Objectives:With the scarcity of organs, a durable, reliable ventricular assist device (VAD) is required. The Berlin Heart EXCOR ® (BH) remains the most established VAD in the paediatric population. Implantable continuous flow (CF) VADs have been introduced to the paediatric field with encouraging early results. In this study, we compared the results of a newly introduced CF VAD (HeartWare VAD [HVAD] ® ) to results in a matched group of BH recipients.Methods:The study included patients aged <16 years who received mechanical left VAD (LVAD) support between December 2005 and January 2016. The preimplant characteristics and postimplant outcomes of patients who received the HVAD were compared with those of a matched group who received the BH. Patients with congenital heart disease were excluded.Results:Thirty patients were included in the study: 13 had received the HVAD and were matched with 17 patients who had received the BH LVAD. The only difference in preimplant characteristics was the need for higher inotropic support in the BH group. There was no difference in the need for right ventricular (RV) support (58.8% for BH vs 53.8% for HVAD, P  = 1.00) or in the incidence of cerebrovascular accidents (12.5% vs 7.7%, respectively, P  = 1.00), though the BH group showed prolonged mechanical ventilation (31.3% vs 0%, P  = 0.047). There were no deaths while on VAD support in either group. Patients with the HVAD showed a bimodal distribution for the primary end point (transplant/explant): All HVAD recipients who also required early RV support reached this end point within 30 days of receiving the implant.Conclusions:Our early experience with the CF intracorporeal LVAD system (HVAD) indicates outcomes comparable to those with the well-established pulsatile flow paracorporeal LVAD (BH). The theoretical durability of the CF device, which might also allow for the possibility of hospital discharge and better quality of life, is yet to be proven.

OBJECTIVES:Adults with failing Fontan circulation (FFC) represent a heterogeneous, high-risk group for cardiac transplantation with poor reported outcomes. We studied the impact of mode of Fontan failure (preserved versus impaired systolic ventricular function) and end-organ dysfunction on early survival in adults undergoing cardiac transplantation for FFC. METHODS:A single-centre, retrospective study of 26 adults (≥16 years) with FFC undergoing cardiac transplantation between 1990 and 2015. Patients were classified by the presence or absence of preserved systolic ventricular function (PVF). End-organ dysfunction was assessed by serum markers, including albumin, liver ultrasound and the presence of varices, ascites, splenomegaly and thrombocytopaenia (VAST score for portal hypertension). RESULTS:Thirty-day survival rate for the entire cohort was 69.2%, with 76.2% survival for the recent era. One-year Kaplan-Meier survival rate was 65.4%. Actuarial survival was poorer in those with PVF or heterotaxy (P = 0.01; log-rank test). Cox multivariable regression analysis confirmed PVF as an independent predictor for death (odds ratio, OR 5.38; confidence interval, CI 1.08-26.96; P = 0.04). In examining the PVF subset further, these patients had significantly higher VAST and liver ultrasound scores and lower serum albumin, compared with patients with impaired function. Patients with PVF and ≥moderate liver fibrosis on ultrasound or VAST score ≥2 accounted for two-thirds of the total mortality. CONCLUSIONS:Favourable cardiac transplantation outcomes can be achieved in adults with failing Fontan circulation. Patients with PVF may represent a distinct subset with more perturbed failing Fontan physiology and higher cardiac transplant mortality. We continue, however, to evolve and refine our strategies and are optimistic concerning future improvement in outcomes even in those with PVF.

OBJECTIVES:Mechanical cardiac support (MCS) can successfully be applied as a bridging strategy for heart transplantation (OHTx) in children with life-threatening heart failure. Emergent use of MCS is often required before establishing the likelihood of OHTx. This can require bridge-to-bridge strategies to increase survival on the waiting list. We compared the outcome of children with heart failure who underwent single MCS with those who required multiple MCS as a bridge to OHTx.METHODS:A retrospective study of patients aged less than 16 years was conducted. From March 1998 to October 2005, we used either a veno-arterial extracorporeal membrane oxygenator (VA-ECMO), or the Medos® para-corporeal ventricular assist device (VAD). From November 2005 onwards, the Berlin Heart EXCOR® (BHE) device was implanted in the majority of cases. Several combinations of bridge-to-bridge strategies have been used: VA-ECMO and then conversion to BHE; BHE and then conversion to VA-ECMO; left VAD and then upgraded to biventricular support (BIVAD); conversion from pulsatile to continuous-flow pumps.RESULTS:A total of 92 patients received MCS with the intent to bridge to OHTx, including 21 (23%) supported with more than one modality. The mean age and weight at support was similar in both groups, but multimodality MCS was used more often in infancy (P = 0.008) and in children less than 10 kg in weight (P = 0.02). The mean duration of support was longer in the multiple MCS group: 40 ± 48 vs 84 ± 43 days (P = 0.0003). Usage of multimodality MCS in dilated cardiomyopathy (19%) and in other diagnoses (29%) was comparable. Incidence of major morbidity (haematological sequelae, cerebrovascular events and sepsis) was similar in both groups. Survival to OHTx/explantation of the device (recovery) and survival to discharge did not differ between single MCS and multiple MCS groups (78 vs 81% and 72 vs 76%, respectively).CONCLUSION:Bridge to OHTx with multiple MCS does not seem to influence the outcome in our population. Infancy and body weight less than 10 kg do not tend to produce higher mortality in the multiple MCS group. However, children receiving more than one modality are supported for longer durations.

Objectives: Berlin Heart EXCOR (BHE) is an important device for bridging children to heart transplantation. However, between the smallest pump (10 ml) and the next size (25 ml) there was still a remarkable gap in terms of perfect pump performance. We describe the first clinical results of post CE-mark early product surveillance (EPS) in Europe for the new 15-ml EXCOR pump. Methods: Nine patients [mean age 1.9 years, mean body surface area (BSA) 0.47 m2] were supported due to severe heart failure. Diagnosis was idiopathic cardiomyopathy in 5 children, ischaemic cardiomyopathy, myocarditis, post-transplant rejection and congenital heart disease in 1, respectively; 78% were in INTERMACS levels I and II. Two children were on veno-arterial extracorporeal membrane oxygenation (VA-ECMO) before BHE (2 and 7 days of support). Data were analysed prospectively in a multicentre approach in 3 European high-volume paediatric heart surgery centres within the EPS (60 days follow-up). Results: All children were included in the study. There were 5 biventricular assist device (BIVAD) and 4 left ventricular assist device (LVAD). Mean length on support was 62 ± 57 days and median 33 days (range 14–182). Thirty-day and 60-day survivals were 100%. Two children were successfully explanted after 15 and 27 days of support. All other patients were successfully transplanted after a mean of 71 days on device. Within EPS time period, there were no device failures or severe adverse events (cerebrovascular accident, haematological sequelae, sepsis, multiorgan failure). Conclusion: The new 15-ml EXCOR pump showed excellent performance in post-market surveillance in Europe and seems to close the gap successfully. Further implantations are needed to confirm these first results.

OBJECTIVES:A significant number of children affected by congenital heart disease (CHD) develop heart failure early or late after surgery, and heart transplantation (OHTx) remains the last treatment option. Due to shortage of donor organs in paediatric group, mechanical circulatory support (MCS) is now routinely applied as bridging strategy to increase survival on the waiting list for OTHx. We sought to assess the impact of MCS as intention to bridge to OHTx in patients with CHD less than 16 years of age.METHODS:From 1998 to 2013, 106 patients received 113 episodes of MCS with paracorporeal devices as intention to bridge to OHTx. Twenty-nine had CHD, 15 (52%) with two-ventricle (Group A) and 14 (48%) with single-ventricle physiology (Group B). In Group A, 5 children had venoarterial extracorporeal membrane oxygenation (VA ECMO), 6 left ventricular assist device (LVAD), 2 biventricular assist device (BIVAD), 1 VA ECMO followed by BIVAD and 1 BIVAD followed by VA ECMO. In Group B, VA ECMO was used in 7 children, univentricular assist device (UVAD) changed to VA ECMO in 4, UVAD in 2 and surgical conversion to two-ventricles physiology with BIVAD support changed to VA ECMO in 1.RESULTS:Twenty-one of 29 (72%) children survived to recovery/OHTx. Seven of 29 (59%) survived to discharge. In Group A, 11/15 (73%) survived to recovery/OHTx and 9/15 (60%) survived to discharge. Four of 15 (27%) died awaiting OHTx. One child had graft failure requiring VA ECMO and was bridged successfully to retransplantation. One child dying after OHTx had acute rejection, was supported with VA ECMO and then BIVAD but did not recover. One patient had an unsuccessful second run on BIVAD 1 year after recovery from VA ECMO. In Group B, 10/14 (71%) survived to recovery/OHTx and 8/14 (57%) survived to discharge. Four of 14 (29%) died awaiting OHTx. Of deaths after OHTx, 1 occurred intraoperatively and 1 was consequent to graft failure and had an unsuccessful second run with VA ECMO.CONCLUSIONS:Children with CHD can be successfully bridged with MCS to heart transplantation. Single-ventricle circulation compared with biventricular physiology does not increase the risk of death before transplant or before hospital discharge.

The use of ventricular assist devices (VADs) as a "bridge to recovery" is increasing in pediatrics.1–3 Long-term implications of transplant, increased waiting times and risks of mechanical support make the potential for functional improvement and device explantation important. Although ways to predict recovery in adults have been suggested,4,5 there are no data to assist in timing of VAD explantation in children. We developed a protocol for assessment of recovery on the Berlin Heart and herein we present our experience with explantation.

BACKGROUND: Mechanical circulatory support (MCS) is used to support children with end-stage heart failure to heart transplant.METHODS: This was a retrospective cohort study of 7 years' experience with the Berlin Heart (BH) EXCOR (Berlin Heart AG, Berlin Germany) paracorporeal ventricular assist device (VAD) in 2 United Kingdom (UK) pediatric heart transplant centers and the effect of this program on the UK pediatric heart transplant service.RESULTS: Of 102 children who received BH support, 84% survived to transplant or BH explant and 81% survived to discharge. Neither age nor duration of support influenced outcome. Stroke, ongoing requirement for ventilation while on BH, and diagnosis other than dilated cardiomyopathy were the only independent mortality risk factors. Children who weighed < 20 kg had significantly (p = 0.03) longer support times than bigger children. The number of children treated with a BH increased over time (p = 0.01). Currently > 50% of pediatric heart transplants are bridged with a BH; however, pediatric transplants per year have not increased significantly (p = 0.07)CONCLUSIONS: BH use in the UK has allowed significant increases in the number of children with end-stage heart failure who can be successfully bridged to transplant and the length of time they can be supported. The total number of transplants has not increased. (C) 2013 International Society for Heart and Lung Transplantation. All rights reserved.

OBJECTIVES:Mechanical circulatory support (MCS) may be required after orthotopic heart transplantation (OHTx) in children for the treatment of failure or rejection. We review the incidence and outcomes of post-transplant MCS in our institution. METHODS:MCS was classified as early (<1 month since transplant) or late (>1 month since transplant) and the support offered was either veno-arterial extra-corporeal membrane oxygenation (VA-ECMO) or a ventricular assist device (VAD). From 2003 to the present, 100 children (<16 years) underwent OHTx. Fifteen (15%) had 17 episodes of MCS. MCS was instituted early in 10 and late in seven episodes. Two children required two episodes of support. VA-ECMO was used in 12 episodes (71%). Two children required VAD support alone (12%). In three (17%) episodes ECMO was subsequently converted from VAD. RESULTS:Among 10 children with early failure, eight were successfully weaned from support with recovery of graft function. In the late failure group, three of six patients died. All but four patients underwent re-transplantation with no perioperative deaths. Overall survival to discharge was 66%. The early failure group shows a better survival rate to hospital discharge compared with the late failure group (78 vs 50%; P < 0.0001). CONCLUSIONS:The incidence of post-transplant MCS for graft failure in our patients was 15%. Early graft failure has a better outcome than late failure. Re-transplantation has good mid-term outcomes in children. A stepwise approach with a multimodality MCS strategy improves survival in this group of patients.

MCS as a bridge to cardiac transplant in children is common. We aimed to review the post transplant course of children on MCS as compared to medical support.

Anticoagulation given to prevent thromboembolic complications in children on mechanical circulatory support can cause bleeding which may be severe enough to neccessitate anticoagulation withdrawal. We aim to identify the incidence of bleeding complications, incidence of anticoagulation withdrawal and outcomes.

Objective: Early failure of the Fontan-type circulation is a potentially fatal complication. We review our experience with cardiac transplantation in children presenting with end-stage heart failure in this scenario. Methods: We performed a retrospective review. Between 1985 and 2003, 6 children aged less than 16 years were referred for cardiac transplantation. The indication for cardiac transplantation was end-stage cardiac failure early after the completion of the Fontan-type operation. Results: All 6 patients listed for transplantation underwent cardiac transplantation; the median interval to transplantation from the operation was 36 days (range, 6-180 days). Four patients had undergone the Fontan procedure, and 2 had one-and-a-half-ventricle repair. All 6 patients were ventilated and inotrope dependant, with varying degrees of multiorgan dysfunction. One patient was bridged to transplantation with extracorporeal membrane oxygenation. The median age at transplantation was 7.1 years (range, 3-12.5 years), and weight was 18.9 kg (range, 11-35 kg). One patient died on the operating table (graft failure and hemorrhage). In 5 survivors the median intensive care unit stay was 10 days (range, 8-61 days). On follow-up of 6 to 81 months, there have been no subsequent deaths, and the quality of life in survivors is good. Conclusion: Rescue cardiac transplantation in the setting of an early failure of the Fontan-type circulation and end-stage cardiac failure is an effective treatment option and can be performed with acceptable early mortality and encouraging short-term to medium-term results.

Berlin Heart (BH) use in children with end stage heart failure as bridge to transplant (BTT) or recovery enables significantly longer cardiac mechanical support times than previously possible.We review coexisting respiratory morbidity in this group.

Background: Mechanical support as a bridge to cardiac transplantation in children is an accepted treatment. With improved devices and increasing experience, the length of time that children can be supported has increased. Donor organs remain scarce and there is significant associated morbidity.Methods: Retrospective review of all children offered mechanical support as a bridge to heart transplant over 10 years in one of the two UK pediatric heart transplant centers. Outcomes during the years 1998 to 2002 were compared with outcomes during the years 2003 to 2007.Results: Forty children in 41 separate patient episodes received mechanical support as a bridge to transplantation or, in 1 case, to recovery. Survival to transplant or recovery was achieved in 29 of 41 (71%); 26 of 40 children (63%) survived to hospital discharge. Devices used were extracorporeal membrane oxygenation (ECMO), the Medos HIAA, the Berlin Heart (from November 2005) and the Levitronix ventricular assist device (VAD) from 2007. All 3 children supported with the Levitronix survived to transplant (median duration of support 10 days). Ten of 13 children (77%) supported by the Berlin Heart survived to transplant or recovery (median duration of support 44 days). Four of 7 (57%) children supported using the Medos device survived to transplant (median duration of support 7 days). Neurologic events were the most common cause of death in both eras (1998 to 2002 and 2003 to 2008).Conclusions: Waiting times to pediatric cardiac transplant in the UK have increased. The Berlin Heart allows children to be bridged to transplant over long periods. Neurologic morbidity remains as a major concern. J Heart Lung Transplant 2009;28:249-54. Copyright (C) 2009 by the International Society for Heart and Lung Transplantation.

Case reports of two patients with unusually late initial presentation of chronic granulomatous disease with fulminant Aspergillus pneumonia.Medical notes; retrospective study.Identical pattern of clinical presentation in two patients referred for support with extracorporeal membrane oxygenation (ECMO). Our Institutional Review Board waived the need for consent.Two school-aged boys presented with features of, and were initially treated, for community-acquired pneumonia. However, the disease course was rapidly progressive to fulminant respiratory failure and because both failed conventional intensive care management, they were referred to ECMO support. Although both died of evolving multiorgan failure, ECMO support allowed open lung biopsy leading to diagnosis of invasive Aspergillus pneumonia and chronic granulomatous disease.Failure of adequate therapy for acute community-acquired pneumonia and rapid progression to respiratory failure should lead to the possibility of fungal etiology. Congenital immunodeficiency may present for the first time late in life, so acute invasive pulmonary aspergillosis in the absence of known risk factors should lead to consideration of chronic granulomatous disease regardless of patient age.