Outcome of pediatric heart transplantation recipients treated with ventricular assist device

This study was conducted to evaluate the long‐term prognosis of pediatric HTx patients treated with VAD before transplantation. The clinical data of six patients bridged to HTx with Berlin Heart EXCOR pediatric device were analyzed retrospectively. Information about graft function, CA results, and EMB findings as well as appearance DSA was collected. Also, information about growth and cognitive function was analyzed. These findings were compared with age‐, gender‐, and diagnosis‐matched HTx patients. During the median follow‐up time of four and half yr after HTx, the prognosis including graft function, number of rejection episodes, and incidence of coronary artery vasculopathy, growth and cognitive development did not differ between VAD‐bridged HTx patients compared with control patients. In both groups, one patient developed positive DSA titer after HTx. Our single‐center experience suggests that the prognosis of pediatric HTx patients treated with VAD before transplantation is not inferior to that of other HTx patients.     

Ventricular assist devices in children: current achievements and future perspectives

Mechanical circulatory support systems for the treatment of acute and chronic heart failure are now available for use in several clinical situations and are designed for different indications and support times. In children, particularly in small infants, extracorporeal membrane oxygenation and centrifugal pumps have been most widely used in the past. These systems are preferred for support after cardiac operations and for use in patients who have concomitant respiratory failure, but they are suitable for short-term application only and intensive care is obligatory. VADs are designed for long-term application and allow patients to be discharged home. Pneumatic pulsatile VADs have been available in pediatric sizes since 1992. Currently at our institution, 74 children have been supported with pediatric extracorporeal VADs for up to 14 months. In the past five yr, a notable rise in survival has been achieved by improvements in pump design and pre- and post-operative management. We have been able to discharge 78% of the infants under one yr old. In this review, our current VAD experience in children will be presented in the light of improvements in decision-making, device technology, and implantation techniques, and in coagulation monitoring and anticoagulation. Additionally, new developments in the field of pediatric assist devices will be presented.     

New era of pediatric ventricular assist devices: Let us go to school

As there is still a shortage of pediatric donor hearts, several techniques have been used to assist pediatric patients to survive until transplantation. VADs provide long‐term support and ability of mobilization for children before a suitable heart becomes available. Several devices such as paracorporeal pumps have been used for this purpose, with acceptable morbidity and mortality rates. However, discharge is not possible, as there is no mobile drive unit for these small‐sized pumps. The possible negative psychosocial impact of long‐term hospitalization, away from home and school, may cause some adjustment problems in the future. In this case series, three pediatric patients that underwent intracorporeal LVAD implantation and returned to school are presented to share clinical experience and also to attract attention to the potential social and psychiatric implications.     

Sub‐acute neonatal hemochromatosis in an infant with hypoplastic left heart syndrome on ventricular assist device awaiting transplantation

Single‐ventricle pediatric patients, amongst other children waiting for OHT, are a vulnerable population, especially if candidacy is established before any palliation. NH is a rare disease with poor prognosis in the post‐natal period. We present a case of sub‐acute NH diagnosed in an infant with HLHS who was listed for OHT while bridged with a pulsatile paracorporeal VAD, with an emphasis on the evolution of the condition throughout the patient's clinical course and the ultimate decision for compassionate deactivation of VAD.     

Biventricular support with HeartWare ventricular assist device in a pediatric patient

Use of ventricular assist devices is increasing in the pediatric population. This has included the extended use of adult continuous‐flow devices in the pediatric population. In a minority of cases, biventricular support may be needed. In these situations, biventricular support with continuous‐flow devices can be surgically challenging, and therefore, only few cases have been reported. Here, we present a case of implantation of two HeartWare HVAD devices for biventricular support for a decompensating patient with acute myocarditis as well as present an alternative implantation surgical strategy.     

Tissue plasminogen activator treatment of bilateral pulmonary emboli in a pediatric patient supported with a ventricular assist device

Bleeding and thrombosis are well‐known potential complications of VAD support. We present a pediatric patient who developed massive bilateral pulmonary emboli while on BiVAD support that was successfully treated with intravenous tPA and bridged to heart transplant.     

Allosensitization does not alter post‐transplant outcomes in pediatric patients bridged to transplant with a ventricular assist device

Patients supported with a VAD are at increased risk for sensitization. We aimed to determine risk factors for sensitization as well as the impact of sensitization on post‐transplant outcomes. The UNOS database (January 2004–June 2014) was used to identify patients (≤18 yrs) supported with a durable VAD. Rates and degree of sensitization in the VAD cohort were calculated. Post‐transplant survival was determined comparing outcomes of sensitized vs. non‐sensitized patients. There were 3097 patients included in the study; 19% (n = 579) were bridged with a VAD. Of these, 41.8% were sensitized vs. 29.9% of the patients who were not bridged with a VAD (p < 0.001). VAD was an independent predictor of sensitization (OR 2.05 [1.63–2.57]; p < 0.001). There was no difference in sensitization based on device type (continuous vs. pulsatile flow, p = 0.990). Post‐transplant survival rates between the sensitized and non‐sensitized VAD patients were not different, including patients with a PRA >50% and VAD patients with a positive DSC (p = 0.280 and 0.160, respectively). In conclusion, pediatric VAD patients are more likely to be sensitized, but there was no difference in sensitization based on device type. In addition, sensitization does not appear to impact outcomes.     

Characteristics of deposits and pump exchange in the Berlin Heart EXCOR ventricular assist device: Experience with 67 cases

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.     

Berlin heart ventricular assist device as a long‐term bridge to transplantation in a Fontan patient with failing single ventricle

The use of VADs as a BTT in children with heart failure has increased due to enhanced device design and reliability, leading to improved survival, functional capacity, and quality of life. However, the use of VADs in patients with Fontan physiology as a BTT is rare with few case reports. Here, we describe a case of implantation of the Berlin VAD as a BTT for 179 days, one of the longest reported periods of mechanical support, in a child with failing single ventricle.     

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??The prevalence of depression and anxiety in children and adolescents with epilepsy was 12%??36%??but the symptoms of depression and anxiety were rarely recognized and treated. Seizure variables??use of polytherapy??demographic characteristics and child and family adaptation are associated with increases in the symptoms. The use of screening measures increases the recognition of symptoms of depression and anxiety. Treatment of epilepsy-specific symptoms of depression and anxiety involves multidisciplinary collaboration. Pharmacological and psychotherapeutic interventions are recommended??with SSRIs as the first-line medications.     

Talking with caregivers of children living in the community with ventricular assist devices

A VAD is a mechanical pump used to support the functioning of a failing heart. As a pediatric therapy, a VAD is used as a temporary solution for poor heart function, a bridge to transplantation or recovery, or a destination therapy. The goal of this qualitative study was to explore the perspectives of family and professional caregivers of children who are supported by VADs in outpatient settings. Semi‐structured interviews were conducted with 22 caregivers of school‐aged children discharged home on VAD support. Interviews were transcribed, and data were analyzed using qualitative content analysis. Caregivers identified issues facing children on VAD support in the contexts of home, school, and other childhood places including being physically connected to a device; experiencing changes; living a medical life; negotiating restrictions; cost of care; family, kinship, and community; and, present and future living. While a child with a VAD may have much in common with other medically complex children, the technological complications and risks of living with a VAD are uniquely identified by caregivers as an issue, especially when considering the way that children with a VAD are connected to their device—implanted yet exterior, mobile yet restricted, and autonomous yet dependent.     

Changes in left ventricular mass index in children and adolescents after renal transplantation

Recent reports indicate a high prevalence of left ventricular hypertrophy (LVH) in children on dialysis and after renal transplantation (Tx), as identified by cross-sectional analysis. However, the evolution of LVH in pediatric patients with end-stage renal disease after renal Tx is not well established. To assess changes of left ventricular mass (LVM), we prospectively performed echocardiography in 23 children and adolescents between November 1998 and July 2000. Each patient had an echocardiographic evaluation while on dialysis (for at least 6 weeks) and a follow-up evaluation at least 6 months after successful renal Tx (i.e. with a measured glomerular filtration rate [GFR] of at least 40 mL/min/1.73 m2). The LVM index was estimated by indexing LVM to height(2.7). Sixteen patients had a cadaveric transplant and seven had a live donor transplant; the mean duration between the two studies was 1.9 +/- 1.6 yr; and the mean GFR was 55.0 +/- 21.4 mL/min/1.73 m2. There was no significant difference in the mean values of the LVM index while on dialysis and after renal Tx (43.9 +/- 17.8 g/m2.7 and 39.3 +/- 12.0 g/m2.7, respectively, p = 0.19), or in the prevalence of LVH (52% and 56%, respectively). Interval changes in the LVM index in individual subjects between the two studies were significantly associated with interval changes in indexed systolic (r = 0.42, p = 0.04) and diastolic (r = 0.42, p = 0.05) blood pressures. Interval changes in hemoglobin, blood urea nitrogen (BUN), creatinine, and duration after Tx did not correlate with changes in the LVM index. There was no significant difference in LVM index change according to the type of dialysis, donor source, and the cause of renal failure. In multivariate analysis, the baseline LVM index and changes in indexed SBP were independent predictors for LVM index change after renal Tx. We conclude that LVH persists in children and adolescents after renal Tx. Control of blood pressure might be an important factor in regression or prevention of progression of LVH in these patients.     

Successful ABO-incompatible heart transplantation in a child despite blood-group sensitization after ventricular assist device support

Abstract:  In the first two yr of life blood-group incompatible (ABO-incompatible) heart transplantation can be performed leading to immune tolerance to donor blood group. Antibody titers should be below 1:4. VAD use is correlated with sensitization toward blood-group antigens. A boy was diagnosed with dilated cardiomyopathy at nine months of age and listed for 0-compatible transplantation. Progressive heart failure required implantation of a left VAD. His listing was extended for ABO-incompatible transplantation despite antibody titers of 1:32 anti-A and 1:8 anti-B. After 26 days on VAD, he was transplanted with a B donor heart. No hyperacute or acute rejection occurred in 12 months post-transplant. Anti-B antibodies rose to a maximum of 1:2. No use of rituximab or plasmapheresis was required. There are no signs of graft vasculopathy. This indicates that inclusion criteria for ABO-incompatible transplantation may be extended to immediate cases. This is the first case with a healthy immune system to show signs of tolerance development after ABO-incompatible heart transplantation with increased prior antibody titers and without specific treatment.     

Solid organ donation in a child after extracorporeal membrane oxygenation,orthotopic heart transplantation,and ventricular assist device support

Gupta P, Blanco C, Madigan M, Dodgen A, Hanson M, Frazier EA, Bhutta AT, Fiser WP. Solid organ donation in a child after extracorporeal membrane oxygenation, orthotopic heart transplantation, and ventricular assist device support. Abstract: Use of high‐risk or marginal donors is the most viable short‐term means to boost the organ supply and bridge the widening gap between the number of patients on the waiting list for organ transplantation and the insufficient numbers of organ donors. Expansion of the donor pool requires an understanding of the impact on survival likely to result from extending one or more high risk factors. Use of extended donor pool results in shorter waiting list times and limits the morbidity and mortality associated with long‐term mechanical support needed to support diseased organs. In this report, we present one such example of expanding donor pool in which a pediatric patient donated a solid organ after two heart transplants and successful use of ECMO and VAD.     

Outcomes of Berlin Heart EXCOR® pediatric ventricular assist device support in patients with restrictive and hypertrophic cardiomyopathy

The outcomes of pediatric ventricular assist device support in patients with diastolic heart failure have not been well described. This study reviews the North American experience with Berlin Heart EXCOR^® ventricular assist device implants in children with such physiology. The Berlin Heart clinical database was reviewed. Patients with primary diastolic dysfunction are included in this study. Twenty pediatric patients with restrictive cardiomyopathy (n = 13), hypertrophic cardiomyopathy (n = 3), or congenital heart disease with restrictive physiology (n = 4) who were supported with EXCOR^® were identified. Of these, nine (45%) were successfully bridged to transplant, one (5%) weaned from support, and 10 (50%) died after support was withdrawn. Of patients under age 3 years (n = 13), 38.5% survived, whereas those aged 3 or older (n = 7) had 71.4% survival (P = .35). Biventricular assist device (BiVAD) patients experienced a 27.3% survival, vs 77.8% for patients with left ventricular assist device only (P = .07). Primary causes of death included stroke, infection, acidosis, multisystem organ failure, and bleeding. Pediatric patients with diastolic heart failure comprise a high‐risk population for mechanical circulatory support. However, half of patients with this physiology have been successfully supported to explant with EXCOR^®. The trends toward higher mortality for younger patients and those receiving BiVAD support warrant consideration.     

Echocardiographic changes and risk factors for left ventricular hypertrophy in children and adolescents after renal transplantation

Long-term consequences of cardiac alteration in children with chronic renal failure and after renal transplantation are largely unknown. In chronic uremia, cardiomyopathy manifests itself as systolic dysfunction, concentric left ventricular hypertrophy (LVH) or left ventricular dilatation. The correction of uremic state by renal transplantation leads to normalization of left ventricular contractility, regression of LVH and improvement of cavity volume and so dialysis patients with uremic cardiomyopathy would benefit from renal transplantation. We studied 73 patients, aged 17 yr or less, who underwent renal transplantation in our center. This cross-sectional study was performed 4.6 yr (median) after transplantation. Of the total, 48 were males and 25 were females. Transthoracic echocardiographic examination was performed for all cases. The effects of clinical, demographic, biochemical and therapeutic data on echocardiographic parameters were assessed. Multivariate analysis was used to assess the relation between the risk factors and the left ventricular muscle mass index. The most common echocardiographic abnormalities were the LVH (47.9%), left atrial enlargement (31.5%) and left ventricular dilatation and systolic dysfunction (13.7% for each). The pretransplant dialysis, arteriovenous fistula, acute rejection, cumulative steroid dose per square meter surface area, post-transplant hypertension, anemia and graft dysfunction were significant risk factors for LVH by univariate analysis. The significant factors by multivariate analysis were pretransplant dialysis, post-transplant hypertension and anemia. From this study we may conclude that LVH is a common problem among renal transplant children and adolescents. Early transplantation, control of hypertension and correction of anemia may be beneficial regarding left ventricular function and structure.