Evolving consequences of right coronary artery to right atrium: coronary cameral fistula-a case report.

Background: Coronary cameral fistula is a rare diagnosis, which may be picked up incidentally on cardiac imaging. While majority of cases is asymptomatic, they can be complicated by myocardial ischaemia, arrhythmias, heart failure, infective endocarditis, and rarely rupture or thrombosis of the fistula leading to sudden death. Case summary: A 73-year-old female presents with fever, lethargy, and examination finding of a continuous cardiac murmur. CT coronary angiogram confirmed the presence of a coronary cameral fistula, with an aneurysmal RCA seen arising from the right coronary sinus, following an extensive tortuous course wrapping around the heart, and terminating at the right atrium. While there was initial streptococcus bacteraemia identified on blood culture sampling, no obvious masses were detected on the valves, chambers, or along the course of the fistula. Over time, she develops anginal chest pain and heart failure symptoms, with progressive dilatation of the right ventricle and functional tricuspid regurgitation secondary to shunting of the fistula into the right chambers. Surgical intervention was then pursued and successfully addressed these complications. Discussion: This case report highlights the importance of advanced imaging modalities for accurate diagnosis of coronary cameral fistulae, addressing late manifestations of the disease and the necessity for a collaborative, multidisciplinary approach in managing complex cardiac anomalies.

Consensus on the assessment of systemic sclerosis-associated primary heart involvement: World Scleroderma Foundation/Heart Failure Association guidance on screening, diagnosis, and follow-up assessment.

Introduction: Heart involvement is a common problem in systemic sclerosis. Recently, a definition of systemic sclerosis primary heart involvement had been proposed. Our aim was to establish consensus guidance on the screening, diagnosis and follow-up of systemic sclerosis primary heart involvement patients. Methods: A systematic literature review was performed to investigate the tests used to evaluate heart involvement in systemic sclerosis. The extracted data were categorized into relevant domains (conventional radiology, electrocardiography, echocardiography, cardiac magnetic resonance imaging, laboratory, and others) and presented to experts and one patient research partner, who discussed the data and added their opinion. This led to the formulation of overarching principles and guidance statements, then reviewed and voted on for agreement. Consensus was attained when the mean agreement was ⩾7/10 and of ⩾70% of voters. Results: Among 2650 publications, 168 met eligibility criteria; the data extracted were discussed over three meetings. Seven overarching principles and 10 guidance points were created, revised and voted on. The consensus highlighted the importance of patient counseling, differential diagnosis and multidisciplinary team management, as well as defining screening and diagnostic approaches. The initial core evaluation should integrate history, physical examination, rest electrocardiography, trans-thoracic echocardiography and standard serum cardiac biomarkers. Further investigations should be individually tailored and decided through a multidisciplinary management. The overall mean agreement was 9.1/10, with mean 93% of experts voting above 7/10. Conclusion: This consensus-based guidance on screening, diagnosis and follow-up of systemic sclerosis primary heart involvement provides a foundation for standard of care and future feasibility studies that are ongoing to support its application in clinical practice.

Accuracy of stress perfusion cardiac magnetic resonance imaging in a district hospital.

Background: The European Society of Cardiology has published updated guidelines regarding pathways for diagnosis and management of obstructive coronary artery disease (CAD). Non-invasive functional assessment, for example, by stress perfusion cardiac magnetic resonance (stress pCMR) is recommended in patients with intermediate pretest probability of disease. Previous pCMR studies were mainly performed in high volume university hospitals with experienced radiologists or cardiologists interpreting the images. Purpose: The aim of the present study was to evaluate the feasibility of establishing a stress pCMR imaging service in a district hospital. Material and Methods: One hundred and thirteen patients with intermediate pretest probability of CAD referred for single-photon emission computed tomography (SPECT) at the regional hospital also underwent adenosine stress pCMR locally. The diagnostic analysis was compared to that of an experienced cardiac magnetic resonance (CMR) center serving as a reference. Results: Inter-rater agreement between local readers and the reference reader was substantial to perfect for late gadolinium enhancement (LGE) (weighted kappa = 0.76 and 0.82), but only fair to moderate for pCMR (k = 0.34 and 0.51). No improvement in agreement between reference reader and local reader during the study was demonstrated. Conclusion: CMR is feasible in patients with intermediate pretest probability of obstructive CAD in the setting of a district hospital. However, as opposed to infarct detection with LGE, the interpretation of stress pCMR was more challenging. To establish this method, we suggest obtaining experience in close collaboration with a reference CMR center.

Chylopericardium associated with constrictive pericarditis assessed by multimodality imaging.

Acute-onset presentation with breathlessness and calcific pericardial thickening encapsulating the heart. Extremely chylous pericardium, which is by itself rare, in combination with constriction assessed with multiple imaging modalities.

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.

CMR feature tracking in cardiac asymptomatic systemic sclerosis: Clinical implications.

BACKGROUND: Impaired myocardial deformation has been sporadically described in cardiac asymptomatic systemic sclerosis (SSc). We aimed to study myocardial deformation indices in cardiac asymptomatic SSc patients using cardiac magnetic resonance feature tracking (CMR-FT) and correlate these findings to the phenotypic and autoimmune background. METHODS: Fifty-four cardiac asymptomatic SSc patients (44 females, 56±13 years), with normal routine cardiac assessment and CMR evaluation, including cine and late gadolinium enhancement (LGE) images, were included. SSc patients were compared to 21 sex- and age- matched healthy controls (17 females; 54±19 years). For CMR-FT analysis, a mid-ventricular slice for LV peak systolic radial and circumferential strain and a 4-chamber view for LV/RV peak systolic longitudinal strain were used. RESULTS: Twenty-four patients had diffuse cutaneous SSc and 30 limited cutaneous SSc. Thirteen patients had digital ulcers. Median disease duration was 3.6 years. LV ejection fraction was higher in SSc patients compared to controls (62±6% vs. 59±5%, p = 0.01). Four patients had no LGE examination; in the remaining patients LGE was absent in 74%, while 18% had RV insertion fibrosis and 8% evidence of subendocardial infarction. LV longitudinal strain differed in those with insertion fibrosis (-18.0%) and infarction (-16.7%) compared to no fibrosis (-20.3%, p = 0.04). Patients with SSc had lower RV longitudinal strain and strain rate compared to controls (p<0.001 and p = 0.01, respectively). All other strain and strain rate measurements were non-significant between patients and controls. CONCLUSIONS: In cardiac asymptomatic SSc patients with normal routine functional indices, CMR-FT identifies subclinical presence of insertion fibrosis and/or myocardial infarction by impaired LV longitudinal strain. RV derived longitudinal indices were impaired in the patient group. CMR FT indices did not correlate to the patients' phenotypic and autoimmune features.

'Image-navigated 3-dimensional late gadolinium enhancement cardiovascular magnetic resonance imaging: feasibility and initial clinical results'.

BACKGROUND: Image-navigated 3-dimensional late gadolinium enhancement (iNAV-3D LGE) is an advanced imaging technique that allows for direct respiratory motion correction of the heart. Its feasibility in a routine clinical setting has not been validated. METHODS: Twenty-three consecutive patients referred for cardiovascular magnetic resonance (CMR) examination including late gadolinium enhancement (LGE) imaging were prospectively enrolled. Image-navigated free-breathing 3-dimensional (3D) T1-weighted gradient-echo LGE and two-dimensional (2D LGE) images were acquired in random order on a 1.5 T CMR system. Images were assessed for global, segmental LGE detection and transmural extent. Objective image quality including signal-to-noise (SNR), contrast-to-noise (CNR) and myocardial/blood sharpness were performed. RESULTS: Interpretable images were obtained in all 2D-LGE and in 22/23 iNAV-3D LGE exams, resulting in a total of 22 datasets and 352 segments. LGE was detected in 5 patients with ischemic pattern, in 7 with non-ischemic pattern, while it was absent in 10 cases. There was an excellent agreement between 2D and 3D data sets with regard to global, segmental LGE detection and transmurality. Blood-myocardium sharpness measurements were also comparable between the two techniques. SNRblood and CNRblood-myo was significantly higher for 2D LGE (P < 0.001, respectively), while SNRmyo was not statistically significant between 2D LGE and iNAV-3D LGE. CONCLUSION: Diagnostic performance of iNAV-3D LGE was comparable to 2D LGE in a prospective clinical setting. SNRblood and CNRblood-myo was significantly lower in the iNAV-3D LGE group.

High-throughput gadobutrol-enhanced CMR: a time and dose optimization study.

BACKGROUND: Reducing time and contrast agent doses are important goals to provide cost-efficient cardiovascular magnetic resonance (CMR) imaging. Limited information is available regarding the feasibility of evaluating left ventricular (LV) function after gadobutrol injection as well as defining the lowest dose for high quality scar imaging. We sought to evaluate both aspects separately and systematically to provide an optimized protocol for contrast-enhanced CMR (CE-CMR) using gadobutrol. METHODS: This is a prospective, randomized, single-blind cross-over study performed in two different populations. The first population consisted of 30 patients with general indications for a rest CE-CMR who underwent cine-imaging before and immediately after intravenous administration of 0.1 mmol/kg body-weight of gadobutrol. Quantitative assessment of LV volumes and function was performed by the same reader in a randomized and blinded fashion. The second population was composed of 30 patients with indication to late gadolinium enhancement (LGE) imaging, which was performed twice at different gadobutrol doses (0.1 mmol/kg vs. 0.2 mmol/kg) and at different time delays (5 and 10 min vs. 5, 10, 15 and 20 min), within a maximal interval of 21 days. LGE images were analysed qualitatively (contrast-to-noise ratio) and quantitatively (LGE%-of-mass). RESULTS: Excellent correlation between pre- and post-contrast cine-imaging was found, with no difference of LV stroke volume and ejection fraction (p = 0.538 and p = 0.095, respectively). End-diastolic-volume and end-systolic-volume were measured significantly larger after contrast injection (p = 0.008 and p = 0.001, respectively), with a mean difference of 3.7 ml and 2.9 ml, respectively. LGE imaging resulted in optimal contrast-to-noise ratios 10 min post-injection for a gadobutrol dose of 0.1 mmol/kg body-weight and 20 min for a dose of 0.2 mmol/kg body-weight. At these time points LGE quantification did not significantly differ (0.1 mmol/kg: 11% (16.4); 0.2 mmol/kg: 12% (14.5); p = 0.059), showing excellent correlation (ICC = 0.957; p < 0.001). CONCLUSION: A standardized CE-CMR rest protocol giving a dose of 0.1 mmol/kg of gadobutrol before cine-imaging and performing LGE 10 min after injection represents a fast low-dose protocol without significant loss of information in comparison to a longer protocol with cine-imaging before contrast injection and a higher dose of gadobutrol. This approach allows to reduce examination time and costs as well as minimize contrast-agent exposure.

Diagnostic performance of image navigated coronary CMR angiography in patients with coronary artery disease.

BACKGROUND: The use of coronary MR angiography (CMRA) in patients with coronary artery disease (CAD) remains limited due to the long scan times, unpredictable and often non-diagnostic image quality secondary to respiratory motion artifacts. The purpose of this study was to evaluate CMRA with image-based respiratory navigation (iNAV CMRA) and compare it to gold standard invasive x-ray coronary angiography in patients with CAD. METHODS: Consecutive patients referred for CMR assessment were included to undergo iNAV CMRA on a 1.5 T scanner. Coronary vessel sharpness and a visual score were assigned to the coronary arteries. A diagnostic reading was performed on the iNAV CMRA data, where a lumen narrowing >50% was considered diseased. This was compared to invasive x-ray findings. RESULTS: Image-navigated CMRA was performed in 31 patients (77% male, 56 ± 14 years). The iNAV CMRA scan time was 7 min:21 s ± 0 min:28 s. Out of a possible 279 coronary segments, 26 segments were excluded from analysis due to stents or diameter less than 1.5 mm, resulting in a total of 253 coronary segments. Diagnostic image quality was obtained for 98% of proximal coronary segments, 94% of middle segments, and 91% of distal coronary segments. The sensitivity and specificity was 86% and 83% per patient, 80% and 92% per vessel and 73% and 95% per segment. CONCLUSION: In this study, iNAV CMRA offered a very good diagnostic performance when compared against invasive x-ray angiography. Due to the short and predictable scan time it can add clinical value as a part of a comprehensive CAD assessment protocol.

Cardiac magnetic resonance feature tracking in Kawasaki disease convalescence.

OBJECTIVE: The objective of this study was to determine whether left ventricular (LV) myocardial deformation indices can detect subclinical abnormalities in Kawasaki disease convalescence. We hypothesized that subclinical myocardial abnormalities due to inflammation represent an early manifestation of the disease that persists in convalescence. BACKGROUND: Myocardial inflammation has been described as a global finding in the acute phase of Kawasaki disease. Despite normal systolic function by routine functional measurements, reduced longitudinal strain and strain rate have been detected by echocardiography in the acute phase. METHODS AND RESULTS: Peak systolic LV myocardial longitudinal, radial, and circumferential strain and strain rate were examined in 29 Kawasaki disease convalescent patients (15 males; mean [standard deviation] age: 11 [6.6] years; median interval from disease onset: 5.8 [5.4] years) and 10 healthy volunteers (5 males; mean age: 14 [3.8] years) with the use of cardiac magnetic resonance (CMR) feature tracking. Routine indices of LV systolic function were normal in both groups. Comparisons were made between normal controls and (i) the entire Kawasaki disease group, (ii) Kawasaki disease subgroup divided by coronary artery involvement. Average longitudinal and circumferential strain at all levels was lower in patients compared to normal controls. In subgroup analysis, both Kawasaki disease patients with and without a history of coronary involvement had similar longitudinal and circumferential strain at all levels and lower when compared to controls. There were lower circumferential and longitudinal values in Kawasaki disease patients with persisting coronary artery lesions when compared to those with regressed ones. CONCLUSION: In this CMR study in Kawasaki disease convalescent patients with preserved routine functional indices, we detected lower circumferential and longitudinal strain values compared to normal controls, irrespective of the coronary artery status.

Cardiovascular magnetic resonance imaging pattern at the time of diagnosis of treatment naïve patients with connective tissue diseases.

BACKGROUND-AIM: Cardiac involvement at diagnosis of connective tissue disease (CTD) has been described by echocardiography. We hypothesized that cardio-vascular magnetic resonance (CMR) detects occult lesions at CTD diagnosis. PATIENTS-METHODS: CMR was performed early after diagnosis in 78 treatment-naïve CTDs (aged 43±11, 59F/19M) without cardiac involvement [5 Takayasu arteritis (TA), 4 Churg Strauss syndrome (CSS), 5 Wegener granulomatosis (WG), 16 systemic lupus erythematosus (SLE), 12 rheumatoid arthritis (RA), 8 mixed connective tissue diseases (MCTD), 12 ankylosing spondylitis (AS), 3 polymyalgia rheumatica (PMR), 8 systemic sclerosis (SSc) and 5 dermatomyositis (DM)]. Acute and chronic lesions were assessed by T2>2 with positive LGE and T2<2 with positive LGE, respectively. RESULTS: In 3/5 TA, 3/4 CSS, 4/5 WG, 10/16 SLE, 9/12 RA, 6/8 MCTD, 4/12 AS, 1/3 PMR, 2/8 SSc and 2/5 DM, the T2 ratio was higher compared to normal (2.78±0.25 vs 1.5±0.2, p<0.01). Myocarditis was identified in 1 TA, 1 SLE, 1 RA, 1 SSc and 2 DM patients; diffuse, subendocardial fibrosis in 1 CSS and 1 RA patient, while subendocardial myocardial infarction in 3 SLE, 1 MCTD, 1 PMR and 2 RA patients. CMR re-evaluation after 6 and 12months of rheumatic and cardiac treatment, available in 28/52 CTDs with increased T2 ratio, showed significant improvement in T2 ratio (p<0.001), non-significant change in LGE extent and normalisation of those with impaired LV function. CONCLUSIONS: Occult CMR lesions, including oedema, myocarditis, diffuse subendocardial fibrosis and myocardial infarction are not unusual in treatment naïve CTDs and may be reversed with appropriate treatment.

Cardiac Magnetic Resonance in Takotsubo Syndrome.

Takotsubo syndrome is an acute, profound but reversible heart failure syndrome of unknown aetiology, usually but not always triggered by physical or emotional stress. Cardiac magnetic resonance has become an important tool for the non-invasive assessment of the syndrome, allowing for a comprehensive, safe and reproducible assessment of functional and anatomical myocardial properties, including perfusion, oedema and necrosis. This review focuses on the emerging role of cardiac magnetic resonance for the characterisation, differential diagnosis as well as risk stratification of patients with Takotsubo syndrome.

Myocardial perfusion in peripheral Raynaud's phenomenon. Evaluation using stress cardiovascular magnetic resonance.

BACKGROUND: Peripheral Raynaud's phenomenon (RP) is either primary (PRP), without any coexisting disease or secondary (SRP), due to connective tissue diseases (CTD). We hypothesized that adenosine stress cardiovascular magnetic resonance (CMR) can assess myocardial perfusion in a population of PRP and SRP. PATIENTS-METHODS: Twenty CTDs, aged 30.6±7.5yrs., 16F/4M, including 9 systemic sclerosis (SSc), 4 systemic lupus erythematosus (SLE), 3 mixed connective tissue disease (MCTD), 2 polymyositis (PM) and 2 rheumatoid arthritis (RA), with SRP, under treatment with calcium blockers, were evaluated by stress CMR and compared with age-sex matched PRP and controls. All RP patients were under treatment with calcium blockers. Stress perfusion CMR was performed by 1.5T system using 140mg/kg/min adenosine for 4min and 0.05mmol/kg Gd-DTPA for first-pass perfusion. A rest perfusion was performed with the same protocol. Late gadolinium enhanced (LGE) images were acquired after another dose of Gd-DTPA. RESULTS: In both PRP, SRP, the myocardial perfusion reserve index (MPRI) was significantly reduced compared with the controls (1.7±0.6 vs 3.5±0.4, p<0.001 and 0.7±0.2 vs 3.5±0.4, p<0.001, respectively). Furthermore, in SRP, MPRI was significantly reduced, compared with PRP (0.7±0.2 vs 1.7±0.6, p<0.001). Subendo-cardial LGE=8.2±1.7 of LV mass was revealed in 1 SLE, 1MCTD and 2 SSc, but in none of PR patients. CONCLUSIONS: MPRI reduction is common in both PRP and SRP, but it is more severe in SRP, even if RP patients are under treatment with calcium blockers. Occult fibrosis may coexist with the reduced MPRI in SRP but not in PRP.

Clinical evaluation of three-dimensional late enhancement MRI.

PURPOSE: To assess the diagnostic value of three-dimensional late enhancement (3D-LGE) for the detection of myocardial necrosis in a routine clinical setting. 3D-LGE has been proposed as a novel magnetic resonance (MR) technique for the accurate detection of myocardial scar in both the ventricles and atria. Its performance in clinical practice has been poorly examined. MATERIALS AND METHODS: Fifty-seven patients referred for cardiac MR examination including scar imaging were prospectively enrolled. Gadolinium enhanced single breathhold 3D T1-weighted gradient-echo inversion recovery sequence and a conventional 2D-LGE sequence were performed using a 1.5 Tesla clinical MR imaging system. The presence, pattern and transmurality of LGE, diagnostic accuracy and level of diagnostic confidence as well as image quality (median quality, mean LGE signal intensity, sharpness, virtual scan time) were graded on a 4-point scale. RESULTS: Interpretable images were obtained in 52/57 2D-LGE and in 47/57 3D high-resolution exams. LGE was detected in 10 patients with ischemic pattern, 9 with nonischemic pattern, while it was absent in 28, resulting in a total of 47 complete datasets. The detection of global and segmental LGE as well as its transmural extent were similar for both techniques (P = 0.65, P = 0.305, and P = 0.15, respectively). Image quality (median quality, LGE/ myocardial and LGE/ blood pool sharpness) was similar for both techniques (P = 0.740, P = 0.34, and P = 1.00, respectively), but LGE signal intensity was higher with 2D (P = 0.020). CONCLUSION: 3D-LGE diagnostic and quality scores were comparable to 2D-LGE in a routine clinical setting. Further technical refinement is required for 3D LGE to offer a reliable alternative for high quality scar imaging. LEVEL OF EVIDENCE: 2 Technical Efficacy: Stage 2 J. MAGN. RESON. IMAGING 2017;45:1675-1683.

Cardiovascular magnetic resonance in rheumatology: Current status and recommendations for use.

Targeted therapies in connective tissue diseases (CTDs) have led to improvements of disease-associated outcomes, but life expectancy remains lower compared to general population due to emerging co-morbidities, particularly due to excess cardiovascular risk. Cardiovascular magnetic resonance (CMR) is a noninvasive imaging technique which can provide detailed information about multiple cardiovascular pathologies without using ionizing radiation. CMR is considered the reference standard for quantitative evaluation of left and right ventricular volumes, mass and function, cardiac tissue characterization and assessment of thoracic vessels; it may also be used for the quantitative assessment of myocardial blood flow with high spatial resolution and for the evaluation of the proximal coronary arteries. These applications are of particular interest in CTDs, because of the potential of serious and variable involvement of the cardiovascular system during their course. The International Consensus Group on CMR in Rheumatology was formed in January 2012 aiming to achieve consensus among CMR and rheumatology experts in developing initial recommendations on the current state-of-the-art use of CMR in CTDs. The present report outlines the recommendations of the participating CMR and rheumatology experts with regards to: (a) indications for use of CMR in rheumatoid arthritis, the spondyloarthropathies, systemic lupus erythematosus, vasculitis of small, medium and large vessels, myositis, sarcoidosis (SRC), and scleroderma (SSc); (b) CMR protocols, terminology for reporting CMR and diagnostic CMR criteria for assessment and quantification of cardiovascular involvement in CTDs; and (c) a research agenda for the further development of this evolving field.

Cardiovascular Magnetic Resonance Imaging clarifies cardiac pathophysiology in early, asymptomatic diffuse systemic sclerosis.

BACKGROUND: Myopericardial inflammation, perfusion's defects and fibrosis are major causes of cardiac disease in scleroderma (SSc). We hypothesized that using inflammation and stress perfusion-fibrosis cardiovascular magnetic resonance (CMR), we can identify the pathophysiology of heart disease in asymptomatic diffuse SSc. PATIENTS-METHODS: 46 recently diagnosed, asymptomatic patients with diffuse SSc had a CMR examination using a 1.5T system. ECG gated breath hold cine and short tau inversion recovery (STIR) T2 images were initially acquired. If T2 ratio<2 a stress perfusion-fibrosis protocol was applied. If T2>2 a myocarditis protocol including early (EGE) and late (LGE) gadolinium imaging was applied. SSc patients' results were compared with age and sex-matched controls and patients with coronary artery disease (CAD). RESULTS: In 2/46 SSc with T2 ratio>2, the myocarditis protocol was positive for acute myocardial inflammation, who developed clinical signs of acute myocarditis shortly after the CMR evaluation. In the rest 44/46 with T2 ratio<2 the stress perfusion-fibrosis CMR identified a significant reduction in Myocardial Perfusion Reserve Index (MPRI) compared with matched controls (0.6±0.4 vs 3.2±0.8, p<0.001), but not with CAD (0.6±0.4 vs 0.86±0.46, p=NS) and correlated only with the presence of digital ulcers (p<0.05). The scar was diffused and greater compared to controls, but did not differ from that assessed in CAD. Two years follow up, available in 11/44 SSc, showed further asymptomatic MPRI deterioration in all and diffuse subendocardial LGE in 8/11, without any change in LV, RV volumes and ejection fractions. CONCLUSION: CMR may reveal severe cardiac involvement in early, asymptomatic diffuse SSc with normal routine cardiac evaluation, presenting either as myocardial inflammation or as severe reduction of MPRI and diffuse fibrosis with further deterioration in the long term follow up.