A spatial human thymus cell atlas mapped to a continuous tissue axis.

T cells develop from circulating precursors, which enter the thymus and migrate throughout specialised sub-compartments to support maturation and selection. This process starts already in early fetal development and is highly active until the involution of the thymus in adolescence. To map the micro-anatomical underpinnings of this process in pre- vs. post-natal states, we undertook a spatially resolved analysis and established a new quantitative morphological framework for the thymus, the Cortico-Medullary Axis. Using this axis in conjunction with the curation of a multimodal single-cell, spatial transcriptomics and high-resolution multiplex imaging atlas, we show that canonical thymocyte trajectories and thymic epithelial cells are highly organised and fully established by post-conception week 12, pinpoint TEC progenitor states, find that TEC subsets and peripheral tissue genes are associated with Hassall's Corpuscles and uncover divergence in the pace and drivers of medullary entry between CD4 vs. CD8 T cell lineages. These findings are complemented with a holistic toolkit for spatial analysis and annotation, providing a basis for a detailed understanding of T lymphocyte development.

EASL-ERN position paper on liver involvement in patients with Fontan-type circulation.

Fontan-type surgery is the final step in the sequential palliative surgical treatment of infants born with a univentricular heart. The resulting long-term haemodynamic changes promote liver damage, leading to Fontan-associated liver disease (FALD), in virtually all patients with Fontan circulation. Owing to the lack of a uniform definition of FALD and the competitive risk of other complications developed by Fontan patients, the impact of FALD on the prognosis of these patients is currently debatable. However, based on the increasing number of adult Fontan patients and recent research interest, the European Association for The Study of the Liver and the European Reference Network on Rare Liver Diseases thought a position paper timely. The aims of the current paper are: (1) to provide a clear definition and description of FALD, including clinical, analytical, radiological, haemodynamic, and histological features; (2) to facilitate guidance for staging the liver disease; and (3) to provide evidence- and experience-based recommendations for the management of different clinical scenarios.

A Novel Strategy for the Mechanical Subpulmonary Support in Failing Fontan Patients.

BACKGROUND: The number of single ventricle patients undergoing Fontan palliation and surviving to adulthood worldwide has steadily increased in recent years. Nevertheless, the Fontan circulation is destined to fail. Ultimately, heart transplantation (HTx) remains the definitive treatment option. Due a shortage of organs, mechanical circulatory support in the form of ventricular assist devices (VADs) is widely used to bridge heart failure patients to HTx, but these devices have been mainly developed to address the needs of normal anatomies. A novel venous cannula has been developed as part of the EXCOR® VAD to provide subpulmonary support in these patients. Its clinical application is investigated in the "Registry to Assess the Safety and Feasibility of the Subpulmonary Support with the Novel Venous Cannula in Patients with Failing/Absence of the Right Heart" (RegiVe study, NCT04782232). METHODS: RegiVe is a multicenter, international, observational, prospective, non-randomized registry aiming to collect the routine clinical data of up to 20 patients. The primary endpoints address device performance and safety, while the secondary endpoints target organ status and overall safety (according to the Interagency Registry for Mechanically Assisted Circulatory Support - INTERMACS - definitions). Data analysis will be performed by means of descriptive statistics. RESULTS: RegiVe has received the favorable opinion of an independent ethics committee and enrollment has recently started. CONCLUSION: RegiVe is the first study evaluating the use of a medical device specifically developed for subpulmonary support of failing Fontan patients. The study will provide important insight and further information on this cohort and help to improve a dedicated VAD strategy.

Outcomes of the Ross procedure in young adults with previous aortic valve interventions.

OBJECTIVES: Contemporarily modified Ross procedure continues to deliver excellent outcomes and remains part of the treatment strategy for aortic valve disease in the young adult population. The aim of this study was to assess whether Ross procedure carried out as a second or subsequent intervention for aortic valve disease carries similar risk and long-term benefit, when compared to Ross procedure for the first-time aortic valve replacement. METHODS: A total of 158 patients aged 16-60 years from a single congenital cardiac centre between 1997 and 2020 were included. The sample was split into 2 subgroups, based on the history of previous aortic valve interventions prior to the Ross procedure. Primary outcomes were defined as survival and pulmonary autograft failure. Coarsened exact matching was used to balance for covariates. RESULTS: A total of 103 patients underwent primary Ross and 55 underwent secondary Ross with a mean follow-up of 7.8 years. Twenty-two patients underwent 28 reoperations during follow-up. Forty-nine well-matched pairs were obtained through matching. Freedom from pulmonary autograft failure at 10 years was 84.5% in the primary group vs 100% in the secondary group (P = 0.021). Five- and ten-year survival were identical, 97.5% in the primary group vs 95.6% in the secondary group (P = 0.53). Male sex and era were found to be predictors of neoaortic root dilatation. CONCLUSIONS: The lower incidence of graft failure seen in secondary Ross could justify a stepwise approach to aortic valve intervention. Ross procedure delivers excellent outcomes in the adult population with no difference in survival for primary or secondary Ross.

A novel approach to stenting a stenotic graft housing a Berlin Heart cannula by utilizing an adapted left ventricular assist device circuit.

BACKGROUND: A 9-year-old boy with Berlin Heart biventricular assist device for diagnosis of dilated cardiomyopathy developed progressive reduction in left ventricular assist device(VAD) emptying and evidence of low cardiac output despite alterations to the device settings. Computed tomography revealed that the Dacron graft attaching the systemic cannula to the ascending aorta was stenosed. METHOD & RESULT: A minimally invasive approach with novel circuit modification was used to achieve antegrade stenting of the stenosed graft. CONCLUSION: This proposed technique provides a large port for minimally invasive access via an adapted VAD circuit allowing stent insertion to the aortic graft with an excellent outcome.

Evolving Approach in Hypoplastic Left Heart Syndrome With Restrictive and Intact Septum.

Background: Hypoplastic left heart syndrome (HLHS) with either intact atrial septum (IS) or highly restrictive interatrial communication (HRIC) is associated with poor survival. Immediate postpartum access to cardiac therapy and timely left atrial decompression (LAD) are paramount to a successful outcome. We describe herein our evolving approach to LAD and report interstage and longer-term results. Methods: We retrospectively identified neonates with HLHS IS/HRIC requiring LAD between 2005 and 2019. All babies had prenatal/postnatal echocardiography. Our LAD strategy evolved over time from attempt at transcatheter balloon atrial septostomy (BAS) to surgical septectomy with inflow occlusion, to hybrid trans-atrial stent implantation. Results: Twelve neonates required LAD at a median time of 14 (0.5-31) hours after birth. Five patients underwent BAS that proved successful in 2 cases. Of the 3 unsuccessful cases, 2 required extra-corporeal membrane oxygenation (ECMO) support and died subsequently; one underwent hybrid trans-atrial stent implantation. Of the remaining 7 patients, 3 underwent surgical septectomy with inflow-occlusion and 4 underwent hybrid trans-atrial stent implantations. Overall, 8 patients survived LAD and reached Norwood palliation. Three of the 8 required ECMO postoperatively. There was no hospital mortality after Norwood stage 1 palliation and interstage survival was 100%. Six patients successfully underwent Glenn shunt (superior cavopulmonary anastomosis) and 5 have completed total cavopulmonary connection. Conclusions: Our experience suggests that prompt postnatal LAD can be safely achieved with careful multidisciplinary planning and accurate antenatal diagnosis. In our hands, hybrid trans-atrial septal stent insertion appears to be a safe approach which combines the versatility of transcatheter techniques together with the effectiveness of surgical control.

Ventricular assist devices in transposition and failing systemic right ventricle: role of tricuspid valve replacement.

OBJECTIVES: Ventricular assist device (VAD) for systemic right ventricular (RV) failure patients post-atrial switch, for transposition of the great arteries (TGA), and those with congenitally corrected TGA has proven useful to reduce transpulmonary gradient and bridge-to-transplantation. The purpose of this study is to describe our experience of VAD in systemic RV failure and our move towards concomitant tricuspid valve replacement (TVR). METHODS: This is a single-centre retrospective study of consecutive adult patients receiving HeartWare VAD for systemic RV failure between 2010 and 2019. From 2017, concomitant TVR was performed routinely. Demographic, clinical variables and echocardiographic and haemodynamic measurements pre- and post-VAD implantation were recorded. Complications on support, heart transplantation and survival rates were described. RESULTS: Eighteen patients underwent VAD implantation. Moderate or severe systemic tricuspid regurgitation was present in 83.3% of patients, and subpulmonic left ventricular impairment in 88.9%. One-year survival was 72.2%. VAD implantation was technically feasible and successful in all but one. Post-VAD, transpulmonary gradient fell from 16 (15-22) to 10 (7-13) mmHg (P = 0.01). Patients with TVR (n = 6) also demonstrated a reduction in mean pulmonary and wedge pressures. Furthermore, subpulmonic left ventricular end-diastolic dimension (44.3 vs 39.6 mm; P = 0.03) and function improved in this group. After 1 year of support, 72.2% of patients were suitable for transplantation. CONCLUSIONS: VAD is an effective strategy as bridge-to-candidacy and bridge-to-transplantation in patients with end-stage systemic RV failure. Concomitant TVR at the time of implant is associated with better early haemodynamic and echocardiographic results post-VAD.

Pediatric Ventricular Assist Device Implantation: An Anesthesia Perspective.

BACKGROUND: Ventricular assist devices (VADs) are increasingly being implanted in children, yet there is little literature to guide anesthetic management for these procedures. AIMS: To describe the pediatric population presenting for VAD implantation and the anesthetic management these patients receive. To compare (a) children under and over 12 months of age and (b) children with and without congenital heart disease. METHODS: Retrospective review of patients aged 0 to 17 years who underwent VAD implantation at a single center between 2014 and 2019. RESULTS: Seventy-seven VADs were implanted in 68 patients (46 left VADs, 24 biventricular VADs, 6 right VADs, and 1 univentricular VAD). One procedure was abandoned. Preoperatively, 20 (26%) patients were supported with extracorporeal membrane oxygenation and 57 (73%) patients were ventilated. Intraoperative donor blood products were required in 74 (95%) cases. Postimplantation inotropic support was required in 66 (85%) cases overall and 46 (100%) patients receiving a left VAD. Infants under 12 months were more likely to require preoperative extracorporeal membrane oxygenation (42% vs 19%), have femoral venous access (54% vs 28%), receive an intraoperative vasoconstrictor (42% vs 24%), and have delayed sternal closure (63 vs 22%). Mortality was higher in patients under 12 months (25% vs 19%) and in patients with congenital heart disease (25% vs 20%). CONCLUSIONS: Children undergoing VAD implantation require high levels of preoperative organ support, high-dose intraoperative inotropic support, and high-volume blood transfusion. Children under 12 months and those with congenital heart disease are particularly challenging for anesthesiologists and have worse overall outcomes.

HeartWare Explant After Recovery 6 Years After Implant in a 3-Year-Old Child: Has the Game Changed?

The use of continuous-flow mechanical circulatory support in preschool children remains anecdotal. This case report describes the sequel to the implantation with a HeartWare HVAD system (Medtronic, Minneapolis, MN) in a 3-year old child. A 3-year-old boy with myocarditis-related cardiomyopathy underwent implantation with a HeartWare device. After an uncomplicated postoperative course, the patient was discharged home. Serial echocardiography showed progressive left ventricular recovery. After 6 months, the device was decommissioned, and the outflow graft was tied off. Six years after insertion, the device was explanted uneventfully. The HeartWare ventricular assist device offers viable long-term mechanical circulatory support in selected children that results in sustainable care and good quality of life.

Improving outcomes for transplantation in failing Fontan-what is the next target?

Objective: To identify the key contributors to postoperative mortality in patients undergoing orthotopic heart transplantation (OHT) for late Fontan failure. Methods: This retrospective review of failing Fontan patients who underwent OHT in our tertiary care center between 2007 and 2019 included adult patients with congenital heart disease and single ventricle physiology who were palliated with a Fontan circulation for >1 year. We excluded patients undergoing combined heart-liver transplantation. Results: The study cohort comprised 31 patients, including 18 males (58.1%), with a mean weight of 58.4 kg, median age at Fontan of 6.9 years (interquartile range [IQR], 2-38 years), and a median age at OHT of 27.1 years (IQR, 16.7-53.3). Almost all (93.5%) of the patients were in New York Heart Association class III-IV, and the majority (74.2%) were in Interagency Registry for Mechanically Assisted Circulatory Support class 3. Overall survival at 30 days, 1 year, and 5 years after OHT was 81%, 71%, and 67%, respectively. Major intraoperative bleeding was associated with increased mortality after OHT (odds ratio, 30; 95% confidence interval, 2.8-322; P = .002). Neither preoperative systemic ventricular function nor the development of primary graft dysfunction (PGD) was significantly associated with postoperative death. Nevertheless, PGD determined significant morbidity of this population. Conclusions: In our cohort, major intraoperative bleeding was the key factor associated with mortality after OHT for late Fontan failure. Novel strategies for the prevention and management of postoperative bleeding will improve outcomes in this group of patients.

A cell atlas of human thymic development defines T cell repertoire formation.

The thymus provides a nurturing environment for the differentiation and selection of T cells, a process orchestrated by their interaction with multiple thymic cell types. We used single-cell RNA sequencing to create a cell census of the human thymus across the life span and to reconstruct T cell differentiation trajectories and T cell receptor (TCR) recombination kinetics. Using this approach, we identified and located in situ CD8αα+ T cell populations, thymic fibroblast subtypes, and activated dendritic cell states. In addition, we reveal a bias in TCR recombination and selection, which is attributed to genomic position and the kinetics of lineage commitment. Taken together, our data provide a comprehensive atlas of the human thymus across the life span with new insights into human T cell development.

Paediatric donation after circulatory determined death heart transplantation using donor normothermic regional perfusion and ex situ heart perfusion: A case report.

This is a report of a unique DCD paediatric heart transplant whereby normothermic regional perfusion was used to assess DCD heart function after death followed by ex situ heart perfusion of the graft during transportation from donor to recipient hospitals. The DCD donor was a 9-year-old boy weighing 84 kg. The recipient was 7-year-old boy with failing Fontan circulation and weighed 23 kg. It was an ABO-compatible heart transplantation. The DCD heart was reperfused and assessed using normothermic regional perfusion followed by portable ex situ heart perfusion during transportation. The orthotopic heart transplantation was successful with good graft function and no evidence of rejection on endomyocardial biopsy at 30 days post-transplant. At 1-year follow-up, excellent graft function is maintained, and he is attending school with a good quality of life. DCD heart transplantation in children is a promising solution to reducing paediatric waiting times. The case demonstrates the feasibility of using normothermic regional perfusion in the donor and ex situ heart perfusion during graft transportation. This combination allowed a functional assessment whilst minimizing warm ischaemia resulting in a successful outcome. More research and long-term follow-up are needed in order to benefit from the huge potential that paediatric DCD heart transplantation has to offer.

Outcome following heart transplant assessment in adults with congenital heart disease.

OBJECTIVES: Adults with congenital heart disease (ACHD) are a growing group with end-stage heart failure. We aim to describe the outcomes of ACHD patients undergoing assessment for orthotopic heart transplant (OHT). METHODS: Case notes of consecutive ACHD patients (>16 years) assessed for OHT between 2000 and 2016 at our centre were reviewed. Decision and outcome were reported as of 2017. Data were analysed in three groups: systemic left ventricle (LV), systemic right ventricle (RV) and single ventricle (SV). RESULTS: 196 patients were assessed (31.8 years, 27% LV, 29% RV, 44% SV). 89 (45%) patients were listed for OHT and 67 (34%) were transplanted. 41 (21%) were unsuitable or too high risk and 36 (18%) were too well for listing. Conventional surgery was undertaken in 13 (7%) and ventricular assist device in 17 (9%) with 7 (4%) bridged to candidacy. Survival from assessment was 84.2% at 1 year and 69.7% at 5 years, with no difference between groups. Patients who were considered unsuitable for OHT (HR 11.199, p<0.001) and listed (HR 3.792, p=0.030) were more likely to die than those who were considered too well. Assessments increased over the study period. CONCLUSIONS: The number of ACHD patients assessed for OHT is increasing. A third are transplanted with a small number receiving conventional surgery. Those who are unsuitable have a poor prognosis.

Closing the gap in paediatric ventricular assist device therapy with the Berlin Heart EXCOR® 15-ml pump.

OBJECTIVES: The Berlin Heart EXCOR ® (EXCOR) paediatric ventricular assist device is used worldwide for mechanical support of infants and small children with end-stage heart failure. A clinically important gap between the smallest EXCOR blood pump (10 ml) and the next larger size (25 ml) limited the choice of pump size in patients with a body surface area (BSA) between 0.33 and 0.5 m 2 . We present the first clinical experience from the early product surveillance (EPS) of the new EXCOR 15-ml blood pump. METHODS: After CE and U.S. Food and Drug Administration approval in January 2013, 20 patients with a mean age of 1.6 years (range 0.5-3.5 years) and a mean BSA of 0.45 m 2 (range 0.33-0.59 m 2 ) were enrolled in the EPS. The main diagnosis was idiopathic cardiomyopathy in 13 patients; the majority ( n = 16) of children were in INTERMACS level 1 or 2. Data from high-volume paediatric transplant centres were collected prospectively for a defined follow-up period of 60 days after device implantation. RESULTS: Mean time on the EXCOR 15-ml blood pump was 43 days; the survival rate was 100% at the end of the EPS period. Seven patients underwent a heart transplant from the device; 2 children were weaned; and 11 patients remained on support. Infection of cannula exit sites occurred in 3 patients. Two patients had minor thromboembolic strokes but made a complete neurological recovery. CONCLUSIONS: The new EXCOR 15-ml blood pump demonstrated optimal ventricular assist device support of children with a BSA of 0.33-0.5 m 2 .

Comparison of paracorporeal and continuous flow ventricular assist devices in children: preliminary results.

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.

Current management of double-outlet left ventricle: towards biventricular repair in infancy.

BACKGROUND: Double-outlet left ventricle (DOLV) is an extremely rare type of ventriculoarterial connection, mostly reported anecdotally with wide variations in morphology, surgical strategy and clinical outcome. The aim of the present study was to define current morbidity and mortality of biventricular repair and discuss surgical management in infancy. METHODS: Inclusion criteria were English literature publication; balanced ventricular development and completion of biventricular repair. Institutional experience was also reported. Study end points were survival and freedom from cardiovascular events. Univariate analysis to identify risk factors for complications was performed. RESULTS: During a 45-year interval, 37 cases of DOLV were identified. Median age at repair was 48 months (range 0.3-336); seven patients were infants. Extracardiac repair was performed in 21 (57%) patients, right ventricular outflow patch in seven, pulmonary translocation in six, and intraventricular repair in three. There were three (8%) hospital deaths, due to right heart failure, and seven (21%) patients experienced 10 perioperative complications. Univariate analysis showed year of operation (P = 0.02) and outflow patch technique (P = 0.003) as risk factors for hospital morbidity and mortality. During a median follow-up of 26 months (range 1-192), there was one death and seven reoperations. Ten-year survival and freedom from adverse cardiovascular events were 87 ±â€Š6 and 70 ±â€Š10%, respectively. CONCLUSION: Although seldom reported in the past, repair of DOLV in infancy does not increase surgical risk. Early and late morbidity are mostly due to right heart complications and more common after right ventricular outflow patch repair. Paucity of follow-up information may underestimate actual late adverse event rate.

Hybrid transatrial stent insertion for left atrial decompression in hypoplastic left heart syndrome with intact atrial septum.

OBJECTIVES: To describe a novel strategy for the management of infants with hypoplastic left heart syndrome (HLHS) and intact atrial septum. Antenatally diagnosed infants are delivered in cardiac theatre and immediate left atrial decompression (LAD) using hybrid transatrial stent insertion (HTSI) via sternotomy is carried out. BACKGROUND: HLHS with intact atrial septum is a condition incompatible with life following placental separation. Despite a number of reported strategies the survival of these infants remains much worse than those born with an adequate atrial communication. Immediate postnatal LAD is mandatory to allow consideration of active treatment. METHODS: Single institution retrospective review of intention to carry out HTSI for LAD in infants with an antenatal diagnosis of HLHS intact atrial septum. RESULTS: Two patients were delivered by planned caesarean section and transferred immediately to the prepared team in the adjacent cardiothoracic theatre. Birth weights were 3.2 Kg and 2.96 Kg. Clinical condition was poor with mean arterial PaO2 2.8kPa intubated with 100% inspired oxygen. HTSI was performed using premounted 7 × 17 mm stents (Visi-Pro™, eV3 Endovascular, Plymouth, MN). Mean arterial PaO2 improved to 6.2 kPa. Mean time from surgical incision to LAD was 26 min. Bilateral pulmonary artery bands (BPAB) were then placed. No procedural complications occurred and both patients underwent subsequent surgical stage 1 Norwood at 6 and 10 days. CONCLUSIONS: In this high-risk anatomical substrate, careful planning from accurate fetal diagnosis underpins the success of initial management. This early experience suggests that HTSI offers rapid and successful postnatal LAD with no procedural morbidity facilitating successful subsequent palliation. © 2015 Wiley Periodicals, Inc.

Management of the failing Fontan.

With and increasing number of early survivors after the palliation of the single ventricle physiology there is a burgeoning Fontan population worldwide that will pose unique challenges because of the inevitable sequelae related to the absence of the alleged "needless" sub-pulmonic ventricle. The increasing number and older-age single-ventricle patients highlights the results of successful contemporary surgical palliation in children, leading to the development of an adult single-ventricle population with unpredictable socio-economic and health service impacts. The wide variability in clinical status of patients with Fontan circulation reflects not only the broadened spectrum of morphological substrates involved, but also the evolving surgical techniques during the last four decades. This has come in the wake of a gradual understanding of an incredibly tricky physiology. The magnitude of the disease, the physio-pathological mechanisms, and the therapeutic options to optimize the "failing Fontan" status and to delay the irreversible deterioration of "Fontan failure" condition are discussed in this review.

Outcome of mechanical cardiac support in children using more than one modality as a bridge to heart transplantation.

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.