-pediatric-dilated-cardiomyopathy
#6
Pamela A Long, Jeanne L Theis, Yu-Huan Shih, Joseph J Maleszewski, Patrice C Abell Aleff, Jared M Evans, Xiaolei Xu, Timothy M Olson
Non-ischemic dilated cardiomyopathy (DCM) has been recognized as a heritable disorder for over 25 years, yet clinical genetic testing is non-diagnostic in > 50% of patients, underscoring the ongoing need for DCM gene discovery. Here, whole exome sequencing uncovered a novel molecular basis for idiopathic end-stage heart failure in two sisters who underwent cardiac transplantation at three years of age. Compound heterozygous recessive mutations in TAF1A, encoding an RNA polymerase I complex protein, were associated with marked fibrosis of explanted hearts and gene-specific nucleolar segregation defects in cardiomyocytes, indicative of impaired ribosomal RNA synthesis...
May 2, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28465118/development-and-validation-of-a-major-adverse-transplant-event-mate-score-to-predict-late-graft-loss-in-pediatric-heart-transplantation
#7
Christopher S Almond, Helena Hoen, Joseph W Rossano, Chesney Castleberry, Scott R Auerbach, Lingyao Yang, Ashwin K Lal, Melanie D Everitt, Matthew Fenton, Seth A Hollander, Elfriede Pahl, Elizabeth Pruitt, David N Rosenthal, Doff B McElhinney, Kevin P Daly, Manisha Desai
BACKGROUND: There is inadequate power to perform a valid clinical trial in pediatric heart transplantation (HT) using a conventional end-point, because the disease is rare and hard end-points, such as death or graft loss, are infrequent. We sought to develop and validate a surrogate end-point involving the cumulative burden of post-transplant complications to predict death/graft loss to power a randomized clinical trial of maintenance immunosuppression in pediatric HT. METHODS: Pediatric Heart Transplant Study (PHTS) data were used to identify all children who underwent an isolated orthotopic HT between 2005 and 2014 who survived to 6 months post-HT...
March 24, 2017: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/28455909/emergency-department-utilization-in-pediatric-heart-transplant-recipients
#8
Carol A Wittlieb-Weber, Joseph W Rossano, David R Weber, Kimberly Y Lin, Chitra Ravishankar, Christopher E Mascio, Robert E Shaddy, Matthew J O'Connor
We used the NEDS database (2010) to evaluate ED utilization in PED HT recipients compared to other patient populations with focus on characteristics of ED visits, risk factors for admission, and charges. We analyzed 433 ED visits by PED HT recipients (median age 8 [range: 0-18] years). The most common primary diagnosis category was infectious (n=163, 37.6%), with pneumonia being the most common infectious etiology. When compared to all PED visits, HT visits were more likely to result in hospital admission (32...
June 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28453909/cardiotoxicity-and-cardiomyopathy-in-children-and-young-adult-survivors-of-hematopoietic-stem-cell-transplant
#9
REVIEW
Seth J Rotz, Thomas D Ryan, Joel Hlavaty, Stephen A George, Javier El-Bietar, Christopher E Dandoy
Cardiomyopathy is common in long-term survivors of pediatric hematopoietic stem cell transplant (HSCT). Events occurring before and after HSCT when combined with specific insults during HSCT likely contribute to long-term risk. Strategies for detecting subclinical cardiomyopathy prior to patients developing overt heart failure are under investigation. Changes in HSCT preparative regimens and cardioprotective medications administered during chemotherapy may alter the risk for cardiomyopathy. Interventions in long-term survivors such as lifestyle modification and cardioactive medications are of increasing importance...
April 28, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28450351/prospective-study-of-adenosine-on-atrioventricular-nodal-conduction-in-pediatric-and-young-adult-patients-after-heart-transplant
#10
Jonathan N Flyer, Warren A Zuckerman, Marc E Richmond, Brett R Anderson, Tamar G Mendelsberg, Jennie M McAllister, Leonardo Liberman, Linda J Addonizio, Eric S Silver
Background -Supraventricular tachycardia (SVT) is common after heart transplant. Adenosine, the standard therapy for treating SVT in children and adults without transplant, is relatively contraindicated post-transplant due to a presumed risk of prolonged atrioventricular (AV) block in denervated hearts. This study tested whether adenosine caused prolonged asystole after transplant and if it was effective in blocking AV nodal conduction in these patients. Methods -This was a single center prospective clinical study including healthy heart transplant recipients ages 6 months - 25 years presenting for routine cardiac catheterization during 2015 - 2016...
April 27, 2017: Circulation
https://www.readbyqxmd.com/read/28424442/clinical-improvement-and-no-further-need-of-transplant-after-closure-of-pda-with-transcatheter-approach-in-an-end-stage-heart-failure-patient-with-hypertrabeculation
#11
Serhat Koca, Feyza Ayşenur Paç, Ajda Mutlu Mıhçıoğlu, Vedat Kavurt, Denizhan Bağrul
Although heart failure is managed medically most of the time, heart transplantation is still last resort for selected end-stage heart failure patients with noncompaction cardiomyopathy. Presently described for the first time is case of pediatric patient with noncompaction cardiomyopathy who was initially referred to our hospital for heart transplant but underwent PDA repair and improved clinically without need for heart transplant.
March 2017: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
https://www.readbyqxmd.com/read/28421185/changes-in-composition-of-the-gut-bacterial-microbiome-after-fecal-microbiota-transplantation-for-recurrent-clostridium-difficile-infection-in-a-pediatric-heart-transplant-patient
#12
Kyle L Flannigan, Taylor Rajbar, Andrew Moffat, Leanna S McKenzie, Frank Dicke, Kevin Rioux, Matthew L Workentine, Thomas J Louie, Simon A Hirota, Steven C Greenway
The microbiome is increasingly recognized as an important influence on human health and many of the comorbidities that affect patients after solid organ transplantation (SOT) have been shown to involve changes in gut bacterial populations. Thus, microbiome changes in an individual patient may have important health implications after SOT but this area remains understudied. We describe changes in the composition of the fecal microbiome from a pediatric heart transplant recipient before and >2.5 years after he underwent repeated fecal microbiota transplantation (FMT) for recurrent Clostridium difficile infection (CDI)...
2017: Frontiers in Cardiovascular Medicine
https://www.readbyqxmd.com/read/28419703/exercise-capacity-following-pediatric-heart-transplantation-a-systematic-review
#13
Sara Peterson, Jennifer A Su, Jacqueline R Szmuszkovicz, Robert Johnson, Barbara Sargent
Pediatric HTs account for 13% of all HTs with >60% of recipients surviving at least 10 years post-HT. The purpose of this systematic review is to synthesize the literature on exercise capacity of pediatric HT recipients to improve understanding of the mechanisms that may explain the decreased exercise capacity. Six databases were searched for studies that compared the exercise capacity of HT recipients ≤21 years old with a control group or normative data. Sixteen studies were included. Pediatric HT recipients, as compared to controls or normative data, exhibit significantly higher resting HR, and at peak exercise exhibit significantly decreased HR, VO2 , power, work, minute ventilation, and exercise duration...
April 17, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28410774/maintaining-the-gift-of-life-achieving-adherence-in-adolescent-heart-transplant-recipients
#14
Rachael Steuer, Sabrina Opiola McCauley
Since the beginning of United Network of Organ Sharing data collection in 1987, a total of 8,333 pediatric patients have received a heart transplant in the United States. Because these patients now have longer graft success with improved care and immunosuppression, many of them are entering adolescence and young adulthood. Primary care pediatric nurse practitioners need to be alert to the prevalence of noncompliance with treatment in heart transplant patients, which continues to be highest in adolescence. Low compliance in adolescence increases morbidity, contributes to decreasing quality of life, and is the leading reason for graft failure and mortality in this age group...
April 12, 2017: Journal of Pediatric Health Care
https://www.readbyqxmd.com/read/28396164/dose-escalation-of-total-marrow-irradiation-in-high-risk-patients-undergoing-allogeneic-hematopoietic-stem-cell-transplantation
#15
Susanta Hui, Claudio Brunstein, Yutaka Takahashi, Todd DeFor, Shernan G Holtan, Veronika Bachanova, Christopher Wilke, Darren Zuro, Celalettin Ustun, Daniel Weisdorf, Kathryn Dusenbery, Michael R Verneris
Patients with refractory leukemia or minimal residual disease (MRD) at transplantation are at increased risk of relapse. Augmentation of irradiation, especially to sites of disease (ie, bone marrow) is one potential strategy for overcoming this risk. We studied the feasibility of radiation dose escalation in high-risk patients using total marrow irradiation (TMI) in a phase I dose-escalation trial. Four pediatric and 8 adult patients received conditioning with cyclophosphamide and fludarabine in conjunction with image-guided radiation to the bone marrow at 15 Gy and 18 Gy (in 3-Gy fractions), while maintaining the total body irradiation (TBI) dose to the vital organs (lungs, hearts, eyes, liver, and kidneys) at <13...
April 7, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28394814/percutaneous-mechanical-circulatory-support-using-impella%C3%A2-devices-for-decompensated-cardiogenic-shock-a-pediatric-heart-center-experience
#16
Dhaval Parekh, Aamir Jeewa, Sebastian C Tume, William J Dreyer, Ricardo Pignatelli, David Horne, Henri Justino, Athar M Qureshi
Cardiogenic shock remains a significant cause of mortality and morbidity in children with heart failure. Percutaneous mechanical circulatory support may be an additional tool to augment left heart support and decompression in addition to conventional therapies. This report aims to review the clinical and hemodynamic outcomes of the Impella® device at a pediatric center. A retrospective review of all implants between October 2014 and November 2016 was conducted. Clinical outcomes, device implant techniques, complications, and hemodynamic data were collected...
April 6, 2017: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/28378408/cancer-recurrence-and-mortality-after-pediatric-heart-transplantation-for-anthracycline-cardiomyopathy-a-report-from-the-pediatric-heart-transplant-study-phts-group
#17
Matthew J Bock, Elfriede Pahl, Paolo G Rusconi, Gerard J Boyle, John J Parent, Clare J Twist, James K Kirklin, Elizabeth Pruitt, Daniel Bernstein
We aimed to determine whether malignancy after pediatric HTx for ACM affects overall post-HTx survival. Patients <18y listed for HTx for ACM in the PHTS database between 1993 and 2014 were compared to those with DCM. A 2:1 matched DCM cohort was also compared. Wait-list and post-HTx survival, along with freedom from common HTx complications, were compared. Eighty subjects were listed due to ACM, whereas 1985 were listed for DCM. Although wait-list survival was higher in the ACM group, post-HTx survival was lower for the ACM cohort...
April 4, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28366553/implications-and-outcomes-of-cardiac-grafts-refused-by-pediatric-centers-but-transplanted-by-adult-centers
#18
Farhan Zafar, Raheel Rizwan, Angela Lorts, Roosevelt Bryant, James S Tweddell, Clifford Chin, David L Morales
BACKGROUND: According to Organ Procurement Transplant Network policy, hearts from donors age <18 years are offered to pediatric recipients before being offered to adults of the same health status. We aimed to analyze differences in the use of adolescent donor hearts between adult and pediatric candidates and also to analyze the outcomes of pediatric candidates in which an adolescent donor heart was refused and later used in an adult recipient. METHODS: All adolescent donors (age 12-17 years) for 2000 to 2015 were identified using the standard United Network of Organ Sharing dataset and matched against the Potential Transplant Recipient dataset...
March 11, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28365178/pediatric-heart-donor-assessment-tool-ph-dat-a-novel-donor-risk-scoring-system-to-predict-1-year-mortality-in-pediatric-heart-transplantation
#19
Farhan Zafar, Robert D Jaquiss, Christopher S Almond, Angela Lorts, Clifford Chin, Raheel Rizwan, Roosevelt Bryant, James S Tweddell, David L S Morales
BACKGROUND: In this study we sought to quantify hazards associated with various donor factors into a cumulative risk scoring system (the Pediatric Heart Donor Assessment Tool, or PH-DAT) to predict 1-year mortality after pediatric heart transplantation (PHT). METHODS: PHT data with complete donor information (5,732) were randomly divided into a derivation cohort and a validation cohort (3:1). From the derivation cohort, donor-specific variables associated with 1-year mortality (exploratory p-value < 0...
March 7, 2017: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/28363739/functional-status-of-united-states-children-supported-with-a-left-ventricular-assist-device-at-heart-transplantation
#20
Anica Bulic, Katsuhide Maeda, Yulin Zhang, Sharon Chen, Doff B McElhinney, John C Dykes, Amanda M Hollander, Seth A Hollander, Jenna Murray, Olaf Reinhartz, Mary Alice Gowan, David N Rosenthal, Christopher S Almond
BACKGROUND: As survival with pediatric left ventricular assist devices (LVADs) has improved, decisions regarding the optimal support strategy may depend more on quality of life and functional status (FS) rather than mortality alone. Limited data are available regarding the FS of children supported with LVADs. We sought to compare the FS of children supported with LVADs vs vasoactive infusions to inform decision making around support strategies. METHODS: Organ Procurement and Transplant Network data were used to identify all United States children aged between 1 and 21 years at heart transplant (HT) between 2006 and 2015 for dilated cardiomyopathy and supported with an LVAD or vasoactive infusions alone at HT...
March 2, 2017: Journal of Heart and Lung Transplantation
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