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Pediatric cardiac transplant

Yoshihiro Ohara, Hitoshi Ohto, Tetsunori Tasaki, Hideki Sano, Kazuhiro Mochizuki, Mitsuko Akaihata, Shogo Kobayashi, Tomoko Waragai, Masaki Ito, Mitsuaki Hosoya, Kenneth E Nollet, Kazuhiko Ikeda, Chitose Ogawa, Takahiro Kanno, Yayoi Shikama, Atsushi Kikuta
BACKGROUND: Pediatric apheresis for peripheral blood stem cell transplantation should be carried out with due concern for low corporeal blood volume and vulnerability to hypocalcemia-related complications, hypovolemic shock, and hypervolemic cardiac overload. STUDY DESIGN AND METHODS: We retrospectively investigated a total of 267 apheresis procedures from 1990 to 2013 on 93 children between 0 and 10 years old, including 89 patients and 4 healthy donors, with body weights of 6...
September 30, 2016: Transfusion and Apheresis Science
Suma P Goudar, G Hamilton Baker, Shahryar M Chowdhury, Kimberly J Reid, Girish Shirali, Mark A Scheurer
BACKGROUND: Adult studies demonstrate that echocardiographic measurements of cardiac function using speckle tracking correlate with invasive measurements, but such data in the pediatric population are sparse. Our aim was to compare speckle-derived measures of cardiac function to measurements routinely obtained by cardiac catheterization in children. METHODS: Echocardiograms were performed on the day of cardiac catheterization. Using Tomtec 2D Cardiac Performance Analysis, longitudinal strain (LS), longitudinal strain rate (LSR), early diastolic LSR, and ejection fraction (EF) for the right and left ventricle (RV and LV) were calculated via speckle tracking...
October 14, 2016: Echocardiography
Tanya N Antonini, Stacey S Beer, Tamir Miloh, William J Dreyer, Susan E Caudle
OBJECTIVE: The purpose of this study was to review the current literature on neuropsychological functioning in two groups of children requiring organ transplants (liver or heart) and present recent clinical data collected through the liver and cardiac transplantation programs at a large pediatric academic medical center. METHOD: Data included in this study came from 18 patients who completed evaluations for heart transplant (n = 8) or liver transplant (n = 10) between the ages of 2 and 6 years (inclusive)...
October 11, 2016: Clinical Neuropsychologist
Eva Hlavackova, Martin Liska, Hana Jicinska, Jiri Navratil, Jiri Litzman
The Fontan operation or total cavopulmonal connection (TCPC) is a palliative surgical correction of rare and complex inborn cardiac malformations that are characterized by univentricular circulation. Protein-losing enteropathy (PLE) develops in 4-13% of patients after the Fontan procedure. Fontan-related PLE leads to secondary combined immunodeficiency marked by hypogammaglobulinemia and predominantly CD4+ lymphocytopenia. Here, we present 3 case reports of patients with secondary immunodeficiency after the Fontan operation...
September 30, 2016: International Archives of Allergy and Immunology
Ilan Maizlin, David A Klima, Elizabeth A Beierle
Posttransplant lymphoproliferative disorder (PTLD) is not uncommon in pediatrics. This case report describes a case of intestinal intussusception that occurred secondary to lymphoid hyperplasia in a child with PTLD following a cardiac transplant. This case was unique in its presentation with multiple areas of intussusception and with need for surgical intervention. The diagnosis of intussusception secondary to lead points from PTLD should be strongly considered in pediatric transplant patients presenting with abdominal complaints...
September 2016: American Surgeon
Roosevelt Bryant, Farhan Zafar, Chesney Castleberry, John L Jefferies, Angela Lorts, Clifford Chin, David L S Morales
The Berlin Heart EXCOR pediatric ventricular assist device is approved by the FDA for bridge to cardiac transplantation (BTT) in children. As the clinical outcomes of the EXCOR continue to be evaluated in the U.S., data on post transplant survival are needed. The UNOS database was used to identify patients < 18 years old undergoing orthotopic heart transplantation (OHT) from 6/2004 to 6/2014. Patients undergoing BTT with the EXCOR were identified. A matched cohort of (358) patients undergoing OHT without pre-transplant mechanical circulatory support (no-MCS) was also identified as controls...
September 20, 2016: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
Caroline M Sierra, Robert Tan, Jim Eguchi, Leonard Bailey, Richard E Chinnock
Pediatric heart transplant patients at our institution are immunosuppressed with a CNI and another immune-modulating agent without utilizing corticosteroids. Patients whose renal function worsened and who did not respond to CNI minimization had their CNI discontinued. The clinical history of 35 pediatric heart transplant patients with significant renal insufficiency whose CNI was discontinued was retrospectively analyzed. Data including serum creatinine and weight were collected before, at time of, and every 3-6 months after CNI discontinuation...
September 23, 2016: Pediatric Transplantation
Eyal Reinstein, Ana Gutierrez-Fernandez, Shay Tzur, Concetta Bormans, Shai Marcu, Einav Tayeb-Fligelman, Chana Vinkler, Annick Raas-Rothschild, Dana Irge, Meytal Landau, Mordechai Shohat, Xose S Puente, Doron M Behar, Carlos Lopez-Otın
In the vast majority of pediatric patients with dilated cardiomyopathy, the specific etiology is unknown. Studies on families with dilated cardiomyopathy have exemplified the role of genetic factors in cardiomyopathy etiology. In this study, we applied whole-exome sequencing to members of a non-consanguineous family affected by a previously unreported congenital dilated cardiomyopathy syndrome necessitating early-onset heart transplant. Exome analysis identified compound heterozygous variants in the FLNC gene...
September 7, 2016: European Journal of Human Genetics: EJHG
J Vockley, J Charrow, J Ganesh, M Eswara, G A Diaz, E McCracken, R Conway, G M Enns, J Starr, R Wang, J E Abdenur, J Sanchez-de-Toledo, D L Marsden
Long-chain fatty acid oxidation disorders (LC-FAOD) can cause cardiac hypertrophy and cardiomyopathy, often presenting in infancy, typically leading to death or heart transplant despite ongoing treatment. Previous data on triheptanoin treatment of cardiomyopathy in LC-FAOD suggested a clinical benefit on heart function during acute failure. An additional series of LC-FAOD patients with critical emergencies associated with cardiomyopathy was treated with triheptanoin under emergency treatment or compassionate use protocols...
August 27, 2016: Molecular Genetics and Metabolism
Adlette Inati, Mario Kahale, Nada Sbeiti, Maria Domenica Cappellini, Ali T Taher, Suzanne Koussa, Therese A Nasr, Khaled M Musallam, Hussein A Abbas, John B Porter
BACKGROUND: Iron overload is well documented in patients with β-thalassemia major, and patients who have undergone hematopoietic stem cell transplantation (HSCT) remain at risk as a result of pre- and immediate post-HSCT transfusions. PROCEDURE: This is a prospective, randomized, 1-year clinical trial that compares the efficacy and safety of the once-daily oral iron chelator deferasirox versus phlebotomy for the treatment of iron overload in children with β-thalassemia major following HSCT...
August 31, 2016: Pediatric Blood & Cancer
Steven R Sloan, Robert I Parker
Outside the neonatal period, most platelets that are transfused to pediatric patients are given to those who are thrombocytopenic secondary to malignancy and associated therapy and/or hematopoietic progenitor cell transplant, or to those with significant bleeding associated with surgery, especially cardiac surgery. Indications for platelet transfusion, doses, and other practices for children largely mimic adult platelet transfusion protocols because there are few pediatric-specific studies in this area. Pediatric platelet transfusion practices would benefit from focused pediatric research...
October 2016: Transfusion Medicine Reviews
Asma M Khan, Robert S Green, Irene D Lytrivi, Raj Sahulee
BACKGROUND: Pediatric heart transplantations are limited by the supply of donor allografts. We sought to determine the cardiac allograft utilization rate for pediatric donors and identify donor factors that predict graft use for transplantation. METHODS: The United Network for Organ Sharing deceased donor database was queried from 4/30/2006 to 3/31/2014. Donor risk factors that might affect graft use for cardiac transplantation were evaluated. The pediatric cardiac graft utilization rate was calculated, and logistic regression modeling was performed to determine the risk factors relationship with graft use for transplantation...
August 19, 2016: Transplant International: Official Journal of the European Society for Organ Transplantation
Katherine Taylor, Wooheon Thomas Kim, Malak Maharramova, Victor Figueroa, Smruthi Ramesh, Armando Lorenzo
INTRODUCTION: Smaller children are presenting for renal transplantation as the treatment of choice for end-stage renal disease. Adult donor organs are more successful than pediatric deceased donor organs. An adult kidney may sequester ~75% of the circulating volume of a 5 year-old child and requires significantly increased cardiac output to maintain renal perfusion. Treatment includes volume, inotropic or vasopressor agents, or central neuroaxial blockade for sympatholysis. We describe the perioperative anesthestic management as a guide to clinical outcomes...
October 2016: Paediatric Anaesthesia
Logan G Spector, Jeremiah S Menk, Jeffrey M Vinocur, Matthew E Oster, Brian A Harvey, James D St Louis, James Moller, Lazaros K Kochilas
BACKGROUND: The long-term outcomes of patients undergoing interventions for congenital heart disease (CHD) remain largely unknown. We linked the Pediatric Cardiac Care Consortium (PCCC) with the National Death Index (NDI) and the United Network for Organ Sharing Dataset (UNOS) registries to study mortality and transplant occurring up to 32 years postintervention. The objective of the current analysis was to determine the sensitivity of this linkage in identifying patients who are known to have died or undergone heart transplant...
2016: Journal of the American Heart Association
Nowell M Fine, Sudhir S Kushwaha
The mammalian target of rapamycin (mTOR) inhibitors sirolimus and everolimus are increasingly utilized in cardiothoracic transplantation. Several recent clinical trials have demonstrated their efficacy in combination with reduced cyclosporine dosing in de novo heart transplant recipients, in particular with everolimus. A number of other studies have demonstrated their efficacy for improving renal function and reducing calcineurin inhibitor use, attenuating cardiac allograft vasculopathy progression and reducing cytomegalovirus infections in maintenance heart transplant populations...
August 5, 2016: Transplantation
Joseph W Rossano, Antonio G Cabrera, Robert E Shaddy
OBJECTIVES: Although there have been tremendous advancements in the care of severe pediatric cardiovascular disease, heart transplantation remains the standard therapy for end-stage heart disease in children. As such, these patients comprise an important and often complex subset of patients in the ICU. The purpose of this article is to review the causes and management of allograft dysfunction and the medications used in the transplant population. DATA SOURCES: MEDLINE, PubMed, and Cochrane Database of systemic reviews...
August 2016: Pediatric Critical Care Medicine
Jonathan Byrnes, Chet Villa, Angela Lorts
OBJECTIVES: The objectives of this review are to discuss the process of patient and mechanical device selection, operative management, and postoperative care with a focus on the management of right ventricular failure, anticoagulation strategies, device-related infections and neurologic sequelae. DATA SOURCES: MEDLINE, PubMed. CONCLUSION: The number of patients with advanced heart failure due to either acquired or congenital heart disease continues to increase, necessitating in some mechanical circulatory support and in others cardiac transplantation...
August 2016: Pediatric Critical Care Medicine
Oluwatoyosi A Onwuemene, Deneen M Heath, Carol Hartman, Edward C C Wong
Pediatric cardiac transplant patients with antibody-mediated rejection (AMR) often undergo therapeutic plasma exchange (TPE) to remove pathologic donor specific antibodies (DSA). In cases where DSA persist, it is unclear how long TPE should be continued. We report a case of a 17-year-old cardiac transplant patient with AMR where use of a C1q complement fixing antibody assay helped guide TPE cessation. This report adds to the existing literature that highlights the potential clinical significance of C1q antibodies in AMR management...
July 31, 2016: Journal of Clinical Apheresis
Altan Alim, Yaman Tokat, Yalcin Erdogan, Zafer Gokkaya, Murat Dayangac, Yildiray Yuzer, Arzu Oezcelik
FH is an autosomal dominant genetic disorder characterized by increased TC and LDL level, which leads to xanthomas, atherosclerosis, and cardiac complications even in childhood. The treatment options are diet, medical treatment, lipid apheresis, and LT. The aim of our study was to analyze our data of patients with FH. Between 2004 and 2015, there were 51 patients who underwent pediatric LT at our center. All patients with FH were identified, and the data were retrospectively analyzed. There were eight patients with homozygous FH in the median age of 10 years (IQR 6-12) who underwent LT...
July 20, 2016: Pediatric Transplantation
Zora Meyer, M Fischer, J Koerfer, K T Laser, D Kececioglu, W Burchert, S Ulrich, R Preuss, N A Haas
AIM: Medical imaging by using FDG-PET/CT (PET-CT) can detect, confirm or eliminate with high sensitivity areas of suspected infections in case of persistent fever of unknown origin in combination with other bacteriological examinations. The aim of this study was to assess the potential role of PET-CT in detecting or excluding infections or other inflammatory processes in patients with congenital heart defects (CHD). In addition we wanted to evaluate the practical impact of PET-CT on the subsequent clinical management...
October 1, 2016: International Journal of Cardiology
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