Role of Cine-Magnetic Resonance Imaging in the Assessment of Mediastinal Masses with Uncertain/Equivocal Findings from Pre-Operative Computed Tomography Scanning.

BACKGROUND: Malignant neoplasms originating from or involving the mediastinum represent a diagnostic and therapeutic challenge when they are in contact with nearby cardiovascular structures. We aimed to test the diagnostic accuracy of cine-magnetic resonance imaging (cine-MRI) in detecting the infiltration of cardiovascular structures in cases with uncertain or equivocal findings from contrast-enhanced Computed Tomography (CT) scanning. METHODS: Fifty patients affected by tumors with a suspected invasion of mediastinal cardiovascular structures at the pre-operative chest CT scan stage underwent cine-MRI before surgery at our Institution. Intraoperative findings and the histological post-surgical report were used as a reference standard to define infiltration. Inter- and intra-observer agreement for CT scans and cine-MRI were also computed over a homogenous sample of 14 patients. RESULTS: Cine-MRI had a higher negative predictive value (93% vs. 54%, p < 0.001) than CT scans, higher sensitivity (91% vs. 16%, p < 0.001), as well as greater accuracy (66% vs. 50%, p < 0.001) in detecting cardiovascular invasion. Cine-MRI also showed better inter- and intra-observer agreement for infiltration detection. CONCLUSIONS: Cine-MRI outperforms conventional contrast-enhanced chest CT scans in the preoperative assessment of cardiovascular infiltration by mediastinal or pulmonary tumors, making it a useful imaging modality in the preoperative staging and evaluation of patients with equivocal findings at the chest CT scan stage.

Mitral annulus disjunction in consecutive patients undergoing cardiovascular magnetic resonance: Where is the boundary between normality and disease?

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.

Prognostic value of right ventricular involvement in hypertrophic cardiomyopathy: A systematic review and meta-analysis.

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.

CT imaging post-TAVI: Murphy's first law in action-preparing to recognize the unexpected.

Transfemoral aortic valve implantation (TAVI) has been long considered the standard of therapy for high-risk patients with severe aortic-stenosis and is now effectively employed in place of surgical aortic valve replacement also in intermediate-risk patients. The potential lasting consequences of minor complications, which might have limited impact on elderly patients, could be more noteworthy in the longer term when occurring in younger individuals. That's why a greater focus on early diagnosis, correct management, and prevention of post-procedural complications is key to achieve satisfactory results. ECG-triggered multidetector computed tomography angiography (CTA) is the mainstay imaging modality for pre-procedural planning of TAVI and is also used for post-interventional early detection of both acute and long-term complications. CTA allows detailed morphological analysis of the valve and its movement throughout the entire cardiac cycle. Moreover, stent position, coronary artery branches, and integrity of the aortic root can be precisely evaluated. Imaging reliability implies the correct technical setting of the computed tomography scan, knowledge of valve type, normal post-interventional findings, and awareness of classic and life-threatening complications after a TAVI procedure. This educational review discusses the main post-procedural complications of TAVI with a specific imaging focus, trying to clearly describe the technical aspects of CTA Imaging in post-TAVI and its clinical applications and challenges, with a final focus on future perspectives and emerging technologies. CRITICAL RELEVANCE STATEMENT: This review undertakes an analysis of the role computed tomography angiography (CTA) plays in the assessment of post-TAVI complications. Highlighting the educational issues related to the topic, empowers radiologists to refine their clinical approach, contributing to enhanced patient care. KEY POINTS: Prompt recognition of TAVI complications, ranging from value issues to death, is crucial. Adherence to recommended scanning protocols, and the optimization of tailored protocols, is essential. CTA is central in the diagnosis of TAVI complications and functions as a gatekeeper to treatment.

Arrhythmic Mitral Valve Prolapse Phenotype: An Unsupervised Machine Learning Analysis Using a Multicenter Cardiac MRI Registry.

Purpose To use unsupervised machine learning to identify phenotypic clusters with increased risk of arrhythmic mitral valve prolapse (MVP). Materials and Methods This retrospective study included patients with MVP without hemodynamically significant mitral regurgitation or left ventricular (LV) dysfunction undergoing late gadolinium enhancement (LGE) cardiac MRI between October 2007 and June 2020 in 15 European tertiary centers. The study end point was a composite of sustained ventricular tachycardia, (aborted) sudden cardiac death, or unexplained syncope. Unsupervised data-driven hierarchical k-mean algorithm was utilized to identify phenotypic clusters. The association between clusters and the study end point was assessed by Cox proportional hazards model. Results A total of 474 patients (mean age, 47 years ± 16 [SD]; 244 female, 230 male) with two phenotypic clusters were identified. Patients in cluster 2 (199 of 474, 42%) had more severe mitral valve degeneration (ie, bileaflet MVP and leaflet displacement), left and right heart chamber remodeling, and myocardial fibrosis as assessed with LGE cardiac MRI than those in cluster 1. Demographic and clinical features (ie, symptoms, arrhythmias at Holter monitoring) had negligible contribution in differentiating the two clusters. Compared with cluster 1, the risk of developing the study end point over a median follow-up of 39 months was significantly higher in cluster 2 patients (hazard ratio: 3.79 [95% CI: 1.19, 12.12], P = .02) after adjustment for LGE extent. Conclusion Among patients with MVP without significant mitral regurgitation or LV dysfunction, unsupervised machine learning enabled the identification of two phenotypic clusters with distinct arrhythmic outcomes based primarily on cardiac MRI features. These results encourage the use of in-depth imaging-based phenotyping for implementing arrhythmic risk prediction in MVP. Keywords: MR Imaging, Cardiac, Cardiac MRI, Mitral Valve Prolapse, Cluster Analysis, Ventricular Arrhythmia, Sudden Cardiac Death, Unsupervised Machine Learning Supplemental material is available for this article. © RSNA, 2024.

Multiparametric Mapping via Cardiovascular Magnetic Resonance in the Risk Stratification of Ventricular Arrhythmias and Sudden Cardiac Death.

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.

CMR Mapping: The 4th-Era Revolution in Cardiac Imaging.

Cardiac magnetic resonance (CMR) imaging has witnessed substantial progress with the advent of parametric mapping techniques, most notably T1 and T2 mapping. These advanced techniques provide valuable insights into a wide range of cardiac conditions, including ischemic heart disease, cardiomyopathies, inflammatory cardiomyopathies, heart valve disease, and athlete's heart. Mapping could be the first sign of myocardial injury and oftentimes precedes symptoms, changes in ejection fraction, and irreversible myocardial remodeling. The ability of parametric mapping to offer a quantitative assessment of myocardial tissue properties addresses the limitations of conventional CMR methods, which often rely on qualitative or semiquantitative data. However, challenges persist, especially in terms of standardization and reference value establishment, hindering the wider clinical adoption of parametric mapping. Future developments should prioritize the standardization of techniques to enhance their clinical applicability, ultimately optimizing patient care pathways and outcomes. In this review, we endeavor to provide insights into the potential contributions of CMR mapping techniques in enhancing the diagnostic processes across a range of cardiac conditions.

Deep Learning Image Reconstruction Algorithm for CCTA: Image Quality Assessment and Clinical Application.

OBJECTIVE: The increasing number of coronary computed tomography angiography (CCTA) requests raised concerns about dose exposure. New dose reduction strategies based on artificial intelligence have been proposed to overcome limitations of iterative reconstruction (IR) algorithms. Our prospective study sought to explore the added value of deep-learning image reconstruction (DLIR) in comparison with a hybrid IR algorithm (adaptive statistical iterative reconstruction-veo [ASiR-V]) in CCTA, even in clinical challenging scenarios, as obesity, heavily calcified vessels and coronary stents. METHODS: We prospectively included 103 consecutive patients who underwent CCTA. Data sets were reconstructed with ASiR-V and DLIR. For each reconstruction signal-to-noise ratio (SNR) and contrast-to-noise ratio (CNR) was calculated, and qualitative assessment was made with a four-point Likert scale by two independent and blinded radiologists with different expertise. RESULTS: Both SNR and CNR were significantly higher in DLIR (SNR-DLIR median value [interquartile range] of 13.89 [11.06-16.35] and SNR-ASiR-V 25.42 [22.46-32.22], P < 0.001; CNR-DLIR 16.84 [9.83-27.08] vs CNR-ASiR-V 10.09 [5.69-13.5], P < 0.001).Median qualitative score was 4 for DLIR images versus 3 for ASiR-V ( P < 0.001), with a good interreader reliability [intraclass correlation coefficient(2,1)e intraclass correlation coefficient(3,1) 0.60 for DLIR and 0.62 and 0.73 for ASiR-V].In the obese and in the "calcifications and stents" groups, DLIR showed significantly higher values of SNR (24.23 vs 11.11, P < 0.001 and 24.55 vs 14.09, P < 0.001, respectively) and CNR (16.08 vs 8.04, P = 0.008 and 17.31 vs 10.14, P = 0.003) and image quality. CONCLUSIONS: Deep-learning image reconstruction in CCTA allows better SNR, CNR, and qualitative assessment than ASiR-V, with an added value in the most challenging clinical scenarios.

Fabry Disease: More than a Phenocopy of Hypertrophic Cardiomyopathy.

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.

Cardiovascular Magnetic Resonance in Patients with Cardiac Electronic Devices: Evidence from a Multicenter Study.

BACKGROUND: Most recent cardiac implantable electronic devices (CIEDs) can safely undergo a cardiovascular magnetic resonance (CMR) scan under certain conditions, but metal artifacts may degrade image quality. The aim of this study was to assess the overall diagnostic yield of CMR and the extent of metal artifacts in a multicenter, multivendor study on CIED patients referred for CMR. METHODS: We analyzed 309 CMR scans from 292 patients (age 57 ± 16 years, 219 male) with an MR-conditional pacemaker (n = 122), defibrillator (n = 149), or loop recorder (n = 38); CMR scans were performed in 10 centers from 2012 to 2020; MR-unsafe implants were excluded. Clinical and device parameters were recorded before and after the CMR scan. A visual analysis of metal artifacts was performed for each sequence on a segmental basis, based on a 5-point artifact score. RESULTS: The vast majority of CMR scans (n = 255, 83%) were completely performed, while only 32 (10%) were interrupted soon after the first sequences and 22 (7%) were only partly acquired; CMR quality was non-diagnostic in 34 (11%) scans, poor (<1/3 sequences were diagnostic) in 25 (8%), or acceptable (1/3 to 2/3 sequences were diagnostic) in 40 (13%), while most scans (n = 201, 68%) were of overall good quality. No adverse event or device malfunctioning occurred, and only nonsignificant changes in device parameters were recorded. The most affected sequences were SSFP (median score 0.32 [interquartile range 0.07-0.91]), followed by GRE (0.18 [0.02-0.59]) and LGE (0.14 [0.02-0.55]). ICDs induced more artifacts (median score in SSFP images 0.87 [0.50-1.46]) than PMs (0.11 [0.03-0.28]) or ILRs (0.11 [0.00-0.56]). Moreover, most artifacts were located in the anterior, anteroseptal, anterolateral, and apical segments of the LV and in the outflow tract of the RV. CONCLUSIONS: CMR is a versatile imaging technique, with a high safety profile and overall good image quality even in patients with MR-conditional CIEDs. Several strategies are now available to optimize image quality, substantially enhancing overall diagnostic yield.

Multimodality imaging in cardio-oncology: the added value of CMR and CCTA.

During the last 30 years, we have assisted to a great implementation in anticancer treatment with a subsequent increase of cancer survivors and decreased mortality. This has led to an ongoing interest about the possible therapy-related side-effects and their management to better guide patients therapy and surveillance in the chronic and long-term setting. As a consequence cardio-oncology was born, involving several different specialties, among which radiology plays a relevant role. Till the end of August 2022, when European Society of Cardiology (ESC) developed the first guidelines on cardio-oncology, no general indications existed to guide diagnosis and treatment of cancer therapy-related cardiovascular toxicity (CTR-CVT). They defined multimodality imaging role in primary and secondary prevention strategies, cancer treatment surveillance and early CTR-CVT identification and management. Cardiac computed tomography angiography (CCTA) has acquired a central role in coronary assessment, as far as coronary artery disease (CAD) exclusion is concerned; but on the side of this well-known application, it also started to be considered in left ventricular function evaluation, interstitial fibrosis quantification and cardiac perfusion studies. Cardiac magnetic resonance (CMR), instead, has been acknowledged as the gold standard alternative to trans-thoracic echocardiography (TTE) poor acoustic window in quantification of heart function and strain modifications, as well as pre- and post-contrast tissue characterization by means of T1-T2 mapping, early Gadolinium enhancement (EGE), late Gadolinium enhancement (LGE) and extracellular volume (ECV) evaluation. Our review is intended to provide a focus on the actual role of CMR and CCTA in the setting of a better understanding of cardiotoxicity and to draw some possible future directions of cardiac imaging in this field, starting from the recently published ESC guidelines.

Solving the Riddle of Sudden Cardiac Death in Hypertrophic Cardiomyopathy: The Added Role of Cardiac Magnetic Resonance.

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.

Nebivolol versus placebo in patients undergoing anthracyclines (CONTROL Trial): rationale and study design.

AIMS: Anthracyclines are the chemotherapeutic agents most frequently associated with cardiotoxicity, while remaining widely used. Different neurohormonal blockers have been tested as a primary prevention strategy to prevent or attenuate the onset of cardiotoxicity, with mixed results. However, prior studies were often limited by a nonblinded design and an assessment of cardiac function based only on echocardiographic imaging. Moreover, on the basis of an improved mechanistic understanding of anthracycline cardiotoxicity mechanisms, new therapeutic strategies have been proposed. Among cardioprotective drugs, nebivolol might be able to prevent the cardiotoxic effects of anthracyclines, through its protective properties towards the myocardium, endothelium, and cardiac mitochondria. This study aims to evaluate the cardioprotective effects of the beta blocker nebivolol in a prospective, placebo-controlled, superiority randomized trial in patients with breast cancer or diffuse large B cell lymphoma (DLBCL) who have a normal cardiac function and will receive anthracyclines as part of their first-line chemotherapy programme. METHODS: The CONTROL trial is a randomized, placebo-controlled, double-blinded, superiority trial. Patients with breast cancer or a DLBCL, with a normal cardiac function as assessed by echocardiography, scheduled for treatment with anthracyclines as part of their first-line chemotherapy programme will be randomized 1 : 1 to nebivolol 5 mg once daily (o.d.) or placebo. Patients will be examined with cardiological assessment, echocardiography and cardiac biomarkers at baseline, 1 month, 6 months and 12 months. A cardiac magnetic resonance (CMR) assessment will be performed at baseline and at 12 months. The primary end point is defined as left ventricular ejection fraction reduction assessed by CMR at 12 months of follow-up. CONCLUSION: The CONTROL trial is designed to provide evidence to assess the cardioprotective role of nebivolol in patients undergoing chemotherapy with anthracyclines. CLINICAL TRIAL REGISTRATION: The study is registered in the EudraCT registry (number: 2017-004618-24) and in the ClinicalTrials.gov registry (identifier: NCT05728632).

The prognostic role of right ventricular dysfunction in patients with hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) primarily affects the left ventricle (LV) sparing the right ventricle (RV) in vast majority of cases. However, several studies employing CMR have revealed that myocardial hypertrophy may also involve the RV. To assess RV size and function in a large prospectively cohort of HCM patients and to evaluate whether these parameters in association with other MR findings can predict cardiac events. Two participating centers prospectively included patients with known or suspected HCM between 2011 and 2017. CMR studies were performed with three different scanners. Outcome measures were a composite of ventricular arrhythmias, hospitalization for HF and cardiac death. Of 607 consecutive patients with known or suspected HCM, 315 had complete follow-up information (mean 65 ± 20 months). Among them, 115 patients developed major cardiac events (MACE) during follow-up. At CMR evaluation, patients with events had higher left atrium (LA) diameter (41.5 ± 8 mm vs. 37.17 ± 7.6 mm, p < 0.0001), LV mass (156.7 vs. 144 g, p = 0.005) and myocardial LGE (4.3% vs. 1.9%, p = 0.001). Similarly, patients with events had lower RV stroke volume index (42.7 vs. 47.0, p = 0.0003) and higher prevalence of both RV hypertrophy (16.4% vs. 4.7%, p = 0.0005) and reduced RV ejection fraction (12.2% vs. 4.4%, p = 0.006). In the multivariate analysis, LA diameter and RV stroke volume index were the strongest predictors of events (p < 0.001 and p = 0.0006, respectively). Anatomic and functional RV anomalies detected and characterized with CMR may have may have a major role in predicting the prognosis of HCM patients.

Myocardial Fibrosis at Cardiac MRI Helps Predict Adverse Clinical Outcome in Patients with Mitral Valve Prolapse.

Background Patients with mitral valve prolapse (MVP) may develop adverse outcomes even in the absence of mitral regurgitation or left ventricular (LV) dysfunction. Purpose To investigate the prognostic value of mitral annulus disjunction (MAD) and myocardial fibrosis at late gadolinium enhancement (LGE) cardiac MRI in patients with MVP without moderate-to-severe mitral regurgitation or LV dysfunction. Materials and Methods In this longitudinal retrospective study, 118 144 cardiac MRI studies were evaluated between October 2007 and June 2020 at 15 European tertiary medical centers. Follow-up was from the date of cardiac MRI examination to June 2020; the minimum and maximum follow-up intervals were 6 months and 156 months, respectively. Patients were excluded if at least one of the following conditions was present: cardiomyopathy, LV ejection fraction less than 40%, ischemic heart disease, congenital heart disease, inflammatory heart disease, moderate or worse mitral regurgitation, participation in competitive sport, or electrocardiogram suggestive of channelopathies. In the remainder, cardiac MRI studies were reanalyzed, and patients were included if they were aged 18 years or older, MVP was diagnosed at cardiac MRI, and clinical information and electrocardiogram monitoring were available within 3 months from cardiac MRI examination. The end point was a composite of adverse outcomes: sustained ventricular tachycardia (VT), sudden cardiac death (SCD), or unexplained syncope. Multivariable Cox regression analysis was performed. Results A total of 474 patients (mean age, 47 years ± 16 [SD]; 244 women) were included. Over a median follow-up of 3.3 years, 18 patients (4%) reached the study end point. LGE presence (hazard ratio, 4.2 [95% CI: 1.5, 11.9]; P = .006) and extent (hazard ratio, 1.2 per 1% increase [95% CI: 1.1, 1.4]; P = .006), but not MAD presence (P = .89), were associated with clinical outcome. LGE presence had incremental prognostic value over MVP severity and sustained VT and aborted SCD at baseline (area under the receiver operating characteristic curve, 0.70 vs 0.62; P = .03). Conclusion In contrast to mitral annulus disjunction, myocardial fibrosis determined according to late gadolinium enhancement at cardiac MRI was associated with adverse outcome in patients with mitral valve prolapse without moderate-to-severe mitral regurgitation or left ventricular dysfunction. © RSNA, 2022 Online supplemental material is available for this article. See also the editorial by Gerber in this issue.

Value of 3D echocardiography in the diagnosis of arrhythmogenic right ventricular cardiomyopathy.

AIMS: The 2010 Task Force Criteria (TFC) require that both right ventricular (RV) regional wall-motion abnormalities (WMA) and specific RV size cut-offs be met in order to fulfil one of the major criterion for arrhythmogenic right ventricular cardiomyopathy (ARVC) diagnosis. Currently, 2D echocardiography (2DE) and cardiovascular magnetic resonance imaging (cMRI) are used to determine if these criteria are met. Little is known about the diagnostic value of 3D echocardiography (3DE) in ARVC. The aim of this study was to determine whether a combination of 2DE-3DE is non-inferior to the currently used 2DE-cMRI combination in the diagnosis of patients with ARVC. METHODS AND RESULTS: Thirty-nine individuals (47±15 years) with suspected ARVC underwent evaluation of the RV with cMRI, 2DE, and 3DE. 3DE and cMRI were independently used to obtain RV volumes, ejection fraction (EF) and determine the presence of segmental RV WMA. Studies were blindly classified as meeting criteria for ARVC in accordance with the 2010 TFC. Kappa statistics were used to test the concordance between 2DE-cMRI and 2DE-3DE approaches. Using the 2DE-cMRI approach, 3/39 were not affected, 5/39 possible, 8/39 borderline, and 23/39 definite ARVC. The proposed 2DE-3DE approach yielded 5/39 not affected, 7/39 possible, 8/39 borderline, and 19/39 definite diagnoses. The two approaches were highly concordant (k = 0.71; 95% confidence interval: 0.44-0.84). Although 3DE underestimated RV volumes in comparison with cMRI, interfering, in some instances with the fulfilment of a major criterion, it was able to identify more RV WMA (28/39) than 2DE (11/39), with a detection-rate comparable to cMRI (33/39) highlighting a unique advantage. CONCLUSION: The combination of 2DE-3DE for ARVC diagnosis is comparable to the conventional 2DE-cMRI approach. 3DE should be performed in all suspected ARVC patients to aide in the detection of WMA.