Ruolo della RM cardiaca nel follow-up a lungo termine della MIS-C

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Abstract
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In this retrospective multicentre study based on cardiac MR, the cardiological outcome of MIS-C patients does appear favourable regardless of severity of the cardiac involvement during the acute phase.

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  • Research Article
  • 10.1161/circinterventions.113.001090
Circulation: Cardiovascular Interventions Editors’ Picks
  • Dec 1, 2013
  • Circulation: Cardiovascular Interventions
  • The Editors

<i>Circulation: Cardiovascular Interventions</i> Editors’ Picks

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  • Cite Count Icon 1
  • 10.1161/circimaging.117.007179
Noninvasive Imaging Post-ST-Segment-Elevation Myocardial Infarction: Towards Targeted Therapy or Targeted End Points?
  • Nov 1, 2017
  • Circulation. Cardiovascular imaging
  • J Ranjit Arnold + 1 more

Recent decades have witnessed a remarkable decline in the incidence and mortality of ST-segment–elevation myocardial infarction (STEMI).1 In Western countries, improvements in acute care have seen in-hospital mortality from STEMI decrease dramatically from ≈20% in the 1980s to ≈5% in the present day.1,2 However, declining mortality engenders an increasing number of survivors with residual cardiac damage and its associated sequelae. Infarct-related left ventricular (LV) remodeling predisposes to systolic dysfunction and the development of heart failure. Consequently, there is an increasing global incidence of ischemic heart failure, a condition whose mortality rate, in contrast to that of STEMI, has barely changed in the last 2 decades (53% survival at 5 years).3 Identifying those at risk of adverse remodeling and heart failure early may allow individualized, targeted treatment to ameliorate heart failure and thereby improve survival. See Article by Stiermaier et al Noninvasive imaging plays a key role in risk stratification post-STEMI. LV ejection fraction (EF) is an independent predictor of survival and forms the basis of treatment stratification (eg, device therapy, aldosterone antagonists).4 The prognostic impact of LV dilatation (as measured by end-diastolic and end-systolic volumes) in historical echocardiographic studies is also well established.5 A relatively recent addition to the diagnostic armamentarium is cardiovascular magnetic resonance (CMR). As well as providing a highly accurate and reproducible depiction of myocardial injury and function, CMR also affords additional prognostic markers. Infarct size (IS) as determined by gadolinium imaging is a stronger predictor of adverse remodeling than EF or volumetric indices and serves as a useful surrogate end point in clinical trials.6,7 Risk prediction is further enhanced by CMR assessment of microvascular obstruction (MVO). Arising …

  • Research Article
  • 10.1161/circulationaha.112.001342
Circulation: Cardiovascular Imaging Editors’ Picks
  • Feb 19, 2013
  • Circulation
  • The Editors

<i>Circulation: Cardiovascular Imaging</i> Editors’ Picks

  • Research Article
  • Cite Count Icon 1
  • 10.5812/ijradiol-138650
Imaging Evaluation of Clinical and Subclinical Myocarditis in Multisystem Inflammatory Syndrome of Children with COVID-19 Using Cardiac Magnetic Resonance: The Results and Prognosis
  • Mar 26, 2024
  • Iranian Journal of Radiology
  • Afrouz Alipour + 4 more

Background: Coronavirus disease 2019 (COVID-19) has emerged as the most significant health crisis in recent years, leading to over 6 million deaths globally due to the disease. Objectives: Given the prevalence of multisystem inflammatory syndrome in children (MISC) following the COVID-19 pandemic, this study aims to examine the imaging findings and prognoses of clinical and subclinical myocarditis in children with MISC through cardiac magnetic resonance (CMR) imaging. Patients and Methods: This prospective cohort study carried out over eighteen months from May 2021 to November 2022, included 14 patients who underwent CMR imaging. A census of all eligible patients during the study period served as the sampling method. Inclusion criteria were patients with confirmed COVID-19 infection through serological tests, polymerase chain reaction (PCR), or recent exposure to COVID-19 patients. Exclusion criteria included patients with a history of congenital heart disease (CHD) or pulmonary disease. Additional diagnostic tests performed included blood sample tests, chest X-ray (CXR), electrocardiogram (ECG), and echocardiography. CMR imaging was conducted on patients with cardiac involvement. A diagnosis of myocardial inflammation was made if a patient met at least two of the Lake Louise Criteria. The Chi-square, Fisher's exact, and Mann-Whitney tests were used to examine the relationship between quantitative variables and treatment outcomes. Additionally, the Wilcoxon signed rank, and McNemar’s tests assessed changes in echocardiography findings from admission to follow-up. A significance level of 0.05 was set. Results: Among the 14 patients studied, 8 (57.10%) were girls and 6 (42.90%) were boys. The average age was 6.03 ± 3.71 years. The median time to CMR imaging after symptom onset was 4 weeks (interquartile range (IQR): 2 - 12, range: 30). Global function assessment using left ventricular ejection fraction (LVEF) showed that 5 (35.70%), 3 (21.40%), and 6 (42.90%) patients had normal function, mild dysfunction, and significant LV dysfunction, respectively. 71.40% of patients who recovered had mild tricuspid regurgitation (TR) and no cardiomegaly. Significant differences in mean values of polymorphonuclear neutrophil (PMN) (37.71 ± 11.75 vs. 81.44 ± 13.06), lymphocytes (48.71 ± 20.08 vs. 12.51 ± 7.26), hemoglobin (Hb) (12.60 ± 1.55 vs. 10.10 ± 1.62), mean corpuscular volume (MCV) (85.90 ± 5.67 vs. 79.37 ± 5.23), erythrocyte sedimentation rate (ESR) (8.86 ± 13.60 vs. 30.29 ± 21.33), and C-reactive protein (CRP) (18.91 ± 27.25 vs. 100.57 ± 85.67) were observed between non-recovered and recovered patients, respectively (P &lt; 0.05). However, no statistically significant association was found between other variables, including N-terminal pro–B-type natriuretic peptide (NT-proBNP), D-dimer, and Troponin I (TPI), with treatment outcomes (P &gt; 0.05). Conclusion: Our findings indicate that a negative COVID-19 test does not exclude an established clinical COVID-19 infection in children with MISC. The results suggest that all children with MISC and a history of COVID-19 infection should undergo assessment for myocardial fibrosis, regardless of ejection fraction (EF) as determined by Echocardiography, laboratory tests, and COVID-19 test results. Strain analysis, conducted during both the acute phase and subsequent follow-ups through CMR imaging or Echocardiography, is recommended to enhance the understanding of the prognosis.

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  • Cite Count Icon 1
  • 10.1136/heartjnl-2024-bcs.185
190 Quantification of myocardial infarction by cardiovascular magnetic resonance late gadolinium enhancement imaging: comparison of magnitude-based versus phase-sensitive inversion recovery
  • May 27, 2024
  • Heart
  • Aida Moafi + 5 more

IntroductionLate gadolinium enhancement (LGE) imaging with cardiovascular magnetic resonance (CMR) is considered the non-invasive gold standard for identifying and quantifying myocardial scar. The diagnostic and prognostic importance of myocardial scar...

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  • Cite Count Icon 4
  • 10.1080/23744235.2023.2286289
Post-acute midterm follow-up cardiac MRI findings and clinical outcomes in patients with COVID-19 vaccine-associated myocarditis: a comprehensive systematic review and meta-analysis
  • Nov 23, 2023
  • Infectious diseases (London, England)
  • Parham Samimisedeh + 3 more

Purpose Although previous investigations revealed favourable in-hospital outcomes of COVID-19 vaccine-related myocarditis, the mid-term prognosis is still unclear. Hence, we aim to summarise existing evidence on the follow-up imaging and clinical findings in patients with COVID-19 vaccine-related myocarditis. Methods We performed a systematic search in online databases using relevant key terms covering COVID-19 vaccine, myocarditis, follow-up, and cardiac MRI. We included all observational studies that reported cardiac MRI findings of patients with myocarditis following COVID-19 vaccination in both acute and follow-up phases. Data on clinical outcomes and cardiac MRI findings were extracted and pooled using a random-effect model. Results A total of 27 studies (126 patients) met our eligibility criteria. At the time of follow-up, myocarditis symptoms were resolved in all patients, but abnormal electrocardiography and elevated troponin levels were detected in 18.7% and 3.8% of them, respectively. Median imaging follow-up times varied from 3 to 6.3 months. On follow-up cardiac MRI, the persistence of LGE was observed in 76% (95%CI: 62 to 85%), but its extension declined compared to the baseline in almost all patients. Persistent LGE was accompanied by myocardial edoema in six patients, and it was consistent with myocardial fibrosis (LGE without edoema) in the remaining cases. Mean changes (95%CI) of cardiac MRI left ventricular ejection fraction (LVEF) (%) was +2.97 (+1.59 to +4.34) from baseline. Conclusion In conclusion, although most patients likely experience favourable clinical outcomes without serious complications, cardiac MRI abnormalities, mainly LGE, may persist in a notable proportion of them beyond the acute phase.

  • Discussion
  • 10.1002/ejhf.2318
Arrhythmic risk management after acute myocarditis: never too early, only too late.
  • Aug 4, 2021
  • European journal of heart failure
  • Mihai Strachinaru + 2 more

Arrhythmic risk management after acute myocarditis: never too early, only too late.

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  • Cite Count Icon 22
  • 10.1038/s41598-020-76432-4
Prognostic value of texture analysis from cardiac magnetic resonance imaging in patients with Takotsubo syndrome: a machine learning based proof-of-principle approach
  • Nov 25, 2020
  • Scientific Reports
  • Manoj Mannil + 14 more

Cardiac magnetic resonance (CMR) imaging has become an important technique for non-invasive diagnosis of takotsubo syndrome (TTS). The long-term prognostic value of CMR imaging in TTS has not been fully elucidated yet. This study sought to evaluate the prognostic value of texture analysis (TA) based on CMR images in patients with TTS using machine learning. In this multicenter study (InterTAK Registry), we investigated CMR imaging data of 58 patients (56 women, mean age 68 ± 12 years) with TTS. CMR imaging was performed in the acute to subacute phase (median time after symptom onset 4 days) of TTS. TA of the left ventricle was performed using free-hand regions-of-interest in short axis late gadolinium-enhanced and on T2-weighted (T2w) images. A total of 608 TA features adding the parameters age, gender, and body mass index were included. Dimension reduction was performed removing TA features with poor intra-class correlation coefficients (ICC ≤ 0.6) and those being redundant (correlation matrix with Pearson correlation coefficient r > 0.8). Five common machine-learning classifiers (artificial neural network Multilayer Perceptron, decision tree J48, NaïveBayes, RandomForest, and Sequential Minimal Optimization) with tenfold cross-validation were applied to assess 5-year outcome including major adverse cardiac and cerebrovascular events (MACCE). Dimension reduction yielded 10 TA features carrying prognostic information, which were all based on T2w images. The NaïveBayes machine learning classifier showed overall best performance with a sensitivity of 82.9% (confidence interval (CI) 80–86.2), specificity of 83.7% (CI 75.7–92), and an area-under-the receiver operating characteristics curve of 0.88 (CI 0.83–0.92). This proof-of-principle study is the first to identify unique T2w-derived TA features that predict long-term outcome in patients with TTS. These features might serve as imaging prognostic biomarkers in TTS patients.

  • Research Article
  • Cite Count Icon 160
  • 10.1161/circimaging.109.875021
Multimodality Noninvasive Imaging for Assessment of Congenital Heart Disease
  • Jan 1, 2010
  • Circulation: Cardiovascular Imaging
  • Ashwin Prakash + 2 more

Major advances in the field of pediatric cardiology and cardiac surgery over the last several decades have led to a dramatic improvement in survival rates for most forms of congenital heart disease (CHD). For example, hypoplastic left heart syndrome, a previously lethal defect, now has early survival rates up to 90% at major centers.1 These improved outcomes have produced a growing population of survivors with complex CHD who are now reaching adulthood (Figure 1). During this period, improvements in surgical and medical treatments have been accompanied by developments in diagnostic modalities. Echocardiography has replaced catheterization as the primary diagnostic modality, and it is now uncommon for newborn infants to undergo catheterization for purely diagnostic purposes. Although echocardiography remains the bedrock of noninvasive cardiac imaging, the array of diagnostic modalities and techniques available continue to grow and this has spawned the specialty of “noninvasive cardiac imaging” and the need for the “cardiac imager” to be adept in all the different modalities. Figure 1. Percentage of patients under the age of 1 year (grey bars) and over the age of 18 years (black bars) undergoing echocardiography at Children’s Hospital Boston from 1983 through 2006. Note the reverse trends of these age groups reflecting the steady increase in the proportion of adult patients with congenital heart disease. Although the absolute number of infants undergoing echocardiography during this time period has increased, their proportion has steadily declined. Echocardiography, cardiac magnetic resonance (CMR), and cardiac computed tomography (CCT) are the primary modalities used for noninvasive cardiac imaging in patients with CHD. Nuclear scintigraphy is used in selected circumstances. The Table summarizes the strengths and weaknesses of each modality. Figure 2 shows temporal trends in utilization for the various noninvasive cardiac imaging techniques at our center. It is clear that echocardiography is the most frequently …

  • Research Article
  • Cite Count Icon 3
  • 10.4037/ccn2009907
Takotsubo Cardiomyopathy: A Nurse’s Guide
  • Oct 1, 2009
  • Critical Care Nurse
  • Scott Griffin + 1 more

Takotsubo Cardiomyopathy: A Nurse’s Guide

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  • Cite Count Icon 1
  • 10.1097/cm9.0000000000002385
The prevalence and associated factors of myocardial involvement in Duchenne muscular dystrophy patients in the first decade of life.
  • May 5, 2023
  • Chinese Medical Journal
  • Rong Xu + 15 more

The prevalence and associated factors of myocardial involvement in Duchenne muscular dystrophy patients in the first decade of life.

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  • Cite Count Icon 1
  • 10.1002/jcu.23415
Cardiovascular magnetic resonance imaging in COVID-19 patients: An important tool in everyday clinical practice.
  • Jan 27, 2023
  • Journal of Clinical Ultrasound
  • Johannes Kersten

Myocarditis is a typical complication of many viral diseases1 and has also been described in other coronavirus diseases other than COVID-19.2, 3 However, it is only one possible complication of cardiac involvement in COVID-19 and requires an endomyocardial biopsy or a combination of suggestive clinical and imaging findings to reach a definitive diagnosis.4 Cardiovascular magnetic resonance (CMR) imaging is a cornerstone of the non-invasive diagnosis of myocarditis. Clinical studies have reported the diagnosis of myocarditis by CMR both in acute infections and in the post-acute phase.5 Cardiac involvement in COVID-19 was described early in the pandemic as a risk factor for poor outcomes.6 Inflammatory, thromboembolic and ischemic events are relevant complications of acute SARS-CoV-2 infection. However, even months after acute infection, many patients describe cardiopulmonary symptoms,7 and CMR indicates inflammatory processes,5 although their contribution to often complex clinical syndromes is questionable in some cases. Palmisano et al. investigated cardiac changes on CMR in 39 patients in the acute or post-acute phase of COVID-19 and found inflammatory changes in most patients.8 However, ischemic, valvular or other cardiomyopathies could also be diagnosed on the basis of CMR. The authors mention the heterogeneous patient population as a limitation of their study. On the contrary, this demonstrates the real additional impact of CMR since it has been shown that it can be used in a broad range of clinical scenarios. CMR can lead to a diagnosis independent of other accompanying factors and allows the exclusion or confirmation of possible differential diagnoses in the case of suspected virus-associated myocarditis. This is one of the most important tasks of cardiac imaging in the context of COVID-19. Especially potentially pre-existing but unknown cardiovascular diseases can be exacerbated in the context of SARS-CoV-2 infection and should therefore be excluded. Three major factors in Palmisano et al.'s study should be emphasized. First, the rate of diagnosed myocarditis (53%) appears to be very high. Although early studies on survivors of SARS-CoV-2 infection postulated high myocarditis rates, this could not be confirmed in actual autopsy studies,9 in which the use of histological criteria for the diagnosis of myocarditis showed a high degree of cardiac involvement but actual myocarditis in only 7.2% of the cases. In Palmisano et al.'s study, this can likely be explained by the good clinical pre-specification of the patients with a high clinical pre-test probability. This illustrates that CMR contributes to confirming the diagnosis, especially when it is well indicated. Second, the authors examined 17 patients with acute SARS-CoV-2 infection using CMR over a period of 5 months. Experience has shown that at several hospitals, this has not been done in fear of exposing staff to the risk of infection. This demonstrates the feasibility of CMR for infectious patients in a clinical setting. Third, with mostly normal biventricular function, non-invasive tissue characterization with an appropriate pre-test probability offers insights that goes far beyond pure functional or volumetric analyses. CMR diagnosis is not the first-line imaging method for diagnosis of myocarditis in mildly ill patients. However, in patients with a clinically justifiable diagnosis due to abnormal ECG and particularly echocardiographic findings and/or abnormal troponin levels accompanied with typical symptoms, CMR should be a diagnostic cornerstone in the acute and post-acute phase. The ongoing debate about short- and long-term cardiovascular consequences of COVID-19 needs to be put in a broader perspective than just the treatment of patients during pandemic. The great scientific effort and gained knowledge can potentially be used for other post-infectious syndromes, such as sequelae of Lyme disease, influenza and pneumococcal pneumonia. Moreover, this and similar studies showed that cardiovascular imaging in general and particularly advanced methods such as CMR and T1-derived parametric mapping should become a clinical routine that will serve not only for better understanding of cardiac involvement in COVID-19, but in overall better acceptance of CMR in everyday clinical practice in large spectrum of cardiovascular diseases. Open Access funding enabled and organized by Projekt DEAL.

  • Research Article
  • 10.1093/ehjci/ehaa946.2949
Cardiac magnetic resonance features of acute myocarditis presenting as acute coronary syndrome
  • Nov 1, 2020
  • European Heart Journal
  • L Szabo + 10 more

Background Clinical presentation of myocarditis varies, one specific form of myocarditis appears with the clinical signs of acute coronary syndrome (ACS). Cardiac magnetic resonance (CMR) is an important method for assessing ventricular function and morphology, additionally provides accurate tissue specific and functional information of the heart. Aims Our aim was to investigate the characteristics, and prognosis of myocarditis presenting with ACS symptoms. Methods 113 patients with the clinical signs of ACS but nonobstructed coronary arteries in whom the CMR revealed acute myocarditis were included in our study. CMR was performed in acute phase and at 3–6-month follow-up. Left ventricular (LV) volumes, mass and strain parameters expressing myocardial deformity were determined. Additional images were taken to represent tissue specific information. Relationships between laboratory and CMR parameters were investigated. Parameters predicting changes in LV ejection fraction (LVEF) were analyzed by logistic regression. Results A total of 113 patients with myocarditis (98 males, 31±11 years) underwent acute and follow-up CMR. Sixty two patients reported fever or infection before the beginning of their complaints, most commonly gastroenteritis (33%) and pharyngitis (32%). The creatinine kinase MB value measured in the acute phase showed positive correlation with the extent of necrosis, and the global longitudinal- and circumferential strain. The extent of the LV necrosis showed negative correlation with LVEF and positive correlation with global circumferential strain (GCS) (p&amp;lt;0.05). On the control CMR examination LVEF and all global strain values improved, fibrosis persisted in 82% of cases but shrank (15±11 vs 5±4 g) and LV mass decreased (p&amp;lt;0.01) compared to the acute phase. Compared to the acute phase, 21% of the patients had lower LVEF on the follow-up CMR. Lower initial LVEF, worse acute GCS, and greater LV necrosis were independent predictors of LVEF reduction in the logistic regression model. During a median follow-up of 6-years of patients treated at our clinic (n=39) no patient suffered cardiac death, heart failure, or documented ventricular arrhythmia but 21% of them had recurrent myocarditis. Conclusion Myocarditis mimicking ACS affects predominantly young men and shows functional improvement and good prognosis on follow-up, but it may reoccur in some cases. The reduction of LV function on control CMR may be predicted by worse initial LVEF, GCS, and a larger LV scar. Strain, LGE in acute phase and follow-up Funding Acknowledgement Type of funding source: Public grant(s) – National budget only. Main funding source(s): Project no. NVKP_16-1-2016-0017 has been implemented with the support provided from the National Research, Development and Innovation Fund of Hungary, financed under the NVKP_16 funding scheme. This project was supported by a grant from the National Research, Development and Innovation Office (NKFIH) of Hungary (K 120277).

  • Research Article
  • Cite Count Icon 64
  • 10.1001/jamacardio.2022.2108
Association of Left Ventricular Remodeling Assessment by Cardiac Magnetic Resonance With Outcomes in Patients With Chronic Aortic Regurgitation
  • Jul 20, 2022
  • JAMA Cardiology
  • Go Hashimoto + 14 more

Chronic aortic regurgitation (AR) causes left ventricular (LV) volume overload, which results in progressive LV remodeling negatively affecting outcomes. Whether cardiac magnetic resonance (CMR) volumetric quantification can provide incremental risk stratification over standard clinical and echocardiographic evaluation in patients with chronic moderate or severe AR is unknown. To compare LV remodeling measurements by CMR and echocardiography between patients with and without heart failure symptoms and to verify the association of remodeling measurements of patients with chronic moderate or severe AR but no or minimal symptoms with clinical outcomes receiving medical management. This multicenter retrospective cohort study included consecutive patients with at least moderate chronic native AR evaluated by 2-dimensional transthoracic echocardiography and CMR examination within 90 days from each other between January 2012 and February 2020 at Allina Health System. Data were analyzed from June 2021 to January 2022. Clinical evaluation and risk stratification by CMR. The end point was a composite of death, heart failure hospitalization, or progression of New York Heart Association functional class while receiving medical management, censoring patients at the time of aortic valve replacement (when performed) or at the end of follow-up. Of the 178 included patients, 119 (66.9%) were male, 158 (88.8%) presented with no or minimal symptoms (New York Heart Association class I or II), and the median (IQR) age was 58 (44-69) years. Compared with patients with no or minimal symptoms, symptomatic patients had greater LV end-systolic volume index (LVESVi) by CMR (median [IQR], 66 [46-85] mL/m2 vs 42 [30-58] mL/m2; P < .001), while there were no significant differences by echocardiography (LVESVi: median [IQR], 38 [30-58] mL/m2 vs 27 [20-42] mL/m2; P = .07; LV end-systolic diameter index: median [IQR], 21 [17-25] mm/m2 vs 18 [15-22] mm/m2; P = .17). During the median (IQR) follow-up of 3.3 (1.6-5.8) years, 50 patients with no or minimal symptoms receiving medical management developed the composite end point, which, in multivariate analysis adjusted for age and EuroSCORE II, was independently associated with LVESVi of 45 mL/m2 or greater and aortic regurgitant fraction of 32% or greater, the latter adding incremental prognostic value to CMR volumetric assessment. In patients with chronic moderate or severe AR, patients presenting with heart failure symptoms have greater LVESVi by CMR than those with no or minimal symptoms. In patients with no or minimal symptoms, CMR quantification of LVESVi and AR severity may identify those at risk of death or incident heart failure and therefore should be considered in the clinical evaluation and decision-making of these patients.

  • Research Article
  • 10.1161/circ.150.suppl_1.4119187
Abstract 4119187: Association of CMR derived Global Longitudinal Strain with adverse Outcomes in Arrhythmogenic Cardiomyopathy.
  • Nov 12, 2024
  • Circulation
  • Ali Bahbah + 8 more

Background: Arrhythmogenic cardiomyopathy (ACM) is associated with high risk of ventricular arrhythmias (VA) and heart failure (HF). Different predictive models and diagnostic criteria include parameters related to myocardial structural abnormalities and right ventricular (RV) ejection fraction (EF). Nevertheless, the value of global longitudinal strain (GLS) ascertained from cardiac magnetic resonance (CMR) for risk-stratification in the context of ACM is unknown. Aim: We aim to study the association of low CMR-derived GLS with adverse outcomes in ACM patients. Methods: This was a retrospective cohort study of ACM patients seen at a single quaternary center. ACM diagnosis followed the modified 2010 ARVC modified task force criteria. Primary outcome was a composite of life-threatening VAs, heart transplant or HF hospitalization during follow up. We excluded patients who suffered events prior to diagnosis, and those with missing or inadequate CMR protocol. Imaging parameters were acquired by CMR including EF, GLS, and late gadolinium enhancement (LGE). Results: A total of 82 patients with suspected ACM were included, with a median age of 40 years, and 43 (52.4%) were females. Most common reason of initial visit was family history of genetic cardiomyopathy in 46 (56%), while 16 (20%) patients visited our clinic due to history of syncope. Out of 60 patients who had genetic testing, 21 (35%) had mutations of DSP gene. Left ventricular involvement (non-ischemic LV-LGE by CMR) was present in 42 (56.8%) out of 74 patients who had CMR with gadolinium. Median LVEF was 62% (56, 66), and RVEF of 53% (48, 59), both by CMR. Median LVGLS was -17.01% (-14.71, -18.43), while median RVGLS was -20.5% (-18.3, -24.4). A total of 15 (18%) patients suffered the composite endpoint during an average follow up period of 8 years. Patients who suffered the composite endpoint had significantly lower mean LVGLS (-15.04% vs -16.70%, p= 0.048), and lower average RVGLS (-18.45% vs -21.33%, p=0.035) compared to patients free from the adverse outcome. Meanwhile there was no significant difference in LVEF (60.13% vs 61.18%, p=0.67), nor in RVEF (49.13% vs 54.01%, p=0.065) between the two groups. Conclusion: Lower GLS was significantly associated with VAs and HF hospitalizations in our patient population. CMR derived GLS can be used as an important prognostic parameter of adverse outcomes in patients with suspected ACM. Especially in those with LV involvement and high genetic predisposition as our cohort.

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