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Background and Aims To explore male-female differences in aneurysm growth and clinical outcomes in a two-centre retrospective Dutch cohort study of adult patients with ascending aortic aneurysm (AscAA). Methods Adult patients in whom imaging of an AscAA (root and/or ascending: >= 40 mm) was performed between 2007 and 2022 were included. Aneurysm growth was analysed using repeated measurements at the sinuses of Valsalva (SoV) and tubular ascending aorta. Male-female differences were explored in presentation, aneurysm characteristics, treatment strategy, survival, and clinical outcomes. Results One thousand eight hundred and fifty-eight patients were included (31.6% female). Median age at diagnosis was 65.4 years (interquartile range: 53.4-71.7) for females and 59.0 years (interquartile range: 49.3-68.0) for males (P < .001). At diagnosis, females more often had tubular ascending aortic involvement (75.5% vs. 70.2%; P = .030) while males more often had SoV involvement (42.8% vs. 21.6%; P < .001). Maximum absolute aortic diameter, at any location, at diagnosis did not differ between females (45.0 mm) and males (46.5 mm; P = .388). In females, tubular ascending growth was faster (P < .001), whereas in males, SoV growth was faster (P = .005), corrected for covariates. Unadjusted 10-year survival was 72.5% [95% confidence interval (CI) 67.8%-77.6%] for females and 78.3% (95% CI 75.3%-81.3%) for males (P = .010). Twenty-three type A dissections occurred, with an incidence rate of 8.2/1000 patient-years (95% CI 4.4-14.1) in females and 2.4/1000 patient-years (95% CI 1.2-4.5) in males [incidence rate ratio females/males: 3.4 (95% CI 1.5-8.0; P = .004)]. Conclusions In patients having entered a diagnostic programme, involvement of aortic segments and age- and segment-related growth patterns differ between women and men with AscAA, particularly at an older age. Unravelling of these intertwined observations will provide a deeper understanding of AscAA progression an

OBJECTIVES: To describe the clinical outcome and quality of life up to 50 years after surgical atrial septal defect (ASD) closure at young age. Primary outcome is defined as MACE (all-cause mortality, cardiac re-interventions, ischemic stroke, endocarditis, heart failure and symptomatic arrhythmia).METHODS: Single-center, longitudinal cohort-study evaluating 135 consecutive patients who underwent ASD-closure before the age of 15 years between 1968 and 1980. Participants were invited for extensive cardiac evaluation and assessment of quality-of-life every 10 years.RESULTS: Eighty patients (86%) of 93 eligible survivors were included in this study (mean age 52 ± 5 years (range 41-63), 40% male). Median follow-up since surgery was 45 years (range 40-51). Cumulative survival after 50 years was 86% and comparable to the normal Dutch population. Cumulative event-free survival after 45 and 50-years was 59% and 46% respectively (re-intervention in 6, symptomatic arrhythmia in 25, and pacemaker implantation in 10 patients). Right ventricular ejection fraction on CMR was diminished in 6%. Exercise capacity was normal in 77%. There was no pulmonary hypertension. NT-proBNP was elevated in 61%. Quality of life was comparable with the general population. No predictors for late events were identified.CONCLUSION: Long-term survival after surgical ASD-closure in childhood is good and not statistically different at 50 years compared to the normal Dutch population. Re-intervention rate is low, there is no pulmonary hypertension. Right ventricular function was diminished in 6%, exercise capacity was good and stable over time with quality of life comparable to the general population. However, supraventricular tachycardia is common.

OBJECTIVES: In complex double outlet right ventricle (DORV) patients, the optimal surgical approach may be difficult to assess based on conventional 2-dimensional (2D) ultrasound (US) and computed tomography (CT) imaging. The aim of this study is to assess the added value of 3-dimensional (3D) printed and 3D virtual reality (3D-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 3 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 ventricular septum defect patch closure could be determined best using 3D-VR reconstructions (3D-VR 92%, 3D print 66% and US/CT 46%, P < 0.01). The percentage of proposed surgical plans corresponding to the performed surgical approach was 66% for plans based on US/CT, 78% for plans based on 3D printing and 80% for plans based on 3D-VR visualization. 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.

AIMS: To support decision-making in children undergoing aortic valve replacement (AVR), by providing a comprehensive overview of published outcomes after paediatric AVR, and microsimulation-based age-specific estimates of outcome with different valve substitutes. METHODS AND RESULTS: A systematic review of published literature reporting clinical outcome after paediatric AVR (mean age <18 years) published between 1/1/1990 and 11/08/2021 was conducted. Publications reporting outcome after paediatric Ross procedure, mechanical AVR (mAVR), homograft AVR (hAVR), and/or bioprosthetic AVR were considered for inclusion. Early risks (<30d), late event rates (>30d) and time-to-event data were pooled and entered into a microsimulation model. Sixty-eight studies, of which one prospective and 67 retrospective cohort studies, were included, encompassing a total of 5259 patients (37 435 patient-years; median follow-up: 5.9 years; range 1-21 years). Pooled mean age for the Ross procedure, mAVR, and hAVR was 9.2 ± 5.6, 13.0 ± 3.4, and 8.4 ± 5.4 years, respectively. Pooled early mortality for the Ross procedure, mAVR, and hAVR was 3.7% (95% CI, 3.0%-4.7%), 7.0% (5.1%-9.6%), and 10.6% (6.6%-17.0%), respectively, and late mortality rate was 0.5%/year (0.4%-0.7%/year), 1.0%/year (0.6%-1.5%/year), and 1.4%/year (0.8%-2.5%/year), respectively. Microsimulation-based mean life-expectancy in the first 20 years was 18.9 years (18.6-19.1 years) after Ross (relative life-expectancy: 94.8%) and 17.0 years (16.5-17.6 years) after mAVR (relative life-expectancy: 86.3%). Microsimulation-based 20-year risk of aortic valve reintervention was 42.0% (95% CI: 39.6%-44.6%) after Ross and 17.8% (95% CI: 17.0%-19.4%) after mAVR. CONCLUSION: Results of paediatric AVR are currently suboptimal with substantial mortality especially in the very young with considerable reintervention hazards for all valve substitutes, but the Ross procedure provides a survival benefit over mAVR. Pros and cons of subs

Early postoperative sinus node dysfunction (SND) is common in paediatric patients undergoing surgical correction of congenital heart disease (CHD). At present, the pathophysiology of these arrhythmias is incompletely understood. In this case series, we present 3 paediatric patients in whom we performed intraoperative epicardial mapping and who developed early postoperative 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 postoperative SND. Such insights into the development of arrhythmias are crucial as it may be the first step in identifying high-risk patients.

Atrial fibrillation (AF) resumes within 90 s in 27% of patients after sinus rhythm (SR) restoration. The aim of this study is to compare conduction heterogeneity during the supervulnerable period immediately after electrical cardioversion (ECV) with long-term SR in patients with AF. Epicardial mapping of both atria was performed during SR and premature atrial extrasystoles in patients in the ECV (N = 17, age: 73 ± 7 years) and control group (N = 17, age: 71 ± 6 years). Inter-electrode conduction times were used to identify areas of conduction delay (CD) (conduction times 7-11 ms) and conduction block (CB) (conduction times ≥ 12 ms). For all atrial regions, prevalences and length of longest CB and continuous CDCB lines, magnitude of conduction disorders, conduction velocity, biatrial activation time, and voltages did not differ between the ECV and control group during both SR and premature atrial extrasystoles (p ≥ 0.05). Hence, our data suggest that there may be no difference in biatrial conduction characteristics between the supervulnerable period after ECV and long-term SR in AF patients. The supervulnerable period after AF termination is not determined by conduction heterogeneity during SR and PACs. It is unknown to what extent intra-atrial conduction is impaired during the supervulnerable period immediately after ECV and whether different right and left atrial regions are equally affected. This high-resolution epicardial mapping study (upper left panel) of both atria shows that during SR the prevalences and length of longest CB and cCDCB lines (upper middle panel), magnitude of conduction disorders, CV and TAT (lower left panel), and voltages did not differ between the ECV and control group. Likewise, these parameters were comparable during PACs between the ECV and control group (lower left panel). †Non-normally distributed. cm/s = centimeters per second; mm = millimeter; ms = millisecond; AF = atrial fibrillation; AT = activation time; BB = Bachmann's bundle

OBJECTIVES A small percentage of paediatric patients supported with a ventricular assist device (VAD) can have their device explanted following myocardial recovery. The goal of this systematic review is to summarize the current literature on the clinical course in these children after weaning. METHODS A systematic literature search was performed on 27 May 2022 using Embase, Medline ALL, Web of Science Core Collection, Cochrane Central Register of Controlled Trials and Google Scholar to include all literature on paediatric patients supported by a durable VAD during the last decade. Overlapping study cohorts and registry-based studies were filtered out. RESULTS Thirty-seven articles were included. Eighteen of them reported on the incidence of recovery in cohort studies, with an overall incidence rate of 8.7% (81/928). Twenty-two of the included articles reported on clinical outcomes after VAD explantation (83 patients). The aetiologies varied widely and were not limited to diseases with a natural transient course like myocarditis. Most of the patients in the included studies (70; 84.3%) were supported by a Berlin Heart EXCOR, and in 66.3% (55/83), only the left ventricle had to be supported. The longest follow-up period was 19.1 years, and multiple studies reported on long-term myocardial recovery. Fewer than half of the reported deaths had a cardiac cause. CONCLUSIONS Myocardial recovery during VAD support is dependent on various contributing components. The interactions among patient-, device-, time- and hospital-related factors are complex and not yet fully understood. Long-term recovery after VAD support is achievable, even after a long duration of VAD support, and even in patients with aetiologies different from myocarditis or post-cardiotomy heart failure. More research is needed on this favourable outcome after VAD support.

The study objective was to analyze survival and incidence of Fontan completion of patients with single-ventricle and concomitant unbalanced atrioventricular septal defect. Data from 4 Dutch and 3 Belgian institutional databases were retrospectively collected. A total of 151 patients with single-ventricle atrioventricular septal defect were selected; 36 patients underwent an atrioventricular valve procedure (valve surgery group). End points were survival, incidence of Fontan completion, and freedom from atrioventricular valve reoperation. Median follow-up was 13.4 years. Cumulative survival was 71.2%, 70%, and 68.5% at 10, 15, and 20 years, respectively. An atrioventricular valve procedure was not a risk factor for mortality. Patients with moderate-severe or severe atrioventricular valve regurgitation at echocardiographic follow-up had a significantly worse 15-year survival (58.3%) compared with patients with no or mild regurgitation (89.2%) and patients with moderate regurgitation (88.6%) (P = .033). Cumulative incidence of Fontan completion was 56.5%, 71%, and 77.6% at 5, 10, and 15 years, respectively. An atrioventricular valve procedure was not associated with the incidence of Fontan completion. In the valve surgery group, freedom from atrioventricular valve reoperation was 85.7% at 1 year and 52.6% at 5 years. The long-term survival and incidence of Fontan completion in our study were better than previously described for patients with single-ventricle atrioventricular septal defect. A concomitant atrioventricular valve procedure did not increase the mortality rate or decrease the incidence of Fontan completion, whereas patients with moderate-severe or severe valve regurgitation at follow-up had a worse survival. Therefore, in patients with single-ventricle atrioventricular septal defect when atrioventricular valve regurgitation exceeds a moderate degree, the atrioventricular valve should be repaired.

OBJECTIVES: Atrial fibrillation (AF) is a risk factor for mortality and cerebrovascular accidents (CVAs) and is common in patients with heart failure. This study evaluated survival and adverse events in patients with a left ventricular assist device (LVAD) and a history of AF in the European Registry for Patients with Mechanical Circulatory Support. METHODS: Patients with a continuous-flow LVAD, AF or sinus rhythm (SR) and a follow-up were included. Kaplan-Meier analyses for survival (including a propensity-scored matched analysis), freedom from CVA, pump thrombosis, bleeding and a composite of pump thrombosis/CVA were performed. To correct for covariate imbalance, a Kaplan-Meier (KM) analysis was performed after propensity score (PS) matching the groups. Finally, a Cox regression was performed for predictors of lower survival. RESULTS: Overall, 1821 patients (83% male) were included, with a median age of 57 years and a median follow-up of 13.1 months (interquartile range: 4.3-27.7). Preoperative Electrocardiogram (ECG) rhythm was AF in 421 (23.1%) and SR in 1400 (76.9%) patients. Patients with pre-LVAD AF had a lower ≤90-day (81.9% vs 87.1%, P = 0.0047) and 4-year (35.4% vs 44.2%, P = 0.0083) survival compared to SR. KM analysis with PS matching groups revealed a trend (P = 0.087) towards decreased survival. Univariable analyses confirmed pre-LVAD AF as a predictor for mortality, but the multivariable analysis did not. No difference in the rate of adverse events was found. An analysis of patients at 24 months revealed a higher rate of CVAs for pre-LVAD AF patients (77% vs 94.3%, P < 0.0001). CONCLUSIONS: Patients with pre-LVAD AF undergoing LVAD implantation had a worse survival. However, after performing a multivariate analysis, and PS matching analysis, AF was no longer significant, indicating a worser preoperative condition in these patients. Concerning thrombo-embolic events, only patients with pre-LVAD AF alive beyond 24 months have a higher risk of CVAs