Background Infective endocarditis (IE) remains a severe condition with high morbidity and mortality despite advances in diagnostics and therapy. Health-related quality of life (HRQL) is an important outcome, yet high-quality data on HRQL in patients with IE are limited as most studies focused exclusively on surgically treated patients and did not assess HRQL at a defined time point. Methods A cross-sectional analysis was performed on HRQL data from 259 patients diagnosed with IE between 11/2019 and 09/2023, derived from the prospective German DERIVE cohort. HRQL was evaluated 12 months after diagnosis using the EQ-5D-5L questionnaire and the EQ visual analog scale (EQ-VAS). Descriptive and nonparametric analyses were applied to compare HRQL across subgroups. Results Of 338 one-year survivors, complete HRQL data were available for 259 patients (77%). The median EQ-5D index (interquartile range) was 0.91 (0.73-0.97), indicating persistent impairment compared with the general population. Female patients had lower HRQL than males (median EQ-5D index, 0.89 vs 0.91) and higher impairments in anxiety, pain, and usual activities. Younger patients (<55 years) experienced higher overall HRQL but more pain and anxiety, while patients older than 65 years reported greater physical limitations. No significant difference in EQ-5D index was observed between surgically and conservatively treated patients (0.91 vs 0.90); however, surgically treated patients reported higher levels of anxiety 1 year after diagnosis. Conclusions Patients with IE experience persistent reductions in HRQL 1 year after diagnosis, affecting nearly all domains of daily life. The data highlight the importance of incorporating patient-reported outcomes into clinical management and long-term follow-up.

Exercise training is generally discouraged in patients with severe symptomatic aortic stenosis (AS) undergoing transcatheter aortic valve implantation (TAVI) due to safety concerns. However, whole-body vibration (WBV) exercise could offer a novel approach to improve exercise capacity and quality of life, though its effects remain unclear in this population. Thirty patients with AS scheduled for TAVI were prospectively and randomly assigned to either the WBV group (12 sessions, 30 min each over 4 weeks) or a control group. Assessments of cardiopulmonary exercise testing (CPET), 6-min walking distance (6MWD) and health-related quality of life (HRQoL) questionnaires were conducted at baseline (V1), one day before TAVI (V2) and at short-term follow-up (V3). WBV was conducted between V1 and V2. For the analysis at V1 and V2 16 patients in the WBV group and 14 in the control group were included. Mean age was 79.7 ± 5.22 years, with a mean aortic valve area of 0.75 ± 0.21 cm2. Peak V’O2 increased by 0.3 mL*min-1*kg-1 in the WBV group versus a decrease of − 1.4 mL*min-1*kg-1 in the control group (difference, 1.7 mL*min-1*kg-1; 95

Aims Valve-in-valve transcatheter aortic valve replacement (ViV-TAVR) procedures are increasingly used. Specific recommendations on antithrombotic strategies following ViV-TAVR are lacking. We aimed to assess the efficacy of different antithrombotic strategies following ViV-TAVR. Methods and results We performed a retrospective analysis of German Statutory Health Claims data following ViV-TAVR stratified by antithrombotic strategies according to prescription within 90 days. Antithrombotic regimens included antiplatelet therapy (APT), direct oral anticoagulants (DOACs) or vitamin K antagonists (VKAs). The composite endpoint was all-cause mortality, stroke and/or systemic embolism (SSE) and mechanical complication of heart valve prosthesis at 12 months. Cox proportional hazard regression models were used to compare outcomes. In total, 908 patients between 2005 and 2022 were identified. Of these, 286 received DOACs, 99 received VKAs, 351 received APT exclusively and 172 had no prescription. The incidence of the composite endpoint was 20.8% in the APT group, 20.3% in the DOAC group and 25.3% in the VKA group which was not statistically significantly different. The rate of SSE in the acetylsalicylic acid (ASA) mono group was higher compared to the dual antiplatelet therapy (DAPT) group (27.3% vs. 12.4%, univariable HR 0.42, 95% CI [0.19, 0.95], p = 0.03). Conclusion In this analysis of German Health Claims data, DOACs seemed to be a safe alternative to VKAs and APT. ASA monotherapy was associated with higher rates of SSE compared to DAPT. Given the high risk of bias of this retrospective analysis and the growing use of valve-in-valve procedures, randomized controlled trials are needed to confirm these findings.

BACKGROUND:Data on the prevalence of mitral annular disjunction (MAD) in Marfan syndrome (MFS) based on cardiovascular magnetic resonance (CMR) is sparse. The purpose of this study was to assess prevalence, extent, and distribution of MAD in MFS using CMR and to examine its association with left heart parameters, aortic dimensions, and cardiovascular events. METHODS:This retrospective multicenter study included CMR studies of patients treated for MFS at four tertiary care medical centers with a (likely) pathogenic fibrillin-1 gene variant. Two radiologists (5 and 8 years of experience in CMR) evaluated datasets for MAD (at 4 points around the annulus, including measurement of extent) and mitral valve prolapse (MVP). Further assessment comprised volumetric and functional analysis of the left ventricle (LV), left atrial size, and aortic root diameters. Cardiovascular events included aortic (aortic surgery or aortic dissection), arrhythmic (sustained ventricular tachycardia or sudden cardiac death), and mitral events (mitral valve surgery, MVS). RESULTS:Among 91 patients [(28.9±14.0 years, 47.3% female (n = 43/91)]81.3% (n = 74/91) had MAD (extent: 6.1±2.6 mm). MAD was mostly found at the inferior insertion (72.5% of patients, n = 66/91) and usually affected all sites (39.6% of patients, n = 36/91). Left heart parameters and aortic dimensions did not differ between MAD and no MAD groups (P>0.05). MAD extent and localizations showed significant correlations with LV dilatation (e.g., inferior MAD: r=0.62 for end-diastolic volume index), decreased LV ejection fraction (e.g., anterolateral MAD: r=-0.46), and MVP (e.g., MAD distance: r=0.83), which was found in 44.6% of patients (n = 33/74) with MAD while only affecting 11.8% (n = 2/17) without MAD (P=0.017). Based on receiver operating characteristic analysis for the prediction of MVP prevalence, a threshold of 7.1 mm MAD extent was identified as the optimal cut-off value (sensitivity: 77.1%, specificity: 89.3%). Additionally, subgroup analysis applying different thresholds of MAD extent revealed a significantly larger displacement of MVP and LV volumes as well as higher aortic root z scores for a threshold of ≥8 mm. After a mean follow-up of 4.0±3.0years, cardiovascular events (aortic: n=13/91 [14.3%], arrhythmic: n= 2/91 [2.2%], and mitral: n=2/91 [2.2%] of patients) did not differ significantly (all P>0.05) between no MAD and MAD groups regardless of applied thresholds although MVS was observed exclusively in patients with MAD. CONCLUSION:The high prevalence, large extent, and predominantly pan-annular distribution of MAD suggest a systemic annular pathology in MFS. Overall presence of MAD was not associated with changes to left heart parameters, aortic dimensions, and cardiovascular events. However, MAD, taking into account its extent and affected insertion sites, could serve as a potential marker of disease progression given the shown association of localizations and distance with LV dysfunction and remodeling as well as aortic enlargement and the formation of MVP.

Background: Advances in diagnosis and treatment have led to a growing population of adults with congenital heart disease (ACHD). Despite increasing life expectancy, their clinical needs-especially in older age-remain poorly defined. Cardiac and non-cardiac comorbidities are prevalent, and emerging evidence suggests accelerated biological aging compared to the general population. However, data on older patients and geriatric patients with CHD are limited. Objectives: This study aimed to characterize patients with CHD aged ≥50 years, focusing on functional status, comorbidities, sex-specific differences, and therapeutic patterns. Methods: The PATHFINDER-CHD Registry is a prospective, observational, multicenter registry enrolling patients with CHD with manifest heart failure (HF), HF history, or high HF risk. Data include anatomy, prior treatments, comorbidities, and medication use. Results: Among 1935 patients, 297 were ≥50 years old. Most had acyanotic CHD (62%); Tetralogy of Fallot (21%) was the most frequent diagnosis. A morphologic right systemic ventricle was present in 12%, and 5% had univentricular hearts. HF was manifest in 21%; 44% were classified as ACC/AHA stage B, 51% as stage C, yet 77% were in Perloff class I/II. Common cardiovascular comorbidities included aortopathy (55%), hypertension (37%), and arrhythmia (33%). Non-cardiac comorbidities included thyroid dysfunction (25%), renal impairment (18%), and neurological disease (13%). Sex-specific differences were observed. Despite HF burden, SGLT2 inhibitors and ARNIs were used in only 17% and 8.4%, respectively. Conclusions: Older patients with CHD represent a clinically complex cohort with high comorbidity burden. The findings support the concept of accelerated aging and emphasize the need for tailored interdisciplinary care strategies.

BACKGROUND:Mitral valve transcatheter edge-to-edge repair (M-TEER) has emerged as a viable therapy option in patients with severe mitral regurgitation and high surgical risk. Although atrial fibrillation is common among patients undergoing M-TEER, the optimal anticoagulatory treatment after the intervention is unknown. METHODS:A single-center retrospective observational analysis was conducted using data from the M-TEER registry at the University Hospital Cologne collected from 2019 untill 2021 including patients undergoing M-TEER between November 2012 and April 2019. Patients with atrial fibrillation receiving consistent anticoagulation following M-TEER were categorized into a direct oral anticoagulant or a vitamin K antagonist (VKA) group. The primary end point was a composite of ischemic cerebrovascular and bleeding events. Additionally, overall survival was assessed. RESULTS:Among 613 patients undergoing M-TEER, 206 met the inclusion criteria, with 61 receiving direct oral anticoagulants and 145 receiving VKAs. After a median follow-up of 833 (interquartile range, 355-1271) days, the incidence of the composite primary end point did not differ between direct oral anticoagulant and VKA groups (hazard ratio [HR], 0.51 [95% CI, 0.23-1.12]; P=0.07). Similarly, rates of ischemic cerebrovascular events and bleeding events were similar between groups. However, the overall mortality rate was higher in the VKA group (HR, 2.56 [95% CI, 1.54-4.26]; P=0.002). In the multivariable analysis, oral anticoagulation with a VKA was an independent predictor for death (adjusted HR, 2.23 [95% CI, 1.08-5.06]; P=0.03). CONCLUSIONS:Our findings suggest that direct oral anticoagulants may offer comparable efficacy and safety to VKAs in preventing thromboembolic events following M-TEER in patients with atrial fibrillation. Further randomized trials are needed to confirm these results and establish optimal anticoagulation strategies in this patient population.

AbstractAimsIn pulmonary arterial hypertension (PAH), upfront combination therapy with ERA and PDE5i is associated with a reduction in morbidity and mortality events and improves standard haemodynamics, but data remain limited. Aims of this study were (i) to capture detailed haemodynamic effects of rapid sequential dual combination therapy in patients with newly diagnosed PAH; (ii) to monitor the impact of treatment initiation on clinical variables and patients' risk status, and (iii) to compare the treatment effect in patients with ‘classical PAH’ and ‘PAH with co‐morbidities’.MethodsFifty patients (median age 57 [42–71] years, 66% female) with newly diagnosed PAH (76% idiopathic) were treated with a PD5i/sGC‐S or ERA, followed by addition of the respective other drug class within 4 weeks. All patients underwent repeat right heart catheterization (RHC) during early follow‐up.ResultsAt early repeat RHC (7 ± 2 months), there were substantial reductions in mean pulmonary artery pressure (mPAP: 52.2 ± 13.5 to 39.0 ± 10.6 mmHg; −25.3%), and pulmonary vascular resistance (PVR: 12.1 ± 5.7 to 5.8 ± 3.1 WU; −52.1%), and an increase in cardiac index (2.1 ± 0.4 to 2.7 ± 0.7 mL/min/m2; +32.2%) (all P < 0.05). Haemodynamic improvements correlated with improved clinical parameters including 6‐min walking distance (336 ± 315 to 389 ± 120 m), NTproBNP levels (1.712 ± 2.024 to 506 ± 550 ng/L, both P < 0.05) and WHO‐FC at 12 months, resulting in improved risk status, and were found in patients with few (n = 37) or multiple cardiovascular co‐morbidities (BMI > 30 kg/m2, hypertension, diabetes, coronary artery disease [≥3]; n = 13), albeit baseline PVR in PAH patients with multiple co‐morbidities was lower (9.3 ± 4.4 vs. 13.1 ± 5.9 WU) and PVR reduction less pronounced compared with those with few co‐morbidities (−42.7% vs. −54.7%). However, comprehensive haemodynamic assessment considering further variables of prognostic relevance such as stroke volume index and pulmonary artery compliance showed similar improvements among the two groups (SVI: +50.0% vs. +49.2%; PAC: 91.7% vs. 100.0%). Finally, the 4‐strata risk assessment approach was better able to capture treatment response as compared with other approaches, particularly in patients with co‐morbidities.ConclusionsRapid sequential combination therapy with PDE5i/sGC‐S and ERA substantially ameliorates cardiopulmonary haemodynamics at early follow‐up in patients without, and to a lesser extent, with cardiovascular co‐morbidities. This occurs in line with improvements of clinical parameters and risk status.

Purpose: Evaluate optic nerve sheath and pial diameters (ONSD, ONPD) via sonography and computed tomography (CT) after out-of-hospital cardiac arrest (CA) and to compare their prognostic significance with other imaging and laboratory biomarkers. Materials and methods: A prospective observational study enrolling patients after successful resuscitation between December 2017 and August 2021. ONSD and ONPD were measured with sonography. Additionally, ONSD, and also grey-to-white ratio at basal ganglia (GWRBG) and cerebrum (GWRCBR), were assessed using CT. Lactate and neuron specific enolase (NSE) blood levels were measured. Results: Sonographically measured ONSD and ONPD yielded no significant difference between survival and nonsurvival (p values >= 0.4). Meanwhile, CT assessed ONSD, GWRBG, GWRCBR, and NSE levels significantly differed regarding both, survival (p values <= 0.005) and neurological outcome groups (p values <= 0.04). For survival prognosis, GWRBG, GWRCBR, and NSE levels appeared as excellent predictors; in predicting a good neurological outcome, NSE had the highest accuracy. Conclusions: CT diagnostics, in particular GWRBG and GWRCBR, as well as NSE as laboratory biomarker, appear as excellent outcome predictors. Meanwhile, our data lead us to recommend caution in utilizing sonography assessed ONSD and ONPD for prognostic decision-making post-CA.

Abstract Background The advent of novel targeted cancer therapies have revolutionized cancer treatment. However, concerns about their impact on cardiovascular health persist and current guidelines recommend cardiac monitoring. Yet, prospective data on incidence rates of cardiovascular events are lacking. The Registry for Cardiovascular Events under new cancer Therapies (RECENT) aims to systematically assess clinical as well as subclinical cardiovascular incidents in patients undergoing these novel therapies to identify baseline risk factors and derive individualized monitoring strategies. Methods This ongoing single-center prospective registry enrolls cancer patients receiving BTK inhibitors, ICI, or RAF/MEK inhibitors. Baseline cardiovascular assessment, risk stratification according to HFA-ICOS risk score, and follow up visits for 12 months at three-monthly intervals (including ECG, biomarkers and echocardiography) during treatment are conducted. Treatment-related events, in particular hypertension, arrhythmias, cancer therapy-related cardiac dysfunction (CTRCD), and (peri-) myocarditis, as defined according to current guideline recommendations, are recorded. Results We report data of the first 85 patients (60.9 ± 14.7 years, 52.9% male) included in this ongoing registry. Of those, 17/85 patients received treatment with BTK inhibitors (20%), 45/85 with ICI (53%) and 23/85 with RAF/MEK inhibitors (27%). Most common treatment indication were dermatological (39/85, 45.9%), hematologic (17/85, 20%), urological (13/85, 15.3%) and head and neck (11/85, 12.9%) cancers. Before treatment, the majority of patients were stratified as low-risk (31/85) or moderate-risk (32/85), and 22/85 (25.9%) as high-risk. 7/85 (8.2%) patients were newly diagnosed with cardiovascular disease at baseline visit, resulting in a change in risk category and subsequent initiation of cardiovascular treatment. This includes 3 patients who had been initially categorized as low-risk and would not have been referred for further cardiac workup due to current guideline recommendations. The six months follow up visit has been completed in 44/85 patients. Among these, 1/20 patients (5%) with ICI developed a perimyocarditis and 2/11 patients (18.2%) with BTK inhibitors developed atrial fibrillation. 1/13 patients (7.7%) with RAF/MEK inhibitor therapy, classified as low-risk at baseline, developed asymptomatic CTRCD, which resolved after initiation of cardioprotective drug therapy. Conclusion This interim analysis of the RECENT registry indicates benefit of a structured baseline cardiac assessment prior to cancer treatment in all patients, irrespective of the initial risk stratification. Contrary to current guidelines, which recommend routine echocardiographic monitoring in patients receiving RAF/MEK inhibitors only in high-risk patients, the current data advocate for extending this also to low-risk patients to detect subclinical deterioration of the cardiovascular status.

Background Adults with congenital heart defects (ACHD) globally constitute a notably medically underserved patient population. Despite therapeutic advancements, these individuals often confront substantial physical and psychosocial residua or sequelae, requiring specialized, integrative cardiological care throughout their lifespan. Heart failure (HF) is a critical challenge in this population, markedly impacting morbidity and mortality. Aims The primary aim of this study is to establish a comprehensive, prospective registry to enhance understanding and management of HF in ACHD. Named PATHFINDER-CHD, this registry aims to establish foundational data for treatment strategies as well as the development of rehabilitative, prehabilitative, preventive, and health-promoting interventions, ultimately aiming to mitigate the elevated morbidity and mortality rates associated with congenital heart defects (CHD). Methods This multicenter survey will be conducted across various German university facilities with expertise in ACHD. Data collection will encompass real-world treatment scenarios and clinical trajectories in ACHD with manifest HF or at risk for its development, including those undergoing medical or interventional cardiac therapies, cardiac surgery, inclusive of pacemaker or ICD implantation, resynchronization therapy, assist devices, and those on solid organ transplantation. Design The study adopts an observational, exploratory design, prospectively gathering data from participating centers, with a focus on patient management and outcomes. The study is non-confirmatory, aiming to accumulate a broad spectrum of data to inform future hypotheses and studies. Processes Regular follow-ups will be conducted, systematically collecting data during routine clinical visits or hospital admissions, encompassing alterations in therapy or CHD-related complications, with visit schedules tailored to individual clinical needs. Assessments Baseline assessments and regular follow-ups will entail comprehensive assessments of medical history, ongoing treatments, and outcomes, with a focus on HF symptoms, cardiac function, and overall health status. Discussion of the design The design of the PATHFINDER-CHD Registry is tailored to capture a wide range of data, prioritizing real-world HF management in ACHD. Its prospective nature facilitates longitudinal data acquisition, pivotal for comprehending for disease progression and treatment impacts. Conclusion The PATHFINDER-CHD Registry is poised to offer valuable insights into HF management in ACHD, bridging current knowledge gaps, enhancing patient care, and shaping future research endeavors in this domain.

Background: Recently, a disease modifying therapy has become available for transthyretin amyloid cardiomyopathy (ATTR-CM). A validated monitoring concept of treatment is lacking, but a current expert consensus recommends three clinical domains (clinical, biomarker and ECG/imaging) assessed by several measurable features to define disease progression. Methods: We retrospectively analyzed data of wild-type ATTR-CM patients initiating tafamidis therapy assessed within our local routine protocol at baseline and 6-months follow-up with respect to the frequency of values beyond the proposed thresholds defining disease progression. Additionally, associations of cardiac magnetic resonance (CMR) tomography with clinical domains were examined within a subgroup. Results: Sixty-two ATTR-CM patients were included (88.7% male, mean age 79 years). In total, 16.1% of patients had progress in the clinical and functional domain, 33.9% in the biomarker domain and 43.5% in the imaging/electrocardiography (ECG) domain, with the latter driven by deterioration of the diastolic dysfunction grade and global longitudinal strain. In total, 35.5% of patients showed progress in none, 35.5% in one, 29.0% in two and no patient in three domains, the latter indicating overall disease progression. A subgroup analysis of twenty-two patients with available baseline and follow-up CMR data revealed an increase in CMR-based extracellular volume by more than 5% in 18.2% of patients, with no significant correlation with progress in one of the clinical domains. Conclusions: We provide first frequency estimates of the markers of disease progression according to a recent expert consensus statement, which might help refine the multiparametric monitoring concept in patients with ATTR-CM.

Background Two subtypes of atrial functional mitral regurgitation (AFMR) have been described, one is characterized by Carpentier type I and the other by Carpentier type IIIb leaflet motion. Objectives The authors sought to analyze echocardiographic characteristics and outcomes of AFMR subtypes undergoing mitral valve transcatheter edge-to-edge repair (M-TEER). Methods Of 1,047 consecutive patients who underwent M-TEER, the authors identified those with isolated mitral annulus dilation (Carpentier I), termed AFMR-IAD, and those with atriogenic hamstringing characterized by restricted posterior mitral leaflet motion (Carpentier IIIb), termed AFMR-AH. Echocardiographic baseline characteristics and outcomes up to 1-year were analyzed. Results A total of 128 patients (12.2%) met AFMR criteria; 75 (58.6%) were identified as AFMR-IAD and 53 (41.4%) as AFMR-AH. AFMR-AH displayed greater left atrial and left ventricular volumes, greater mitral annulus, shorter and steeper posterior mitral leaflet, and more pronounced MR (all P < 0.05). Technical success was achieved in 98.7% (AFMR-IAD) and 86.8% (AFMR-AH) of patients (P = 0.009). At discharge, device detachments were exclusively observed in AFMR-AH (10.0%). MR ≤II was achieved in 95.6% and 78.6% at 30 days (P = 0.009) and in 93.0% and 74.1% at 1 year (P = 0.038) in patients with AFMR-IAD and AFMR-AH, respectively. AFMR-AH was associated with procedural failure (OR: 1.17 [95% CI: 1.00-1.38]; P = 0.045) at 30 days (43.4% vs 24.0%; P = 0.023) and all-cause mortality (HR: 2.54 [95% CI: 1.09-5.91]; P = 0.031) at 1 year (77% vs 92%, Kaplan-Meier estimated 1-year survival; P = 0.017). Conclusions AFMR-AH shows worse procedural and clinical outcomes following M-TEER than AFMR-IAD. Thus, vigilance regarding this pathology is warranted and alternative mitral valve therapies might need to be considered.

Background: In Germany, a total of 38 547 heart valve procedures were performed in 2022. With a growing number of patients undergoing the surgical and interventional implantation of heart valves, the incidence of prosthetic endocarditis is also rising.Methods: We summarize the current state of the prophylaxis, diagnosis, and treatment of prosthetic endocarditis in a selective review of the literature.Results: Prosthetic endocarditis accounts for 10-30% of all cases of endocarditis. As its echocardiographic and microbiologic findings are often less specific than those of native endocarditis, its diagnosis now increasingly relies on alternative imaging modalities such as F-18-FDG PET-CT. Anti-infective and surgical treatment are made more difficult by biofilm formation on the prosthetic valve and the frequent formation of perivalvular abscesses.Conclusion: Increased awareness of this clinical entity in the outpatient setting will promote the earlier initiation of appropriate diagnostic studies. Proper diagnostic evaluation is an essential prerequisite for the early detection and timely treatment of prosthetic endocarditis, with the goal of preventing progressive destruction and thus improving the outcome. Preventive and educative measures should be intensified, and certified, multidisciplinary endocarditis teams should be established. Antibiotic prophylaxis is now given much more restrictively than in earlier years; the risk of infection must be weighed against the potential development of both individual and collective resistance to antibiotic drugs.

Patients with left heart disease (LHD) often display pulmonary hypertension (PH), which impacts morbidity and mortality. The pathophysiology of PH is complex and entails pulmonary congestion due to elevated left-sided filling pressures, pulmonary vasoconstriction as well as vascular remodeling. The recent ESC/ERS Guidelines on pulmonary hypertension updated the hemodynamic definitions of pulmonary hypertension in general, and the subclassification of post-capillary PH. This review summarizes recent advances in the diagnostic work-up and management strategies of PH associated with LHD. Specifically, we summarize revisited hemodynamic definitions and the characteristics of isolated post-capillary PH (IpcPH) and combined post- and pre-capillary PH (CpcPH). Furthermore, we review the current knowledge on the pathogenesis of PH-LHD, the prognostic relevance of hemodynamic parameters, and the management strategies, differentiating between treatment of the underlying left heart disease and therapies targeting the pulmonary circulation. The article emphasises the need for precise diagnostic work-up and individualized treatment strategies in patients with PH-LHD.

Objective: To compare the measurement of aortic diameters using a novel flow-independent MR-Angiography (3D modified Relaxation-Enhanced Angiography without Contrast and Triggering (modified REACT)) and trans-thoracic echocardiography (TTE) in Marfan syndrome (MFS) patients.Material and methods: This retrospective, single-center analysis included 46 examinations of 32 MFS patients (mean age 37.5 +/- 11.3 years, 17 women, no prior aortic surgery) who received TTE and 3D modified REACT (ECG-and respiratory-triggering, Compressed SENSE factor 9 for acceleration of image acquisition) of the thoracic aorta. Aortic diameters (sinus of Valsalva (SV), sinotubular junction (STJ), and ascending aorta (AoA)) were independently measured by two cardiologists in TTE (leading-edge) and two radiologists in modified REACT (inner-edge, using multiplanar reconstruction). Intraclass correlation coefficient, Bland-Altman analyses, and Pearson's correlation (r) were used to assess agreement between observers and methods.Results: Interobserver correlation at the SV, STJ, and AoA were excellent for both, TTE (ICC = 0.95-0.98) and modified REACT (ICC = 0.99-1.00). There was no significant difference between TTE and modified REACT for diameters measured at the SV (39.24 +/- 3.24 mm vs. 39.63 +/- 3.76 mm; p = 0.26; r = 0.78) and the STJ (35.16 +/- 4.47 mm vs. 35.37 +/- 4.74 mm; p = 0.552; r = 0.87). AoA diameters determined by TTE were larger than in modified REACT (34.29 +/- 5.31 mm vs. 30.65 +/- 5.64 mm; p < 0.01; r = 0.74). The mean scan time of modified REACT was 05:06 min +/- 02:47 min, depending on the patient's breathing frequency and heart rate.Conclusions: Both TTE and modified REACT showed a strong correlation for all aortic levels; however, at the AoA, diameters were larger using TTE, mostly due to the limited field of view of the latter with measurements being closer to the aortic valve. Given the excellent interobserver correlation and the strong agreement with TTE, modified REACT represents an attractive method to depict the thoracic aorta in MFS patients.

Abstract Background Pulmonary arterial hypertension (PAH) occurs in approximately 10% of adults with congenital heart disease (ACHD), usually develops secondary to intra- or extra-cardiac shunts resulting in pressure and/or volume overload in the pulmonary circulation, and is associated with increased morbidity and mortality. Treatment strategies for PAH-ACHD include disease targeting therapies (DTT), such as phosphodiesterase-5 inhibitors (PDE5i) and endothelin receptor antagonists (ERA). Data regarding the impact of DTT on cardiopulmonary haemodynamics in PAH-ACHD are scarce. Purpose Aim of this study was to quantify effects of DTT on cardiopulmonary haemodynamics (assessed by right heart catheterisation [RHC]) and on key non-invasive functional parameters contributing to ESC/ERS risk status in PAH-ACHD patients, as applied in real-world clinical practice. Methods We retrospectively analysed the effect of DTT in PAH-ACHD patients who had undergone RHC in adulthood at baseline (treatment-naïve) between 2000 and 2022, with at least one follow-up RHC during DTT, in six expert centres in Europe. Clinical characteristics (WHO functional class [WHO-FC], 6-minute walking distance [6MWD], N-terminal pro-brain natriuretic peptide [NTproBNP] and measures of RHC (systemic/ pulmonary cardiac output, systolic/diastolic/mean pulmonary arterial pressure [sPAP, dPAP, mPAP], and pulmonary arterial wedge pressure [PAWP]) were collected. Pulmonary vascular resistance/arterial compliance (PVR+PAC) and stroke volume index (SVI) were calculated. Results 44 patients (70% female, mean age 45±15 years) were included. Underlying CHD was a pre- or post-tricuspid shunt in 35% and 63%, respectively. 14% had complex CHD, 7% had innate left heart disease, and 21% presented with Eisenmenger´s syndrome. At baseline 71% were in WHO-FC III/IV, 6MWD was 371±105 m, and NTproBNP was 673 [446–1021] ng/l. On RHC, mPAP was 58±15 mmHg, PVR 11 [7–17] wood units (WU), SVI 32 [27–44] ml/min/m2, CI 2.7±1.0 l/min/m2, and PAC 1.0 [0.7–1.4] ml/mmHg. DTT was started in all patients after baseline RHC. 81% received monotherapy (34% PDE5i, 66% ERA) and 19% received upfront combination therapy (PDE5i+ERA). Follow-up RHC was performed 15 [5–33] months after baseline RHC. At follow-up, WHO-FC and NTproBNP (562 [428-762] ng/l) significantly improved (p<0.001 and p=0.021, respectively), as did hemodynamic measures: mPAP (53±15 mmHg, p<0.001), PVR (9 [5–10] WU, p<0.001), SVI (37 [30–49] ml/min/m2, p=0.010), PAC (1.3 [1.0–1.7] ml/mmHg, p<0.001), and CI (3.1±1.1 l/min/m2, p=0.001). In contrast, 6MWD showed subtle change (379±111 m, p=0.164). After follow-up RHC, DTT was escalated in 78% of patients. Conclusions In PAH-ACHD patients, DTT was associated with a moderate but sustained haemodynamic and functional improvement after 15 months. Repeat RHC triggered treatment escalation in most patients. Therefore, ongoing vigilance for PAH-ACHD patients and subsequent treatment optimisation is warranted.

Abstract Background Transcatheter aortic valve implantation (TAVI) has developed to the therapy of choice for patients with symptomatic severe aortic stenosis who are inoperable or at high or intermediate operative risk for surgical aortic valve replacement. However, the optimal anticoagulant therapy post-TAVI still remains a matter of debate and individual patient characteristics and comorbidities lead to the prescription of different regimens in clinical practice. Purpose This study investigates current anticoagulant treatment patterns and clinical outcomes in patients after TAVI in a real-world population. Methods The German Aortic Valve Registry (GARY) is a prospective, multicenter all-comers registry enrolling patients undergoing invasive treatment for aortic valve disease. From January 2011 to December 2019, 141,790 patients from 92 hospitals performing TAVI procedures in Germany were enrolled. Different anticoagulant treatment regimens were assessed at hospital discharge for patients after TAVI procedures. All-cause mortality and the combined endpoint "cardiac and cerebrovascular events" containing myocardial infarction (MI), stroke, transient ischaemic attack (TIA), aortic prosthesis re-intervention and all-cause mortality in the first year after TAVI were examined by treatment regimen. Results The study population comprised 45,598 patients with TAVI between 2011 and 2019: 46.5 % received dual antiplatelet therapy (DAPT), 25.7 % oral anticoagulation (OAC) plus antiplatelet therapy, 15.0 % single antiplatelet therapy (SAPT), 6.8 % OAC monotherapy, 4.7 % triple therapy and 1.3 % did not receive any anticoagulant medication. The majority of patients with oral anticoagulation were prescribed VKA (66.8 %) compared to DOAC (33.2 %). After adjustment for baseline confounders, cardiac and cerebrovascular event free survival did not differ significantly between patients with SAPT and DAPT (HR 0.94, 95% CI 0.89-1.00, p = 0.07), albeit the risk of all-cause mortality was slightly increased in SAPT (HR 1.24, 95% CI 1.12-1.37, p <0.001) and all regimens containing oral anticoagulation (OAC mono: HR 1.24, 95% CI 1.08-1.42, p = 0.002, OAC duo: HR 1.23, 95% CI 1.13-1.35, p < 0.001, OAC triple: HR 1.25, 95% CI 1.08-1.45, p = 0.003) compared to dual antiplatelet regime. In patients with OAC, the risk of all-cause mortality (HR 0.99, 95% CI 0.89-1.10, p = 0.81) and cardiac and cerebrovascular event free survival (HR 1.07, 95% CI 0.97-1.18, p = 0.17) was not significantly different in patients with DOAC-containing regimens when compared to VKA-containing regimens. Conclusions In an analysis from one of the world’s largest national registries of patients undergoing TAVI, we found comparative effectiveness of SAPT vs. DAPT and DOACs vs. VKA after TAVI with respect to cardiac and cerebrovascular events. The increased risk of all-cause mortality in patients with antiplatelet monotherapy might be confounded by unmeasured morbidity but requires further attention.Multivariate: Reference group DAPTMultivariate: Reference group VKA