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Background Since 1998, there has been a national programme for paediatric heart transplantations (HT) in the Netherlands. In this study, we investigated waiting list mortality, survival post-HT, the incidence of common complications, and the patients’ functional status during follow-up. Methods All children listed for HT from 1998 until October 2020 were included. Follow-up lasted until 1 January 2021. Data were collected from the patient charts. Survival, post-operative complications as well as the functional status (Karnofsky/Lansky scale) at the end of follow-up were measured. Results In total, 87 patients were listed for HT, of whom 19 (22%) died while on the waiting list. Four patients were removed from the waiting list and 64 (74%) underwent transplantation. Median recipient age at HT was 12.0 (IQR 7.2–14.4) years old; 55% were female. One-, 5‑, and 10-year survival post-HT was 97%, 95%, and 88%, respectively. Common transplant-related complications were rejections (50%), Epstein-Barr virus infections (31%), cytomegalovirus infections (25%), post-transplant lymphoproliferative disease (13%), and cardiac allograft vasculopathy (13%). The median functional score (Karnofsky/Lansky scale) was 100 (IQR 90–100). Conclusion Children who undergo HT have an excellent survival rate up to 10 years post-HT. Even though complications post-HT are common, the functional status of most patients is excellent. Waiting list mortality is high, demonstrating that donor availability for this vulnerable patient group remains a major limitation for further improvement of outcome.

Mechanical device malfunction remains a known issue in left ventricular assist devices (LVADs). We investigated the incidence of mechanical device malfunction in the HeartMate II and HeartMate 3 LVADs. We conducted a retrospective study of all HeartMate II and HeartMate 3 LVADs implanted in our center. We evaluated major malfunction, potential major malfunction, minor malfunction, and need of device exchange. In total, 163 patients received an LVAD; in 63 (39%) a HeartMate II, and in 100 (61%) a HeartMate 3, median support time of respectively 24.6 months (interquartile range [IQR]: 32.4) and 21.1 months [IQR: 27.2]. Mechanical device malfunction, consisting of both major and potential major malfunction, occurred significantly less in the HeartMate 3 patients with a hazard ratio (HR) of 0.37 (95% confidence interval [CI]: 0.15-0.87, p = 0.022). Major malfunction alone occurred significantly less in HeartMate 3 patients with a HR of 0.18 (95% CI: 0.05-0.66, p = 0.009). HeartMate 3 patients had a significantly decreased hazard of a pump or outflow graft exchange (HR 0.13, 95% CI: 0.08-0.81, p = 0.008). System controller defects occurred significantly less in HM 3 patients (p = 0.007), but battery-clips defects occurred significantly more in HM 3 patients (p = 0.039). Major device malfunction including pump or outflow graft exchange occurred significantly less in HeartMate 3 compared to HeartMate II, while minor malfunctions were similar. Periodical assessment of the technical integrity of the device remains necessary during long-term LVAD support.

Objectives: New temperature management concepts of moderate and mild hypothermic circulatory arrest during aortic arch surgery have gained weight over profound cooling. Comparisons of all temperature levels have rarely been performed. We performed direct and indirect comparisons of deep hypothermic circulatory arrest (DHCA) (≤20°C), moderate hypothermic circulatory arrest (MHCA) (20.1–25°C), and mild hypothermic circulatory arrest (mild HCA) (≥25.1°C) in a network meta-analysis. Methods: The literature was systematically searched for all papers published through February 2022 reporting on clinical outcomes after aortic arch surgery utilizing DHCA, MHCA and mild HCA. The primary outcome was operative mortality. The secondary outcomes were postoperative stroke and acute kidney failure (AKI). Results: A total of 34 studies were included, with a total of 12,370 patients. DHCA was associated with significantly higher postoperative incidence of stroke when compared with MHCA (odds ratio [OR], 1.46, 95% confidence interval [CI], 1.19–1.78) and mild HCA: (OR, 1.50, 95% CI, 1.14–1.98). Furthermore, DHCA and MHCA were associated with higher operative mortality when compared with mild HCA (OR 1.71, 95% CI, 1.23–2.39 and OR 1.50, 95% CI, 1.12–2.00, respectively). Separate analysis of randomized and propensity score matched studies showed sustained increased risk of stroke with DHCA in contrast to MHCA and mild HCA (OR, 1.61, 95% CI, 1.18–2.20, p value =.0029 and OR, 1.74, 95% CI, 1.09–2.77, p value =.019). Conclusions: In the included studies, the moderate to mild hypothermia strategies were associated with decreased operative mortality and the risk of postoperative stroke. Large-scale prospective studies are warranted to further explore appropriate temperature management for the treatment of aortic arch pathologies.

Background and Objective: The cardiac autonomic nervous system (CANS) plays an important role in the pathophysiology of atrial fibrillation (AF). Cardiovascular disease can cause an imbalance within the CANS, which may contribute to the initiation and maintenance of AF. Increased understanding of neuromodulation of the CANS has resulted in novel emerging therapies to treat cardiac arrhythmias by targeting different circuits of the CANS. Regarding AF, neuromodulation therapies targeting the vagus nerve have yielded promising outcomes. However, targeting the vagus nerve can be both pro-arrhythmogenic and anti-arrhythmogenic. Currently, these opposing effects of vagus nerve stimulation (VNS) have not been clearly described. The aim of this review is therefore to discuss both pro-arrhythmogenic and anti-arrhythmogenic effects of VNS and recent advances in clinical practice and to provide future perspectives for VNS to treat AF. Materials and Methods: A comprehensive review of current literature on VNS and its pro-arrhythmogenic and anti-arrhythmogenic effects on atrial tissue was performed. Both experimental and clinical studies are reviewed and discussed separately. Results: VNS exhibits both pro-arrhythmogenic and anti-arrhythmogenic effects. The anatomical site and stimulation settings during VNS play a crucial role in determining its effect on cardiac electrophysiology. Since the last decade, there is accumulating evidence from experimental studies and randomized clinical studies that low-level VNS (LLVNS), below the bradycardia threshold, is an effective treatment for AF. Conclusion: LLVNS is a promising novel therapeutic modality to treat AF and further research will further elucidate the underlying anti-arrhythmogenic mechanisms, optimal stimulation settings, and site to apply LLVNS.

OBJECTIVES In complex double outlet right ventricle (DORV) patients, the optimal surgical approach may be difficult to assess based on conventional two-dimensional (2D) ultrasound (US) and computed tomography (CT) imaging. The aim of this study is to assess the added value of 3D printed and 3D Virtual Reality (VR) models of the heart used for surgical planning in DORV patients, supplementary to the gold standard 2D imaging modalities. METHODS Five patients with different DORV-subtypes and high-quality CT scans were selected retrospectively. 3D prints and 3D-VR models were created. Twelve congenital cardiac surgeons and paediatric cardiologists, from three different hospitals, were shown 2D-CT first, after which they assessed the 3D print and 3D-VR models in random order. After each imaging method, a questionnaire was filled in on the visibility of essential structures and the surgical plan. RESULTS Spatial relationships were generally better visualized using 3D methods (3D printing/3D-VR) than in 2D. The feasibility of VSD patch closure could be determined best using 3D-VR reconstructions (3D-VR 92%, 3D print 66%, and US/CT 46%, P CONCLUSIONS This study shows that both 3D printing and 3D-VR have additional value for cardiac surgeons and cardiologists over 2D imaging, because of better visualization of spatial relationships. As a result, the proposed surgical plans based on the 3D visualizations matched the actual performed surgery to a greater extent.

Background Nowadays, more than 90% of patients with congenital heart disease (CHD) reach adulthood. However, long-term impact on neurodevelopment and executive functioning in adults with CHD are not completely understood. Purpose To investigate the self- and informant-reported executive functioning in adults with CHD operated in childhood. Material and methods Longitudinal study of a cohort of patients (n = 194, median age: 49.9 [46.1–53.8]) who were operated in childhood (< 15 years old) between 1968 and 1980 (median follow-up time: 45 [40–53] years) for one of the following diagnoses: atrial septal defect (ASD), ventricular septal defect (VSD), pulmonary stenosis (PS), tetralogy of Fallot (ToF) or transposition of the great arteries (TGA). Behavior Rating Inventory of Executive Function-Adult version (BRIEF-A) questionnaire was used to assess self- and informant-reported executive functioning. Results 40–53 years after surgery, the CHD group did show significantly better executive functioning compared to the norm data. No significant difference was found between mild CHD (ASD, VSD and PS) and moderate/severe CHD (ToF and TGA). Higher education, NYHA class 1 and better exercise capacity were associated with better self-reported executive functioning, whereas females or patients taking psychiatric or cardiac medications reported worse executive functioning. Conclusions Our findings suggest favorable outcomes (comparable to normative data) regarding executive functioning in adults with CHD, both self- and informant-reported. However, further study is warranted to explore more in detail the different cognitive domains of executive functioning in these patients. Graphical abstract

Purpose Multiple randomized controlled trials have presented SGLT2 inhibitors (SGLT2i) as novel pharmacological therapy for patients with heart failure, resulting in reductions in hospitalization for heart failure and mortality. Given the absence of SGLT2 receptors in the heart, mechanisms of direct cardioprotective effects of SGLT2i are complex and remain to be investigated. In this study, we evaluated the direct biomechanical effects of SGLT2i empagliflozin on isolated myocardium from end-stage heart failure patients. Methods Ventricular tissue biopsies obtained from 7 patients undergoing heart transplantation or ventricular assist device implantation surgery were cut into 27 living myocardial slices (LMS) and mounted in custom-made cultivation chambers with mechanical preload and electrical stimulation, resulting in cardiac contractions. These 300 µm thick LMS were subjected to 10 µM empagliflozin and with continuous recording of biomechanical parameters. Results Empagliflozin did not affect the maximum contraction force of the slices, however, increased total contraction duration by 13% (p = 0.002) which was determined by prolonged time to peak and time to relaxation (p = 0.009 and p = 0.003, respectively). Conclusion The addition of empagliflozin to LMS from end-stage heart failure patients cultured in a biomimetic system improves contraction and relaxation kinetics by increasing total contraction duration without diminishing maximum force production. Therefore, we present convincing evidence that SGLT2i can directly act on the myocardium in absence of systemic influences from other organ systems.

BACKGROUNDSex does have an effect on disease perception and outcomes after cardiac surgery.OBJECTIVESThe aim of this study was to quantify the differences in cardiovascular risk profiles within an age-matched cohort and assess the long-term survival differences in males and females who underwent surgical aortic valve replacement (SAVR) with or without concomitant coronary artery bypass surgery.METHODSAll-comers patients who underwent SAVR with or without coronary artery bypass surgery were included. Characteristics, clinical features and survival up to 30 years were compared between female and male patients. Propensity matching and age matching using propensity scores were used to compare both groups.RESULTSDuring the total study period between 1987 and 2017, there were 3462 patients {mean age 66.8 [standard deviation (SD): 11.1] years, 37.1% female} who underwent SAVR with or without coronary artery bypass surgery at our institution. In general, female patients were older than male patients (69.1 (SD : 10.3) versus 65.5 (SD : 11.3), respectively). In the age-matched cohort, female patients were less likely to have multiple comorbidities and undergo concomitant coronary artery bypass surgery. Twenty-year survival following the index procedure was higher in age-matched female patients (27.1%) compared to male patients (24.4%) in the overall cohort (P = 0.018).CONCLUSIONSSubstantial sex differences in cardiovascular risk profile exist. However, when SAVR with or without coronary artery bypass surgery is performed, extended long-term mortality is comparable between males and females. More research regarding sex-dimorphic mechanisms of aortic stenosis and coronary atherosclerosis would promote more awareness in terms of sex-specific risk factors after cardiac surgery and contribute to more guided personalized surgery in the future.

OBJECTIVES The Berlin Heart EXCOR (BHE) offers circulatory support across all paediatric ages. Clinically, the necessary care and the outcomes differ in various age groups. The EUROMACS database was used to study age- and size-related outcomes for this specific device. METHODS All patients RESULTS In total, 303 patients were included [mean age: 2.0 years (interquartile range: 0.6–8.0, males: 48.5%)]. Age and BSA were not significantly associated with mortality (n = 74, P = 0.684, P = 0.679). Factors associated with a transplant (n = 175) were age (hazard ratio 1.07, P = 0.006) and aetiology other than congenital heart disease (hazard ratio 1.46, P = 0.020). Recovery rates (n = 42) were highest in patients with a BSA of CONCLUSIONS Mortality rates in Berlin Heart-supported patients cannot be predicted by age or BSA. Recovery rates are remarkably high in the smallest patient category (BSA

Early post-operative sinus node dysfunction (SND) is common in paediatric patients undergoing surgical correction of congenital heart defects (CHD). At present, the pathophysiology of these arrhythmias is incompletely understood. In this case series, we present three paediatric patients in whom we performed intraoperative epicardial mapping and who developed early post-operative SND. All patients had either an inferior or multiple sinoatrial node (SAN) exit sites, in addition to extensive conduction disorders at superior and inferior right atrium. Our findings contribute to the hypothesis that pre-existing alterations in SAN exit sites in combination with atrial conduction disorders may predispose paediatric patients with CHD for early post-operative SND. Such insights in the development of arrhythmias are crucial as it may be the first step in identifying high-risk patients.

OBJECTIVES When surgical resection is indicated for a congenital lung abnormality (CLA), lobectomy is often preferred over segmentectomy, mostly because the latter is associated with more residual disease. Presumably, this occurs in children because sublobar surgery often does not adhere to anatomical borders (wedge resection instead of segmentectomy), thus increasing the risk of residual disease. This study investigated the feasibility of identifying eligible cases for anatomical segmentectomy by combining virtual reality (VR) and artificial intelligence (AI). METHODS Semi-automated segmentation of bronchovascular structures and lesions were visualized with VR and AI technology. Two specialists independently evaluated via a questionnaire the informative value of regular computed tomography versus three-dimensional (3D) VR images. RESULTS Five asymptomatic, non-operated cases were selected. Bronchovascular segmentation, volume calculation and image visualization in the VR environment were successful in all cases. Based on the computed tomography images, assignment of the CLA lesion to specific lung segments matched between the consulted specialists in only 1 out of the cases. Based on the three 3D VR images, however, the localization matched in 3 of the 5 cases. If the patients would have been operated, adding the 3D VR tool to the preoperative workup would have resulted in changing the surgical strategy (i.e. lobectomy versus segmentectomy) in 4 cases. CONCLUSIONS This study demonstrated the technical feasibility of a hybridized AI–VR visualization of segment-level lung anatomy in patients with CLA. Further exploration of the value of 3D VR in identifying eligible cases for anatomical segmentectomy is therefore warranted.

Background The objective of this international multicenter study was to investigate both early and late outcomes of cardiac resynchronization therapy (CRT) in patients with a systemic right ventricle (SRV) and to identify predictors for congestive heart failure readmissions and mortality. Methods and Results This retrospective international multicenter study included 13 centers. The study population comprised 80 adult patients with SRV (48.9% women) with a mean age of 45±14 (range, 18–77) years at initiation of CRT. Median follow‐up time was 4.1 (25th–75th percentile, 1.3–8.3) years. Underlying congenital heart disease consisted of congenitally corrected transposition of the great arteries and dextro‐transposition of the great arteries in 63 (78.8%) and 17 (21.3%) patients, respectively. CRT resulted in significant improvement in functional class (before CRT: III, 25th–75th percentile, II–III; after CRT: II, 25th–75th percentile, II–III; P =0.005) and QRS duration (before CRT: 176±27; after CRT: 150±24 milliseconds; P =0.003) in patients with pre‐CRT ventricular pacing who underwent an upgrade to a CRT device (n=49). These improvements persisted during long‐term follow‐up with a marginal but significant increase in SRV function (before CRT; 30%, 25th–75th percentile, 25–35; after CRT: 31%, 25th–75th percentile, 21–38; P =0.049). In contrast, no beneficial change in the above‐mentioned variables was observed in patients who underwent de novo CRT (n=31). A quarter of all patients were readmitted for heart failure during follow‐up, and mortality at latest follow‐up was 21.3%. Conclusions This international experience with CRT in patients with an SRV demonstrated that CRT in selected patients with SRV dysfunction and pacing‐induced dyssynchrony yielded consistent improvement in QRS duration and New York Heart Association functional status, with a marginal increase in SRV function.

BackgroundThe ligament of Marshall (LOM) may play a role in the pathophysiology of several tachyarrhythmias and accurate electrophysiological localization of this structure is crucial for effective ablation therapy. This study therefore quantifies electrophysiological properties of the LOM, and identifies which electrogram (EGM) recording (uni- or bipolar) and processing technologies [local activation time (LAT) and/or voltage mapping] are most suitable for accurate localization of the LOM.MethodsThe LOM was electrophysiologically identified in 19 patients (mean age 66 ± 14 years; 12 male) undergoing elective cardiac surgery using intra-operative high-density epicardial mapping, to quantify and visualize EGM features during sinus rhythm.ResultsOnly a third of LOM potentials that were visualized using unipolar EGMs, were still visible in bipolar activation maps. Unipolar LOM potentials had lower voltages (P50: LOM: 1.51 (0.42–4.29) mV vs. left atrium (LA): 8.34 (1.50–17.91) mV, p < 0.001), less steep slopes (P50: LOM: –0.48 (–1.96 to –0.17) V/s vs. LA: –1.24 (–2.59 to –0.21) V/s, p < 0.001), and prolonged activation duration (LOM: 20 (7.5–30.5) ms vs. LA: 16.5 (6–28) ms, p = 0.008) compared to LA potentials. Likewise, bipolar LOM voltages were also smaller (P50: LOM: 1.54 (0.48–3.28) mV vs. LA: 3.12 (0.50–7.19) mV, p < 0.001).ConclusionThe LOM was most accurately localized in activation and voltage maps by using unipolar EGMs with annotation of primary deflections in case of single potentials and secondary deflections in case of double or fractionated potentials.

Background Bachmann's Bundle (BB) is the main preferential route for interatrial conduction and plays an important role in initiation and perpetuation of atrial tachyarrhythmia such as atrial fibrillation (AF). Especially in patients with inter- and intra-atrial conduction disorders, the conventional site for placement of right atrial (RA) pacing lead in the RA appendage (RAA) has been questioned as it results in a higher incidence of AF. However, it remains unknown how pacing from various sites affects conduction at BB. Purpose To examine the impact of programmed stimulation from the left and right atrium on activation and electrophysiological features of BB. Methods Intra-operative high-resolution epicardial mapping of BB was performed during sinus rhythm (SR) and programmed stimulation in 32 patients (29 male, 65±10 years). Programmed stimulation included a fixed rate sequence of average SR cycle length minus 50 ms provided from the RAA, junction of the RA with inferior caval vein (RAinf) and left atrial appendage (LAA). Electrophysiological features included unipolar voltage, conduction velocity, potential type (single, short/long double and fractionated) and conduction delay/block (CDCB). Results The average cycle length during SR was 962 [869–1085] ms. Pacing from RAA resulted in comparable activation patterns as during SR. As expected, pacing from LAA resulted in left-to-right conduction across BB. However, during pacing from RAinf in most patients activation started in the centre of BB. Compared to SR, the least amount of differences in electrophysiological features was found during pacing from the RAA, followed by pacing from RAinf and LAA. Unipolar voltages, conduction velocity and potential type distribution were similar. However, during pacing from RAinf, total activation time decreased compared to SR (44 [38–62] ms vs 60 [51–67] ms, P=0.012), while total activation time increased by pacing from LAA (66 [60–72] ms, P=0.020). The total activation time during pacing from RAinf was lower compared to LAA (P Conclusions Pacing from the inferior RA results in a remarkable decrease of total activation time and conduction disorders compared to pacing from the LAA or RAA. As specific patients may benefit more from inferior RA pacing, locations for pacing lead placement should be individualized. Funding Acknowledgement Type of funding sources: None.

Background For children with congenital heart disease (CHD), subtle neuropsychological deficits have been reported. However, very little is known about executive functioning in their adulthood. Purpose To investigate the self- and informant-reported executive functioning in adults with CHD operated in childhood ( Material and methods A cohort study of 194 patients (age 50 [46–54] years), operated in childhood between 1968 and 1980 for one of the following diagnosis: atrial septal defect (ASD), ventricular septal defect (VSD), pulmonary stenosis (PS), tetralogy of Fallot (ToF) or transposition of the great arteries (TGA), were evaluated 40–53 years after surgery. The “Behavior Rating Inventory of Executive Functions – Adult version” (BRIEF-A) questionnaire was used to assess self- and informant-reported executive functioning and compared to the general population. Results The CHD group did not show worse executive functioning compared to normative data. In addition, no significant difference was found between simple CHD (ASD, VSD and PS) and moderate/complex CHD (ToF and TGA). Higher education and NYHA class 1 were associated with better self-reported executive functioning, while male patients and patients taking psychiatric or cardiac medications reported worse executive functioning. Conclusions Overall, our findings suggest reassuring outcomes regarding executive functioning in adults with CHD. However, these findings should be confirmed with neuropsychological assessment studies. Funding Acknowledgement Type of funding sources: Foundation. Main funding source(s): Thorax Foundation

Background Despite improvement in (timing of) of intervention or surgery, atrial fibrillation (AF) still occurs more often and at a younger age in patients with congenital heart disease (CHD) than in the general population. This suggests that in this population atrial conduction is already affected in an early stage. Purpose To unmask early, enhanced non-uniform anisotropic atrial conduction in pediatric patients with CHD by examining the impact of spontaneous aberrant atrial extrasystoles (AES) on atrial conduction. Methods Twenty-one pediatric patients with various CHD (mild 43%, moderate 24% and severe 33%) (median age=1.8 years [0.2–7.3]) undergoing primary cardiac surgery were included for an intra-operative epicardial mapping of both atria, including Bachmann's bundle. Conduction velocity (CV), localized areas of conduction delay and/or block (CD, CB and continuousCDCB) and unipolar electrogram (EGM) characteristics (voltages and amount of fractionated potentials) were quantified during sinus rhythm (SR) and compared with corresponding AES beats. Results During 41 AES median unipolar voltage and CV decreased compared to corresponding SR beats (7.08 [5.50–9.10] mV vs. 5.71 [3.95–7.48] mV, p0.05). Conclusions AES in pediatric patients with CHD cause localized areas of conduction slowing and block, potentials with lower unipolar voltages and a higher degree of fractionation compared to SR potentials, independent of its prematurity. Our findings indicate that in pediatric patients with CHD, early, enhanced non-uniform anisotropic atrial conduction is already present and can be unmasked by AES. These insights are the first step in further understanding the time course of AF in CHD patients. Funding Acknowledgement Type of funding sources: Public grant(s) – National budget only. Main funding source(s): CVON-AFFIPNWO-Vidi

Summary OBJECTIVES The emergence of big cardio-thoracic surgery datasets that include not only short-term and long-term discrete outcomes but also repeated measurements over time offers the opportunity to apply more advanced modelling of outcomes. This article presents a detailed introduction to developing and interpreting linear mixed-effects models for repeated measurements in the setting of cardiothoracic surgery outcomes research. METHODS A retrospective dataset containing serial echocardiographic measurements in patients undergoing surgical pulmonary valve replacement from 1986 to 2017 in Erasmus MC was used to illustrate the steps of developing a linear mixed-effects model for clinician researchers. RESULTS Essential aspects of constructing the model are illustrated with the dataset including theories of linear mixed-effects models, missing values, collinearity, interaction, nonlinearity, model specification, results interpretation and assumptions evaluation. A comparison between linear regression models and linear mixed-effects models is done to elaborate on the strengths of linear mixed-effects models. An R script is provided for the implementation of the linear mixed-effects model. CONCLUSIONS Linear mixed-effects models can provide evolutional details of repeated measurements and give more valid estimates compared to linear regression models in the setting of cardio-thoracic surgery outcomes research.

IntroductionThis study aims to provide an overview of outcomes after right ventricular outflow tract (RVOT) reconstruction using different valve substitutes in different age groups for different indications.MethodsThe literature was systematically searched for articles published between January 2000 and June 2021 reporting on clinical and/or echocardiographic outcomes after RVOT reconstruction with valve substitutes. A random-effects meta-analysis was conducted for outcomes, and time-related outcomes were visualized by pooled Kaplan–Meier curves. Subgroup analyses were performed according to etiology, implanted valve substitute and patient age.ResultsTwo hundred and seventeen articles were included, comprising 37,078 patients (age: 22.86 ± 11.29 years; 31.6% female) and 240,581 patient-years of follow-up. Aortic valve disease (Ross procedure, 46.6%) and Tetralogy of Fallot (TOF, 27.0%) were the two main underlying etiologies. Homograft and xenograft accounted for 83.7 and 32.6% of the overall valve substitutes, respectively. The early mortality, late mortality, reintervention and endocarditis rates were 3.36% (2.91–3.88), 0.72%/y (95% CI: 0.62–0.82), 2.62%/y (95% CI: 2.28–3.00), and 0.38%/y (95%CI: 0.31–0.47) for all patients. The early mortality for TOF and truncus arteriosus (TA) were 1.95% (1.31–2.90) and 10.67% (7.79–14.61). Pooled late mortality and reintervention rate were 0.59%/y (0.39–0.89), 1.41%/y (0.87–2.27), and 1.20%/y (0.74–1.94), 10.15%/y (7.42–13.90) for TOF and TA, respectively. Endocarditis rate was 0.21%/y (95% CI: 0.16–0.27) for a homograft substitute and 0.80%/y (95%CI: 0.60–1.09) for a xenograft substitute. Reintervention rate for infants, children and adults was 8.80%/y (95% CI: 6.49–11.95), 4.75%/y (95% CI: 3.67–6.14), and 0.72%/y (95% CI: 0.36–1.42), respectively.ConclusionThis study shows RVOT reconstruction with valve substitutes can be performed with acceptable mortality and morbidity rates for most patients. Reinterventions after RVOT reconstruction with valve substitutes are inevitable for most patients in their life-time, emphasizing the necessity of life-long follow-up and multidisciplinary care. Follow-up protocols should be tailored to individual patients because patients with different etiologies, ages, and implanted valve substitutes have different rates of mortality and morbidity.Systematic review registration[www.crd.york.ac.uk/prospero], identifier [CRD42021271622].

Background Ventricular performance is temporarily reduced following surgical atrial septal defect closure. Cardiopulmonary bypass and changes in loading conditions are considered important factors, but this phenomenon is incompletely understood. We aim to characterize biventricular performance following surgical and percutaneous atrial septal defect closure and to relate biomarkers to ventricular performance following intervention. Methods and Results In this multicenter prospective study, children scheduled for surgical or percutaneous atrial septal defect closure were included. Subjects were assessed preoperatively, in the second week postintervention (at 2‐weeks follow‐up), and 1‐year postintervention (1‐year follow‐up). At each time point, an echocardiographic study and a panel of biomarkers were obtained. Sixty‐three patients (median age, 4.1 [interquartile range, 3.1–6.1] years) were included. Forty‐three patients underwent surgery. At 2‐weeks follow‐up, right ventricular global longitudinal strain was decreased for the surgical, but not the percutaneous, group (−17.6±4.1 versus −27.1±3.4; P P =0.040). At 1‐year follow‐up, left ventricular performance returned to baseline, whereas right ventricular performance improved, but did not reach preintervention levels. Eight biomarkers relating to cardiovascular and immunological processes differed across study time points. Of these biomarkers, only NT‐proBNP (N‐terminal pro‐B‐type natriuretic peptide) correlated with less favorable left ventricular global longitudinal strain at 2‐weeks follow‐up. Conclusions Right, and to a lesser degree left, ventricular performance was reduced early after surgical atrial septal defect closure. Right ventricular performance at 1‐year follow‐up remained below baseline levels. Several biomarkers showed a pattern over time similar to ventricular performance. These biomarkers may provide insight into the processes that affect ventricular function. Registration URL: https://www.trialregister.nl/ ; Unique identifier: NL5129

OBJECTIVES A third paediatric report has been generated from the European Registry for Patients with Mechanical Circulatory Support (EUROMACS). The purpose of EUROMACS, which is operated by the European Association for Cardio-Thoracic Surgery, is to gather data related to durable mechanical circulatory support for scientific purposes and to publish reports with respect to the course of mechanical circulatory support therapy. Since the first report issued, efforts to increase compliance and participation have been extended. Additionally, the data provided the opportunity to analyse patients of younger age and lower weight. METHODS Participating hospitals contributed pre-, peri- and long-term postoperative data on mechanical circulatory support implants to the registry. Data for all implants in paediatric patients (, European Association for Cardio-Thoracic Surgery (EACTS), The European Association for Cardio-Thoracic Surgery (EACTS) supported this study and made resources available to execute this work.

Living myocardial slices (LMS) are beating sections of intact human myocardium that maintain 3D microarchitecture and multicellularity, thereby overcoming most limitations of conventional myocardial cell cultures. We introduce a novel method to produce LMS from human atria and apply pacing modalities to bridge the gap between in-vitro and in-vivo atrial arrhythmia studies. Human atrial biopsies from 14 patients undergoing cardiac surgery were dissected to tissue blocks of ~ 1 cm2 and cut to 300 µm thin LMS with a precision-cutting vibratome. LMS were placed in a biomimetic culture chamber, filled with standard cell culture medium, under diastolic preload (1 mN) and continuous electrical stimulation (1000 ms cycle length (CL)), resulting in 60 beating LMS. Atrial LMS refractory period was determined at 192 ± 26 ms. Fixed rate pacing with a CL of 333 ms was applied as atrial tachyarrhythmia (AT) model. This novel state-of-the-art platform for AT research can be used to investigate arrhythmia mechanisms and test novel therapies.

OBJECTIVES: Ventricular assist device support as a bridge to transplant or recovery is a well-established therapy in children on the cardiac transplant waiting list. The goal of this study was to investigate the incidence of and the associated factors for cerebrovascular accidents in paediatric patients supported by a Berlin Heart EXCOR. METHODS: All patients, European Association for Cardio-Thoracic Surgery, The European Association for Cardio-Thoracic Surgery supported this study and made resources available to execute this work. No funding was received for this work.

Background:Sinus node dysfunction (SND) and atrial tachyarrhythmias frequently co-exist in the aging patient with congenital heart disease (CHD), even after surgical correction early in life. We examined differences in electrophysiological properties of the sino-atrial node (SAN) area between pediatric and adult patients with CHD.MethodsEpicardial mapping of the SAN was performed during sinus rhythm in 12 pediatric (0.6 [0.4–2.4] years) and 15 adult (47 [40–55] years) patients. Unipolar potentials were classified as single-, short or long double- and fractionated potentials. Unipolar voltage, relative R-to-S-amplitude ratio and duration of all potentials was calculated. Conduction velocity (CV) and the amount of conduction block (CB) was calculated.ResultsSAN activity in pediatric patients was solely observed near the junction of the superior caval vein and the right atrium, while in adults SAN activity was observed even up to the middle part of the right atrium. Compared to pediatric patients, the SAN region of adults was characterized by lower CV, lower voltages, more CB and a higher degree of fractionation. At the earliest site of activation, single potentials from pediatrics consisted of broad monophasic S-waves with high amplitudes, while adults had smaller rS-potentials with longer duration which were more often fractionated.ConclusionsCompared to pediatric patients, adults with uncorrected CHD have more inhomogeneous conduction and variations in preferential SAN exit site, which are presumable caused by aging related remodeling. Long-term follow-up of these patients is essential to demonstrate whether these changes are related to development of SND and also atrial tachyarrhythmias early in life.

OBJECTIVES This retrospective study investigated the correlation between the angular position of the left ventricular assist device (LVAD) inflow cannula and relapsing low-flow alarms. METHODS Medical charts were reviewed of all patients with HeartMate 3 LVAD support for relapsing low-flow alarms. A standardized protocol was created to measure the angular position with a contrast-enhanced computed tomography scan. Statistics were done using a gamma frailty model with a constant rate function. RESULTS For this analysis, 48 LVAD-supported patients were included. The majority of the patients were male (79%) with a median age of 57 years and a median follow-up of 30 months (interquartile range: 19–41). Low-flow alarm(s) were experienced in 30 (63%) patients. Angulation towards the septal–lateral plane showed a significant increase in low-flow alarms over time with a constant rate function of 0.031 increase in low-flow alarms per month of follow-up per increasing degree of angulation (P = 0.048). When dividing this group using an optimal cut-off point, a significant increase in low-flow alarms was observed when the septal–lateral angulation was 28° or more (P = 0.001). Anterior–posterior and maximal inflow cannula angulation did not show a significant difference. CONCLUSIONS This study showed an increasing number of low-flow alarms when the degrees of LVAD inflow cannula expand towards the septal–lateral plane. This emphasizes the importance of the LVAD inflow cannula angular position to prevent relapsing low-flow alarms with the risk of diminished quality of life and morbidity.

OBJECTIVES In patients supported by a durable left ventricular assist device (LVAD), infections are a frequently reported adverse event with increased morbidity and mortality. The purpose of this study was to investigate the possible association between infections and thromboembolic events, most notable cerebrovascular accidents (CVAs), in LVAD patients. METHODS An analysis of the multicentre European Registry for Patients Assisted with Mechanical Circulatory Support was performed. Infections were categorized as VAD-specific infections, VAD-related infections and non-VAD-related infections. An extended Kaplan–Meier analysis for the risk of CVA with infection as a time-dependent covariate and a multivariable Cox proportional hazard model were performed. RESULTS For this analysis, 3282 patients with an LVAD were included with the majority of patients being male (83.1%). During follow-up, 1262 patients suffered from infection, and 457 patients had a CVA. Cox regression analysis with first infection as time-dependent covariate revealed a hazard ratio (HR) for CVA of 1.90 [95% confidence interval (CI): 1.55–2.33; P CONCLUSIONS Infection during LVAD support is associated with an increased risk of developing an ischaemic or haemorrhagic CVA, particularly in the setting of VAD-related or VAD-specific infections. This suggests the need of a stringent anticoagulation management and adequate antibiotic treatment during an infection in LVAD-supported patients.