Competency based curriculum for cardiovascular magnetic resonance: A position statement of the Society for Cardiovascular Magnetic Resonance.

This position statement guides cardiovascular magnetic resonance (CMR) imaging program directors and learners on the key competencies required for Level II and III CMR practitioners, whether trainees come from a radiology or cardiology background. This document is built upon existing curricula and was created and vetted by an international panel of cardiologists and radiologists on behalf of the Society for Cardiovascular Magnetic Resonance (SCMR).

Canadian Association of Radiologists Cardiovascular Imaging Referral Guideline.

The Canadian Association of Radiologists (CAR) Cardiovascular Expert Panel is made up of physicians from the disciplines of radiology, cardiology, and emergency medicine, a patient advisor, and an epidemiologist/guideline methodologist. After developing a list of 30 clinical/diagnostic scenarios, a rapid scoping review was undertaken to identify systematically produced referral guidelines that provide recommendations for one or more of these clinical/diagnostic scenarios. Recommendations from 48 guidelines and contextualization criteria in the Grading of Recommendations, Assessment, Development, and Evaluations (GRADE) for guidelines framework were used to develop 125 recommendation statements across the 30 scenarios (27 unique scenarios as 2 scenarios point to the CAR Thoracic Diagnostic Imaging Referral Guideline and the acute pericarditis subscenario is included under 2 main scenarios). This guideline presents the methods of development and the referral recommendations for acute chest pain syndromes, chronic chest pain, cardiovascular screening and risk stratification, pericardial syndromes, intracardiac/pericardial mass, suspected valvular disease cardiomyopathy, aorta, venous thrombosis, and peripheral vascular disease.

Peripartum Cardiomyopathy is Associated With Abnormalities of Myocardial Deformation and Late Gadolinium Enhancement.

Purpose: Peripartum cardiomyopathy (PPCM) affects women in late pregnancy and postpartum. Cardiovascular magnetic resonance (CMR) can contribute to PPCM diagnosis and management. We explored CMR findings in PPCM, including myocardial strain and late gadolinium enhancement (LGE) patterns. Materials and Methods: This retrospective single-centre study included patients with PPCM who underwent CMR from 2010 to 2018. Exclusions were other cardiomyopathy causes. CMR parameters, including ventricular function, LGE, and myocardial strain, were compared between the PPCM group and healthy controls. Transthoracic echocardiographic data were reviewed to assess functional improvement in PPCM patients. Results: Thirty-two women with PPCM (mean age 42 ± 6 years) and 26 controls (mean age 43 ± 14 years) were included. PPCM patients had significantly lower left ventricular (LV) ejection fractions (median 37.5% vs 60.5%, P < .001), higher LV end-diastolic volumes (median 108 ml/m² vs 76 ml/m², P < .001), and reduced global LV strain compared to controls. Eighteen PPCM patients (58%) had non-ischaemic pattern LGE, with no LGE in controls besides hingepoint LGE (23%). LGE was most prevalent in the basal and mid anteroseptum. LGE patterns included linear mid-wall, subepicardial, and right ventricular side of the septum. Twenty-four patients (92%) showed improvement in LVEF at follow-up echocardiogram (mean LVEF 28% ± 1.9% at diagnosis and 45% ± 3% at follow-up, P < .001). Conclusion: We identified a non-ischaemic pattern LGE that is nonspecific in isolation but could suggest PPCM in the correct clinical context along with abnormal CMR strain values. Future studies should evaluate the clinical application of these findings to facilitate earlier diagnosis and enhance management.

Breast Arterial Calcifications on Mammography: Awareness and Reporting Preferences Amongst Referring Physicians in Canada.

Purpose: Breast arterial calcifications (BAC) on mammography have been correlated with increased cardiovascular risk. The Canadian Society of Breast Imaging released a position statement on BAC reporting in January 2023. This study evaluates the awareness of the clinical significance of BAC and reporting preferences of referring physicians in Canada. Methods: A 15-question survey was distributed to Canadian physicians who may review mammography results via regional and subspecialty associations and on social media following local institutional ethical approval. Responses were collected over 10 weeks from February to April 2023. Results: Seventy-two complete responses were obtained. We are unable to determine the response rate, given the means of distribution. Only 17% (12/72) of responding physicians were previously aware of the association between BAC and increased cardiovascular risk, and 51% (37/72) preferred the inclusion of BAC in the mammography report. Fifty-six percent (40/72) indicated that BAC reporting would prompt further investigation, and 63% (45/72) would inform patients that their mammogram showed evidence of BAC. Sixty-nine percent (50/72) would find grading of BAC beneficial and 71% (51/72) agreed that there is a need for national guidelines. Conclusion: Less than a quarter of responding Canadian referring physicians were previously aware of the association between BAC and cardiovascular risk, although half of respondents indicated a preference for BAC reporting on mammography. Most participating physicians would inform their patients of the presence of BAC and consider further cardiovascular risk management. There was consensus that a national BAC grading system and clinical management guidelines would be beneficial.

CAR and CSTR Cardiac Computed Tomography (CT) Practice Guidelines: Part 2-Non-Coronary Imaging.

The cardiac computed tomography (CT) practice guidelines provide an updated review of the technological improvements since the publication of the first Canadian Association of Radiologists (CAR) cardiac CT practice guidelines in 2009. An overview of the current evidence supporting the use of cardiac CT in the most common clinical scenarios, standards of practice to optimize patient preparation and safety as well as image quality are described. Coronary CT angiography (CCTA) is the focus of Part I. In Part II, an overview of cardiac CT for non-coronary indications that include valvular and pericardial imaging, tumour and mass evaluation, pulmonary vein imaging, and imaging of congenital heart disease for diagnosis and treatment monitoring are discussed. The guidelines are intended to be relevant for community hospitals and large academic centres with established cardiac CT imaging programs.

CAR and CSTR Cardiac Computed Tomography (CT) Practice Guidelines: Part 1 Coronary CT Angiography (CCTA).

Imaging the heart is one of the most technically challenging applications of Computed Tomography (CT) due to the presence of cardiac motion limiting optimal visualization of small structures such as the coronary arteries. Electrocardiographic gating during CT data acquisition facilitates motion free imaging of the coronary arteries. Since publishing the first version of the Canadian Association of Radiologists (CAR) cardiac CT guidelines, many technological advances in CT hardware and software have emerged necessitating an update. The goal of these cardiac CT practice guidelines is to present an overview of the current evidence supporting the use of cardiac CT in various clinical scenarios and to outline standards of practice for patient safety and quality of care when establishing a cardiac CT program in Canada.

Cardiac Investigation Trends Among Primary Care Physicians: A Paradigm Shift.

OBJECTIVE: While numerous recent guidelines support coronary computed tomography angiography (CTA) as a first-line test for stable chest pain, it remains underutilized by primary care physicians (PCPs). We aimed to evaluate cardiac investigation ordering practices following education sessions, as well as the total number of downstream tests and time to diagnosis for patients presenting with stable chest pain. METHODS: A retrospective chart review was completed for eligible patients assessed at the Women's College Hospital Family Practice Health Centre between 2017 and 2019 following the education sessions. The outcome measures were first-choice cardiac investigation, additional downstream testing, time from presentation to first investigation, and time to final diagnosis. RESULTS: 419 patients were included in the final analysis (74.70% female; mean age 61 ± 11 years). Coronary CTA requests by PCPs increased between 2017 and 2019 (18 vs 72 tests; P < .0001). When coronary CTA was the first-line test, patients were less likely to receive additional downstream testing when compared to those receiving other first-line investigations (P < .0001). Coronary CTA was associated with longer time to diagnosis than stress echocardiography (47 ± 45 vs 27 ± 36 days; P = .0068) due to limited availability of coronary CTA appointment times. There was no significant difference in time to final diagnosis among the cardiac imaging modalities observed in the cohort (P = .0623). CONCLUSION: Utilization of coronary CTA as the first-line test for stable chest pain increased following our education sessions targeting PCPs. Coronary CTA was associated with less downstream testing compared to other non-invasive cardiac investigations.

Correlation Between Breast Arterial Calcifications and Higher Cardiovascular Risk: Awareness and Attitudes Amongst Canadian Radiologists Who Report Mammography.

Background: Breast arterial calcification (BAC) on mammography correlates with increased cardiovascular risk. Reporting BAC is not standard practice. Our study evaluates the awareness of Canadian radiologists who report mammography of the clinical significance of BAC and their attitudes towards reporting BAC compared to their European and American counterparts. Methods: Following local institutional ethics approval, a 25 question survey (SurveyMonkey) was disseminated to Canadian radiologists via provincial and national society email lists. Responses were collected over 5 weeks (April-June 2022). Results: One hundred and eighty-six complete responses were collected. Sixty percent (112/186) were aware of the association between BAC and cardiovascular risk and 16% (29/186) document its presence in mammogram reports. Thirty five percent (65/186) occasionally document BAC if severe or in a young patient. Four percent (7/186) had local departmental guidelines on BAC reporting and 82% (153/186) agreed there is a need for national BAC reporting guidelines. Fewer Canadian radiologists were aware of the association between BAC and cardiovascular risk compared to European radiologists (60% vs 81%), report the presence of BAC compared to both European (15% vs 62%) and American (15% vs 35%) radiologists, and inform the patient of the presence of BAC compared to European radiologists (1% vs 46%). Conclusion: Canadian radiologists who report mammography were less aware of the association between BAC and cardiovascular risk than their European and American counterparts and were less likely to document the presence of BAC. Given the correlation of BAC with increased cardiovascular event risk, there is increased need for awareness as well as national BAC reporting guidelines.

Promoting Equity, Diversity, and Inclusion in Medicine: A Comprehensive Toolkit for Change in Radiology.

This toolkit presents a comprehensive framework for a toolkit intended to increase equity, diversity, and inclusion (EDI) within the medical field and recommendations. We advocate for clear, comprehensive definitions and interpretations of fundamental EDI terms, laying the groundwork necessary for initiating and maintaining EDI initiatives. Furthermore, we offer a systematic approach to establishing EDI committees within medical departments, accentuating the pivotal role these committees play as they drive and steer EDI strategies. This toolkit also explores strategies tailored for the recruitment of a diverse workforce. This includes integral aspects such as developing inclusive job advertisements, implementing balanced search methods for candidates, conducting unbiased appraisals of applications, and structuring diverse hiring committees. The emphasis on these strategies not only augments the diversity within medical institutions but also sets the stage for a more holistic approach to healthcare delivery. Therefore, by adopting the recommended strategies and guidelines outlined in this framework, medical institutions and specifically radiology departments can foster an environment that embodies inclusivity and equity, thereby enhancing the quality of patient care and overall health outcomes.

Canadian Association of Radiologists/Canadian Association for Interventional Radiology/Canadian Society of Thoracic Radiology Guidelines on Thoracic Interventions.

Thoracic interventions are frequently performed by radiologists, but guidelines on appropriateness criteria and technical considerations to ensure patient safety regarding such interventions is lacking. These guidelines, developed by the Canadian Association of Radiologists, Canadian Association for Interventional Radiology and Canadian Society of Thoracic Radiology focus on the interventions commonly performed by thoracic radiologists. They provide evidence-based recommendations and expert consensus informed best practices for patient preparation; biopsies of the lung, mediastinum, pleura and chest wall; thoracentesis; pre-operative lung nodule localization; and potential complications and their management.

Visual Ordinal Scoring of Coronary Artery Calcium on Contrast-Enhanced and Noncontrast Chest CT: A Retrospective Study of Diagnostic Performance and Prognostic Utility.

BACKGROUND. Current guidelines recommend visual evaluation of coronary artery calcium (CAC) on all nongated noncontrast chest CT examinations. However, chest CT examinations are often performed with contrast material administration. OBJECTIVE. The purpose of our study was to evaluate diagnostic performance, prognostic utility, and interobserver agreement of visual CAC assessment on chest CT performed for other indications. METHODS. This retrospective study included 260 patients (158 men, 102 women; mean age, 60 ± 11 [SD] years) who underwent both nongated chest CT (contrast-enhanced in 116 patients; noncontrast in 144 patients) and cardiac calcium score CT within a 12-month interval. A cardiothoracic radiologist visually assessed CAC on chest CT using an ordinal scale (absent, mild, moderate, or severe). Cardiac CT Agatston calcium scores were quantified according to established guidelines and were categorized as CAC absent (0), mild CAC (1-99), moderate CAC (100-299), or severe CAC (≥ 300). The diagnostic performance of chest CT for the presence of CAC was assessed using cardiac CT as the reference standard. Major adverse cardiac events (MACE) were assessed as a composite of cardiovascular death and myocardial infarction and were evaluated using Cox proportional hazards models. A second cardiothoracic radiologist performed visual CAC assessments in a random subset of 50 chest CT examinations to assess interob-server agreement. RESULTS. For the presence of any CAC on cardiac CT, contrast-enhanced and non-contrast chest CT had sensitivity of 83% (62/75) and 89% (85/95) (p = .20) and specificity of 100% (41/41) and 100% (49/49) (p = .99). CAC present on cardiac CT was misclassified as absent on 13 contrast-enhanced and 10 noncontrast chest CT examinations; Agatston score was less than 30 in all such patients, and none experienced any MACE. The visual ordinal CAC score was associated with MACE for contrast-enhanced chest CT (hazard ratio [HR] = 4.5 [95% CI, 1.2-16.4], p = .02) and noncontrast chest CT (HR = 3.4 [95% CI, 1.5-7.8], p = .003). Interobserver agreement was excellent for contrast-enhanced (κ = 0.89) and noncontrast (κ = 0.95) chest CT. CONCLUSION. Visual ordinal CAC assessment on both contrast-enhanced and non-contrast chest CT has high diagnostic performance, prognostic utility, and interobserver agreement. CLINICAL IMPACT. Routine reporting of CAC on all chest CT examinations regardless of clinical indication and contrast material administration could identify a large number of patients with previously unknown CAC who might benefit from preventive treatment.

Computed Tomography Angiography Assessment of Acute Aortic Syndromes: Classification, Differentiating Imaging Features, and Imaging Interpretation Pitfalls.

An acute aortic syndrome (AAS) is an important life-threatening condition that requires early detection and management. Acute intramural hematoma (IMH), aortic dissection (AD) and penetrating atherosclerotic ulcer (PAU) are included in AAS. ADs can be classified using the well-known Stanford or DeBakey classification systems. However, these classification systems omit description of arch dissections, anatomic variants, and morphologic features that impact outcome. The Society for Vascular Surgery and Society of Thoracic Surgeons (SVS-STS) have recently introduced a classification system that classifies ADs according to the location of the entry tear (primary intimomedial tear, PIT) and the proximal and distal extent of involvement, but does not include description of all morphologic features that may have diagnostic and prognostic significance. This review describes these classification systems for ADs and other AAS entities as well as their limitations. Typical computed tomography angiography (CTA) imaging appearance and differentiating features of ADs, limited intimal tears (LITs), IMHs, intramural blood pools (IBPs), ulcer-like projections (ULPs), and PAUs will be discussed. Furthermore, this review highlights common imaging interpretation pitfalls, what should be included in a comprehensive CTA report, and provides a brief overview of current management options.

Canadian Society of Thoracic Radiology/Canadian Association of Radiologists Best Practice Guidance for Investigation of Acute Pulmonary Embolism, Part 1: Acquisition and Safety Considerations.

Acute pulmonary embolism (APE) is a well-recognized cause of circulatory system compromise and even demise which can frequently present a diagnostic challenge for the physician. The diagnostic challenge is primarily due to the frequency of indeterminate presentations as well as several other conditions which can have a similar clinical presentation. This often obliges the physician to establish a firm diagnosis due to the potentially serious outcomes related to this disease. Computed tomography pulmonary angiography (CTPA) has increasingly cemented its role as the primary investigation tool in this clinical context and is widely accepted as the standard of care due to several desired attributes which include great accuracy, accessibility, rapid turn-around time and the ability to suggest an alternate diagnosis when APE is not the culprit. In Part 1 of this guidance document, a series of up-to-date recommendations are provided to the reader pertaining to CTPA protocol optimization (including scan range, radiation and intravenous contrast dose), safety measures including the departure from breast and gonadal shielding, population-specific scenarios (pregnancy and early post-partum) and consideration of alternate diagnostic techniques when clinically deemed appropriate.

Canadian Society of Thoracic Radiology/Canadian Association of Radiologists Best Practice Guidance for Investigation of Acute Pulmonary Embolism, Part 2: Technical Issues and Interpretation Pitfalls.

The investigation of acute pulmonary embolism is a common task for radiologists in Canada. Technical image quality and reporting quality must be excellent; pulmonary embolism is a life-threatening disease that should not be missed but overdiagnosis and unnecessary treatment should be avoided. The most frequently performed imaging investigation, computed tomography pulmonary angiogram (CTPA), can be limited by poor pulmonary arterial opacification, technical artifacts and interpretative errors. Image quality can be affected by patient factors (such as body habitus, motion artifact and cardiac output), intravenous (IV) contrast protocols (including the timing, rate and volume of IV contrast administration) and common physics artifacts (including beam hardening). Mimics of acute pulmonary embolism can be seen in normal anatomic structures, disease in non-vascular structures and pulmonary artery filling defects not related to acute pulmonary emboli. Understanding these pitfalls can help mitigate error, improve diagnostic quality and optimize patient outcomes. Dual energy computed tomography holds promise to improve imaging diagnosis, particularly in clinical scenarios where routine CTPA may be problematic, including patients with impaired renal function and patients with altered cardiac anatomy.

Diagnostic performance of chest radiography measurements for the assessment of cardiac chamber enlargement.

BACKGROUND: The cardiothoracic ratio (CTR) is commonly assessed on chest radiography for detection of cardiac chamber enlargement, but the traditional cutpoint of 0.5 has low specificity. We sought to evaluate the diagnostic accuracy of new measurement techniques for the detection of cardiac enlargement on chest radiographs. METHODS: We obtained retrospective cross-sectional data on consecutive patients who underwent both chest radiography and cardiac magnetic resonance imaging (MRI) within a 14-day interval between 2006 and 2016 at a large academic hospital network. We established the presence of cardiac chamber enlargement using cardiac MRI as the reference standard. We evaluated the diagnostic performance of different techniques for measuring heart size and CTR on frontal chest radiographs. RESULTS: Of 152 patients included, 81 (53%) were men and the mean age was 52 years. Maximum heart diameter had the highest area under the receiver operating characteristic curve for detection of cardiac enlargement (0.827, 95% confidence interval 0.760-0.894). In the subgroup of posteroanterior chest radiography studies (n = 101), a CTR cutpoint of 0.50 had only moderate sensitivity (72%) and specificity (72%). In men, a maximum heart diameter cutpoint of 15 cm had a sensitivity of 86% and a negative likelihood ratio of 0.24, and a cutpoint of 19 cm had a specificity of 100% and a positive likelihood ratio of infinity. In women, a maximum heart diameter cutpoint of 13 cm had a sensitivity of 91% and a negative likelihood ratio of 0.15, and a cutpoint of 17 cm had a specificity of 91% and a positive likelihood ratio of 3.5. INTERPRETATION: A traditional CTR cutpoint of 0.5 has limited diagnostic value. Simple heart diameter measurements have higher diagnostic performance measures than CTR.

Type 2 bridging bronchus with left pulmonary artery sling.

Bridging bronchi are the rarest of the major airway anomalies reported in the literature. In this brief report, we present a case of a symptomatic adult male patient presenting with a type 2 bridging bronchus associated with left pulmonary artery sling.

Canadian Society of Thoracic Radiology/Canadian Association of Radiologists Clinical Practice Guidance for Non-Vascular Thoracic MRI.

Historically thoracic MRI has been limited by the lower proton density of lung parenchyma, cardiac and respiratory motion artifacts and long acquisition times. Recent technological advancements in MR hardware systems and improvement in MR pulse sequences have helped overcome these limitations and expand clinical opportunities for non-vascular thoracic MRI. Non-vascular thoracic MRI has been established as a problem-solving imaging modality for characterization of thymic, mediastinal, pleural chest wall and superior sulcus tumors and for detection of endometriosis. It is increasingly recognized as a powerful imaging tool for detection and characterization of lung nodules and for assessment of lung cancer staging. The lack of ionizing radiation makes thoracic MRI an invaluable imaging modality for young patients, pregnancy and for frequent serial follow-up imaging. Lack of familiarity and exposure to non-vascular thoracic MRI and lack of consistency in existing MRI protocols have called for clinical practice guidance. The purpose of this guide, which was developed by the Canadian Society of Thoracic Radiology and endorsed by the Canadian Association of Radiologists, is to familiarize radiologists, other interested clinicians and MR technologists with common and less common clinical indications for non-vascular thoracic MRI, discuss the fundamental imaging findings and focus on basic and more advanced MRI sequences tailored to specific clinical questions.

Canadian Society of Thoracic Radiology/Canadian Association of Radiologists Consensus Statement Regarding Chest Imaging in Suspected and Confirmed COVID-19.

On March 11, 2020, the World Health Organization declared infection related to a novel coronavirus (SARS-CoV-2) a pandemic. The role and impact of imaging predates this declaration and continues to change rapidly. This article is a consensus statement provided by the Canadian Society of Thoracic Radiology and the Canadian Association of Radiologists outlining the role of imaging in COVID-19 patients. The objectives are to answer key questions related to COVID-19 imaging of the chest and provide guidance for radiologists who are interpreting such studies during this pandemic. The role of chest radiography (CXR), computed tomography (CT), and lung ultrasound is discussed. This document attempts to answer key questions for the imager when dealing with this crisis, such as "When is CXR appropriate in patients with suspected or confirmed COVID-19 infection?" or "How should a radiologist deal with incidental findings of COVID-19 on CT of the chest done for other indications?" This article also provides recommended reporting structure for CXR and CT, breaking diagnostic possibilities for both CXR and CT into 3 categories: typical, nonspecific, and negative based on imaging findings with representative images provided. Proposed reporting language is also outlined based on this structure. As our understanding of this pandemic evolves, our appreciation for how imaging fits into the workup of patients during this unprecedented time evolves as well. Although this consensus statement was written using the most recent literature, it is important to maintain an open mind as new information continues to surface.

CT-guided Microcoil Pulmonary Nodule Localization prior to Video-assisted Thoracoscopic Surgery: Diagnostic Utility and Recurrence-Free Survival.

Background CT-guided microcoil localization has been shown to reduce the need for thoracotomy or video-assisted thoracoscopic surgery (VATS) anatomic resection. However, only short-term follow-up after CT-guided microcoil localization and lung resection has been previously reported. Purpose To assess the diagnostic utility and recurrence-free survival over a minimum of 2 years following CT-guided microcoil localization and VATS. Materials and Methods Among 1950 VATS procedures performed in a single tertiary institution from October 2008 through April 2016, 124 consecutive patients with CT-guided microcoil localization were retrospectively evaluated. Patient demographics, nodule characteristics, and histopathologic findings were recorded. The primary end point was recurrence-free survival after 2 or more years of CT surveillance. Statistical analysis included Kaplan-Meier survival curves and Cox regression. Results In 124 patients (men, 35%; mean age, 65 years ± 12) with a nodule found at CT, microcoil localization and VATS resection were performed for a total of 126 nodules (mean size, 13 mm ± 6; mean distance to pleura, 20 mm ± 9). On presurgical CT evaluation, 42% (53 of 126) of nodules were solid, 33% (41 of 126) were ground glass, and 24% (30 of 126) were subsolid. VATS excisional biopsy altered cytopathologic diagnosis in 21% (five of 24) of patients with prior diagnostic premicrocoil CT-guided biopsy. At histopathologic examination, 17% (21 of 126) of the nodules were adenocarcinoma in situ, 17% (22 of 126) were minimally invasive adenocarcinoma, 30% (38 of 126) were invasive lung primary tumors, and 22% (28 of 126) were metastases. Among the 72 patients with malignancy at histopathologic examination and at least 2 years of CT surveillance, local recurrence occurred in 7% (five of 72), intrathoracic recurrence in 22% (16 of 72), and extrathoracic recurrence in 18% (13 of 72) after 2 or more years of CT surveillance. There was no recurrence for adenocarcinoma in situ, minimally invasive adenocarcinoma, or invasive lung tumors measuring less than 1 cm. After multivariable adjustment, nodule location at a distance greater than 10 mm from the pleura was an independent predictor of time to recurrence (hazard ratio, 2.9 [95% confidence interval: 1.1, 7.4]; P = .03). Conclusion CT-guided microcoil localization and video-assisted thoracoscopic surgical resection alter clinical management and were associated with excellent recurrence-free survival for superficial premalignant, minimally invasive, and small invasive lung tumors. © RSNA, 2019 Online supplemental material is available for this article.

Loss of base-to-apex circumferential strain gradient assessed by cardiovascular magnetic resonance in Fabry disease: relationship to T1 mapping, late gadolinium enhancement and hypertrophy.

BACKGROUND: Cardiac involvement is common and is the leading cause of mortality in Fabry disease (FD). We explored the association between cardiovascular magnetic resonance (CMR) myocardial strain, T1 mapping, late gadolinium enhancement (LGE) and left ventricular hypertrophy (LVH) in patients with FD. METHODS: In this prospective study, 38 FD patients (45.0 ± 14.5 years, 37% male) and 8 healthy controls (40.1 ± 13.7 years, 63% male) underwent 3 T CMR including cine balanced steady-state free precession (bSSFP), LGE and modified Look-Locker Inversion recovery (MOLLI) T1 mapping. Global longitudinal (GLS) and circumferential (GCS) strain and base-to-apex longitudinal strain (LS) and circumferential strain (CS) gradients were derived from cine bSSFP images using feature tracking analysis. RESULTS: Among FD patients, 8 had LVH (FD LVH+, 21%) and 17 had LGE (FD LGE+, 45%). Nineteen FD patients (50%) had neither LVH nor LGE (FD LVH- LGE-). None of the healthy controls had LVH or LGE. FD patients and healthy controls did not differ significantly with respect to GLS (- 15.3 ± 3.5% vs. - 16.3 ± 1.5%, p = 0.45), GCS (- 19.4 ± 3.0% vs. -19.5 ± 2.9%, p = 0.84) or base-to-apex LS gradient (7.5 ± 3.8% vs. 9.3 ± 3.5%, p = 0.24). FD patients had significantly lower base-to-apex CS gradient (2.1 ± 3.7% vs. 6.5 ± 2.2%, p = 0.002) and native T1 (1170.2 ± 37.5 ms vs. 1239.0 ± 18.0 ms, p < 0.001). Base-to-apex CS gradient differentiated FD LVH- LGE- patients from healthy controls (OR 0.42, 95% CI: 0.20 to 0.86, p = 0.019), even after controlling for native T1 (OR 0.24, 95% CI: 0.06 to 0.99, p = 0.049). In a nested logistic regression model with native T1, model fit was significantly improved by the addition of base-to-apex CS gradient (χ2(df = 1) = 11.04, p < 0.001). Intra- and inter-observer agreement were moderate to good for myocardial strain parameters: GLS (ICC 0.849 and 0.774, respectively), GCS (ICC 0.831 and 0.833, respectively), and base-to-apex CS gradient (ICC 0.737 and 0.613, respectively). CONCLUSIONS: CMR reproducibly identifies myocardial strain abnormalities in FD. Loss of base-to-apex CS gradient may be an early marker of cardiac involvement in FD, with independent and incremental value beyond native T1.