Children Undergoing Heart Transplantation Research Articles

Heart transplantation in single-ventricle physiology with failed fontan circulation

Methods Between 1986 and 2011, 179 children underwent heart transplantation for cardiomyopathy and end-stage congenital heart disease. Fifteen patients had single-ventricle physiology and underwent Fontan procedure. Five patients (mean age 6.6±1.2, range 2-14, years old) had failed Fontan circulation. Interval between Fontan procedure and failing circulation was 8-14 years. Indications for heart transplantation were ventricular failure (n=4), and protein-losing enteropathy (n=1).

Regional Variation in Survival Before and After Pediatric Heart Transplantation—An Analysis of The UNOS Database

Geographic variation occurs in a variety of health outcomes. Regional influences on outcomes before and after listing for pediatric heart transplantation have not been assessed. Review of the UNOS dataset identified 5398 pediatric (≤18 years) patients listed for heart transplantation 2000–2011. Patients were stratified based on the region of listing. Regional-level variables were correlated with individual risk-adjusted outcomes. Mean time spent on the waitlist varied from 91.0 ± 163 days (Region 6 [R6]) to 248.1 ± 493 days (R4, p < 0.0001). Regions with more transplant centers (p < 0.0001) and fewer transplants (p = 0.0015) had higher waitlist mortality. Risk-adjusted individual waitlist mortality varied from 6.9% (R1, CI 6.2–7.8) to 19.2% (R5, CI 18.0–20.6). Waitlist mortality was higher for individuals awaiting transplant in regions with more listings per center (OR 1.04, CI 1.01–1.08) and lower in regions with more donors per center (OR 0.95, CI 0.90–0.99 per donor). Posttransplant risk-adjusted survival varied across regions (R4: 5.4%, CI 4.2–7.4; R7: 18.0%, CI 12.4–32.5), but regional variables were not correlated with outcomes. Outcomes among children undergoing heart transplantation vary by region. Factors leading to increased competition for donor allografts are associated with poorer waitlist survival. Equitable allocation of cardiac allografts requires further investigation of these findings.

Pediatric Ventricular Assist Device Use as a Bridge to Transplantation Does Not Affect Long-Term Quality of Life

Purpose Fifteen-percent of the more than 500 children that receive a heart transplant annually require a ventricular assist device (VAD) as a bridge to transplantation. With recent data showing improved long-term survival after transplantation, it is appropriate to focus attention on whether children requiring a VAD as a bridge to transplantation fair as well as those who went straight to transplantation in terms of their long-term quality of life. Methods and Materials This study used a cross-sectional design to evaluate the long-term quality of life in children who required long-term VAD support (greater than two weeks) as a bridge to transplantation, compared to children undergoing heart transplantation without VAD support, employing the Core and Cardiac modules of the Pediatric Quality of Life Inventory (PedsQL) survey, via a telephone interview. In a secondary analysis, factors associated with worse quality of life outcome among VAD patients were also investigated. Results Among 21 children who required a ventricular assist device as a bridge to transplantation and 42 children who went straight to transplantation, there were no significant differences in quality of life as measured by their Psychosocial Health Summary Score, Physical Health Summary Score, or Total Score in the PedsQL Core module, nor were there any differences in any of the six dimensions of quality of life assessed by the PedsQL Cardiac module. Among patients who required a ventricular assist device, only the presence of a neurological complication was associated with worse quality of life, in the form of decreased Physical Health Summary and decreased Cardiac Communication scores. Conclusions Over the long-term, surviving children who required a long-term ventricular assist device as a bridge to transplantation experience a similar quality of life as those who went straight to transplantation.

Outcomes with High Pre-Transplant PRA in Pediatric Heart Transplantation at a Single Center

Purpose Acceptance and management of pediatric heart transplant (Tx) candidates with high pre-Tx PRA remains center dependent, largely due to lack of medium and longterm followup on outcomes. Some centers proceed only with a negative crossmatch, essentially guaranteeing no suitable donor in the worst cases. Other centers proceed across a positive crossmatch, feeling mortality is more likely waiting for a suitable donor, but with uncertain outcomes. We review outcomes after pediatric heart Tx with positive pre-Tx PRA (>10%) at a center that has not used prospective crossmatch, and more recently has chosen to transplant across a positive virtual crossmatch for those with markedly elevated PRA. Methods and Materials This is a retrospective chart review of all children undergoing heart transplantation at our center. Results We identified 19 recipients with pre-Tx PRA >10%: 11 class I, 2 class II, and 6 both class I and II. PRA class I mean was 43% (range 0-100) and class II mean was 27% (0-100). 11 of 18 tested had a positive retrospective crossmatch; 1 had no crossmatch. Only the 2 most recent recipients had specific intra-operative therapy to reduce antibody. Anti-thymocyte antibody induction is used. Post-Tx medications are often modified for better B cell coverage in this group. There were 5 deaths: rejection 2.3 y; rejection 3.2 y; PTLD 3.4 y; infection 7.0 y; and suicide 10.4 y. Both rejection deaths were linked to noncompliance. None of the high PRA recipients had documented coronary vasculopathy. Actuarial survival was 100% at 1 y; 79% at 5 y; 69% at 10y. 7 of 19 had rejection: 3 cellular, 2 AMR and 2 diagnosed clinically without biopsy. Time to first rejection was 1 y in 3. 3 recipients had >1 rejection episode. Positive crossmatch did not predict those likely to have rejection or death from rejection. Conclusions Reasonable outcomes in pediatric heart Tx recipients with high PRA, with or without a positive crossmatch, support offering Tx across a positive virtual crossmatch for those unlikely to otherwise undergo Tx.

PEDIATRIC VENTRICULAR ASSIST DEVICE USE AS A BRIDGE TO TRANSPLANTATION DOES NOT AFFECT LONG-TERM QUALITY OF LIFE

Objective The present study sought to determine the long-term quality of life (QOL) of children who required long-term ventricular assist device (VAD) support as a bridge to transplantation (BTT) compared with children who underwent heart transplantation without VAD support. Currently, 20% of children undergoing heart transplantation have required a VAD as a BTT. Few data have been published assessing how children requiring a VAD as a BTT will fair in terms of their long-term QOL. Methods The present study used a cross-sectional design, using the Core and Cardiac modules of the Pediatric Quality of Life Inventory survey. In a secondary analysis, the factors associated with worse QOL outcomes among the VAD patients were also investigated. Results At follow-up (median, 4.2 years), between the 21 children who required a VAD as a BTT and 42 who went straight to transplantation, no significant differences were found in the QOL as measured using the Psychosocial Health Summary Score, Physical Health Summary Score, or Total Score in the survey's Core Module, nor were any differences found in the outcomes assessed using the survey's Cardiac Module. Of the patients who required a VAD, only the presence of a neurologic complication was associated with worse QOL, which was demonstrated by decreased Physical Health Summary and Cardiac Communication scores. Conclusions Over the long term, surviving children who required a long-term VAD as a BTT experience a similar QOL as those who went straight to transplantation.

State of the art in paediatric heart transplantation: the Berlin experience

Enormous progress has been made in paediatric heart transplantation since the first unsuccessful effort by Kantrowitz in 1967. Early reports of children undergoing heart transplantation showed alarmingly high perioperative mortality rates of 25-60%, with the diagnosis of congenital heart disease (CHD) representing a particularly high-risk subset compared with cardiomyopathy. Many of these early failures were related to poor patient selection, suboptimal immunosuppression and technical problems. We learned a great deal from these earlier difficulties. Presently, with more refined techniques, better-defined patient selection criteria, excellent graft rejection monitoring and optimal immunosuppression, the ISHLT 2011 registry reported a 10-year survival rate of 60% for patients transplanted for end-stage CHD and >70% for those transplanted for cardiomyopathy. The technical dilemmas in complex CHD were overcome by surgical ingenuity and creativity, innovative solutions and careful surgical planning, adapting the complex recipient anatomy to the normal donor anatomy. The miniaturized Berlin Heart pulsatile ventricular assist devices in children as a bridge to transplantation have revolutionized treatment and become a significant contribution in heart-failure therapy. The intramyocardial electrogram and echocardiographic strain rate imaging have been employed as non-invasive techniques of rejection monitoring. Immunosuppressive drugs have a major impact on the development and progression of cardiac allograft vasculopathy, the main cause of cardiac allograft loss and a leading cause of mortality after the first year post-transplantation. The questions of whether a transplanted heart in a newborn grows to adult size along with the child and whether the dimensional cardiac growth allows adequate function over time have been largely answered in our previous investigations. As more transplanted children reach adulthood, concerns about their life expectancy when they have reached 10 years of life post-transplant are raised, particularly with respect to establishing partnerships and families, their ability to earn a living and the fulfilment of personal life perspectives. Some heart-transplanted patients require retransplantation to remain alive. The disparity between the demand for and supply of donor hearts makes retransplantation an ethical issue. We 'do not refuse' any patient who needs retransplantation. Mechanical circulatory support devices for long-term use are now largely available to accommodate such cases.

The Impact of Tacrolimus as Rescue Therapy in Children Using a Double Immunosuppressive Regimen After Heart Transplantation

Organ transplant recipients with refractory rejection or intolerance to the prescribed immunosuppressant may respond to rescue therapy with tacrolimus. We sought to evaluate the clinical outcomes of children undergoing heart transplantation who required conversion from a cyclosporine-based, steroid-free therapy to a tacrolimus-based regimen. We performed a prospective, observational, cohort study of 28 children who underwent conversion from cyclosporine-based, steroid-free therapy to a tacrolimus-based therapy for refractory or late rejection or intolerance to cyclosporine. There was complete resolution of refractory rejection episodes and adverse side effects in all patients. The incidence rate (×100) of rejection episodes before and after conversion was 7.98 and 2.11, respectively (P ≤ .0001). There was a 25% mortality rate in patients using tacrolimus after a mean period of 60 months after conversion. Tacrolimus is effective as rescue therapy for refractory rejection and is a therapeutic option for pediatric patients.

135 Neuro-Cognitive Performance of Children Undergoing Heart Transplantation in the US Analysis of Data from the Organ Procurement and Transplant Network

135 Neuro-Cognitive Performance of Children Undergoing Heart Transplantation in the US Analysis of Data from the Organ Procurement and Transplant Network

Perceived quality of life of children after successful bridging to heart transplantation

Mechanical circulatory support is increasingly used to bridge children with end-stage heart failure to transplant. Quality of life (QoL) has not been systematically evaluated in children bridged to heart transplant. All children transplanted for cardiomyopathy during 2001 to 2008 and currently being followed at our center (n = 84) had QoL assessed during 2006 to 2009, at a median of 3 years post-transplant, using a validated generic measure (PedsQL4.0). Twenty-six children, aged 2.7 to 18 (median 7.4) years who were bridged to transplant, were compared with 58 children, aged 2.0 to 18.0 (median 13.0) years, who were transplanted in the same era without bridging. There were no significant differences between the 2 groups on any domains of QoL assessed by children or parents, although the small number of bridged patients increases the likelihood of a Type II error. Bridged children who were younger (r = 0.48, p = 0.02) or more recently transplanted (r = 0.42, p = 0.04) were scored by their parents as having poorer emotional QoL. Regression analysis indicated that age at transplant was the only medical or demographic variable associated with parent-reported total QoL scores (β = 0.27, p = 0.01). With few links between QoL scores and medical or demographic factors, other subjective psychologic factors may be of greater salience in determining QoL. Despite greater severity of illness, children who required mechanical bridging to transplantation report a QoL comparable to that of other children undergoing heart transplantation. Younger children may require greater psychologic support to reach their full potential in terms of QoL.

The profile of inflammatory and metabolic response in children undergoing heart transplantation

Inflammatory and metabolic response is an important factor to determine clinical outcomes. However, it remains unknown in children undergoing heart transplantation (HTx). We examined the perioperative changes in the inflammatory and metabolic response markers C-reactive protein (CRP) and prealbumin (PA) in 38 heart-transplanted children. Data obtained prior to and within one month after HTx included CRP, PA, total and differential white blood cell counts, doses of inotropes and immunosuppressants, cultures of blood and body fluids, duration of cardiopulmonary bypass (CPB), aortic cross clamp and donor heart ischemia, and days in the intensive care unit (ICU) and hospital. CRP was 32±49 mg/L before HTx, increased to 130±55 mg/L on postoperative day 1-2, and decreased to 21±31 mg/L by one month after HTx. PA was 0.15±0.06 g/L before HTx, decreased to 0.12±0.03 g/L on postoperative day 1-2, and then gradually increased to 0.21±0.10 g/L by one month after HTx. Postoperative CRP positively correlated with epinephrine dosage and CPB duration. PA positively correlated with age. In conclusion, inflammatory and metabolic response is present before HTx and acutely intensified after HTx. It may be mainly influenced by CPB duration and cardiovascular function status.

Heart Transplantation in Children With a Fontan Procedure

Previous studies have reported that children with a prior Fontan procedure have decreased survival after heart transplantation. We examined 190 primary pediatric heart transplants. Since 1988, 27 (14.2%) of 190 children less than 18 years old undergoing primary heart transplantation had a Fontan procedure 3.7 ± 4.3 years before transplantation. Compared with 163 (85.8%) non-Fontan primary transplants, the Fontan patients were similar in age (8.2 ± 5.0 vs 6.5 ± 6.0 years), presensitization, and pretransplant clinical status. More Fontan patients had prior operations (100% vs 50%; p < 0.0001) and needed pulmonary artery reconstruction (100% vs 23.5%; p < 0.0001). Twelve (44%) had protein-losing enteropathy. Donor ischemic times (211 ± 72 vs 170 ± 61 minutes; p = 0.0018) and cardiopulmonary bypass times (197 ± 91 vs 121 ± 53 minutes; p < 0.0001) were greater in the Fontan group as were durations of ventilator support (4.9 ± 6.6 vs 2.6 ± 3.9 days; p = 0.018) and hospital stay (20.2 ± 17.5 vs 14.3 ± 12.4 days; p = 0.0435). The Fontan group had one 30-day mortality. One-year actuarial survival (81.5% vs 84.6%, Fontan vs non-Fontan) and five-year actuarial survival (65.5% vs 66.2%, Fontan vs non-Fontan) were similar, as was rejection incidence at one year (2.0 ± 2.0 vs 1.7 ± 1.9 episodes per patient; p = 0.3972). Five Fontan patients (18.5%) required retransplantation 4.9 ± 3.6 years posttransplant compared with 18 non-Fontan patients (11.0%) retransplanted 5.2 ± 3.4 years posttransplant (p = 0.3346). Contrary to prior reports, we did not identify any early or midterm disadvantage for children undergoing heart transplantation after a previous Fontan procedure despite more complex transplant operations. We contend that carefully selected children with a failing Fontan circulation can do as well as other children with heart transplantation.

Children undergoing heart transplant are at increased risk for postoperative vasodilatory shock*

To determine the incidence of vasodilatory shock (VDS) in children after cardiopulmonary bypass (CPB), and to describe this syndrome of post-CPB VDS in children. Prospective, observational. Pediatric and neonatal intensive care units in a tertiary care, children's hospital. Three hundred children undergoing CPB. None. Three hundred subjects undergoing CPB were evaluated for clinical evidence of VDS following CPB. The incidence of post-CPB VDS was 3%. Characteristics of children who developed VDS: higher peak lactate (6.2 +/- 2.6 vs. 3.0 +/- 2.1 mmol/L; p = 0.0002), higher peak serum blood urea nitrogen (18.5 +/- 4.6 vs. 15.6 +/- 7.2 mg/dL; p = 0.04), lower urine output (1.7 +/- 0.8 vs. 2.6 +/- 0.2 mL/kg/hr; p = 0.04), and fewer intensive care unit free days (14.9 +/- 9.0 vs. 21.1 +/- 7.2 days; p = 0.01). Univariate predictors for the development of post-CPB VDS included children who had heart transplantation (HT) (relative risk [RR], 9.8; 95% confidence interval [CI], 2.7-35.2) or ventricular assist device (VAD) placed (RR, 17.9; 95% CI, 3.8-84.1), a cardiomyopathy diagnosis (RR, 8.5; 95% CI, 2.3-31), age >12 years (RR, 4.5; 95% CI, 1.2-17.0), CPB time >180 minutes (RR, 7.1; 95% CI, 1.9-26.2), and preoperative ventricular dysfunction (RR, 3.7; 95% CI, 1.0-13.4). By stratified analysis, the only independent predictor for the development of VDS was undergoing HT/VAD. Post-CPB VDS is uncommon in children. However, children who undergo HT or VAD placement are at high risk for developing post-CPB VDS. Recognition that the overall incidence of post-CPB is low-except in the HT/VAD population-may help guide therapy in the pediatric post-CPB patient.

Assessment of the Cylex ImmuKnow Cell Function Assay in Pediatric Heart Transplant Patients

The Cylex ImmuKnow (Cylex, Columbia, MD) cell function assay (CICFA) is a commercially available test of immune response that purportedly identifies solid organ transplant patients at risk for either acute rejection (AR) or infection. Data on the utility of this test in pediatric heart transplant patients are very limited. This study tested the hypothesis that CICFA is a clinically useful test in this transplant population. All children undergoing heart transplantation at the study center (1989-2006) for whom CICFA levels were obtained were reviewed. The association of CICFA levels with episodes of AR and significant infections was determined. Among 83 patients (34 girls, 41%), 367 CICFA levels were obtained (median, 4.0; interquartile range [IQR], 2.0-6.0 per patient). There were 26 episodes of AR in 17 patients (20%) and 38 infections in 34 patients (41%). CICFA levels were similar among patients with AR at the time of the CICFA measurement (median, 325 [IQR, 163-480] adenosine triphosphate [ATP] ng/ml) vs patients without AR (median, 330 [IQR, 227-441] ATP ng/ml; p = 0.36). CICFA levels were similar among patients with infections within 1 month of CICFA measurement (median, 295 [IQR, 216-366] ATP ng/ml) and those without infections (median, 330 [IQR, 226-453] ATP ng/ml; p = 0.24). The CICFA is not predictive of AR or significant infections in pediatric heart transplant patients. On the basis of the available evidence, this assay cannot be recommended as part of the routine management of pediatric heart transplant patients.

Cardiac Transplant Outcomes in Pediatric Patients with Pre-formed Anti-Human Leukocyte Antigen Antibodies and/or Positive Retrospective Crossmatch

Children undergoing heart transplantation who have preformed anti-human leucocyte antigen (HLA) panel reactive antibodies (PRA) or positive retrospective crossmatch (XM) may be at increased risk for rejection and graft failure. We assessed outcomes of transplant recipients with either positive PRA before transplant or positive retrospective XM. A review of 148 heart transplant patients between 1990 and 2006 was undertaken, identifying transplants in patients with pre-transplant PRA > 1% and/or a positive XM. Demographic information and detailed post-transplant outcomes including episodes of rejection, infection, and graft failure were recorded. There were 11 PRA positive (PRA+) transplants, 135 PRA negative (PRA-) transplants, and no PRA data on 2. There were 14 XM+ transplants, 115 XM- transplants, and no XM data on 19. Kaplan-Meier graft survival was better in XM- than XM+ patients (p < 0.015), but not different between PRA+ and PRA- Groups. Timing of first rejection and number of rejection episodes were not different between XM+ and XM- Groups or between PRA+ and PRA- Groups. Infections were not different between PRA or XM Groups. Four patients were PRA+/XM- (all PRA, 1%-10%), 7 were PRA-/XM+, and 7 were PRA+/XM+ (6 of 7 PRA >10%). Pediatric heart transplant patients whose retrospective XM is positive are at significantly increased risk for graft failure. Elevated pre-transplant PRA may not predict increased risk of graft failure, although markedly positive PRA (>10%) predicts a positive retrospective XM. Improved treatment for pediatric transplant patients with a positive retrospective XM is needed.

Positive Pretransplantation Cytomegalovirus Serology Is a Risk Factor for Cardiac Allograft Vasculopathy in Children

Cytomegalovirus (CMV) infection has been implicated as a cause of posttransplantation coronary artery disease in adults. The purpose of this retrospective observational study was to evaluate the effect of CMV on outcome after heart transplantation in children. Risk factors tested were recipient age, sex, and pretransplantation CMV serology; use of anti-CMV prophylaxis; posttransplantation evidence of CMV infection; and donor CMV serology. Transplantations were stratified traditionally according to CMV risk as low risk (recipient negative/donor negative), intermediate risk (recipient positive), and high risk (recipient negative/donor positive). Primary outcome measures were (1) development of coronary artery vasculopathy, (2) mortality (or graft loss) that occurred outside the early postoperative period, and (3) death (or graft loss) due to vasculopathy. Analysis was by proportional hazards modeling. A total of 165 children underwent heart transplantation, with a mean age at transplantation of 7.8 (SD 5.6) years. Thirty-two children had laboratory evidence of CMV infection after transplantation, but only 6 developed CMV disease or syndrome. Traditional CMV risk stratification correlated well with CMV infection but did not predict mortality, coronary artery disease, or coronary death. In contrast, positive recipient CMV was the only independent predictor of all 3 outcome measures: coronary artery disease (hazard ratio=3.6), all-cause mortality (partial hazard ratio=4.1), and coronary death (hazard ratio=4.6). In children, pretransplantation recipient CMV status is a more powerful predictor for the development of clinically significant vasculopathy and subsequent death than traditional risk stratification. This phenomenon warrants further investigation.

568: Cardiac transplant outcomes in pediatric patients with preformed anti-HLA antibodies and/or positive retrospective crossmatch

Purpose: Children undergoing heart transplantation (HTx) who have preformed anti-HLA antibodies (PRA) and/or a positive retrospective crossmatch (XM) may have increased complication rates. Pragmatic limitations preclude routine prospective donor XM. This study assessed the outcome of patients that received HTx at our institution who had either positive PRA before transplant or a positive retrospective XM.

212: Ultrasound cardiac output monitor measurements in pediatric cardiac transplant patients

Purpose: The ultrasound cardiac output monitor (USCOM) is an FDA approved non-invasive device that can quickly determine cardiac output based on outflow tract velocity measured by continuous doppler and outflow valvular surface area deduced by patients’ height and weight. This investigation examines USCOM measurements in pediatric cardiac transplant patients. Methods and Materials: Following IRB approval and informed consent, USCOM readings were obtained during routine outpatient visits from June to August 2006. Measurements included cardiac index (CI), aortic valve diameter (AVD) and velocity through the aortic valve (VAV). Aortic valve velocities were compared to readings obtained by echocardiogram (ECHO) performed using the paired t-test with statistical significance at p-value 0.05. Results: 16 boys and 15 girls met study entry criteria. Mean age was 11.5 years and mean weight was 42.2 kg (range 12.3 to 146 kg). A single USCOM reading was obtained on each patient. Each USCOM measurement took less than 10 minutes to perform. USCOM derived mean CI was 4.42 L/min/m( /1.74). Mean USCOM VAV (1.13 /0.36 m/sec) was no different than that measured by ECHO (1.14 /0.16 m/sec), p 0.46. However, mean USCOM derived AVD (1.69 cm) was smaller than that measured by ECHO (2.32 cm). This is attributable to fact that the cardiac grafts are typically larger than the recipients’ expected AVD calculated using height and weight. All children appeared healthy and active on discharge from clinic. One child later requiring ICU admission for sepsis had a 50% decrement in CI from his USCOM baseline which also correlated with moderate to severe decrease in graft function on ECHO. Conclusions: VAV in children undergoing heart transplantation obtained by the USCOM device appeared no different from that measured by ECHO, though measured and calculated AVD may vary. USCOM derived CI appears to reasonably portray these patients’ clinical condition. USCOM may be a useful rapid screening tool to assess deviations from the baseline in pediatric heart transplant recipients.

Infant Heart Transplantation Ten Years Later—Where Are They Now?

Many uncertainties regarding the fate of children undergoing heart transplantation as infants were present when we and others embarked on this program. Although no truly long-term results are available, a significant cohort of children has now reached preteen and early teenage status. We reviewed our group of infants transplanted more than 10 years ago to assess survival and quality of life as they approach their teenage years. We retrospectively reviewed the medical records of all infant (younger than 6 months of age) heart transplant recipients, transplanted between 1988 and 1995, to ascertain survival statistics, incidence of complications, and current health status. A total of 42 patients were identified. The majority of these underwent transplantation for hypoplastic left heart syndrome. Eleven patients have died, 4 early and 7 late. The actual survival at 10 years is 76%. Twenty-seven of the 31 long-term survivors attend regular school; 4 are in special education classes owing to developmental delay. Five patients take medication for attention-deficit disorder. Malignancies have been discovered in 5, and 1 died secondary to this. Six patients have significant renal insufficiency, 1 of whom has undergone renal transplantation. One patient has undergone retransplantation for coronary artery disease. One patient required reoperation for supravalvar aortic stenosis. Other general medical problems that are being treated include sleep apnea (n = 1), hypertension (n = 5), and recurrent pneumonias (n = 1). Although these children require ongoing medical attention, including daily medications and regular follow-up visits, most have a satisfactory quality of life and behave much like normal children.

Pediatric Cardiac Transplantation in Children With High Panel Reactive Antibody

Background Elevated panel reactive antibody (PRA) may be considered a risk factor precluding pediatric orthotopic heart transplantation. We retrospectively reviewed our management strategy and outcome data for children undergoing heart transplantation with high PRA (> 10%). Methods Sixty consecutive children (median age = 130.5 days) underwent heart transplantation. Diagnoses included hypoplastic left heart syndrome (HLHS) (30 patients), cardiomyopathy (18 patients), and postoperative complex congenital heart disease (CCHD) (12 patients). Standard induction immunosuppressive therapy included pulse steroids, gamma globulin, and polyclonal rabbit antithymocyte globulin. Initial immunosuppression is a calcinurin inhibitor and an antiproliferative agent. Eight children exhibited elevated PRA (group P). Fifty-two exhibited nonelevated PRA (group N). Immunosuppression was modified in group P as follows: preoperative intravenous immunoglobulin G (IVIG) and/or cyclophosphamide or mycophenolate mofetil and preoperative and postoperative exchange transfusions or plasmapheresis. In group P, cyclophosphamide was the initial antiproliferative agent. Results Group P = 4 HLHS patients (all status post [s/p] prior cardiac surgery) and 4 postoperative CCHD patients. Group N = 26 HLHS patients (4 patients s/p prior cardiac surgery), 18 cardiomyopathy patients, and 8 postoperative CCHD patients. Group P patients were older and weighed more than group N patients. Waiting time for donor heart, cardiac ischemic time, and length of hospital stay were similar in both groups. Thirty-day mortality for group P was 25% and for group N it was 7.9% ( p = 0.178). Overall mortality for group P was 50% and for group N it was 15.4% ( p = 0.043). Conclusions Although heart transplantation can offer children with end-stage heart failure and elevated PRA their only chance of survival, these patients remain high risk despite aggressive immunosuppression.

Basiliximab in critically ill children undergoing heart transplantation: initial data

major risk factor for CMV was D /R(p 0.0001). Among all other D/R combinations, the freedom from CMV infection ranged from 90–98% at 6 months. In all D/R combinations (including D /R-), there was no significant reduction in CMV infection with any form of prophylaxis. Conclusions: CMV infection is infrequent except in CMV negative recipients with CMV positive donors. Death from CMV infection is uncommon. There does not appear to be a demonstrable benefit of any type of CMV prophylaxis in our study.