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Two Viable Surgical Options for Varying Degree of Pulmonary Vein Stenosis in Pediatric Heart Transplant Recipients
Abstracts S325 Purpose: In children, there is little evidence for the comparative utility of biomarkers in clinical assessment of heart failure (HF). Plasma levels of Asymmetric Dimethyl-arginine (ADMA), a nitric oxide synthase inhibitor, have been correlated with HF severity and risk of adverse outcomes in adult HF populations. This pilot study assessed the comparative utility of brain natriuretic peptide (BNP), N-terminal pro-BNP (NT-pro-BNP) and ADMA, a novel biomarker pediatric HF, to risk stratify outcomes. Methods: Twenty consecutive patients with severe HF defined as ventricular dysfunction and treatment with intravenous inotropic medications were enrolled from October 2013 to July 2014 in this prospective, observational pilot study. At the time of presentation, plasma ADMA, BNP and NT-pro-BNP were measured, and ejection fraction (EF) by echocardiogram and inotrope score (IS) were recorded. Primary outcomes were hospital discharge (DISC), or cardiac transplantation or death (Tx/D). Results: Patient ages ranged 0.08-17 years, median 1.7 years. Diagnoses included cardiomyopathy (12), ventricular failure in congenital heart disease (4), myocarditis (3) and graft rejection (1). 8 patients had outcome of DISC, 12 Tx/D. Plasma ADMA levels on presentation were not elevated compared to normal pediatric values. There was no difference in ADMA level between outcome groups (p= 0.22), and no correlation between ADMA level and EF or IS. BNP and NT-pro-BNP levels were elevated (BNP 63-5000, median 2529; NT-pro-BNP 544-70000, median 10600), without significant differences between outcome groups (p= 0.48 and p= 0.62, respectively.) BNP and NT-pro-BNP levels were strongly correlated (R= 0.83, p< 0.0001). Plasma ADMA levels were moderately correlated with BNP and NT-pro-BNP (R= 0.52, p= 0.019 and R= 0.65, p= 0.003, respectively). Conclusion: Unlike in adults with HF, plasma ADMA was not elevated in children with severe acute HF. Despite a moderate correlation with BNP and NT-pro-BNP; lack of correlation with outcome, EF or IS suggests that ADMA level does not describe severity of HF. As expected, BNP and NT-proBNP levels were both elevated and strongly correlated. The strength of correlation suggests that a larger study is needed to assess the superiority of one natriuretic peptide over the other in clinical assessment and risk stratification for children with heart failure. 9( 03) Effect of Induction Therapy on Graft Survival in Primary Pediatric Heart Transplantation: A Propensity Score Analysis of the UNOS Database R.J. Butts , M. Davis, A. Savage, A. Burnette, M. Kavarana, A. Atz, P. Nietert. Medical Univ of Sourth Carolina, Charleston, SC. Purpose: Induction therapy use in pediatric heart transplantation has increased. The aim of this study is to investigate the effect of induction therapy on graft survival. Methods: The UNOS database was queried for isolated pediatric heart transplants (age < 18 years) from January 1, 1994 to December 31, 2013. Propensity scores for induction treatment were calculated by estimating probability of induction using a logistic regression model. Transplants were matched between induction treatment groups based upon the propensity score, reducing potential biases. Using only propensity score matched transplants, the effect of induction therapy on graft survival was investigated using Cox-proportional hazards. Results: Of 3741 pediatric heart transplants with complete data for use in the propensity score, 2792 transplants were successfully propensity score matched (induction n= 1396, no induction n= 1396). There were no significant differences in transplant and pre-transplant covariates between induction groups (Table 1). Graft survival in the induction group at 1, 5 and 10 years post-transplant was 91%, 76%, and 61%, respectively, versus 88%, 72%, and 56% graft survival in the no induction cohort. In the Cox-proportional hazards model, the use of induction of was not associated with graft loss (HR 1.14 95%CI 0.98-1.33, p-value = 0.09). Conclusion: Induction therapy is not associated with improved graft survival in primary pediatric heart transplantation. Further studies should investigate if different subgroups of pediatric heart transplant recipients benefit from induction therapy.
Comparison of Induction versus no-Induction cohorts
Female (%) Caucasian (%) Negative Crossmatch (%) High Degree of HLA match (%) Mechanical Ventilation (%) ECMO support (%) Inotropes (%) Age (yr, ±SD) Donor-Recipient Weight Ratio (±SD) Ischemic Time (hr, ±SD) PRA (%, ±SD) Clearance (mL/min/1.73m2, ±SD)
No Induction (n= 1396)
Induction (n= 1396)
43.6 81.8 84.9 14.5 16.1 4.1 50.4 6.6 ± 6.3 1.4 ± 0.5 3.7 ± 1.3 9.8 ± 23.4 123.1 ± 58.9
44.9 81.6 84.7 14.3 15.8 4.5 50.1 6.6 ± 6.3 1.4 ± 0.5 3.7 ± 1.2 10.0 ± 23.4 123.5 ± 58.5
9( 04) New Onset Diabetes Mellitus After Heart Transplant in Children S. Sehgal ,1 M.J. Bock,1 H.L. Palac,2 J.G. Gossett,1 B.S. Marino,1 C.L. Backer,1 E. Pahl.1 1Ann and Robert H. Lurie Children’s Hospital of Chicago, Chicago, IL; 2Department of Preventative Medicine, Northwestern University, Chicago, IL. Purpose: Diabetes mellitus is a recognized complication of solid organ transplantation in adults and is associated with decreased graft and patient survival. However, little is known about the incidence and risk factors of new onset diabetes mellitus (NODM) in pediatric heart transplant recipients. We aimed to characterize the incidence and describe risk factors for development of new onset diabetes mellitus after heart transplant in children. We investigated the outcomes of patients with NODM compared to those without. Methods: All children younger than 18 yrs of age who developed diabetes after heart transplant were identified from the Organ Procurement and Transplantation network (OPTN) database (1987-2014). Patients with preexisting diabetes and/or re-transplantation were excluded. Baseline demographic, clinical data (BMI, medical care utilization and immunosuppressive strategy) as well as follow up data [treated episodes of rejection, coronary allograft vasculopathy (CAV), renal dysfunction] were compared between patients with and without diabetes. Wilcoxon rank-sum was used for continuous variables and chi-square test for categorical variables. Multiple logistic regression was used to control for covariates. Results: There was a 8% incidence of NODM (443/5824); 77% were insulin dependent. Those who developed NODM were older at the time of transplant (median age 12 vs. 4 yr, p< 0.0001), had higher BMI (20 vs. 17, p< 0.001), and were sicker pre-transplant (more likely to have dialysis, ECMO or VAD placed), p< 0.01. The incidence of NODM was significantly higher in the recent era (2001-2014) compared to older era (1987-2000) (55 vs. 45%, p< 0.001). Patients with NODM had higher number of treated episodes of rejection (0.9 vs. 0.3, p< 0.001). After transplant, patients with NODM were twice as likely to develop CAV (OR 2.2, 95% CI: 1.8-2.7) and 3x as likely to develop renal dysfunction requiring dialysis (OR 3.0, 95% CI: 2.4-3.7) even after controlling for age and time since transplant (p< 0.0001). Conclusion: NODM is a serious comorbidity after pediatric heart transplant with higher incidence in the recent era. Risk factors for NODM include older recipient age, higher BMI, sicker recipients and higher number of treated rejection episodes. NODM is associated with increased risk of CAV and renal failure, thus aggressive treatment for tight glycemic control may be warranted in this population. 9( 05) Two Viable Surgical Options for Varying Degree of Pulmonary Vein Stenosis in Pediatric Heart Transplant Recipients E. Jean-St-Michel , O. Honjo, C. Manlhiot, A. Dipchand. Hospital for Sick Children, Toronto, ON, Canada. Purpose: Pulmonary vein (PV) stenosis is usually progressive and despite the current surgical technique, “sutureless repair”, it still has a high mortality rate and remains a relative contraindication for heart transplantation (HTx).
S326
The Journal of Heart and Lung Transplantation, Vol 34, No 4S, April 2015
We sought to evaluate the outcomes of pediatric patients diagnosed with PV stenosis before HTx with and without PV repair at the time of HTx. Methods: We performed a retrospective review of all 288 HTx performed between 1993 and 2013 at a single institution. Results: Overall, 12 (4%) patients had PV stenosis and all had complex congenital heart disease (AVSD (5), tricuspid atresia (1), Ebstein’s (1), hypoplastic left heart variant (3), CCTGA (1), critical AS (1)). Five of the patients had PV atresia (2 left PV atresia, 2 right PV atresia and 1 left lower PV atresia), therefore HTx into a single lung was purposely done. One patient with right PV atresia also needed left PVs repair at time of HTx. The other 7 patients all had “sutureless repair” at the time of HTx (2 bilateral PV repairs, 1 left and right upper PV repair, 4 left PVs repairs). The median ICU and hospital stay were 12 (IQR 6-14) and 25 (IQR 19-29) days. The mean ischemic and bypass times were 268±70.0 min and 152±53.2 min. The median intubation duration was 4 (IQR 3-12) days; only 2 patients required nitric oxide post transplant. Two patients required long-term supplemental oxygen, 3 had recurrent pneumonias and 2 had severe pulmonary hypertension. For the 5 patients with PV atresia, 2 (40%) are alive (4 and 12 years post-transplant); 2 (40%) died of pulmonary hypertension at 4 and 6 months post-transplant and 1 (20%) died of rejection. For the 7 patients with PV stenosis, there was only 1 death (14%) from graft failure (2 year post HTx); 1 patient (14%) had recurrence of severe left PV stenosis with flow to the left lung of 0.1ml/min/m2 but no sign of pulmonary hypertension. Death secondary to pulmonary hypertension was only associated with PV atresia and both patients had developed stenosis on the contralateral side. Those 2 patients were also transplanted younger than the other PV atresia patients (1 yr old vs. 4, 5 and 6 yrs of age). Conclusion: Heart transplant is a viable option for patients with PV stenosis or atresia. Death secondary to pulmonary hypertension, even in the setting of virtually no flow to one lung, was seen only in patients with PV atresia who developed stenosis on the contralateral side. 9( 06) Cirrhosis in Patients Following the Fontan Operation: Incidence and Long-Term Outcomes K. Pundi ,1 K.N. Pundi,2 J.N. Johnson,2 Z. Li,3 D.J. Driscoll,2 F. Cetta.2 1Mayo Clinic College of Medicine, Mayo Clinic, Rochester, MN; 2Department of Pediatrics, Division of Pediatric Cardiology, Mayo Clinic, Rochester, MN; 3Biomedical Statistics and Informatics, Mayo Clinic, Rochester, MN. Purpose: Hepatic complications including cirrhosis are increasingly recognized after the Fontan operation. We examined the incidence and predictors of cirrhosis after Fontan operation. Methods: We reviewed records of all patients who had a Fontan operation from 1973-2012 at our institution (n = 1052). 449 patients had available post-operative liver data. Patients were classified as having cirrhosis only if they had a diagnostic liver biopsy or characteristic findings on magnetic resonance imaging (MRI) or magnetic resonance elastography (MRE). Patients with isolated blood liver function tests or ultrasound abnormalities were not considered to have proven cirrhosis. Results: The overall 10, 20, and 30-year freedom from cirrhosis was 99%, 96%, and 76%, respectively. Among the 25 patients with cirrhosis, the mean age at Fontan operation was 11.4 ± 9.7 years. The average duration from the Fontan operation to diagnosis of cirrhosis was 19.5 ± 7.8 years. Of these 25 patients, 13 had an atriopulmonary connection, 6 had a lateral tunnel, 2 had an extracardiac conduit, and 4 had other Fontan connections. Only 2 patients had a fenestration. Atrial or ventricular arrhythmias were present in 20/25 patients (80%), diagnosed a mean of 9.7 ± 5.0 years prior to cirrhosis. MRE-derived mean liver stiffness data was available on 8/25 patients with an average of 6.0 ± 1.8 kPa (> 5 kPa interpreted as stage 4 fibrosis or cirrhosis). Using multivariate analysis, a diagnosis of asplenia and preop digoxin use were the only factors associated with the development of cirrhosis. Survival following the diagnosis of cirrhosis was 71%, 25% and 17% at 1, 5 and 10 years respectively. The cause of death was known for 4 patients (3 multiorgan failure, 1 metastatic hepatocellular carcinoma). Conclusion: Patients with asplenia are at the highest risk of developing cirrhosis during follow-up, though some heterotaxy patients may have liver disease prior to Fontan. Long-term outcome after diagnosis of cirrhosis is poor, warranting noninvasive hepatic screening strategies in patients after Fontan. Ultimately our study may be underestimating the burden of liver
disease in these patients. Therapy is difficult and novel multiorgan transplant strategies may benefit this patient population. 9( 07) How Should the Effect of Persantine Be Measured Using Thromboelastography: Correlation and Agreement Between Percent ADP Inhibition and ADP Net G M. Massicotte ,1 J. Conway,2 L. May,3 H. Buchholz,4 C. Lo,5 A. Bruce,6 T. Tesoro,7 D. Rosenthal,3 C. Almond.8 1Thrombosis KIDCLOT, University of Alberta/Stollery Childrens Hospital, Edmonton, AB, Canada; 2Cardiology, University of Alberta/Stollery Childrens Hospital, Edmonton, AB, Canada; 3Cardiology, Stanford University, Stanford, CA; 4Cardiac Surgery, University of Alberta/Stollery Childrens Hospital, Edmonton, AB, Canada; 5Hematology, Stanford University, Stanford, CA; 6Hematology, University of Alberta/Stollery Childrens Hospital, Edmonton, AB, Canada; 7Pharmacy, Stanford University, Stanford, CA; 8Cardiology, Stanford University, Stanford, CA, Canada. Purpose: Controversy exists as to whether percent ADP (%ADP) inhibition or ADP Net G should be used as the primary laboratory target to adjust the dose of platelet inhibitors such as dipyridamole in children supported with Pediatric ventricular assist devices (VAD). The purpose of this study was to examine the agreement between these two values derived from Thromboelastography with Platelet Mapping® (TEG/PM) with respect to dose-adjustment recommendations. Methods: All pediatric patients supported with a pediatric VAD between 2006 and 2014 with TEG/PM data available at two pediatric VAD centers were identified using their respective hospital databases and merged under an IRB approved protocol. For each specimen, %ADP inhibition result (target 70-95%) was compared to ‘ADP NET G’ value (target 4-8), defined as the product of the baseline G (citrated kaolin with heparinase) and (100%-%ADP inhibition)/100. Agreement was evaluated using the Kappa statistic. Results: Overall there were a total of 665 TEG/PM specimens performed in 51 children across both centers. The median age of patients was 11 months (IQR 6 mo, 5 years), median weight 8.9 kg (IQR 6.2, 17.7); 29% had congenital heart disease (CHD) including 8% with single-ventricle disease, 49% received a temporary circulatory support device pre-implant, and 65% received an LVAD. Overall the mean %ADP inhibition was 49%±35% (SD) and mean ADP Net G 5±3.9 with a Pearson’s correlation of 0.78 for simultaneous values. Using %ADP inhibition of 70-95 as the target, 15.3% were classified as in-range, 66% sub-therapeutic and 18% supra-therapeutic; using ADP Net G of 4-8 as the target, 43.5% of values were classified as in-range, 41% sub-therapeutic and 14% supra-therapeutic. Correlation between the two classification systems was good (R 0.71, P< 0.001); agreement on dosing recommendation was poor (Kappa 0.39, P< 0.001) related in large part to the NET G value classifying significantly more patients as in-range. Conclusion: Overall, %ADP inhibition and ADP Net G values correlate well however there is poor agreement on dosing recommendations when an ADP Net G target of 4-8 is compared to a %ADP inhibition of 70-95%. Further research is needed to determine the optimal ADP target for pediatric VAD patients. 9( 08) Immune Profiling Pre/Post Berlin VAD Implant and Pre/Post Transplantation of Pediatric Heart Failure Patients R.H. Kerman ,1 P. Jindra,2 A. Jeewa,3 S. Burki,3 C. Fraser,3 I. Adachi.3 1Baylor College of Medicine, Houston, TX; 2Surgery, Baylor College of Medicine, Houston, TX; 3Texas Children’s Hospital and Baylor College of Medicine, Houston, TX. Purpose: The Berlin Heart Excor Ventricular Assist Device (Berlin VAD) was designed for use in children with heart failure and has allowed for successful bridging to heart transplantation (HTx). Blood products and VAD components exposure during VAD implant, may result in the production of antiHLA antibodies (Abs) which are a risk factor for rejection in HTx patients. Methods: We immunologically profiled 35 pediatric Berlin VAD patients (19 males, 16 females with a mean age of 6 ± 6 years of age) between 1999-2012 pre/post VAD implant and pre/post HTx. Immune profiling consisted of determining Flow panel reactive antibodies (PRAs), presence of donor specific antibody (DSA) and antibody fixation to complement (C1q assay). Assays were perfomed using One Lambda, Inc. reagents according to manufacturer’s instructions. Patients were excluded when immune data was unavailable.
Comparison of Induction versus no-Induction cohorts
Female (%) Caucasian (%) Negative Crossmatch (%) High Degree of HLA match (%) Mechanical Ventilation (%) ECMO support (%) Inotropes (%) Age (yr, ±SD) Donor-Recipient Weight Ratio (±SD) Ischemic Time (hr, ±SD) PRA (%, ±SD) Clearance (mL/min/1.73m2, ±SD)
No Induction (n= 1396)
Induction (n= 1396)
43.6 81.8 84.9 14.5 16.1 4.1 50.4 6.6 ± 6.3 1.4 ± 0.5 3.7 ± 1.3 9.8 ± 23.4 123.1 ± 58.9
44.9 81.6 84.7 14.3 15.8 4.5 50.1 6.6 ± 6.3 1.4 ± 0.5 3.7 ± 1.2 10.0 ± 23.4 123.5 ± 58.5
9( 04) New Onset Diabetes Mellitus After Heart Transplant in Children S. Sehgal ,1 M.J. Bock,1 H.L. Palac,2 J.G. Gossett,1 B.S. Marino,1 C.L. Backer,1 E. Pahl.1 1Ann and Robert H. Lurie Children’s Hospital of Chicago, Chicago, IL; 2Department of Preventative Medicine, Northwestern University, Chicago, IL. Purpose: Diabetes mellitus is a recognized complication of solid organ transplantation in adults and is associated with decreased graft and patient survival. However, little is known about the incidence and risk factors of new onset diabetes mellitus (NODM) in pediatric heart transplant recipients. We aimed to characterize the incidence and describe risk factors for development of new onset diabetes mellitus after heart transplant in children. We investigated the outcomes of patients with NODM compared to those without. Methods: All children younger than 18 yrs of age who developed diabetes after heart transplant were identified from the Organ Procurement and Transplantation network (OPTN) database (1987-2014). Patients with preexisting diabetes and/or re-transplantation were excluded. Baseline demographic, clinical data (BMI, medical care utilization and immunosuppressive strategy) as well as follow up data [treated episodes of rejection, coronary allograft vasculopathy (CAV), renal dysfunction] were compared between patients with and without diabetes. Wilcoxon rank-sum was used for continuous variables and chi-square test for categorical variables. Multiple logistic regression was used to control for covariates. Results: There was a 8% incidence of NODM (443/5824); 77% were insulin dependent. Those who developed NODM were older at the time of transplant (median age 12 vs. 4 yr, p< 0.0001), had higher BMI (20 vs. 17, p< 0.001), and were sicker pre-transplant (more likely to have dialysis, ECMO or VAD placed), p< 0.01. The incidence of NODM was significantly higher in the recent era (2001-2014) compared to older era (1987-2000) (55 vs. 45%, p< 0.001). Patients with NODM had higher number of treated episodes of rejection (0.9 vs. 0.3, p< 0.001). After transplant, patients with NODM were twice as likely to develop CAV (OR 2.2, 95% CI: 1.8-2.7) and 3x as likely to develop renal dysfunction requiring dialysis (OR 3.0, 95% CI: 2.4-3.7) even after controlling for age and time since transplant (p< 0.0001). Conclusion: NODM is a serious comorbidity after pediatric heart transplant with higher incidence in the recent era. Risk factors for NODM include older recipient age, higher BMI, sicker recipients and higher number of treated rejection episodes. NODM is associated with increased risk of CAV and renal failure, thus aggressive treatment for tight glycemic control may be warranted in this population. 9( 05) Two Viable Surgical Options for Varying Degree of Pulmonary Vein Stenosis in Pediatric Heart Transplant Recipients E. Jean-St-Michel , O. Honjo, C. Manlhiot, A. Dipchand. Hospital for Sick Children, Toronto, ON, Canada. Purpose: Pulmonary vein (PV) stenosis is usually progressive and despite the current surgical technique, “sutureless repair”, it still has a high mortality rate and remains a relative contraindication for heart transplantation (HTx).
S326
The Journal of Heart and Lung Transplantation, Vol 34, No 4S, April 2015
We sought to evaluate the outcomes of pediatric patients diagnosed with PV stenosis before HTx with and without PV repair at the time of HTx. Methods: We performed a retrospective review of all 288 HTx performed between 1993 and 2013 at a single institution. Results: Overall, 12 (4%) patients had PV stenosis and all had complex congenital heart disease (AVSD (5), tricuspid atresia (1), Ebstein’s (1), hypoplastic left heart variant (3), CCTGA (1), critical AS (1)). Five of the patients had PV atresia (2 left PV atresia, 2 right PV atresia and 1 left lower PV atresia), therefore HTx into a single lung was purposely done. One patient with right PV atresia also needed left PVs repair at time of HTx. The other 7 patients all had “sutureless repair” at the time of HTx (2 bilateral PV repairs, 1 left and right upper PV repair, 4 left PVs repairs). The median ICU and hospital stay were 12 (IQR 6-14) and 25 (IQR 19-29) days. The mean ischemic and bypass times were 268±70.0 min and 152±53.2 min. The median intubation duration was 4 (IQR 3-12) days; only 2 patients required nitric oxide post transplant. Two patients required long-term supplemental oxygen, 3 had recurrent pneumonias and 2 had severe pulmonary hypertension. For the 5 patients with PV atresia, 2 (40%) are alive (4 and 12 years post-transplant); 2 (40%) died of pulmonary hypertension at 4 and 6 months post-transplant and 1 (20%) died of rejection. For the 7 patients with PV stenosis, there was only 1 death (14%) from graft failure (2 year post HTx); 1 patient (14%) had recurrence of severe left PV stenosis with flow to the left lung of 0.1ml/min/m2 but no sign of pulmonary hypertension. Death secondary to pulmonary hypertension was only associated with PV atresia and both patients had developed stenosis on the contralateral side. Those 2 patients were also transplanted younger than the other PV atresia patients (1 yr old vs. 4, 5 and 6 yrs of age). Conclusion: Heart transplant is a viable option for patients with PV stenosis or atresia. Death secondary to pulmonary hypertension, even in the setting of virtually no flow to one lung, was seen only in patients with PV atresia who developed stenosis on the contralateral side. 9( 06) Cirrhosis in Patients Following the Fontan Operation: Incidence and Long-Term Outcomes K. Pundi ,1 K.N. Pundi,2 J.N. Johnson,2 Z. Li,3 D.J. Driscoll,2 F. Cetta.2 1Mayo Clinic College of Medicine, Mayo Clinic, Rochester, MN; 2Department of Pediatrics, Division of Pediatric Cardiology, Mayo Clinic, Rochester, MN; 3Biomedical Statistics and Informatics, Mayo Clinic, Rochester, MN. Purpose: Hepatic complications including cirrhosis are increasingly recognized after the Fontan operation. We examined the incidence and predictors of cirrhosis after Fontan operation. Methods: We reviewed records of all patients who had a Fontan operation from 1973-2012 at our institution (n = 1052). 449 patients had available post-operative liver data. Patients were classified as having cirrhosis only if they had a diagnostic liver biopsy or characteristic findings on magnetic resonance imaging (MRI) or magnetic resonance elastography (MRE). Patients with isolated blood liver function tests or ultrasound abnormalities were not considered to have proven cirrhosis. Results: The overall 10, 20, and 30-year freedom from cirrhosis was 99%, 96%, and 76%, respectively. Among the 25 patients with cirrhosis, the mean age at Fontan operation was 11.4 ± 9.7 years. The average duration from the Fontan operation to diagnosis of cirrhosis was 19.5 ± 7.8 years. Of these 25 patients, 13 had an atriopulmonary connection, 6 had a lateral tunnel, 2 had an extracardiac conduit, and 4 had other Fontan connections. Only 2 patients had a fenestration. Atrial or ventricular arrhythmias were present in 20/25 patients (80%), diagnosed a mean of 9.7 ± 5.0 years prior to cirrhosis. MRE-derived mean liver stiffness data was available on 8/25 patients with an average of 6.0 ± 1.8 kPa (> 5 kPa interpreted as stage 4 fibrosis or cirrhosis). Using multivariate analysis, a diagnosis of asplenia and preop digoxin use were the only factors associated with the development of cirrhosis. Survival following the diagnosis of cirrhosis was 71%, 25% and 17% at 1, 5 and 10 years respectively. The cause of death was known for 4 patients (3 multiorgan failure, 1 metastatic hepatocellular carcinoma). Conclusion: Patients with asplenia are at the highest risk of developing cirrhosis during follow-up, though some heterotaxy patients may have liver disease prior to Fontan. Long-term outcome after diagnosis of cirrhosis is poor, warranting noninvasive hepatic screening strategies in patients after Fontan. Ultimately our study may be underestimating the burden of liver
disease in these patients. Therapy is difficult and novel multiorgan transplant strategies may benefit this patient population. 9( 07) How Should the Effect of Persantine Be Measured Using Thromboelastography: Correlation and Agreement Between Percent ADP Inhibition and ADP Net G M. Massicotte ,1 J. Conway,2 L. May,3 H. Buchholz,4 C. Lo,5 A. Bruce,6 T. Tesoro,7 D. Rosenthal,3 C. Almond.8 1Thrombosis KIDCLOT, University of Alberta/Stollery Childrens Hospital, Edmonton, AB, Canada; 2Cardiology, University of Alberta/Stollery Childrens Hospital, Edmonton, AB, Canada; 3Cardiology, Stanford University, Stanford, CA; 4Cardiac Surgery, University of Alberta/Stollery Childrens Hospital, Edmonton, AB, Canada; 5Hematology, Stanford University, Stanford, CA; 6Hematology, University of Alberta/Stollery Childrens Hospital, Edmonton, AB, Canada; 7Pharmacy, Stanford University, Stanford, CA; 8Cardiology, Stanford University, Stanford, CA, Canada. Purpose: Controversy exists as to whether percent ADP (%ADP) inhibition or ADP Net G should be used as the primary laboratory target to adjust the dose of platelet inhibitors such as dipyridamole in children supported with Pediatric ventricular assist devices (VAD). The purpose of this study was to examine the agreement between these two values derived from Thromboelastography with Platelet Mapping® (TEG/PM) with respect to dose-adjustment recommendations. Methods: All pediatric patients supported with a pediatric VAD between 2006 and 2014 with TEG/PM data available at two pediatric VAD centers were identified using their respective hospital databases and merged under an IRB approved protocol. For each specimen, %ADP inhibition result (target 70-95%) was compared to ‘ADP NET G’ value (target 4-8), defined as the product of the baseline G (citrated kaolin with heparinase) and (100%-%ADP inhibition)/100. Agreement was evaluated using the Kappa statistic. Results: Overall there were a total of 665 TEG/PM specimens performed in 51 children across both centers. The median age of patients was 11 months (IQR 6 mo, 5 years), median weight 8.9 kg (IQR 6.2, 17.7); 29% had congenital heart disease (CHD) including 8% with single-ventricle disease, 49% received a temporary circulatory support device pre-implant, and 65% received an LVAD. Overall the mean %ADP inhibition was 49%±35% (SD) and mean ADP Net G 5±3.9 with a Pearson’s correlation of 0.78 for simultaneous values. Using %ADP inhibition of 70-95 as the target, 15.3% were classified as in-range, 66% sub-therapeutic and 18% supra-therapeutic; using ADP Net G of 4-8 as the target, 43.5% of values were classified as in-range, 41% sub-therapeutic and 14% supra-therapeutic. Correlation between the two classification systems was good (R 0.71, P< 0.001); agreement on dosing recommendation was poor (Kappa 0.39, P< 0.001) related in large part to the NET G value classifying significantly more patients as in-range. Conclusion: Overall, %ADP inhibition and ADP Net G values correlate well however there is poor agreement on dosing recommendations when an ADP Net G target of 4-8 is compared to a %ADP inhibition of 70-95%. Further research is needed to determine the optimal ADP target for pediatric VAD patients. 9( 08) Immune Profiling Pre/Post Berlin VAD Implant and Pre/Post Transplantation of Pediatric Heart Failure Patients R.H. Kerman ,1 P. Jindra,2 A. Jeewa,3 S. Burki,3 C. Fraser,3 I. Adachi.3 1Baylor College of Medicine, Houston, TX; 2Surgery, Baylor College of Medicine, Houston, TX; 3Texas Children’s Hospital and Baylor College of Medicine, Houston, TX. Purpose: The Berlin Heart Excor Ventricular Assist Device (Berlin VAD) was designed for use in children with heart failure and has allowed for successful bridging to heart transplantation (HTx). Blood products and VAD components exposure during VAD implant, may result in the production of antiHLA antibodies (Abs) which are a risk factor for rejection in HTx patients. Methods: We immunologically profiled 35 pediatric Berlin VAD patients (19 males, 16 females with a mean age of 6 ± 6 years of age) between 1999-2012 pre/post VAD implant and pre/post HTx. Immune profiling consisted of determining Flow panel reactive antibodies (PRAs), presence of donor specific antibody (DSA) and antibody fixation to complement (C1q assay). Assays were perfomed using One Lambda, Inc. reagents according to manufacturer’s instructions. Patients were excluded when immune data was unavailable.