Bedside right ventricle quantification using three-dimensional echocardiography in children with congenital heart disease: A comparative study with cardiac magnetic resonance imaging.

BACKGROUND: Accurate quantification of right ventricular (RV) volumes and function is crucial for the management of congenital heart diseases. AIMS: We aimed to assess the feasibility and accuracy of bedside analysis using new RV quantification software from three-dimensional transthoracic echocardiography in children with or without congenital heart disease, and to compare measurements with cardiac magnetic resonance imaging. METHODS: We included paediatric patients with congenital heart disease (106 patients) responsible for RV volume overload and a control group (30 patients). All patients underwent three-dimensional transthoracic echocardiography using a Vivid E95 ultrasound system. RV end-diastolic and end-systolic volumes and RV ejection fraction were obtained using RV quantification software. Measurements were compared between RV quantification and cardiac magnetic resonance imaging in 27 patients. RESULTS: Bedside RV quantification analysis was feasible in 133 patients (97.8%). Manual contour adjustment was necessary in 126 patients (93%). The mean time of analysis was 62±42s. RV end-diastolic and end-systolic volumes were larger in the congenital heart disease group than the control group: median 85.0 (interquartile range 29.5) mL/m2 vs 55.0 (interquartile range 20.5) mL/m2 for RV end-diastolic volume and 42.5 (interquartile range 15.3) mL/m2 vs 29.0 (interquartile range 11.8) mL/m2 for RV end-systolic volume, respectively. Good agreement for RV end-diastolic and end-systolic volumes and RV ejection fraction was found between RV quantification and magnetic resonance imaging measurements. RV quantification software underestimated RV end-diastolic volume/body surface area by 3mL/m2 and RV ejection fraction by 2.1%, and overestimated RV end-systolic volume/body surface area by 0.2mL/m2. CONCLUSIONS: We found good feasibility and accuracy of bedside RV quantification analysis from three-dimensional transthoracic echocardiography in children with or without congenital heart disease. RV quantification could be a reliable and non-invasive method for RV assessment in daily practice, facilitating appropriate management and follow-up care.

The 2023 European Task Force Criteria for Diagnosis of Arrhythmogenic Cardiomyopathy: Historical Background and Review of Main Changes.

Arrhythmogenic cardiomyopathy (ACM) is a cardiac disease featured by non-ischemic myocardial scarring linked to ventricular electrical instability. As there is no single gold-standard test, diagnosing ACM remains challenging and a combination of specific criteria is needed. The diagnostic criteria were first defined and widespread in 1994 and then revised in 2010, approaching and focusing primarily on right ventricular involvement without considering any kind of left ventricular variant or phenotype. Years later, in 2020, with the purpose of overcoming previous limitations, the Padua Criteria were introduced by an international expert report. The main novel elements were the introduction of specific criteria for left ventricular variants as well as the use of cardiac magnetic resonance for tissue characterization and scar detection. The last modifications and refinement of these criteria were published at the end of 2023 as the European Task Force criteria, by a "head-quarter" of ACM international experts, proving the emerging relevance of this condition besides its difficult diagnosis. In this review, emphasizing the progress in understanding the aetiology of the cardiomyopathy, an analysis of the new criteria is presented. The introduction of the term "scarring/arrhythmogenic cardiomyopathy" sets an important milestone in this field, underlying how non-ischemic myocardial scarring-typical of ACM-and arrhythmic susceptibility could be the main pillars of numerous different phenotypic variants regardless of etiology.

Prognostic Implications of Clinical and Imaging Diagnostic Criteria for Myocarditis.

BACKGROUND: The European Society of Cardiology (ESC), the American College of Cardiology, the American Heart Association, and expert consensus documents provide different diagnostic criteria for myocarditis. Their overlap and prognostic value have never been compared. OBJECTIVES: This study aims to assess and compare the predictive value of ESC criteria for clinically suspected myocarditis, updated Lake-Louise criteria (LLC), American Heart Association criteria for probable acute myocarditis (pAM), and expert consensus criteria for acute myocarditis (AM) and complicated myocarditis (CM). METHODS: Patients with a clinical suspicion of myocarditis referred for cardiac magnetic resonance were enrolled at 2 centers. Those with any prior cardiomyopathy were excluded. The association of composite outcome events (heart failure hospitalization, recurrent myocarditis, sustained ventricular tachycardia, or death) with ESC diagnostic criteria, LLC, pAM, AM, and CM were compared. RESULTS: Among 1,557 consecutive patients referred for cardiac magnetic resonance with possible myocarditis, 1,050 (62.6% male; 48.9 ± 16.8 years of age) were without an alternative diagnosis. Of those, 938 (89.3%) met ESC criteria for clinically suspected myocarditis, 299 (28.5%) LLC, and 356 (33.9%), 216 (20.6%), and 77 (7.3%) pAM, AM, and CM, respectively. Adverse events occurred in 161 patients (15.3%) during a median follow-up of 3.4 years. The highest annualized event rates (6.6%) were observed in patients meeting LLC, whereas negative ESC criteria indicated excellent prognosis (0.7% annualized event rate). Among all myocarditis definitions, ESC criteria and LLC were the strongest multivariable outcome predictors and had independent and incremental prognostic value (HRadjusted: 3.87; 95% CI: 1.22-12.2; P = 0.021, and HRadjusted: 2.53; 95% CI: 1.83-3.49; P < 0.001, respectively) when adjusted for clinical characteristics. CONCLUSIONS: In a real-world cohort of patients with possible myocarditis, diagnosis was reached in most patients using ESC criteria whereas only approximately one-quarter of patients reached a diagnosis with LLC. The independent prognostic value of ESC-criteria and LLC highlights the complementary role of clinical and CMR-based findings in the diagnosis and risk stratification of myocarditis.

Crinkle-Cut Coronary Arteries in a Patient With Granulomatosis With Polyangiitis.

Granulomatosis with polyangiitis (GPA) is a rare type of small to medium vessel necrotizing vasculitis that usually affects vessels of the upper or lower airways and kidneys. Cardiac involvement in GPA is often subclinical and if clinically significant has been rarely reported, even less so as an initial presentation. We describe the case of a 44-year-old man who presented with what appeared to be inferior ST-segment elevation myocardial infarction and was found to have small vessel vasculitis of the coronary arteries with associated myocarditis as a presenting manifestation of GPA, which was ultimately treated with steroids, rituximab, and avacopan.

Long-Standing Chronic Recurrent Pericarditis Managed With Advanced Diagnostic and Therapeutic Methods.

A 65-year-old woman with a history of idiopathic pericarditis presented with chronic recurrent pericarditis. Because of the inability to taper off anakinra without recurrent flares, she transitioned to rilonacept, which led to symptom abatement. Her positive response to rilonacept therapy correlated with an improvement in inflammatory changes noted on cardiac magnetic resonance imaging.

Coronary Vasospasm Presenting in a Catastrophic Way.

Coronary artery vasospasm is an under-recognized yet fatal condition that can manifest as sudden cardiac arrest. A 51-year-old man presented with out-of-hospital cardiac arrest due to ventricular fibrillation during exercise. Coronary angiogram showed severe disease at ostial left main coronary artery and left anterior descending artery (LAD), that was not present anymore on repeated angiography and intravascular ultrasound assessment. Cardiac magnetic resonance demonstrated evidence of prior myocardial infarction in the LAD territory. Acetylcholine challenge test was performed that induced severe left main coronary artery and LAD spasm. Coronary artery vasospasm has contributed to a significant proportion of patients presenting with coronary artery disease. Establishing the correct diagnosis is detrimental to future management of patients. This case highlighted a multimodality approach in the diagnosis of coronary vasospasm.

Hepatic T1 Mapping as a Novel Cardio-Hepatic Axis Imaging Biomarker Early after STEMI.

BACKGROUND: The hepatic response after ST-elevation myocardial infarction (STEMI) may be associated with mortality and morbidity. We aimed to assess the cardio-hepatic axis post-STEMI using cardiovascular magnetic resonance (CMR). METHODS: This prospective, observational, single-centre study included consecutive STEMI patients who underwent CMR after primary angioplasty from January 2015 to January 2019. Standard infarct characteristics were analysed, and hepatic T1 and hepatic extracellular volume (ECV) were assessed using pre- and post-contrast T1-mapping sequences. The primary endpoint was the relationship between native hepatic T1-values and ischemic right ventricular (RV) involvement, determined by RV ejection fraction (EF) dysfunction and/or the presence of RV acute myocardial infarction (AMI). The diagnostic performance of hepatic T1 values for detecting RV involvement was assessed using the area under the receiver operating characteristic curve (AUC). RESULTS: Of 177 consecutive STEMI patients undergoing CMR, 142 were included. Patients with RV ischemic involvement, compared to those without, had significantly higher native hepatic T1 (p < 0.001) and hepatic ECV (p = 0.016). Hepatic T1 values demonstrated a good diagnostic performance in detecting RV involvement (AUC 0.826, p < 0.001) and correlated positively with NT-proBNP values (r = 0.754, p < 0.001). Patients with high hepatic T1 values (>605 ms) had significantly higher NT-proBNP levels (<0.001), larger RV end-diastolic volume (p < 0.001), lower RV-EF (p < 0.001), and a higher prevalence of RV AMI (p = 0.022) compared to those with hepatic T1 ≤ 605 ms, while left ventricular EF and infarct size were similar. Multivariable logistic regression analysis identified RV-EF (p = 0.010) and NT-proBNP values (p < 0.001) as independent predictors of increased hepatic T1 values. Patients with increased hepatic T1 values had a higher rate of re-hospitalization for heart failure at 17-month follow-up (12.1% vs 2.0%, p = 0.046). CONCLUSIONS: Hepatic T1 mapping has emerged as a possible novel imaging biomarker of the cardio-hepatic axis in STEMI, being associated with RV involvement and increased NT-proBNP values.

Machine learning and radiomics for ventricular tachyarrhythmia prediction in hypertrophic cardiomyopathy: insights from an MRI-based analysis.

BACKGROUND: Myocardial fibrosis is often detected in patients with hypertrophic cardiomyopathy (HCM), which causes left ventricular (LV) dysfunction and tachyarrhythmias. PURPOSE: To evaluate the potential value of a machine learning (ML) approach that uses radiomic features from late gadolinium enhancement (LGE) and cine images for the prediction of ventricular tachyarrhythmia (VT) in patients with HCM. MATERIAL AND METHODS: Hyperenhancing areas of LV myocardium on LGE images were manually segmented, and the segmentation was propagated to corresponding areas on cine images. Radiomic features were extracted using the PyRadiomics library. The least absolute shrinkage and selection operator (LASSO) method was employed for radiomic feature selection. Our model development employed the TabPFN algorithm, an adapted Prior-Data Fitted Network design. Model performance was evaluated graphically and numerically over five-repeat fivefold cross-validation. SHapley Additive exPlanations (SHAP) were employed to determine the relative importance of selected radiomic features. RESULTS: Our cohort consisted of 60 patients with HCM (73.3% male; median age = 51.5 years), among whom 17 had documented VT during the follow-up. A total of 1612 radiomic features were extracted for each patient. The LASSO algorithm led to a final selection of 18 radiomic features. The model achieved a mean area under the receiver operating characteristic curve of 0.877, demonstrating good discrimination, and a mean Brier score of 0.119, demonstrating good calibration. CONCLUSION: Radiomics-based ML models are promising for predicting VT in patients with HCM during the follow-up period. Developing predictive models as clinically useful decision-making tools may significantly improve risk assessment and prognosis.

Prediction of left ventricular thrombus after myocardial infarction: a cardiac magnetic resonance-based prospective registry.

BACKGROUND: Left ventricular thrombus (LVTh) is a severe complication after ST-segment elevation myocardial infarction (STEMI). OBJECTIVES: We aim to predict LVTh occurrence by cardiac magnetic resonance (CMR) using clinical, echocardiographic, and electrocardiographic (ECG) variables readily available at admission. METHODS: We included 590 reperfused STEMI patients who underwent early (1-week) and/or late (6-month) CMR in our institution. Baseline clinical, echocardiographic (left ventricular ejection fraction -LVEF-) and ECG data (summatory of ST-segment elevation -sum-STE- and Q-wave and residual ST-elevation >1 mm -Q-STE-) during admission were registered. Multivariate binary logistic regression models and receiver operating characteristic curves were computed for LVTh prediction. RESULTS: LVTh was detected by CMR in 43 (7.3 %) patients and was predicted by previous chronic coronary syndrome (CCS, HR 4.74 [1.82-12.35], p = 0.001), anterior STEMI (HR 10.93 [2.47-48.31], p = 0.002), LVEF (HR 0.96 [0.93-0.99] per %, p = 0.008), maximum sum-STE (HR 1.04 [1.01-1.07] per mm, p = 0.04), and Q-STE (HR 1.31 [1.08-1.6] per lead, p = 0.008). High-risk patients with both major (anterior STEMI and Q-STE in ≥1 leads) and 1-3 minor (CCS, maximum sum-STE >10 mm, LVEF <50%) factors showed the highest LVTh risk (19.6 % within 6 months). The model showed excellent discrimination ability (area under the curve=0.85 [0.81-0.9], p < 0.001). Simplified 4-variable (excluding sum-STE) and 3-variable (also excluding CCS) risk scores showed similar discrimination ability and were externally validated. CONCLUSIONS: LVTh within 6 months post-STEMI can be predicted using pre-discharge clinical (anterior infarction and CCS), echocardiographic (LVEF), and ECG (sum-STE and Q-STE) data. Our results can help select patients who should undergo CMR after STEMI for LVTh detection.

Quality control of cardiac magnetic resonance imaging segmentation, feature tracking, aortic flow, and native T1 analysis using automated batch processing in the UK Biobank study.

Aims: Automated algorithms are regularly used to analyse cardiac magnetic resonance (CMR) images. Validating data output reliability from this method is crucial for enabling widespread adoption. We outline a visual quality control (VQC) process for image analysis using automated batch processing. We assess the performance of automated analysis and the reliability of replacing visual checks with statistical outlier (SO) removal approach in UK Biobank CMR scans. Methods and results: We included 1987 CMR scans from the UK Biobank COVID-19 imaging study. We used batch processing software (Circle Cardiovascular Imaging Inc.-CVI42) to automatically extract chamber volumetric data, strain, native T1, and aortic flow data. The automated analysis outputs (∼62 000 videos and 2000 images) were visually checked by six experienced clinicians using a standardized approach and a custom-built R Shiny app. Inter-observer variability was assessed. Data from scans passing VQC were compared with a SO removal QC method in a subset of healthy individuals (n = 1069). Automated segmentation was highly rated, with over 95% of scans passing VQC. Overall inter-observer agreement was very good (Gwet's AC2 0.91; 95% confidence interval 0.84, 0.94). No difference in overall data derived from VQC or SO removal in healthy individuals was observed. Conclusion: Automated image analysis using CVI42 prototypes for UK Biobank CMR scans demonstrated high quality. Larger UK Biobank data sets analysed using these automated algorithms do not require in-depth VQC. SO removal is sufficient as a QC measure, with operator discretion for visual checks based on population or research objectives.

Noninvasive Assessment in Takotsubo Syndrome: A Diagnostic Challenge.

Takotsubo syndrome (TTS) is a clinical syndrome characterized by a transient left ventricular dysfunction whose diagnosis can be challenging due to its resemblance to acute myocardial infarction (AMI). Despite the growing recognition of TTS, acute complications and long-term mortality rates are comparable to those observed in AMI patients. In this context, a systematic diagnostic approach is imperative for an accurate patient assessment, with due consideration of the distinctive characteristics and optimal timing of each imaging modality. Coronary angiography with ventriculography may be reserved for cases presenting with ST-segment elevation, whereas in all other cases, the use of multimodality noninvasive imaging allows for a comprehensive evaluation of typical diagnostic features and detection of acute complications while also providing prognostic insights. The aim of this review is to evaluate the current research on non-invasive modalities and to propose a diagnostic algorithm that will facilitate the identification and management of TTS.

Recurrent Pericarditis and Paradigm Shift in Cardiovascular Imaging and Targeted Therapeutics.

Recurrent pericarditis poses a significant challenge to patients and clinicians given its high morbidity and health care burden. Since the last iteration of European Society of Cardiology Guidelines in 2015, further insights have been gained into the pathophysiology, multimodality imaging assessment, and treatment of this condition. The purpose of this review is to discuss each of these aspects and highlight the role of imaging-guided therapy and interleukin-1 inhibitors in autoinflammatory phenotypes that together have transformed the care of these patients. Although future investigations are needed to optimize diagnostic surveillance and timing of therapy, recent evidence points at an encouraging paradigm shift in the treatment of recurrent pericarditis.

Extracellular Volume and Fibrosis Volume of Left Ventricular Myocardium Assessed by Cardiac Magnetic Resonance in Vaccinated and Unvaccinated Patients with a History of SARS-CoV-2 Infection.

Cardiac magnetic resonance (CMR) enables the assessment of tissue characteristics of the myocardium. Changes in the extracellular volume (ECV) and fibrosis volume (FV) of the myocardium are sensitive and early pathogenetic markers and have prognostic significance. The aim of the study was to assess ECV and FV of left ventricular myocardium in T1 mapping sequence in patients with a history of SARS-CoV-2 infection, considering vaccination status against COVID-19. The study group consisted of 97 patients (52.54 ± 8.31 years, 53% women and 47% men). The participants were divided into three subgroups: A) patients with a history of symptomatic SARS-CoV-2 infection, unvaccinated against COVID-19 (n = 39), B) patients with a history of symptomatic SARS-CoV-2 infection, with a full vaccination schedule against COVID-19 (n = 22), and C) persons without a history of SARS-CoV-2 infection constituting the control subgroup (C, n = 36). All patients underwent 1.5 T cardiac magnetic resonance. In subgroup A compared to subgroups B and C, both the ECV whole myocardium and ECV segments 2, 5-6, 8, and 10-11 were statistically significantly higher. In addition, the ECV segment 16 was statistically significantly higher in subgroup A than in subgroup C. Also, the FV whole myocardium was statistically significantly higher in subgroup A in comparison to subgroups B and C. There were no significant differences in ECV and FV between subgroups B and C. In summary, unvaccinated against COVID-19 patients with a history of symptomatic SARS-CoV-2 infection have higher myocardial ECV and FV values in the T1 mapping sequence, compared to those without COVID-19 and those suffering from COVID-19, previously vaccinated with the full vaccination schedule.

Diagnostic Performance of Quantitative Perfusion Cardiac Magnetic Resonance Imaging in Patients with Prior Coronary Artery Disease.

AIMS: The diagnostic performance of quantitative perfusion cardiac magnetic resonance (QP-CMR) imaging has scarcely been evaluated in patients with a history of coronary artery disease (CAD) and new onset chest pain. The present study compared the diagnostic performance of automated QP-CMR for detection of fractional flow reserve (FFR) defined hemodynamically significant CAD with visual assessment of first-pass stress perfusion CMR (v-CMR) and quantitative [15O]H2O positron emission tomography (PET) imaging in a true head-to-head fashion in patients with prior CAD. METHODS AND RESULTS: This PACIFIC-2 substudy included 145 symptomatic chronic coronary symptom patients with prior myocardial infarction (MI) and/or percutaneous coronary intervention (PCI). All patients underwent dual-sequence, single bolus perfusion CMR and [15O]H2O PET perfusion imaging followed by invasive coronary angiography with three-vessel FFR. Hemodynamically significant CAD was defined as an FFR ≤0.80. QP-CMR, v-CMR and PET exhibited a sensitivity of 66%, 67%, and 80%, respectively, whereas specificity was 60%, 62%, and 63%. Sensitivity of QP-CMR was lower than PET (P=0.015), whereas specificity of QP-CMR and PET was comparable. Diagnostic accuracy and area under the curve (AUC) of QP-CMR (64% and 0.66) was comparable to both v-CMR (66% [P=NS] and 0.67 (P=NS]) and PET (74% [P=NS] and 0.78 [P=NS]). CONCLUSIONS: In patients with prior MI and/or PCI, the diagnostic performance of QP-CMR was comparable to visual assessment of first-pass stress perfusion CMR and quantitative [15O]H2O PET for the detection of hemodynamically significant CAD as defined by FFR.

Cystic cardiac myxoma: a rare variant of cardiac myxoma in a woman with acute stroke.

BACKGROUND: Cardiac myxomas are the most common primary benign tumors of the heart usually presenting as an oval mass attached to the interatrial septum. Mild heterogenous enhancement is typically seen upon contrast administration. Myxomas are benign, however can present with embolic episodes necessitating prompt diagnosis and treatment. CASE PRESENTATION: A middle-aged woman presenting with acute ischemic stroke was detected to have a complex cystic mass in the left atrium on echocardiography. Further evaluation with cardiac magnetic resonance imaging narrowed a differential diagnosis to hemangioma, hemangioendothelioma, sarcoma, hydatid cysts, bronchogenic cysts, and cystic variant of cardiac myxoma. CTA demonstrated arterial supply from the left circumflex artery supplying the lesion. Following surgical excision, histopathology analysis suggested a cystic cardiac myxoma with secondary degeneration. CONCLUSION: Cystic masses in the heart are challenging to diagnose due to similar imaging characteristics, and misdiagnosis may lead to further major downstream complications such as stroke, hemorrhage, and hemodynamic alterations causing syncope. Therefore, surgical excision of a cystic cardiac mass is justified to establish a definitive histopathological diagnosis and prevent further downstream complications. We hereby report a rare case of cystic cardiac myxoma in the left atrium showing intense progressive enhancement in CMR and CTA in a middle-aged woman presenting with acute stroke. This case highlights an exceedingly rare variant of cystic cardiac myxoma.

[Position paper of the Austrian Society for Rheumatology and the Austrian Society for Pneumology on the diagnosis and treatment of sarcoidosis 2024]. / Positionspapier der ÖGR und ÖGP zur Diagnose und Therapie der Sarkoidose 2024.

In many cases sarcoidosis is a multisystemic disease that requires interdisciplinary medical cooperation in the diagnostics, treatment and medical care during follow-up. Due to the often chronic course, it is of utmost importance to include patients with their priorities and wishes at an early stage and extensively in disease management and to establish a shared decision making whenever possible. In the process of writing this joint position paper, the expert group on interstitial and orphan lung diseases of the Austrian Society for Pulmonology and the working group on rheumatological lung disorders of the Austrian Society for Rheumatology and Rehabilitation sought to include patient advocacy groups as well as experts for rare organ manifestations of sarcoidosis. This position paper is not only meant to reflect current scientific and clinical standards but should also focus the national expertise and by networking and exchange to be a first step to strengthen cooperation between stakeholders to ultimately improve care for patients with sarcoidosis.

Late gadolinium enhancement and outcome of cardiac resynchronization therapy in non-ischemic cardiomyopathy.

BACKGROUND: It is uncertain whether CRT with defibrillator (CRTD) is superior to CRT with pacemaker (CRTP) in NICM. Patients with low arrhythmic risk and high probability of response to CRT might be ideal candidates for CRTP. We aimed to evaluate predictors of ventricular arrhythmias and of echocardiographic response to cardiac resynchronization therapy (CRT) in non-ischemic cardiomyopathy (NICM). METHODS: Multicenter, retrospective observational study of NICM patients with left ventricular ejection fraction (LVEF) ≤35 %, cardiac magnetic resonance with analysis of late gadolinium enhancement (LGE) available and de-novo CRT implant. Echocardiographic response to CRT was defined as an improvement in LVEF ≥10 %. The combined arrhythmic endpoint included sustained ventricular tachycardia, appropriate ICD therapy, resuscitated cardiac arrest and sudden death. RESULTS: We included 167 patients, with a median follow-up of 63 months. LGE was present in 77 (46 %). Response to CRT occurred in 68 % of patients, more frequently in LGE- than in LGE+ (81 % vs 53 %, p < 0.001). Absence of LGE (OR 3.4, p = 0.002), was an independent predictor of response to CRT. The arrhythmic endpoint occurred in 19 patients (11 %). Among LGE- patients there were zero arrhythmic events as compared to a 25 % cumulative incidence in LGE+ (p < 0.001). Presence of LGE (HR 22.5, p < 0.001), was an independent predictor of the arrhythmic endpoint. CONCLUSION: Absence of LGE identifies patients at minimal arrhythmic risk and with high probability of response to CRT. Thus, they might be ideal candidates to CRT-P.

Usefulness of Native T1 in Cardiac Magnetic Resonance Imaging and Echocardiographic Strain Parameters for Detecting Early Cardiac Involvement in Fabry Cardiomyopathy.

Background: Non-invasive diagnosis of disease stage in Fabry cardiomyopathy with multimodality imaging is pivotal when deciding on the appropriate time to initiate enzyme replacement therapy. However, this approach has not been well established. Methods and Results: We enrolled 14 patients with Fabry disease. All patients were evaluated using echocardiography and contrast cardiac magnetic resonance (CMR), and were divided into either an early-stage group without left ventricular hypertrophy (LVH; wall thickness >12 mm) or late gadolinium enhancement (LGE; n=7; median age 37 years; 4 female), or an advanced-stage group with LVH and/or LGE (n=7; median age 66 years; 7 female). Strain data from echocardiography and T1 mapping on CMR were compared between the groups. In the advanced-stage group, all strain data were impaired. In the early-stage group, localized longitudinal strain in the basal posterolateral segment was already reduced but both localized and global circumferential strain remained preserved. On CMR analysis, global and localized native T1 shortening were observed in the early-stage group, but were pseudo-normalized in the advanced-stage group. In logistic regression analysis, localized circumferential strain had significant diagnostic value for differentiating between early- and advanced stage (P=0.037) and significantly improved the predictive power of the model containing localized native T1 in CMR. Conclusions: A combination of localized native T1 in CMR and echocardiographic strain parameters could be useful for staging Fabry cardiomyopathy.