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Background: Cardiac involvement represents the main cause of death in patients with Fabry disease (FD). Echocardiography and cardiovascular magnetic resonance (CMR) have an established diagnostic role, but their prognostic value remains unresolved. This systematic review and meta-analysis sought to assess the prognostic implications of imaging parameters in FD., Methods: PubMed, ClinicalTrials.gov, Embase, Cochrane Library and Web of Science databases were searched for studies from inception through 1 May 2024. Studies including FD patients undergoing baseline imaging assessment and clinical follow-up were selected. Pre-defined study outcomes were a cardiovascular endpoint and a composite clinical endpoint. The study protocol was registered in PROSPERO (ID CRD42022342394)., Results: Fourteen studies, including 1713 FD patients (44.7% males), were selected. At pooled analysis, late gadolinium enhancement (hazard ratio [HR]: 4.45; 95% CI: 2.82-7.02), left atrium volume indexed (HR: 1.02 per mL/m 2 ; 95% CI: 1.01-1.03), E/e' (HR: 1.14 per unit increase; 95% CI: 1.08-1.21), left ventricular (LV) mass indexed (HR: 1.01 per mg/m 2 ; 95% CI: 1.00-1.02), maximum LV wall thickness (HR: 1.19 per mm, 95% CI: 1.04-1.36) and LV-global longitudinal strain (HR: 1.20 per unit increase; 95% CI: 1.16-1.25) were significantly associated with the cardiovascular endpoint, whereas T1-mapping and LV-ejection fraction were not. T1-mapping was associated with the composite endpoint (HR: 0.99 per msec increase; 95% CI: 0.98-1.00). Meta-regression analysis did not show any significant interaction between each of the potential effect modifiers., Conclusions: Several imaging parameters were significant predictors of adverse clinical outcomes in patients with FD. Late gadolinium enhancement showed the strongest association with adverse prognosis., (© 2025 The Author(s). European Journal of Clinical Investigation published by John Wiley & Sons Ltd on behalf of Stichting European Society for Clinical Investigation Journal Foundation.)

We demonstrate the case of a man presenting with chest pain in which an initial assessment with echocardiography and, subsequently, with cardiac computed tomography led to a final diagnosis of caseous mitral annular calcification complicated by multiple embolizations., Competing Interests: Declarations. Informed consent: Informed consent was given to the publication of the present clinical case. Conflict of interest: None declared., (© 2024. The Author(s), under exclusive licence to Springer Nature B.V.)

Aims: Transcatheter tricuspid valve interventions (TTVI) are increasingly used to treat patients with significant tricuspid regurgitation (TR). The evolution of concurrent mitral regurgitation (MR) severity after TTVI is currently unknown and may be pivotal for clinical decision-making. The aim of this study was to assess the evolution of MR after TTVI and to identify predictors of MR worsening and improvement., Methods and Results: This analysis is a substudy of the TriValve Registry, an international registry designed to collect data on TTVI. This substudy included all patients with echocardiographic data on MR evolution and excluded those with a concomitant tricuspid and mitral transcatheter valve intervention or with a history of mitral valve intervention. The co-primary outcomes were MR improvement and worsening at two timepoints: pre-discharge and 2-month follow-up. This analysis included 359 patients with severe TR, mostly (80%) treated with tricuspid transcatheter edge-to-edge repair (T-TEER). MR improvement was found in 106 (29.5%) and 99 (34%) patients, while MR worsening was observed in 34 (9.5%) and 33 (11%) patients at pre-discharge and 2-month follow-up, respectively. Annuloplasty and heterotopic replacement were associated with MR worsening. Independent predictors of MR improvement were: atrial fibrillation, T-TEER, acute procedural success, TR reduction, left ventricular end-diastolic diameter> 60 mm, and beta-blocker therapy. Patients with moderate-to-severe/severe MR following TTVI showed significantly higher death rates., Conclusion: MR degree variation is common after TTVI, with most cases showing improvement. Clinical and procedural characteristics may predict the MR evolution, in particular procedural success and T-TEER play key roles in MR outcomes. TTVI may be beneficial, even in the presence of functional MR., Competing Interests: Conflict of interest: RH has served as a consultant for Abbott Vascular, Abbott Structural, NaviGate, Philips Healthcare, Medtronic, Edwards Lifesciences, and GE Healthcare; has been the Chief Scientific Officer for the Echocardiography Core Laboratory at the Cardiovascular Research Foundation for multiple industry-supported trials, for which she receives no direct industry compensation; has received speaker fees from Boston Scientific and Baylis Medical; and has received non-financial support from 3mensio. HA has received consulting fees from Abbott and Edwards LifeSciences. MA has served as a proctor/consultant for and has received speaker fees from Abbott, Edwards LifeSciences, Boston, Zoll, and Medtronic; and has received institutional grants from Edwards Lifesciences, Abbott, Medtronic, and LSI Solutions. DB has received speaker honoraria and travel support from Abbott Vascular. KC has received honoraria from Abbott. PD has served as a consultant for Abbott Vascular, 4Tech, Neovasc, and InnovHeart; and has received honoraria from Abbott and Edwards Lifesciences. RE-L has received speaker fees from Abbott, Boston, and Edwards Lifesciences. JH has received speaker honoraria from Abbott Vascular and Edwards Lifesciences. DH has served as proctor for Edwards Lifesciences and Abbott Vascular. DK has received personal fees from Abbott Medical, Edwards Lifescienes, Medtronic Inc., and Pi-Cardio Ltd. outside the submitted work. MA has been a consultant to Abbott Structural Heart and Medtronic. AL has served on the advisory board for Medtronic, Abbott Vascular Boston Scientific, Edwards Lifesciences, Shifamed, NeoChord Inc., V-dyne, and Philips. EL has received grant support and lecture fees from Abbott; and has received lecture fees from Edwards Lifesciences. SL has received travel compensation from Edwards LifeSciences, advisory fees from Bayer, speaker honoraria from Abbott and serves as consultant for New Valve Technology. PL has received speaker fees from Abbott. FP has received travel expenses from Edwards Lifesciences, Abbott Vascular, and Polares Medical. JR-C has received institutional research grants from Edwards Lifesciences. JS has served as a consultant for Edwards Lifesciences. HS has received study honoraria, travel expenses, and consulting fees from 4Tech Cardio, Abbott, Ablative Solutions, Ancora Heart, Bavaria Medizin Technologie, Bioventrix, Boston Scientific, Carag, Cardiac Dimensions, Celonova, Comed BV, Contego, CVRx, Edwards Lifesciences, Endologix, Hemoteq, Lifetech, Maquet Getinge Group, Medtronic, Mitralign, Nuomao Medtech, Occlutech, PFM Medical, ReCor, Renal Guard, Rox Medical, Terumo, Vascular Dynamics, and Vivasure Medical. GT has served as a consultant, physician advisory board member, and faculty trainer for Abbott Structural Heart; has served as a consultant for Medtronic and NeoChord; and has served as a physician advisory board member for JenaValve. K-PK has been a consultant to Edwards Lifesciences. MM has received the following personal fees of minimal amounts since January 2021: from Amgen, Liva Nova, and Vifor pharma as a member of Executive or Data Monitoring Committees of sponsored clinical trials; and from AstraZeneca, Bayer, Boehringer Ingelheim, Edwards Lifesciences, and Roche Diagnostics for participation to advisory boards and/or speeches at sponsored meetings. PL has been a consultant to Abbott Structural Heart, Edwards Lifesciences, and Medtronic. RSVB has performed unpaid trial activities for Abbott, Edwards Lifesciences, Medtronic, and the University of Göttingen (IIT) and serves as an advisory board or Speakers Bureau member for Abbott Cardiovascular, Edwards Lifesciences, Medtronic, and NeoChord. JW is a consultant for Edwards Lifesciences Inc. SW reports research, travel, or educational grants to the institution from Abbott, Abiomed, Amgen, AstraZeneca, Bayer, Biotronik, Boehringer Ingelheim, Boston Scientific, Bristol Myers Squibb, Cardinal Health, CardioValve, Corflow Therapeutics, CSL Behring, Daiichi Sankyo, Edwards Lifesciences, Guerbet, InfraRedx, Janssen-Cilag, Johnson & Johnson, Medicure, Medtronic, Merck Sharp & Dohme, Miracor Medical, Novartis, Novo Nordisk, Organon, OrPha Suisse, Pfizer, Polares, Regeneron, Aventis, Servier, Sinomed, Terumo, Vifor, and V-Wave. SW serves as an unpaid advisory board member and/or unpaid member of the steering/executive group of trials funded by Abbott, Abiomed, Amgen, AstraZeneca, Bayer, Boston Scientific, Biotronik, Bristol Myers Squibb, Edwards Lifesciences, Janssen, MedAlliance, Medtronic, Novartis, Polares, Recardio, Sinomed, Terumo, V-Wave, and Xeltis, but has not received personal payments by pharmaceutical companies or device manufacturers. He is also a member of the steering/executive committee group of several investigator-initiated trials that receive funding by industry without impact on his personal remuneration. ML has received institutional clinical research grants from Abbott, Boston Scientific, Edwards Lifesciences, and Medtronic. RH has served as a consultant for Abbott Vascular, Abbott Structural, NaviGate, Philips Healthcare, Medtronic, Edwards Lifesciences, and GE Healthcare; has been the Chief Scientific Officer for the Echocardiography Core Laboratory at the Cardiovascular Research Foundation for multiple industry-supported trials, for which she receives no direct industry compensation; has received speaker fees from Boston Scientific and Baylis Medical; and has received nonfinancial support from 3mensio. FM has served as a consultant for and received consulting fees and honoraria from Abbott Vascular, Edwards Lifesciences, Cardiovalve, SwissVortex, Perifect, Xeltis, Transseptal Solutions, Magenta, Valtech, and Medtronic; has reported being a cofounder of 4Tech; has received research grant support from Abbott, Medtronic, Edwards Lifesciences, Biotronik, Boston Scientific, NVT, and Terumo; has received royalties and owns intellectual property rights from Edwards Lifesciences (FMR surgical annuloplasty); and has reported being a shareholder in Cardiovalve, Swiss Vortex, Magenta, Transseptal Solutions, Occlufit, 4Tech, and Perifect. FDM is a consultant and has provided paid expert testimony for Abbott and Boston Scientific. MT has served as a consultant for Abbott Vascular, Boston Scientific, 4Tech, and CoreMedic and has received speaker honoraria from Edwards Lifesciences. SL has received travel compensation from Edwards Lifesciences. GP declares honorarium as speaker and/or institutional research grant from GE Healthcare, Bracco, Heartflow, Menarini, Novartis, Alexion, Novo Nordisk. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Background: Mitral annular disjunction (MAD) is a controversial entity. Recently, a distinction between pseudo-MAD, present in systole and secondary to juxtaposition of the billowing posterior leaflet on the left atrial wall, and true-MAD, where the insertion of the posterior leaflet is displaced on the atrial wall both in diastole or in systole, has been proposed. We investigated the prevalence of pseudo-MAD and true-MAD., Methods: This was a retrospective study, including consecutive patients referred to cardiovascular magnetic resonance (CMR). MAD was defined as a ≥1 mm displacement between the left atrial wall-mitral valve leaflet junction hinge and the top of the left ventricular wall, measured from cine-CMR images in the three long-axis views. Pseudo-MAD and true-MAD were defined as the presence of MAD only in systole or both in systole and diastole, respectively., Results: Two hundred and ninety patients (59 [47-71] years; 181/290 men, 62%) were included. Mitral valve prolapse (MVP) and MAD were found in 24/290 (8%) and 145/290 (50%) patients, of which 100/290 (35%) with true-MAD and 45/290 (16%) with pseudo-MAD. In all measurements, systolic MAD extent (2.3 [1.7-3.0] mm) resulted equal to or greater than diastolic MAD extent (2.0 [1.5-2.9] mm). The most frequent MAD location was the inferior wall (117/290, 40%) and the inferolateral wall was the rarest (50/290, 17%). In patients with MVP, the prevalence of MAD was higher (21/24, 88%), mainly driven by a higher prevalence of pseudo-MAD, as the prevalence of true-MAD did not vary significantly in patients with vs without MVP (p = 0.22), except for the inferolateral wall (9/24, 38% vs 20/266, 8%; p < 0.001). The extent of pseudo-MAD was greater in patients with MVP (4.0 [3.0-5.6] mm) than in those without MVP (2.0 [1.5-3.0]; p < 0.001), whereas the extent of true-MAD did not differ significantly (2.5 [2.0-3.2] mm and 1.9 [1.5-2.9] mm; p = 0.06). At the inferolateral wall, the prevalence of pseudo-MAD was 7/24, 29% vs 14/266, 5% (p < 0.001) in patients with vs without MVP., Conclusion: True-MAD was a common imaging finding in patients undergoing CMR, irrespective of MVP. Patients with MVP showed higher prevalence and extent of pseudo-MAD in all locations and true-MAD in the inferolateral wall., Competing Interests: Declaration of competing interests The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2025 The Authors. Published by Elsevier Inc. All rights reserved.)

Purpose: Mitral valve prolapse (MVP) has been associated with left heart remodeling. This study explored cardiac remodeling in patients with MVP without significant regurgitation, focusing on the right heart., Methods: This single-center study enrolled consecutive patients referred to trans-thoracic-echocardiography (TTE) with MVP, excluding those with significant regurgitation or known cardiovascular or pulmonary diseases. A group of healthy controls was included., Results: Forty-nine patients with MVP and 54 controls were finally selected (mean age of 62, 52-71; 52% males), and echocardiographic parameters were compared among groups. Twenty-nine (41%) patients with MVP showed tricuspid valve prolapse (TVP). Patients with MVP, irrespective of TVP, showed greater tricuspid annulus (systolic annulus: 31±6 vs. 32±5 vs. 27±3 mm for MVP + /TVP - , MVP + /TVP + , and controls, respectively; all p < 0.01) and greater minimum right atrial volume indexed (13, 12-15 mL/m 2  vs. 15, 12-20 mL/m 2 vs. 11, 10-14 mL/m 2 ; all p < 0.05). Right ventricular dimensions and systolic indexes did not differ among groups, except TAPSE, which was significantly greater in MVP + /TVP + patients compared to controls (25±4 vs. 22±3 mm, p = 0.004). A significant correlation (ρ = 0.43; p < 0.001) and an independent association at multivariate analysis (ß = 0.28; 95% CI 0.09-0.47; p = 0.004) were observed between end-systolic tricuspid diameter and TAPSE., Conclusion: In patients with MVP with less-than-moderate mitral or tricuspid regurgitation, dilation of the right atrium and tricuspid annulus was found. The latter finding was associated with increased values of TAPSE, which should then be used with caution, while other indexes may be preferred when assessing the systolic function of these patients., (© 2024 The Author(s). Echocardiography published by Wiley Periodicals LLC.)

The growing awareness of tricuspid regurgitation (TR) and the fast-expanding array of devices aiming to percutaneously repair or replace the tricuspid valve have underscored the central role of multi-modality imaging in comprehensively assessing the anatomical and functional characteristics of TR. Accurate phenotyping of TR, the right heart, and pulmonary vasculature via echocardiography, computed tomography, and, occasionally, cardiovascular magnetic resonance and right heart catheterization is deemed crucial in choosing the most suitable treatment strategy for each patient and achieving procedural success. In the first part of the present review, key imaging factors for patient selection will be discussed. In the ensuing sections, an overview of the most commonly used, commercially available systems for transcatheter repair/replacement will be presented, along with their respective selection criteria and information on intraprocedural imaging guidance; these are edge-to-edge repair, orthotopic and heterotopic replacement, and valve-in-valve procedures.

Background: Right ventricular (RV) involvement has been reported in one out of three patients with hypertrophic cardiomyopathy (HCM), however its prognostic significance remains unknown. We aimed to assess the prognostic value of RV involvement in patients with HCM through a systematic review and meta-analysis., Methods: A literature search was performed on PubMed, ClinicalTrials.gov and Cochrane Library databases from inception through November 15, 2023. Original articles enrolling HCM patients >18 years old and evaluating the association of RV parameters routinely assessed in clinical practice through trans-thoracic echocardiography or cardiac magnetic resonance (i.e., RV hypertrophy, volumes, systolic function, and late gadolinium enhancement) and the risk of a pre-defined composite endpoint including i) all cause death; ii) cardiac death; iii) heart transplantation; iv) heart failure-related hospitalization; v) atrial fibrillation; vi) ventricular tachycardia; and vii) stroke were retrieved. We pooled the effect of RV imaging variables on the combined clinical endpoint in terms of hazard ratio (HR) with 95% confidence interval (CI)., Results: The meta-analysis included 12 articles and 4634 patients. The pooled analysis demonstrated that the presence of RV systolic dysfunction conveyed an increased risk of adverse outcomes (HR 2.46; 95% CI 1.80-3.35; P < 0.001), whereas other RV imaging parameters were not significantly associated with patients' prognosis, except for RV-fractional area change analyzed as a continuous variable (HR 0.96 per % increase; 95% CI 0.93-0.995; P = 0.025)., Conclusions: Our results pinpoint a prognostic role of RV dysfunction, independent of left ventricular involvement, in patients with HCM, and future longitudinal studies, including multi-parametric RV assessment, are encouraged to provide clinically-relevant data to refine risk stratification in patients with HCM., Competing Interests: Declaration of competing interest None., (Copyright © 2024. Published by Elsevier B.V.)

Background: Previous studies have investigated a 1 to 6-month short dual antiplatelet therapy (S-DAPT) after percutaneous coronary intervention (PCI) with modern drug eluting-stents to reduce bleeding events., Objectives: To investigate cardiovascular outcomes in patients at high bleeding risk (HBR) according to the Academic Research Consortium for High Bleeding Risk (ARC-HBR) criteria after PCI with the Synergy bioresorbable-polymer everolimus-eluting stents (EES)., Methods: We applied ARC-HBR criteria in the population of the prospective, single-arm, multicenter POEM (Performance of Bioresorbable Polymer-Coated Everolimus-Eluting Synergy Stent in Patients at HBR Undergoing Percutaneous Coronary Revascularization Followed by 1-Month Dual Antiplatelet Therapy) trial. The primary endpoint was a composite of cardiac death, myocardial infarction, or definite or probable stent thrombosis at 12 months., Results: The original POEM cohort included 356 patients (80.4 %) fulfilling ARC-HBR criteria. Oral anticoagulant (OAC) usage and age ≥75 years were the most frequent major and minor ARC-HBR criteria, respectively. The ARC-HBR group was mainly represented by men (71.1 %), with 74.4 ± 9.3 years and a high burden of cardiovascular risk factors. DAPT was prescribed in 79.3 %, and single antiplatelet (SAPT) with OAC in 18.7 %. 12-month follow-up was completed in 96.2 %. The primary endpoint occurred in 5.2 % (95 % CI 3.29-8.10) of patients, whereas bleeding Academic Research Consortium type 3-5 occurred in 2.7 % (95 % CI, 1.39 %-5.05 %)., Conclusion: Previous results of the POEM trial showed positive outcomes regarding ischemic and bleeding events with an S-DAPT regimen after Synergy EES. These results are also confirmed in sub-group analysis when ARC-HBR criteria are applied., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Percutaneous closure of a patent foramen ovale (PFO), a common variation of interatrial septum anatomy, is a commonly performed procedure in the catheterization laboratory to reduce the risk of recurrent stroke in selected patients and to treat other PFO-related syndromes. In the last twenty years, disc-based devices have represented the armamentarium of the interventional cardiologist; recently, suture-based devices have become an attractive alternative, despite limited data regarding their long-term performance. The present review gives an overview of the current evidence regarding suture-based PFO closure, the device's characteristics, the echocardiographic evaluation of the PFO anatomy, and recommendations for patient selection. A detailed procedural guide is then provided, and potential complications and future developments in the field are discussed., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Aims: Electrolyte imbalances are common in patients with heart failure. Several studies have shown that a low serum chloride level is associated with adverse outcomes in hospitalized patients with acute heart failure and in outpatients with chronic heart failure. We performed a systematic review and meta-analysis to assess the association of hypochloremia with all-cause mortality in patients with heart failure., Methods: Data search was conducted from inception through 1 February 2023, using the following MeSH terms: ('chloride' OR 'hypochloremia') AND 'heart failure'. Studies evaluating the association between serum chloride and all-cause mortality in patients with heart failure were included. The predefined primary outcome was all-cause mortality. Pooled hazard ratios and 95% confidence intervals (CIs) were used as effect estimates and calculated with a random-effects model; fixed-effects model and leave-one-out sensitivity analyses were also performed., Results: A total of 15 studies, involving 25 848 patients, were included. The prevalence of hypochloremia ranged from 8.6 to 31.5%. Follow-up time ranged from 6 to 67 months. Hypochloremia as a categorical variable was associated with an increased risk of all-cause mortality [hazard ratio 1.56; 95% confidence interval (CI) 1.38-1.75; P  < 0.001]. As a continuous variable, serum chloride was associated with all-cause mortality (hazard ratio per mmol/l decrease in serum chloride: 1.06; 95% CI 1.05-1.07; P  < 0.001). Results were confirmed by using several sensitivity analyses., Conclusion: Hypochloremia exhibits a significant prognostic value in patients with heart failure. Serum chloride can be used as an effective tool for risk stratifying in patients with heart failure., (Copyright © 2024 Italian Federation of Cardiology - I.F.C. All rights reserved.)

Purpose To perform a systematic review and meta-analysis to assess the effect of enzyme replacement therapy on cardiac MRI parameters in patients with Fabry disease. Materials and Methods A systematic literature search was conducted from January 1, 2000, through January 1, 2024, in PubMed, ClinicalTrials.gov, Embase, and Cochrane Library databases. Study outcomes were changes in the following parameters: (a) left ventricular wall mass (LVM), measured in grams; (b) LVM indexed to body mass index, measured in grams per meters squared; (c) maximum left ventricular wall thickness (MLVWT), measured in millimeters; (d) late gadolinium enhancement (LGE) extent, measured in percentage of LVM; and (e) native T1 mapping, measured in milliseconds. A random-effects meta-analysis of the pooled mean differences between baseline and follow-up parameters was conducted. The study protocol was registered in PROSPERO (CRD42022336223). Results The final analysis included 11 studies of a total of 445 patients with Fabry disease (mean age ± SD, 41 years ± 11; 277 male, 168 female). Between baseline and follow-up cardiac MRI, the following did not change: T1 mapping (mean difference, 6 msec [95% CI: -2, 15]; two studies, 70 patients, I 2 = 88%) and LVM indexed (mean difference, -1 g/m 2 [95% CI: -6, 3]; four studies, 290 patients, I 2 = 81%). The following measures minimally decreased: LVM (mean difference, -18 g [95% CI: -33, -3]; seven studies, 107 patients, I 2 = 96%) and MLVWT (mean difference, -1 mm [95% CI: -2, -0.02]; six studies, 151 patients, I 2 = 90%). LGE extent increased (mean difference, 1% [95% CI: 1, 1]; three studies, 114 patients, I 2 = 85%). Conclusion In patients with Fabry disease, enzyme replacement therapy was associated with stabilization of LVM, MLVWT, and T1 mapping values, whereas LGE extent mildly increased. Keywords: Fabry Disease, Enzyme Replacement Therapy (ERT), Cardiac MRI, Late Gadolinium Enhancement (LGE) Supplemental material is available for this article. © RSNA, 2024.

Risk stratification for malignant ventricular arrhythmias and sudden cardiac death is a daunting task for physicians in daily practice. Multiparametric mapping sequences obtained via cardiovascular magnetic resonance imaging can improve the risk stratification for malignant ventricular arrhythmias by unveiling the presence of pathophysiological pro-arrhythmogenic processes. However, their employment in clinical practice is still restricted. The present review explores the current evidence supporting the association between mapping abnormalities and the risk of ventricular arrhythmias in several cardiovascular diseases. The key message is that further clinical studies are needed to test the additional value of mapping techniques beyond conventional cardiovascular magnetic resonance imaging for selecting patients eligible for an implantable cardioverter defibrillator.

Background: The presence of mitral annulus disjunction (MAD) has been considered a high-risk feature for sudden cardiac death based on selected study populations. We aimed to assess the prevalence of MAD in consecutive patients undergoing clinically indicated cardiovascular magnetic resonance (CMR), its association with ventricular arrhythmias, mitral valve prolapse (MVP), and other CMR features., Methods: This single-center retrospective study included consecutive patients referred to CMR at our institution between June 2021 and November 2021. MAD was defined as a ≥1 mm displacement between the left atrial wall-mitral valve leaflet junction and the left ventricular wall during end-systole. MAD extent was defined as the maximum longitudinal displacement. Associates of MAD were evaluated at univariable and multivariable regression analysis. The study endpoint, a composite of (aborted) sudden cardiac death, unexplained syncope, and sustained ventricular tachycardia, was evaluated at a 12-month follow-up., Results: Four hundred and forty-one patients 55 ± 18 years, 267/441 (61%) males) were included, and 29/441 (7%) had MVP. The prevalence of MAD ≥1 mm, 4 mm, and 6 mm was 214/441 (49%), 63/441 (14%), and 15/441 (3%), respectively. Patients with MVP showed a higher prevalence of MAD greater than 1 mm (26/29 (90%) vs 118/412 (46%)); p < 0.001), 4 mm (14/29 (48%) vs 49/412 (12%)); p < 0.001), and 6 mm (3/29 (10%) vs 12/412 (3%)); p = 0.03), and a greater MAD extent (4.2 mm, 3.0-5.7 mm vs 2.8 mm, 1.9-4.0 mm; p < 0.001) compared to patients without MVP. MVP was the only morpho-functional abnormality associated with MAD at multivariable analysis (p < 0.001). A high burden of ventricular ectopic beats at baseline Holter-electrocardiogram was associated with MAD ≥4 mm and MAD extent (p < 0.05). The presence of MAD ≥1 mm (0.9% vs 1.8%; p = 0.46), MAD ≥4 mm (1.6% vs 1.3%; p = 0.87), or MVP (3.5% vs 1.2%; p = 0.32) were not associated with the study endpoint, whereas patients with MAD ≥6 mm showed a trend toward a higher likelihood of the study endpoint (6.7% vs 1.2%; p = 0.07)., Conclusion: MAD of limited severity was common in consecutive patients undergoing CMR. Patients with MVP showed higher prevalence and greater extent of MAD. Extended MAD was rarer and showed association with ventricular arrhythmias at baseline. The mid-term prognosis of MAD seems benign; however, prospective studies are warranted to search for potential "malignant MAD extents" to improve patients' risk stratification., Competing Interests: Declaration of competing interests The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.)

An 81-year-old woman presented with acute pulmonary edema. Echocardiography revealed severe functional mitral regurgitation, the mechanism of which was unusual. An atypical bileaflet tethering caused by disharmonic annular remodeling, concomitant aortic dilatation, and reduced aorto-mitral angle without left ventricular dysfunction or dilatation was found. A transcatheter edge-to-edge repair was nonetheless successfully performed., Competing Interests: The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (© 2024 The Authors.)

Fabry disease (FD) is a genetic lysosomal storage disease with frequent cardiovascular involvement, whose presence is a major determinant of adverse clinical outcomes. As a potentially treatable cause of left ventricular hypertrophy (LVH) and heart failure with preserved ejection fraction, the early recognition of FD is crucial to initiate enzyme replacement therapy and improve long-term prognosis. Multimodality imaging plays a central role in the evaluation of patients with FD and helps in the differential diagnosis of other conditions presenting with LVH. In the present review, we explore the current applications of multimodality cardiac imaging, in particular echocardiography and cardiovascular magnetic resonance, in the diagnosis, prognostic assessment, and follow-up of patients with FD.

Background: Cardiovascular sequelae may occur in patients recovered from coronavirus disease 2019 (COVID-19). Recent studies have detected a considerable incidence of subclinical myocardial dysfunction-assessed with speckle-tracking echocardiography-and of long-COVID symptoms in these patients. This study aimed to define the long-term prognostic role of subclinical myocardial dysfunction and long-COVID condition in patients recovered from COVID-19 pneumonia., Methods: We prospectively followed up 110 patients hospitalized at our institution due to COVID-19 pneumonia in April 2020 and then recovered from SARS-CoV-2 infection. A 7-month clinical and echocardiographic evaluation was performed, followed by a 21-month clinical follow-up. The primary outcome was major adverse cardiovascular events (MACE), a composite of myocardial infarction, stroke, heart failure hospitalization, and all-cause mortality., Results: A subclinical myocardial dysfunction-defined as an impairment of left ventricular global longitudinal strain (≥-18%)-was identified at a 7-month follow-up in 37 patients (34%), was associated with an increased risk of long-term MACE with a good discriminative power (area under the curve: .73) and resulted in a strong independent predictor of extended MACE in multivariate regression analyses. Long-COVID condition was not associated with a worse long-term prognosis, instead., Conclusions: In patients recovered from COVID-19 pneumonia, a subclinical myocardial dysfunction is present in one-third of the whole population at 7-month follow-up and is associated with a higher risk of MACE at long-term follow-up. Speckle-tracking echocardiography is a promising tool to optimize the risk-stratification in patients recovered from COVID-19 pneumonia, while the definition of a long-COVID condition has no prognostic relevance., (© 2023 The Authors. Echocardiography published by Wiley Periodicals LLC.)

Cardiac magnetic resonance (CMR) has been recently implemented in clinical practice to refine the daunting task of establishing the risk of sudden cardiac death (SCD) in patients with hypertrophic cardiomyopathy (HCM). We present an exemplificative case highlighting the practical clinical utility of this imaging modality in a 24-year-old man newly diagnosed with an apical HCM. CMR was essential in unmasking a high risk of SCD, which appeared low-intermediate after traditional risk assessment. A discussion examines the essential role of CMR in guiding the patient's therapy and underlines the added value of CMR, including novel and potential CMR parameters, compared to traditional imaging assessment for SCD risk stratification.