Mitral Annulus Disjunction in consecutive patients undergoing Cardiac 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 Cardiac 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. The MAD was defined as a ≥1mm displacement between the left atrial wall-mitral valve leaflet junction and the left ventricular wall during end-systole. The MAD extent was defined as the maximum longitudinal displacement. Associates of MAD were evaluated at uni- and multi-variariable regression analysis. A study endpoint including (aborted) sudden cardiac death, unexplained syncope, and sustained ventricular tachycardia was evaluated at 12-month follow-up. RESULTS: Four-hundred-forty-one patients (55±18 years, 61% males) were included, and 29 (7%) had MVP. The prevalence of MAD ≥1mm, 4mm, and 6mm were 214 (49%), 63 (14%), and 15 (3%), respectively. Patients with MVP showed a higher prevalence of MAD greater than 1mm (90% vs. 46%; p<0.001), 4mm (48% vs. 12%; p<0.001), and 6mm (10% vs. 3%; p=0.03), and a greater MAD extent (4.2mm, 3.0-5.7mm vs. 2.8mm, 1.9-4.0mm; p<0.001) than patients without MVP. The 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-ECG was associated with MAD ≥4mm and MAD extent (p<0.05). The presence of MAD ≥1mm (0.9% vs. 1.8%; p=0.46), MAD ≥4mm (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 ≥6mm showed a trend towards a higher likelihood of the study endpoint (6.7% vs. 1.2%; p=0.07). CONCLUSIONS: A MAD of limited entity 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.

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.

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.

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.

Cardiac Masses and Pseudomasses: An Overview about Diagnostic Imaging and Clinical Background.

A cardiac lesion detected at ultrasonography might turn out to be a normal structure, a benign tumor or rarely a malignancy, and lesion characterization is very important to appropriately manage the lesion itself. The exact relationship of the mass with coronary arteries and the knowledge of possible concomitant coronary artery disease are necessary preoperative information. Moreover, the increasingly performed coronary CT angiography to evaluate non-invasively coronary artery disease leads to a rising number of incidental findings. Therefore, CT and MRI are frequently performed imaging modalities when echocardiography is deemed insufficient to evaluate a lesion. A brief comprehensive overview about diagnostic radiological imaging and the clinical background of cardiac masses and pseudomasses is reported.

Similar clinical and radiographic outcomes after two different hypoallergenic medial unicompartmental knee in patients with metal allergy.

BACKGROUND: The purpose in the present study was to compare clinical and radiological outcomes of patients who had undergone a mobile-bearing unicompartmental knee arthroplasty (UKA) with either titanium niobium nitride (TiNbN) alloy implants or with fixed-bearing oxidized zirconium alloy implants. METHODS: The records of two consecutive cohorts for a total of 86 hypoallergenic implants were prospectively analyzed. The first cohort consisted of 49 consecutive implantations of the hypoallergenic UKA Journey Uni Oxinium (Ox Group), while the second consisted of 37 consecutive series of UKA Oxford (TiNbN Group). All patients were evaluated by two independent surgeons who were not involved in the index surgery. The clinical evaluation consisted of evaluating each patient's Oxford Knee Score and Knee Society Score day before surgery (T0), and with two consecutive follow-ups at T1 (minimum follow-up 9 months) and T2 (minimum follow-up 24 months). RESULTS: The two groups were homogeneous in all preoperative values, except Body Mass Index (BMI) and duration of final follow-up [both statistically higher (p < 0.05) in the TiNbN group]. Both groups showed a clinically significant improvement for all scores at final follow-up (p < 0.05). The only differences between the two groups involved a higher pre-operative Oxford Score in TiNbN group (p = 0.031), and different tibial and femoral angles at the final follow-up. CONCLUSIONS: Both TiNbN and Oxinium UKA procedures enabled patients from good to excellent clinical and radiographic outcomes after the final follow-up, regardless of the age, gender, BMI bearing type, and implant size. LEVEL OF EVIDENCE: LEVEL II: Comparative study.

Prognostic value of tricuspid regurgitation.

Tricuspid regurgitation (TR) has a considerable prevalence in the overall population, that further increases in selected categories of patients. Three morphologic types of TR prevail, namely primary, secondary and atrial TR, mostly, but not always, occurring in different subsets of patients. Recent evidences demonstrate a negative impact of TR on outcomes, irrespective of etiology and even when less than severe in grading. Unfortunately, current surgical standards are void of strong prospective evidence of positive impact on clinical outcomes. While on one hand recent advances in diagnosis and risk stratification of patients with TR are shedding light onto the population that may benefit from intervention and its appropriate timing, on the other hand the arrival on stage of percutaneous treatment options is widening even more the therapeutic options for such population. In this review we will address and discuss the available evidence on the prognostic impact of TR in different clinical contexts encountered in practice.

Cardiac magnetic resonance imaging of myocarditis and pericarditis following COVID-19 vaccination: a multicenter collection of 27 cases.

OBJECTIVES: To assess clinical and cardiac magnetic resonance (CMR) imaging features of patients with peri-myocarditis following Coronavirus Disease 2019 (COVID-19) vaccination. METHODS: We retrospectively collected a case series of 27 patients who underwent CMR in the clinical suspect of heart inflammation following COVID-19 vaccination, from 16 large tertiary centers. Our patient's cohort was relatively young (36.6 ± 16.8 years), predominately included males (n = 25/27) with few comorbidities and covered a catchment area of approximately 8 million vaccinated patients. RESULTS: CMR revealed typical mid-subepicardial non-ischemic late gadolinium enhancement (LGE) in 23 cases and matched positively with CMR T2 criteria of myocarditis. In 7 cases, typical hallmarks of acute pericarditis were present. Short-term follow-up (median = 20 days) from presentation was uneventful for 25/27 patients and unavailable in two cases. CONCLUSIONS: While establishing a causal relationship between peri-myocardial inflammation and vaccine administration can be challenging, our clinical experience suggests that CMR should be performed for diagnosis confirmation and to drive clinical decision-making and follow-up. KEY POINTS: • Acute onset of dyspnea, palpitations, or acute and persisting chest pain after COVID-19 vaccination should raise the suspicion of possible myocarditis or pericarditis, and patients should seek immediate medical attention and treatment to help recovery and avoid complications. • In case of elevated troponin levels and/or relevant ECG changes, cardiac magnetic resonance should be considered as the best non-invasive diagnostic option to confirm the diagnosis of myocarditis or pericarditis and to drive clinical decision-making and follow-up.

Edge-Based Transfer Learning for Classroom Occupancy Detection in a Smart Campus Context.

Studies and systems that are aimed at the identification of the presence of people within an indoor environment and the monitoring of their activities and flows have been receiving more attention in recent years, specifically since the beginning of the COVID-19 pandemic. This paper proposes an approach for people counting that is based on the use of cameras and Raspberry Pi platforms, together with an edge-based transfer learning framework that is enriched with specific image processing strategies, with the aim of this approach being adopted in different indoor environments without the need for tailored training phases. The system was deployed on a university campus, which was chosen as the case study. The proposed system was able to work in classrooms with different characteristics. This paper reports a proposed architecture that could make the system scalable and privacy compliant and the evaluation tests that were conducted in different types of classrooms, which demonstrate the feasibility of this approach. Overall, the system was able to count the number of people in classrooms with a maximum mean absolute error of 1.23.

Cardiac magnetic resonance predicts ventricular arrhythmias in scleroderma: the Scleroderma Arrhythmia Clinical Utility Study (SAnCtUS).

OBJECTIVES: Cardiac rhythm disturbances constitute the most frequent cardiovascular cause of death in SSc. However, electrocardiographic findings are not a part of risk stratification in SSc. We aimed to translate 24 h Holter findings into a tangible risk prediction score using cardiovascular magnetic resonance. METHODS: The Scleroderma Arrhythmia Clinical Utility Study (SAnCtUS) was a prospective multicentre study including 150 consecutive SSc patients from eight European centres, assessed with 24 h Holter and cardiovascular magnetic resonance, including ventricular function, oedema (T2 ratio) and late gadolinium enhancement (%LGE). Laboratory/clinical parameters were included in multivariable corrections. A combined endpoint of sustained ventricular tachycardia requiring hospitalization and sudden cardiac death at a median (interquartile range) follow-up of 1 (1.0-1.4) year was generated. RESULTS: Only T2 ratio and %LGE were significant predictors of ventricular rhythm disturbances, but not of supraventricular rhythm disturbances, after multivariable correction and adjustment for multiple comparisons. Using decision-tree analysis, we created the SAnCtUS score, a four-category scoring system based on T2 ratio and %LGE, for identifying SSc patients at high risk of experiencing ventricular rhythm disturbance at baseline. Increasing SAnCtUS scores were associated with a greater disease and arrhythmic burden. All cases of non-sustained ventricular tachycardia (n = 7) occurred in patients with the highest SAnCtUS score (=4). Having a score of 4 conveyed a higher risk of reaching the combined endpoint in multivariable Cox regression compared with scores 1/2/3 [hazard ratio (95% CI): 3.86 (1.14, 13.04), P = 0.029] independently of left ventricular ejection fraction and baseline ventricular tachycardia occurrence. CONCLUSION: T2 ratio and %LGE had the greatest utility as independent predictors of rhythm disturbances in SSc patients.

RaveGuard: A Noise Monitoring Platform Using Low-End Microphones and Machine Learning.

Urban noise is one of the most serious and underestimated environmental problems. According to the World Health Organization, noise pollution from traffic and other human activities, negatively impact the population health and life quality. Monitoring noise usually requires the use of professional and expensive instruments, called phonometers, able to accurately measure sound pressure levels. In many cases, phonometers are human-operated; therefore, periodic fine-granularity city-wide measurements are expensive. Recent advances in the Internet of Things (IoT) offer a window of opportunities for low-cost autonomous sound pressure meters. Such devices and platforms could enable fine time-space noise measurements throughout a city. Unfortunately, low-cost sound pressure sensors are inaccurate when compared with phonometers, experiencing a high variability in the measurements. In this paper, we present RaveGuard, an unmanned noise monitoring platform that exploits artificial intelligence strategies to improve the accuracy of low-cost devices. RaveGuard was initially deployed together with a professional phonometer for over two months in downtown Bologna, Italy, with the aim of collecting a large amount of precise noise pollution samples. The resulting datasets have been instrumental in designing InspectNoise, a library that can be exploited by IoT platforms, without the need of expensive phonometers, but obtaining a similar precision. In particular, we have applied supervised learning algorithms (adequately trained with our datasets) to reduce the accuracy gap between the professional phonometer and an IoT platform equipped with low-end devices and sensors. Results show that RaveGuard, combined with the InspectNoise library, achieves a 2.24% relative error compared to professional instruments, thus enabling low-cost unmanned city-wide noise monitoring.

Reference values of cardiac volumes, dimensions, and new functional parameters by MR: A multicenter, multivendor study.

PURPOSE: To define reference values of cardiac volumes, dimensions, and new morpho-functional parameters normalized for age, gender, and body surface area by cine-bSSFP (balanced steady-state free-precession) magnetic resonance (MR). MATERIALS AND METHODS: We enrolled 308 healthy subjects subdivided by gender and by six age classes: class I, >15-20 years; class II, >20-30 years; class III, >30-40 years; class IV, >40-50 years; class V, >50-60 years; and class VI >60 years. Dimensional, volumetric and morpho-functional parameters of the left (LV) and right (RV) ventricles were measured using cine-bSSFP MRI at 1.5T. RESULTS: The LV and RV end-diastolic volume indexes (EDVi) were inversely related to age (P < 0.0001 r = -0.34 and P < 0.0001 r = -0.37, respectively). In addition, the LV mass index decreased with age (P = 0.0004, r = -0.21). The LV longitudinal shortening was not significantly different among groups: ≥15% in all populations (95% confidence interval [CI]: 16-31). The sphericity index measured in end-diastole was higher in females than in males (P < 0.03): the upper limit was 40% for males and 42% for females. The normality cutoff of LV global function index was ≥33% in males and ≥35% in females. The end-diastolic volume (EDV) of RV and LV was balanced (RV/LV ratio 0.85-1.15) without differences in the population. The LV EDV/mass was 1.0-1.8 in males and 1.0-2.1 in females. CONCLUSION: This study provides potential age- and gender-specific reference. LEVEL OF EVIDENCE: 2 J. Magn. Reson. Imaging 2017;45:1055-1067.

Does a thrombin-based topical haemostatic agent reduce blood loss and transfusion requirements after total knee revision surgery? A randomized, controlled trial.

PURPOSE: The aim of the present study was to assess the efficacy of a thrombin-based topical haemostatic in reducing blood requirements after total knee replacement (TKR) revision surgery. METHODS: This prospective, randomized, controlled study was designed to evaluate the haemostatic efficacy and safety of a thrombin-based topical haemostatic (Floseal) versus standard treatment in patients receiving total knee revision arthroplasty. The decrease in haemoglobin values postsurgery and the blood units transfused were recorded. The decision to transfuse was made by a surgeon blinded to the patient's group allocation. RESULTS: Forty-eight patients were enroled in the study; twenty-four patients each were randomized to the treatment and control groups, respectively. The median decrease in haemoglobin concentration on the first postoperative day was 2.2 g/dL in the treatment group and 2.7 g/dL in the control group. A significant reduction in units of blood transfused was also observed in the treatment group compared with the control group [1.1 ± 1.13 (range 0-4) vs. 1.9 ± 1.41 (range 0-5) blood units; P = 0.04]. No major treatment-related adverse events were recorded in the study. CONCLUSIONS: This study shows that a thrombin-based topical haemostatic reduces the need for blood transfusion in TKR revision surgery. CLINICAL RELEVANCE: A thrombin-based topical haemostatic agent can be an appropriate solution to enhance haemostasis and vessel sealing at the operative site in TKR revision surgery, in order to reduce the need for blood transfusion after surgery. LEVEL OF EVIDENCE: II.

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.

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.

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.

Heart Involvement in Systemic Sclerosis: the Role of Magnetic Resonance Imaging.

Systemic sclerosis (SSc) is a severe connective tissue disease characterized by diffuse vascular damage and aberrant activation of immune system, resulting in inflammation and fibrosis of skin and internal organs, including the heart. Cardiac involvement is frequent in SSc, even though often unrecognized due to the occult nature at early stages and to the lack of a defined diagnostic algorithm. Once clinically evident, heart involvement is associated with a poor prognosis, representing the leading cause of death in about one third of SSc patients. Thus, its early recognition and monitoring are of crucial importance to allow a prompt therapeutic intervention and to improve patients' outcomes. Cardiac Magnetic Resonance (CMR) is a non-invasive, non-radiating imaging technique of great importance for the assessment of cardiovascular system, and represents the modality of choice for the morpho-functional and structural characterization of the heart. In SSc, CMR allows a precise definition of biventricular and biatrial size and function, and a detailed tissue characterization. CMR has been therefore extensively proposed in SSc as a non-invasive diagnostic tool to characterize heart involvement, particularly myocardial involvement. In this review, we summarize the most recent evidences to support the use of CMR in SSc as an important tool to recognize and characterize scleroderma heart disease. Furthermore, the unmet needs and the future perspectives of a CMR-based approach for the early detection of SSc heart involvement are discussed.