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Competing Interests: Competing interests: None declared.

Background and Aims: Transcatheter pulmonary valve implantation (TPVI) is indicated to treat right-ventricular outflow tract (RVOT) dysfunction related to congenital heart disease (CHD). Outcomes of TPVI with the SAPIEN 3 valve that are insufficiently documented were investigated in the EUROPULMS3 registry of SAPIEN 3-TPVI., Methods: Patient-related, procedural, and follow-up outcome data were retrospectively assessed in this observational cohort from 35 centres in 15 countries., Results: Data for 840 consecutive patients treated in 2014-2021 at a median age of 29.2 (19.0-41.6) years were obtained. The most common diagnosis was conotruncal defect (70.5%), with a native or patched RVOT in 50.7% of all patients. Valve sizes were 20, 23, 26, and 29 mm in 0.4%, 25.5%, 32.1%, and 42.0% of patients, respectively. Valve implantation was successful in 98.5% [95% confidence interval (CI), 97.4%-99.2%] of patients. Median follow-up was 20.3 (7.1-38.4) months. Eight patients experienced infective endocarditis; 11 required pulmonary valve replacement, with a lower incidence for larger valves (P = .009), and four experienced pulmonary valve thrombosis, including one who died and three who recovered with anticoagulation. Cumulative incidences (95%CI) 1, 3, and 6 years after TPVI were as follows: infective endocarditis, 0.5% (0.0%-1.0%), 0.9% (0.2%-1.6%), and 3.8% (0.0%-8.4%); pulmonary valve replacement, 0.4% (0.0%-0.8%), 1.3% (0.2%-2.4%), and 8.0% (1.2%-14.8%); and pulmonary valve thrombosis, 0.4% (0.0%-0.9%), 0.7% (0.0%-1.3%), and 0.7% (0.0%-1.3%), respectively., Conclusions: Outcomes of SAPIEN 3 TPVI were favourable in patients with CHD, half of whom had native or patched RVOTs., (© The Author(s) 2023. Published by Oxford University Press on behalf of the European Society of Cardiology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Aims: Patients with systemic right ventricles are at high risk of sudden cardiac death. Arrhythmia is a significant risk factor. Routine Holter monitoring is opportunistic with poor adherence. The aim of this study was to determine if continuous rhythm monitoring with an implantable loop recorder (ILR) could allow early detection of clinically important arrhythmias., Methods and Results: Implantable loop recorder implantation was offered to patients with atrial switch repair for transposition of the great arteries. Recordings were made with symptoms or, automatically for pauses, significant bradycardia or tachycardia and reviewed by the multi-disciplinary team. Twenty-four out of 36 eligible patients underwent ILR implantation with no complication. Forty-two per cent had preserved ventricular function, 75% were NYHA functional class I, 88% had low sudden cardiac death risk, 33% had previous intra-atrial re-entrant tachycardia (IART), and none had known conduction disease. Eighteen out of 24 (75%) patients made 52 recordings (52% automated) over 39.5 months (1.6-72.5). Thirty-two out of 52 (62%) recordings in 15/24 (63%) of the cohort were clinically significant and included sinus node disease (two patients), atrioventricular block (two patients), IART (seven patients), and IART with sinus node disease or atrioventricular block (four patients). Implantable loop recorder recordings prompted medication change in 11 patients [beta-blockers (n = 9), anti-coagulation (n = 5), and stopping anti-coagulation (n = 1)] and device therapy recommendation in seven patients [five pacemakers (three: atrioventricular block) and two defibrillators]. Two patients declined intervention; one suffered an arrhythmic death. Intra-atrial re-entrant tachycardia and clinically relevant conduction disease were detected in patients irrespective of sudden cardiac death risk., Conclusion: Continuous monitoring with an ILR in patients with systemic right ventricle following atrial switch detects clinically relevant arrhythmias that impact decision-making. In this cohort, clinically relevant arrhythmias did not correlate with sudden cardiac death risk., Competing Interests: Conflict of interest: Dr Stephen Murray has received honoraria from Acutus Medical and Boston Scientific. Dr Louise Coats receives funding from the British Heart Foundation and collaborates with CardiacSense. All remaining authors have declared no conflicts of interest., (© The Author(s) 2023. Published by Oxford University Press on behalf of the European Society of Cardiology.)

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., (© The Author(s) 2022. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

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., (© 2022 Wiley Periodicals LLC.)

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., (© The Author(s) 2022. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

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.

Objective: Our goal was to present 2 decades of our experience with the Ross procedure and its sequential modifications, adopted since 2010, to improve the reoperation rate., Methods: We performed a single-centre, retrospective review of database information and medical notes about the implantation technique: the freestanding root. We compared era 1 (1997-2009) and era 2 (2010-2019)., Results: Between 1997 and 2019, a total of 214 Ross procedures were performed (71% men, median age 24 years) [interquartile range (IQR) 15-38]. Of these, 87% had various forms of congenital-dysplastic aortic valves. The median cross-clamping and bypass times were 173 (IQR 148-202) and 202 (IQR 182-244) min. The median postoperative stay was 6 days (2-77). Thirty-day mortality was 0.5%. The median follow-up time was 8.2 years (IQR 3.9-13.2). Survival at 10 and 20 years was 97% and 95%; freedom from greater than moderate aortic regurgitation or aortic valve intervention was 91% and 80%; and 93% of the patients were in New York Heart Association functional class I. Twenty (21%) patients operated on during era 1 and 6 (9%) during era 2 underwent autograft reoperations. The median follow-up time was 14.3 (IQR 11.5-17.4) and 4.8 (IQR 2.5-7) years. Freedom from autograft reoperation was 87% and 69% at 10 and 20 years, with no significant difference between eras. Freedom from homograft reoperation was 96% and 76% at 10 and 20 years. The presence of aortic regurgitation, infective endocarditis and era 1 were predictors of autograft reoperation. Male gender and era 1 were predictors of neoaortic root dilatation., Conclusions: The contemporary modified Ross procedure continues to deliver excellent results and should remain part of the strategy to treat children and young adults requiring aortic valve replacement., (© The Author(s) 2021. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

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., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2019. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Aims: Most reports on the outcome of children who present with heart failure, due to heart muscle disease, are from an era when ventricular assist devices were not available. This study provides outcome data for the current era where prolonged circulatory support can be considered for most children., Methods & Results: Data was retrieved on 100 consecutive children, who presented between 2010 - 2016, with a first diagnosis of unexplained heart failure. Hospital outcome was classified as either death, transplantation, recovery of function or persistent heart failure. Median age at presentation was 24 months and 58% were < 5 years old. Hospital mortality was 12% and 59% received a heart transplant. Most, 79%, of the transplants were carried out on patients with a device. Recovery of function was observed in 18% and 10% stabilised on oral therapy. Eighty-four percent of the deaths occurred in the <5 year old group. Shorter duration of support was associated with survival (34 days in survivors versus 106 in non-survivors, p = 0.01) and 72% were on an assist device at time of death., Conclusion: Heart failure in children who require referral to a transplant unit is a serious illness with a high chance of either transplantation or death. Modifications in assist devices will be required to improve safety, especially for children < 5 years old where the donor wait may be prolonged. The identification of children who may recover function requires further study.

Aims: Superior sinus venosus atrial septal defect (SVASD) is commonly associated with partial anomalous pulmonary venous drainage (PAPVD). We aimed to describe the first series of percutaneous SVASD and PAPVD correction using a two-step simulation for procedural planning., Methods and Results: Patients with SVASD and right PAPVD with a clinical indication for correction were selected. They underwent an ex vivo procedural simulation on a 3D-printed model followed by an in vivo simulation using balloon inflation in the targeted stent landing zone. The percutaneous procedure consisted in deploying a 10-zig custom-made covered stent in the SVC-RA junction. Five patients were referred for preprocedural evaluation and were deemed suitable for percutaneous correction. The procedure was successful in all patients with no residual interatrial shunt and successful redirection of the pulmonary venous drainage to the left atrium. At a median clinical follow-up of 8.1 months (2.6-19.8), no adverse events were noted, and all patients showed clinical improvement. During follow-up, transthoracic echocardiography and multidetector cardiac tomography in four patients or invasive angiography in one patient demonstrated a patent SVC stent, and no residual SVASD and unobstructed PV drainage in all patients., Conclusions: In selected patients using a two-stage simulation strategy, percutaneous correction of SVASD with PAPVD is feasible and safe, and led to favourable short-term outcomes.

Cardiomyopathy may have a variety of causes and may lead to significant morbidity. Often, there is no "perfect" treatment. New investigative techniques may add insight but retain the possibility of uncertainty. The distinction between restrictive cardiomyopathy and pericardial constriction may be challenging, particularly when considering the incidence of these entities. This distinction may significantly impact patient management and this is becoming increasingly important in the context of donor organ austerity. We present a case of a 17-year-old male to illustrate the overlap highlighting this debate and our subsequent management.

Background: Infants born with cardiac abnormalities causing dependence on the arterial duct for pulmonary blood flow are often palliated with a shunt usually between the subclavian artery and either pulmonary artery. A so-called modified Blalock-Taussig shunt allows progress through early life to an age and weight at which repair or further more stable palliation can be safely achieved. Modified Blalock-Taussig shunts continue to present concern for postprocedural instability and early mortality such that other alternatives continue to be explored. Duct stenting (DS) is emerging as one such alternative with potential for greater early stability and improved survival., Methods: The purpose of this study was to compare postprocedural outcomes and survival to next-stage palliative or reparative surgery between patients undergoing a modified Blalock-Taussig shunt or a DS in infants with duct-dependent pulmonary blood flow. All patients undergoing cardiac surgery and congenital interventions in the United Kingdom are prospectively recruited to an externally validated national outcome audit. From this audit, participating UK centers identified infants <30 days of age undergoing either a Blalock-Taussig shunt or a DS for cardiac conditions with duct-dependent pulmonary blood flow between January 2012 and December 31, 2015. One hundred seventy-one patients underwent a modified Blalock-Taussig shunt, and in 83 patients, DS was attempted. Primary and secondary outcomes of survival and need for extracorporeal support were analyzed with multivariable logistic regression. Longer-term mortality before repair and reintervention were analyzed with Cox proportional hazards regression. All multivariable analyses accommodated a propensity score to balance patient characteristics between the groups., Results: There was an early (to discharge) survival advantage for infants before next-stage surgery in the DS group (odds ratio, 4.24; 95% confidence interval, 1.37-13.14; P =0.012). There was also a difference in the need for postprocedural extracorporeal support in favor of the DS group (odds ratio, 0.22; 95% confidence interval, 0.05-1.05; P =0.058). Longer-term survival outcomes showed a reduced risk of death before repair in the DS group (hazard ratio, 0.25; 95% confidence interval, 0.07-0.85; P =0.026) but a slightly increased risk of reintervention (hazard ratio, 1.50; 95% confidence interval, 0.85-2.64; P =0.165)., Conclusions: DS is emerging as a preferred alternative to a surgical shunt for neonatal palliation with evidence for greater postprocedural stability and improved patient survival to destination surgical treatment., (© 2017 American Heart Association, Inc.)

Objectives: Mortality associated with the modified Blalock-Taussig shunt (MBTS) remains high despite advanced perioperative management. This study was formulated to provide data on (1) current indications, (2) outcomes, and (3) factors affecting mortality and morbidity., Design: A retrospective single center chart review identified 95 children (excluding hypoplastic left heart lesions) requiring a MBTS. Mortality and major morbidity were analyzed using the Kaplan Meier method and risk factor analysis using Cox's proportional hazard regression., Results: Median age was 8 (0-126) days, weight 3.1(1.7-5.4) kg. Seventy-three percent were neonates, 58% duct dependent and 73% had single ventricle physiology. Ninety-seven percent had a sternotomy approach for shunt placement with 70% receiving a 3.5 mm graft. Mean graft index (shunt cross sectional area [mm 2 ]/BSA [m 2 ]) was 44.39 ± 8.04 and shunt size (mm) to body weight (kg) ratio 1.1 ± 0.2. Hospital mortality was 12%, with an interval mortality of 6%. Shunt thrombosis/stenosis occurred in 23% and pulmonary over circulation in 30%, while shunt reoperation was required in 12% and catheter intervention in 8% of the cohort. At 1-year, survival was 82.0% (95% CI [72.7%, 88.4%]), and survival free of major morbidity 61.4% (95% CI [50.7%, 70.5%]). Duct dependency predisposed to mortality (P = .01, HR 6.74 [1.54, 29.53]) and composite outcome (mortality and major morbidity) (P = .04, HR 2.15, CI [1.036, 4.466]) and higher graft index to mortality (P = .005, HR 1.07 [1.02, 1.12])., Conclusions: The commonest indication for a MBTS in the current era was single ventricle palliation. Morbidity and mortality was considerable, partly explained by the higher at risk population. Alternative methods to maintain pulmonary blood flow in place of a MBTS requires further investigation., (© 2017 Wiley Periodicals, Inc.)

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., (© The Author 2016. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

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., (© 2015 Wiley Periodicals, Inc.)