Pancreatitis Following Paediatric Heart Transplantation

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The Journal of Heart and Lung Transplantation, Vol 36, No 4S, April 2017

analysis and Cox proportional hazard modeling. Modified Cabrol bi-atrial or bi-caval implant was performed along with using a long segment of ascending aorta to facilitate anatomic reconstruction of the great vessels. Results: Patient characteristics were identical for the L-TGV and match cohorts. The mean age of patients with L-TGV was 40.8 (range 18.4 to 60.7). 4 out of 7 patients had undergone at least one prior heart operation including 3 with systemic AV valve replacement. All had the onset of class 3-4 heart failure symptoms within 2 years of transplant listing. Heart implantation was facilitated by avoiding concomitant lung donation and preserving as much main and branch pulmonary arteries as possible. In all cases, reconstruction of the great vessels was easily carried out. Long term survival was identical for L-TGV patients (83+/-15%) and matched cohort (76+/-10%, Log-Rank, P= 0.6850). One L-TGV patient died 18 months after HT from rejection due to non compliance. Conclusion: Patients undergoing HT for advanced heart failure due to L-TGV have excellent outcomes. It is imperative to consider the technical demands for operation including donor selection and maintenance of as much aorta and pulmonary artery as possible when planning for HT in this group of patients. 7( 16) Outcomes After Listing for Heart Transplantation in Pediatric and Young Adult Patients with Hypertrophic Cardiomyopathy: An Analysis of the Scientific Recipient Transplant Registry (SRTR) D. Cheong ,1 D.T. Hsu,2 J.M. Lamour.2  1School of Medicine, University of Connecticut Health Center, Farmington, CT; 2Pediatric Cardiology, The Children’s Hospital at Montefiore, Bronx, NY. Purpose: Although hypertrophic cardiomyopathy (HCM) is the most common pediatric cardiomyopathy, only a small percent require heart transplant (HT). Post-HT survival in HCM pts has been reported to be lower than in pts with dilated cardiomyopathy (DCM) and worse survival has been associated with younger age at HT. Risk factors for worse outcome are not well defined. Methods: Demographic and clinical data from pts with HCM age ≤ 25 yrs at listing were identified in the SRTR. Characteristics and outcomes pre- and post-HT were compared between younger (age < 5 yrs) and older children (5-25 yrs). Factors associated with death while waiting in pts with HCM were identified. Post-HT survival was compared between HCM and DCM pts. Results: HCM was present in 401/15,515 (2.6%) pts listed for HT since 1987; 135 were <  5 yrs and 266 were between 5-25 yrs. Characteristics at listing are shown in Table 1. Younger pts were more likely to be in an intensive care unit (ICU) and listed as Status 1A. In older pts, amiodarone and implantable defibrillator (ICD) use were more common and mean pulmonary capillary wedge pressure (mPCWP) was higher. Ventricular assist devices (VAD) were used in < 5% of the entire cohort. Death within 6 months of listing was worse in the younger vs. older pts (24% vs. 10%, p< 0.01). Risk factors for death while waiting included ICU and higher mPCWP. One yrsurvival after HT was no different between older vs. younger pts (95% vs. 88%, p =  0.65). Post-HT survival was no different between HCM and DCM pts in either age group. Conclusion: Among HCM pts listed for HT, survival to HT was worse in infants and toddlers compared to children and young adults. VAD use was rare. Higher mPCWP and ICU were associated with worse survival to transplant. Post-HT survival in both younger and older children with HCM was excellent and not significantly different than post-HT survival in pts with DCM.



7( 17) Variability in Tacrolimus Levels Is Associated with Biopsy Proven Rejection in Pediatric Heart Transplant Recipients M. Cousino ,1 K. Rea,1 M. Zamberlan,2 J. Jordan,1 H. Lim,1 D. Peng,1 K. Schumacher.1  1Pediatrics, C.S. Mott Children's Hospital, Ann Arbor, MI; 2Cardiac Surgery, C.S. Mott Children's Hospital, Ann Arbor, MI. Purpose: Research in pediatric and adult liver transplant has suggested that variability in tacrolimus trough levels is associated with higher rates of late rejection. Variability in tacrolimus levels may be a result of non-adherence to the immunosuppressant regimen, which is a modifiable causal factor that is amenable to intervention. This is the first study to examine associations between variability in tacrolimus levels and health-related outcomes in pediatric heart transplant recipients. Methods: Pediatric heart transplant recipients of any age followed at a single center were eligible. The standard deviation of outpatient tacrolimus trough levels collected in year prior to study enrollment was calculated for each participant. The number of biopsy proven rejection episodes, hospitalizations, and clinic attendance were collected from medical records. Independent samples t-tests and chi-square analyses were conducted to examine group differences between those with a rejection episode and those without. Results: Participants included 46 heart transplant recipients ages 0-22 years (M= 11.4; SD= 5.8) with a mean time since transplant of 6 years (SD= 4.8). There were 14 episodes of biopsy proven rejection in the sample. Individuals with at least one episode of biopsy-proven rejection were significantly more likely to have had higher variability in tacrolimus levels prior to the episode (M= 2.94, SD= 1.73 in rejection patients vs. M= 1.73, SD= 1.10 in non-rejectors; p = .006). Using a cut-off of greater than/equal to 2 SD in tacrolimus trough variability, a high variability group was defined. The odds of having a trough level SD greater than/equal to 2 was 5.4 times higher in the rejectors group. Higher tacrolimus tough variability was also associated with increased number of hospitalizations (p= .01), but not with clinic attendance. Conclusion: Variability in tacrolimus trough levels is associated with biopsy proven rejection in pediatric heart transplant recipients, replicating findings documented in other transplant populations. This easy to calculate variable may help to identify patients in need of interventions to decrease rejection episodes.

7( 18) Pancreatitis Following Paediatric Heart Transplantation T.G. Day ,1 A. Sylvan,2 S. Christoforides,1 E. Hannon,2 J. Curry,2 M. Fenton,1 M. Burch,1 J. Simmonds.1  1Cardiology, Great Ormond Street Hospital for CHildren NHS Foundation Trust, London, United Kingdom; 2Surgery, Great Ormond Street Hospital for CHildren NHS Foundation Trust, London, United Kingdom. Purpose: Pancreatitis is a serious complication that is significantly more frequent in adult heart transplant recipients compared those undergoing other forms of cardiac surgery. Although well described in the adult population, there are relatively few data in paediatric transplant recipients. We aimed to investigate the incidence and outcome of pancreatitis following paediatric heart transplantation in our institution. Methods: Biochemical and radiological results were reviewed from all heart transplant recipients at our institution transplanted from 1st of January 2005 to 1st June 2015. Pancreatitis was defined as an increase in either amylase or lipase to over three times the normal upper reference range (≥  300 or 585 international units/litre respectively), or radiological findings in keeping with

Abstracts S273 pancreatitis. The ages of the pancreatitis and non-pancreatitis groups were compared using the Student’s T test. Results: 266 heart transplants were performed over the time period. 10 cases of pancreatitis were identified (incidence 3.7 %). The mean age of the pancreatic group was 11.9 years, versus 8.3 years in the non-pancreatitis group (p =  0.02). All 10 patients had abnormal biochemistry, either elevated amylase (1 patient), lipase (6 patients), or both amylase and lipase (3 patients). All pancreatitis patients also had imaging of their pancreas attempted using either abdominal computed tomography (CT) or ultrasound, but in 3 cases it was not possible to visualise the pancreas (all ultrasound scans). 2 cases had a normal pancreatic appearance (1 CT and 1 ultrasound). 5 cases had radiological evidence of pancreatitis (3 CT and 2 ultrasound). 2 patients required surgical intervention for pancreatits (1 laparotomy, 1 percutaneous drainage of pancreatic pseudocyst and gastrocystic stenting). Of the 10 patients with pancreatitis, 2 died before discharge from hospital. 1 patient required long term treatment with insulin for impaired glucose tolerance. Conclusion: This is the largest study yet presented on pancreatitis following paediatric heart transplant. Our incidence of pancreatitis is similar to previously published studies in both children and adults. The patients who developed pancreatitis were significantly older than those who did not. Pancreatitis is associated with a substantial pre-discharge mortality, and can result in long-term morbidity for some patients. 7( 19) Improving Outcomes of Cardiac Transplantation in Patients with Cavopulmonary Connection L.E. De León ,1 J.S. Heinle,1 E. McKenzie,1 C.M. Mery,1 L.C. Kane,1 W. Dreyer,2 S.W. Denfield,2 J.F. Price,2 A.G. Cabrera,2 C.D. Fraser Jr.,1 I. Adachi.1  1Division of Congenital Heart Surgery, Texas Children's Hospital; Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX; 2Lillie Frank Abercrombie Section of Pediatric Cardiology, Texas Children's Hospital, Houston, TX. Purpose: Previous studies have reported unfavorable early mortality rates (up to 35%) following transplantation in patients with cavopulmonary connection(s) either with bidirectional Glenn (BDG) or Fontan procedures. We sought to evaluate outcomes of cardiac transplantation in this group of patients at a single pediatric institution. Methods: All patients who had undergone cardiac transplantation after a BDG or Fontan procedure between 1995 and 2016 were included. Actuarial survival was analyzed using the Kaplan-Meier method. Results: The cohort included 53 patients [median age 7 years (7 months - 20 years)], with 25 (47%) at BDG and 28 (53%) at Fontan stage. Six patients (11%) were mechanically bridged. Median time from BDG or Fontan to transplantation was 4 years (2 months - 19 years). Protein losing enteropathy (PLE) and/or plastic bronchitis (PB) were seen in 11 (39%) of Fontan patients. There were 2 (3.7%) operative deaths; one due to graft failure at 43 days postoperatively in a BDG patient who had a positive crossmatch, and the other one due to multi-organ failure at 306 days in a Fontan patient. There were 16 (30%) late mortalities, eight in each group of patients. Actuarial survival at 1- and 5-years after transplant was 89% and 59%, respectively. When compared to all transplant recipients with congenital heart disease (CHD) but without cavopulmonary connections, there was no statistical difference in operative [2/53 (3.7%) vs 1/45 (2.2%), p= 0.55] or late mortality [16/53 (30%) vs 16/45 (36%), p=  0.57]. The Fontan non-survivor had severe PLE/ascites, and these conditions persisted even after restoration of normal cardiac physiology with transplantation. This was in stark contrast to the rest of the Fontan patients, most of whom [9/10 (90%)] experienced resolution of PLE/PB. Conclusion: With appropriate patient selection, cardiac transplantation after BDG and Fontan procedures can be performed with low operative mortality. Resolution of PLE and PB may be expected unless transplantation is offered at a too advanced stage. Although the presence of cavopulmonary connections is not a negative predictor among the CHD population, further improvement is necessary in posttransplant outcomes since late outcomes in this cohort remain suboptimal relative to the overall pediatric transplant outcomes. 7 ( 20) Primary Graft Dysfunction After Pediatric Heart Transplant: A Single Center Experience

K. Mowers , K. Simpson, P. Eghtesady, O. Petrucci, C. Canter, C. Castleberry.  Washington University School of Medicine, St. Louis, MO. Purpose: Primary graft dysfunction (PGD) has been identified as a complication after transplantation (OHT) and a significant cause of mortality, particularly in infant recipients. Lack of standardized definition of PGD in the pediatric population makes prevalence and impact of PGD unclear. Methods: We applied adult ISHLT PGD consensus guidelines, which include number and doses of inotropes and need for mechanical circulatory support (MCS), to 208 pediatric OHT recipients from Jan 2005-May 2016. PGD was defined as: moderate PGD - inotrope score > 10 on post-operative day 1 (24-48 hours), and severe PGD - MCS within 24 hours (in the absence of rejection). Results: PGD occurred in 36 patients (17%); 18 of which had severe PGD (8.5%). All with severe PGD were on ECMO. Multivariate risk factors for PGD included a positive cross match (PCM) (OR 7.23, 95% 1.9,29.8, p< 0.01) and cardiopulmonary bypass time (CPBT) (OR 10.14/10 min, 95% 10.05,10.2, p< 0.01), but not age, weight, ischemic time, or primary diagnosis. Risk factors for severe PGD also included PCM (OR 7.87, 95% 1.26,55.4, p= 0.03) and CPBT (OR 10.3/10 min, 95% 10.05,10.2, p= 0.03). Patients with PGD vs those without PGD, had longer hospital courses (28 vs 10 days (med), p< 0.01); yet when comparing patients with moderate PGD to without PGD, they also had longer courses (26 vs 10 days) but this was not statistically significant (p= 0.08). Patients with PGD had decreased overall survival (69% vs 85%, p= 0.03), which remained significant on multivariate analysis (HR 2.82, 95% CI 1.01, 7.67, p< 0.01 ). Severe PGD was associated with the worst survival; yet survival was comparable between moderate PGD and those without PGD (89% vs 85%, p= 0.65) [Figure 1]. Conclusion: In the current era, infants do not have increased risk of PGD; however, it appears to be related to CPBT and PCM. Need for MCS, but not high dose inotropes, is associated with increased mortality. The association of PCM and severe PGD in the absence of detectable rejection requires further investigation.

7( 21) AMR in the Current Era: Clinical, Pathologic, and Immunologic Features in Pediatric Heart Transplant Recipieints D. Drogalis-Kim ,1 M.J. Hickey,2 M.C. Fishbein,3 J.C. Alejos,1 E.F. Reed,2 N.J. Halnon.1  1Pediatrics, UCLA, Los Angeles, CA; 2Immunogenetics Center, Department of Pathology and Laboratory Medicine, UCLA, Los Angeles, CA; 3Pathology and Laboratory Medicine, UCLA, Los Angeles, CA. Purpose: Right ventricular (RV) endomyocardial biopsy (EMB), an invasive procedure, is currently the mainstay for monitoring for allograft rejection. Over time, criteria for the pathologic diagnosis of antibody mediated rejection (pAMR) have evolved based on new diagnostic methods. However,