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Heart failure is implicated in nearly 300,000 deaths annually, and is the leading cause of death or disability in adults in the United States [1,2]. Patients with heart failure often demonstrate mechanical dyssynchrony, characterized by dyskinetic ventricular contraction, regional myocardial hypokinesis, and perturbations in the natural ventricular geometry. Mechanical dyssynchrony may be associated with electrical dyssynchrony, an abnormal ventricular depolarization pattern, which is often accompanied by a bundle branch block on ECG. Such alterations in the normal contraction pattern of the left ventricle can lead to poor hemodynamics, altered ventricular function, and even changes in myocyte structure. Cardiac resynchronization therapy, which involves the individual pacing of the right atrium as well as both ventricles to provide a more physiologic atrio-ventricular (AV) delay and a more synchronous contraction of the left and right ventricles, re-establishes a more normal contraction pattern and has been shown to be an effective treatment for heart failure due to left ventricular dysfunction in adults. In children, the role of cardiac resynchronization therapy is less well understood. Given the diverse etiologies of dilated cardiomyopathy in children as well as the individual anatomic and physiologic considerations of patients with congenital heart disease, re-establishing physiologic synchrony and improving ventricular function are considerable challenges in pediatric cardiology. This manuscript describes the more recent exploration of cardiac resynchronization therapy in the pediatric population, with emphasis on its emerging use in patients with heart failure associated with congenital heart disease, including the situation of the single ventricle.

BACKGROUND Malnutrition is common in pediatric heart failure and is associated with mortality. The effect of VAD support on malnutrition in children is unknown. We sought to compare the prevalence and severity of malnutrition at HT in children on VAD support vs OMT to inform decisions regarding support strategies. METHODS Retrospective chart review involving all patients

Danon disease (DD) is an X-linked dominant disorder caused by a mutation in the lysosomal-associated membrane protein-2 (LAMP-2) gene coding for the LAMP-2 protein. We report two cases of successful heart transplantation (HT) in adolescent brothers with DD, including one who was bridged to HT for 34 days with a HeartWare left ventricular assist device. In both patients, the post-transplant course was complicated by profound skeletal muscle weakness that resolved with corticosteroid withdrawal. These cases highlight that both HT and ventricular assist device support are feasible in patients with DD. Corticosteroid use may exacerbate skeletal myopathy, and therefore, steroid minimization may be warranted whenever possible.

Per protocol, our institution obtains echocardiograms immediately after each EMB to rule out procedural complication. We sought to determine the incidence of echocardiogram-detected cardiac perforation and TV injury and to evaluate the utility of routine screening echocardiogram after each EMB in the current era. At a single center, 99% (1917/1942) EMB performed in 162 patients were immediately followed by an echocardiogram per protocol. There were five newly diagnosed pericardial effusions, and only one required pericardiocentesis. In the three echocardiograms demonstrating new flail TV, only one patient underwent surgical repair 2 months later. This study demonstrates the very low incidence of significant hemopericardium and TV injury after EMB in pediatric heart transplant recipients and argues against the utility of post-EMB echocardiograms to screen solely for procedural complications.

IntroductionChildren with congenital heart disease now survive well into adolescence and adulthood. As palliative procedures reach the end of their lifespan, chronic conditions related to heart failure such as cardiac cirrhosis are becoming more prevalent. Combined heart-liver transplantation is an

Biopsy-diagnosed pAMR has been observed in over half of pediatric HT recipients within 6 years of transplantation. We report the incidence and outcomes of pAMR at our center. All endomyocardial biopsies for all HT recipients transplanted between 2010 and 2015 were reviewed and classified using contemporary ISHLT guidelines. Graft dysfunction was defined as a qualitative decrement in systolic function by echocardiogram or an increase of ≥3 mm Hg in atrial filling pressure by direct measurement. Among 96 patients, pAMR2 occurred in 7 (7%) over a median follow-up period of 3.1 years, while no cases of pAMR3 occurred. A history of CHD, DSA at transplant, and elevated filling pressures were associated with pAMR2. Five-sixths (83%) of patients developed new C1q+ DSA at the time of pAMR diagnosis. There was a trend toward reduced survival, with 43% of patients dying within 2.3 years of pAMR diagnosis.

Pump exchanges are frequently required in the Berlin Heart EXCOR VAD. We intended to describe the characteristics of pump deposits in a larger patient series and evaluate if changes in our exchange procedure over time have led to increased complications. We reviewed all EXCOR pump exchanges in our institution from July 2004 to October 2014. We gathered data on size and location of pump deposits and exchange procedures. EXCOR devices were implanted in 38 children. Support was LVAD only in 22, BiVAD in 13, and SVAD in 3 cases. Sixty-seven pumps were exchanged. The incidence of pump exchanges per month was higher for smaller pumps and for RVADs vs LVADs. Indications were visible pump deposit in 55, stroke without visible deposit in 5, incorporation of membrane oxygenator in 3, pump size change in 2, and sepsis in 1 case, respectively. Deposits were located in the outflow valve in 73%, inflow valve in 22%, pump body in 3%, and outflow cannula in 3%. EXCOR pumps are predominantly exchanged for deposits, which are most frequently located in the outflow valves. The procedure is now carried out without sedation at the bedside. No major complications were observed during exchanges.

Outpatient experience of children supported with continuous-flow ventricular assist devices (CF-VAD) is limited. We reviewed our experience with children discharged with CF-VAD support. All pediatric patients

ObjectivesNeonates undergoing cardiac surgery are at high risk for adverse outcomes. B-type natriuretic peptide is used as a biomarker in patients with cardiac disease, but the predictive value of B-type natriuretic peptide after cardiac surgery in neonates has not been evaluated. Therefore, the objective of this study was to determine the predictive value of perioperative B-type natriuretic peptide levels for postoperative outcomes in neonates undergoing cardiac surgery.MethodsPlasma B-type natriuretic peptide determinations were made before and 2, 12, and 24 hours after surgery in 36 consecutive neonates. B-type natriuretic peptide levels and changes in perioperative B-type natriuretic peptide were evaluated as predictors of postoperative outcome.ResultsB-type natriuretic peptide levels at 24 hours were lower than preoperative levels (24-h/pre B-type natriuretic peptide ratio < 1) in 29 patients (81%) and higher (24-h/pre B-type natriuretic peptide ratio ≥ 1) in 7 patients (19%). A 24-hour/pre B-type natriuretic peptide level of 1 or greater was associated with an increased incidence of low cardiac output syndrome (100% vs 34%, P = .002) and fewer ventilator-free days (17 ± 13 days vs 26 ± 3 days, P = .002), and predicted the 6-month composite end point of death, an unplanned cardiac operation, or cardiac transplant (57% vs 3%, P = .003). A 24-hour/pre B-type natriuretic peptide level of 1 or greater had a sensitivity of 80% and a specificity of 90% for predicting a poor postoperative outcome (P = .003).ConclusionIn neonates undergoing cardiac surgery, an increase in B-type natriuretic peptide 24 hours after surgery predicts poor postoperative outcome.

Background Life-threatening arrhythmia events (LTEs) occur in ~5% of children with dilated cardiomyopathy (DCM). While prolonged QRS duration has been shown to be associated with LTEs, electrocardiographic (ECG) repolarization findings have not been examined. Objective We sought to determine the associations between ECG repolarization abnormalities and LTEs in children with DCM. Methods A single-center retrospective review of children with DCM was performed. LTEs were defined as documented ventricular tachycardia or fibrillation requiring medical intervention. Three pediatric cardiologists, blinded to clinical events, evaluated ECGs obtained at the time of initial referral. Kaplan-Meier survival and Cox proportional hazards analyses were used to evaluate time to LTEs. Results A total of 137 patients (mean age 7.8 ± 6.7 years; 75(55%) male patients) with DCM (mean ejection fraction 35% ± 16%) were included; 67 patients (49%) had a corrected JT (JTc) interval of ≥340 ms, 72 (53%) had a corrected QT (QTc) interval of ≥450 ms, and 41 (30%) had abnormal T waves. LTEs occurred in 15 patients at a median of 12 months (interquartile range 3–36 months) after the initial ECG. Patients with LTEs had a longer JTc interval (371 ± 77 ms vs 342 ± 41 ms; P = .02) and a longer QTc interval (488 ± 96 ms vs 453 ± 44 ms; P = .01). In survival analysis, a JTc interval of ≥390 ms (hazard ratio [HR] 4.07; 95% confidence interval [CI] 1.12–14.83; P = .03), a QTc interval of ≥510 ms (HR 6.95; 95% CI 1.53–31.49; P = .01), abnormal T-wave inversion (HR 11.62; 95% CI 2.75–49.00; P = .001), and ST-segment depression (HR 6.91; 95% CI 1.25–38.27; P = .03) were associated with an increased risk of LTEs, even after adjusting for QRS duration and amiodarone use. Conclusion Repolarization abnormalities are common in children with DCM. Certain ECG repolarization abnormalities, such as significantly prolonged JTc and QTc intervals, may be useful in identifying patients at risk of LTEs.

Although ventricular assist devices (VADs) restore hemodynamics in those with heart failure, reversibility of end-organ dysfunction with VAD support is not well characterized. Renal function often improves in adults after VAD placement, but this has not been comprehensively explored in children.Sixty-three children on VAD support were studied. Acute kidney injury (AKI) was defined by Kidney Disease: Improving Global Outcomes criteria. Estimated glomerular filtration rate (eGFR) was determined by the Schwartz method. Generalized linear mixed-effects models compared the pre-VAD and post-VAD eGFR for the cohort and sub-groups with and without pre-VAD renal dysfunction (pre-VAD eGFR90 ml/min/1.73 m(2)).The pre-VAD eGFR across the cohort was 84.0 ml/min/1.73 m(2) (interquartile range [IQR] 62.3-122.7), and 55.6% (34 of 63) had pre-VAD renal dysfunction. AKI affected 60.3% (38 of 63), with similar rates in those with and without pre-existing renal dysfunction. Within the cohort, the nadir eGFR occurred 1 day post-operatively (62.9 ml/min/1.73 m(2); IQR, 51.2-88.9 ml/min/1.73 m(2); p0.001). By Day 5, however, the eGFR exceeded the baseline (99.0 ml/min/1.73 m(2); IQR, 59.3-146.7 ml/min/1.73 m(2); p = 0.03) and remained significantly higher through the first post-operative week. After adjusting for age, gender, and AKI, the eGFR continued to increase throughout the entire 180-day study period (β = 0.0025; 95% confidence interval, 0.0015-0.0036; p0.001). Patients with pre-VAD renal dysfunction experienced the greatest improvement in the eGFR (β = 0.0051 vs β = 0.0013, p0.001).Renal dysfunction is prevalent in children with heart failure undergoing VAD placement. Although peri-operative AKI is common, renal function improves substantially in the first post-operative week and for months thereafter. This is particularly pronounced in those with pre-VAD renal impairment, suggesting that VADs may facilitate recovery and maintenance of kidney function in children with advanced heart failure.

Objective We hypothesized that isolated gastrointestinal complaints (abdominal pain, nausea, anorexia, weight loss), in the absence of other symptoms, were a common mode of initial presentation in children with congestive heart failure (CHF). Study design Ninety-eight patients younger than 18 years hospitalized with dilated cardiomyopathy at a single institution between January 1, 2000, and December 31, 2009, were included. Retrospective review of their presenting complaints was recorded and analyzed according to 3 age groups: 0 to 1 year (infants), 1 to 10 years (children), and 11 to 18 years (adolescents) of age. Results Respiratory symptoms were common in all age groups (range, 56%-63%). Gastrointestinal complaints were also common in all age groups (42%, 28%, and 65%, respectively) and were more frequent than respiratory complaints in adolescents. Adolescents were likely to present with abdominal pain as their only complaint (10/43, 23%). Chest pain, syncope, or cardiac arrest occurred rarely. Conclusions Abdominal complaints are a common component of the presenting symptom complex of CHF in pediatric dilated cardiomyopathy in all age groups. In adolescents, abdominal complaints occur more frequently than respiratory complaints and often in the absence of any other symptoms. Unlike CHF in adults, chest pain, arrhythmia, or cardiac arrest occurs rarely at presentation in pediatric patients. Recognition of the different presenting symptoms of heart failure in children by primary providers is crucial to ensuring prompt diagnosis and timely initiation of therapy.

The proteasome inhibitor bortezomib has been used with variable success in the treatment of AMR following heart transplant. There is limited experience with this agent as a pretransplant desensitizing therapy. We report a case of successful HLA desensitization with a bortezomib-based protocol prior to successful heart transplantation. A nine-yr-old boy with dilated cardiomyopathy, not initially sensitized to HLA (cPRA of zero), required three days of ECMO, followed by implantation of a Heartmate II LVAD. Within six wk, the patient developed de novo class I IgG and C1q complement-fixing HLA antibodies with a cPRA of 100%. Two doses of IVIG (2 g/kg) failed to reduce antibody levels, although two courses of a novel desensitization protocol consisting of rituximab (375 mg/m(2) ), bortezomib (1.3 mg/m(2) × 5 doses), and plasmapheresis reduced his cPRA to 0% and 87% by the C1q and IgG assays, respectively. He underwent heart transplantation nearly two months later. The patient is now >one yr post-transplant, is free of both AMR and ACR, and has no detectable donor-specific antibodies by IgG or C1q. Proteasome inhibition with bortezomib and plasmapheresis may be an effective therapy for HLA desensitization pretransplant.

Echocardiogram reports, but not images, are usually available for the evaluation of potential donor hearts. To assess the reliability of local reports of potential pediatric heart donors, we compared echocardiographic measurements of left ventricular (LV) systolic function between local hospitals and a central echocardiography laboratory.We identified all potential donors aged18 years managed by the California Transplant Donor Network from 2009 to 2013. Echocardiograms and reports were obtained from local hospitals. All studies were reviewed in a central laboratory by an experienced pediatric cardiologist blinded to local reports. Local and central measurements of fractional shortening (FS) were compared using the Bland-Altman method (mean difference ± 2 standard deviations). LV function was categorized based on FS as normal or mild, moderately, or severely depressed.There were 70 studies from 59 donors with local and central measurements of FS. The mean difference between local and central FS was 3.9 ± 9.0. The limits of agreement ranged from -14.2 to 22. Twenty-five studies had discordant measurements of LV function, with 17 discordant by 1 category and 8 by 2 or more categories. Of 55 studies categorized as normal by local measurement, 6 were moderately to severely depressed by central review. Of 15 studies categorized as depressed by local measurement, 3 were normal by central review.Local and central measurements of LV systolic function were discordant in 36% of studies. Given such discordance, efforts to obtain and view actual echocardiographic images should be part of the standard evaluation of potential pediatric heart donors.

Because children with heart failure live longer both before and after cardiac transplantation, there is renewed focus on the quality and preservation of their intellectual functioning and psychosocial health. Children with chronic heart failure are at risk for delays in both cognitive development and psychologic functioning, though the extent and permanence of impairment is not well understood. Children with medically managed heart failure have been shown to be at increased risk for anxiety and depression, with a prevalence of emotional disorders similar to that of other children with congenital heart disease. The use of ventricular assist devices as a bridge to transplantation offers both risks and benefits for the preservation of intellectual and emotional function, with an increased risk for ischemic injury to the brain, but offers the advantage of allowing for cognitive stimulation and improved social interactions. A new population of children with heart failure, those outfitted with permanent ventricular assist devices in lieu of cardiac transplantation, may represent a particular risk group regarding social and cognitive function, but as of yet this is not well studied. Early intervention and school accommodations are recommended for those with cognitive, social, or emotional deficits, and brain imaging should be considered for those with persistent difficulties. Whenever possible, patients should be referred to psychologists and developmental specialists with experience in treating this patient population.

Behcet's disease is a rare autoimmune disease characterized by oral and genital ulcers, and by multisystem disease, including arthritis, neurologic complications and vasculitis. Large-vessel and coronary artery aneurysms are often an indication for surgery, but the return of aneurysms, thrombosis, and the tendency to exhibit an exaggerated inflammatory response at puncture sites (pathergy) complicate surgical recovery. As such, cardiac transplantation, which requires atrial and large-vessel anastomoses, has not been reported in patients with Behcet's disease. We report the first orthotopic heart transplant with >1-year survival in a patient with Behcet's disease despite major complications. The investigators remain pessimistic about cardiac transplantation in patients with Behcet's disease until advances in preventing recurrent vascular pathology ensue.

HC is a rare cause of congestive heart failure that typically presents with malignant ventricular arrhythmias in infants, often requiring urgent intervention. Successful heart transplantation in a patient with HC has only been reported once (J Heart Lung Transplant 2004: 23: 902). The combination of HC with concurrent LVNC has only been described three times (Int J Legal Med 2009: 123: 47; Hum Pathol 2005: 36: 403; Pediatr Dev Pathol 2012: 15: 397). We report two rare cases of HC with LVNC in two infants presenting with cardiogenic shock, one requiring ECMO support who was successfully bridged to orthotopic heart transplantation with a Berlin Heart LVAD.

The incidence of sudden death in children with dilated cardiomyopathy has been estimated at1% annually. This number, however, may underestimate the incidence of life-threatening arrhythmias. The objective of this study was to assess the incidence of and identify risk factors for life-threatening arrhythmias in children with dilated cardiomyopathy.We conducted a retrospective record review of 183 children with dilated cardiomyopathy treated at a single center between 2000 and 2011. Life-threatening arrhythmia was defined as any ventricular arrhythmia that resulted in syncope or hypotension and required medical intervention. Risk factors for life-threatening arrhythmias were identified with univariate analyses. A prediction model was constructed with multivariate logistic regression and receiver operating characteristic curves.Nineteen patients experienced life-threatening arrhythmias, representing an annualized rate of 4.9%. Outpatient life-threatening arrhythmias occurred at a rate of 2.2% per year. Predictors of outpatient life-threatening arrhythmias were longer QRS duration (p = 0.003) and increased left ventricular posterior wall (LVPWd) thickness (p = 0.03). Only QRS duration remained significant in multivariate logistic regression (odds ratio, 1.8 for every unit increase in z-score; 95% CI, 1.01-1.9; p = 0.04). For all life-threatening arrhythmias, prolonged QRS duration was the only significant risk factor in multivariate logistic regression (odds ratio, 1.5; 95% CI, 1.1-2.2; p = 0.02).In children with dilated cardiomyopathy, as QRS duration increases, so too does the risk of life-threatening arrhythmias. Life-threatening arrhythmias occurred at an annual rate of 5%, which was much higher than the previously reported rate of sudden cardiac death in this population.

Background— We hypothesized that children with dilated cardiomyopathy who require hospital admission are at increased risk for death or transplantation during their first hospitalization and in the first year that follows. We also assessed the value of routine data collected during that time to predict death or the need for transplantation prior to discharge and within 1 year of admission. Methods and Results— We conducted a retrospective review of 83 pediatric patients with dilated cardiomyopathy whose initial hospitalization fell between 2004 and 2009. The mean age at hospitalization was 7 years. The majority of patients demonstrated moderate or severe left ventricular dysfunction on initial echocardiogram (80%) and/or the need for intravenous inotropes within 7 days of hospital admission (69%). Five patients (6%) died, and 15 (18%) were transplanted in the initial hospitalization. At 1 year, 11/71 (15%) had died, and 27/71 (38%) were transplanted. The overall freedom from death, transplantation, or rehospitalization at 1 year following admission was 21%. Fractional shortening, left ventricular ejection fraction, serum cholesterol, uric acid, mixed venous saturation, and atrial filling pressures were all predictive of death or transplantation during the initial hospitalization. Left ventricular ejection fraction was predictive of death or transplantation at 1 year. Conclusions— The first hospitalization for dilated cardiomyopathy marks a period of high risk for clinical decline, end stage heart failure, and the need for cardiac transplantation. Echocardiographic function and hemodynamic and serum measurements may aid in predicting outcomes. Despite medical management, most patients will be rehospitalized and/or require cardiac transplantation within 1 year of admission.

For patients with end-stage hepatic failure secondary to failing hemodynamics, combined heart-liver transplant (H-LT) remains the only option for long-term survival. We report a series of three pediatric patients who successfully underwent orthotopic H-LT for failed single-ventricle palliation. All three patients are currently living, now two, three, and five years post-transplant, and remain completely free of cardiac cellular allograft rejection despite reduced immunosuppression protocols. One patient, however, did develop acute antibody-mediated rejection in the immediate post-transplant period, suggesting that this protective effect may be less effective in attenuating humoral mechanisms of rejection.

s S161 Survival after lung transplantation did not differ significantly for patients with pPH, pcPH and Non-PH, and no difference in survival was observed between patients with IpcPH and CpcPH (log-rank P= 0.913). Conclusion: Postcapillary pulmonary hypertension occurred in 11.1% of patients, and was not verified as a risk factor for post-transplant survival whether of isolated or combined type. Patients with diastolic dysfunction and preserved systolic ejection fraction should not be excluded from lung transplantation.

Purpose Use of MCS in children has increased rapidly in the past decade. Initial reporting has focused on survival and major morbidities. We describe a novel scoring system to measure clinical status after implantation, which we apply to a test series of 25 children. Methods and Materials The scoring system was developed based upon clinical expertise and literature review to capture heart failure severity in a hospitalized cohort. The score is based upon multiple aspects of heart failure care. The components of the scoring system and the score ranges are shown in the table, with examples. Higher scores represent higher levels of care. Final scores are normalized to 100 points. We applied the score to a retrospective test set of 25 children with MCS. Results We evaluated 24 children with 25 MCS implantations as a bridge to transplant between 2009 and 2011. There were 14 males. Mean (SD) age was 6.7 (6.2) yrs. Support duration was 59 (49) days. Outcomes included: transplant (58%), death (29%), and explantation (13%). Devices include EXCOR (12), Thoratec PVAD (9), and other (4). Support was LVAD (17), RVAD (1), and BIVAD (7). Scores at day -3 (pre-implant), and days 7, 21 and 60 are shown in the figure. [ figure 1 ] Conclusions The Stanford VAD score summarizes overall patient recovery after MCS implantation in children. It can be readily obtained from chart review. Validation with other clinical data is needed. Components of Stanford VAD Score Category Point Range Sample Item Nutrition 0–4 Effective enteral support: 1 pt Respiratory Support 0–3 Mechanical ventilation: 3 pts Physical Activity (if > 5 yrs) 0–4 Bed rest: 4 pts Heart Failure Medications 0–7 IV vasoconstrictors: 2 pts Care Setting 0–3 ICU care: 3 pts Total 0–21 (0–17 if

Purpose With recent advances in pediatric ventricular assist devices (VADs), patients are able to be discharged from the hospital. However, the course of outpatient care following outpatient VAD placement is not well described. Methods and Materials We retrospectively reviewed all patients with the Thoratec Heartmate II LVAS (HMII) at our institution between June 2010 and November 2012. Results Seven patients received HMII; patient age ranged from 10 to 18 (mean, 15). All patients had dilated cardiomyopathy; three had an underlying diagnosis of Becker’s muscular dystrophy and one with congenital heart disease. Two patients were transplanted prior to discharge and one patient is currently still hospitalized. Four patients were successfully discharged to the outpatient transition home 1.3 miles away. Average length of stay was 38 days with clinic visits every five days. Once discharged home, patients were seen every one to three months depending on clinical stability. The distance from the implanting center to each patient’s home ranges from 14 to 231 miles away. These four patients have been supported for 87, 583, 169, and 75 outpatient days respectively. There were a total of six re-hospitalizations for rule out infection, rule out hemolysis, post cardiac catherization recovery, knee effusion and rule out hemearthrosis, bilateral pleural effusions, and decompensated cardiogenic shock and multi-system organ failure. Thus far, three patients continue to be supported on outpatient VADs and one is deceased. Conclusions Pediatric VADs can be successfully transitioned to outpatient care even despite distance from implanting center, however, close follow-up and a multidisciplanry highly coordinated approach are absolute requirements.

Purpose Riding the successful curtails of adult ventricular assist device (VAD) designs, we are now able to discharge pediatric VADs into the community. An important aspect of quality of life for these patients is school re-integration. We sought to review the school re-integration process in this population. Methods and Materials We retrospectively reviewed all the patients at our institution from June 2010 to November 2012 with dischargeble VADs and their school attendance from inpatient to outpatient care. Results Seven patients received the Thoratec Heartmate II LVAS; patient age ranged from 10 to 18 (mean: 15). One patient was enrolled in college prior to admission, so did not participate in hospital school. Median length of inpatient stay was 66 days. For the remaining six patients, bedside instruction began on day 11 post implant and classroom instruction started on day 25. Two patients were transplanted prior to discharge and one patient is currently still hospitalized. Four patients were successfully discharged to the outpatient transition home closeby and concurrently attended hospital school. Upon transition home, one patient returned to his charter school and continues with hospital school as well, two patients returned to their public schools, and one patient received home schooling. One patient has been supported for 583 outpatient days. In this time period, he has only missed 29 school days for clinic visit and re-hospitalizations. Conclusions With proper support, education, and training, school re-integration for pediatric VADs is possible despite the complex care needs of these patients.

Purpose The institution of mechanical circulatory support is often complicated by incessant bleeding as a result of pre-existing coagulopathy or the use of anticoagulation. The use of pro-coagulant factors in these cases carries a high risk for thrombosis or pump malfunction. The pro-coagulant FEIBA has been used for refractory bleeding in cases of factor deficiency, however its use in pediatric mechanical support patients has not been reported. Methods and Materials Retrospective review of FEIBA use in our pediatric mechanical circulatory program between June 2010 and Nov 2012. Results Eleven patients (weight 2.4 to 72 kg) on MCS received low dose FEIBA for uncontrolled postoperative bleeding. Three patients were placed on Heartmate 2® left ventricular assist device, two patients on right ventricular assist device after heart transplant (Biomedicus ® and Revolution ® ), three patients on the Berlin Heart ® ventricular assist device, and three patients on extracorporeal membrane circulatory support after congenital heart surgery. Ages ranged from 7 days to 16 years. Six patients received a single low dose (approximately 10 units/kg), three patients received two low doses, and two patients received greater than two low doses. Blood product utilization and chest tube output were decreased significantly following administration of FEIBA in all patients, although one patient required chest re-exploration. There were no thrombotic events or device malfunctions. There were three deaths unrelated to FEIBA administration. Conclusions Low dose FEIBA administration is effective in controlling bleeding in pediatric MCS patients. In appropriate situations, it can be used safely with a low adverse event rate.