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The Society for Cardiovascular Magnetic Resonance (SCMR) is an international society focused on the research, education, and clinical application of cardiovascular magnetic resonance (CMR). The SCMR web site (https://www.scmr.org) hosts a case series designed to present case reports demonstrating the unique attributes of CMR in the diagnosis or management of cardiovascular disease. Each clinical presentation is followed by a brief discussion of the disease and unique role of CMR in disease diagnosis or management guidance. By nature, some of these are somewhat esoteric, but all are instructive. In this publication, we provide a digital archive of the 2019 Case of the Week series as a means of further enhancing the education of those interested in CMR and as a means of more readily identifying these cases using a PubMed or similar search engine.

Graphical abstract, Highlights • The authors report a novel approach for MV repair. • ULMV is rare in clinical practice. • Comprehensive multimodality cardiac imaging can delineate complex valvular pathology.

Among numerous etiologies of ventricular arrhythmias, arrhythmogenic right ventricular cardiomyopathy (ARVC) and methamphetamine-induced cardiomyopathy (MAC) are 2 of the rarer causes of ventricular arrhythmias.1 ARVC is a disease that is characterized by fibro-fatty replacement of the myocardium.1 Although it was originally described as a right ventricular (RV) disease, left ventricular (LV) and biventricular involvement are also possible in patients with ARVC.1 On the other hand, MAC is a separate clinical entity that is rare and under-recognized.2 We report the first known case of reversible MAC mimicking ARVC with ventricular tachycardia (VT).

60-year-old female with history of 33mm ATS mechanical mitral valve replacement 16 years ago for rheumatic mitral valve disease and permanent Atrial Fibrillation (AF) presented with one-year history of worsening exertional dyspnoea as well as symptom of pre-syncope. She was on warfarin for AF as well as mechanical mitral valve replacement (MVR) with therapeutic anticoagulation in the recent time. Her transthoracic echocardiogram was technically difficult as MVR leaflet motion and aortic valve morphology was not well visualised because of artefact from mechanical mitral valve (Panel 1 A: 4chamber still echo image showing reverberation artefact from mechanical mitral valve leaflets)) and aortic valve calcification. Doppler Echocardiography revealed significant aortic stenosis (AS) and mildly elevated gradients across the MVR with average peak (17 ​mmHg)/mean (6.5 ​mmHg) gradients and Effective Orifice Area (ERO) of 1.9cm2 (normal >2 cm2) at heart rate of 60bpm in AF raising a possibility of only mild mechanical prosthetic mitral valve stenosis.

Graphical abstract, Highlights • LV pseudoaneurysm is a rare but fatal complication of myocardial infarction. • Multimodality imaging is important in the diagnosis. • Surgical repair is the mainstay of treatment. • Conservative management may be necessary in patients with excessive surgical risk.

Graphical abstract, Highlights • LV aneurysm formation and VSR complicated elective PCI. • This rare pathology occurred after occlusion of a

In the intravenous drug user (IVDU) population, infected right-sided valvular lesions are common, and this has been well described in the literature. The Eustachian valve (also known as the valve of the inferior vena cava) is another valve in close proximity to the tricuspid valve, which can, in rare cases, be the focus of infection. Eustachian valve endocarditis may be an under-recognised complication of Staphylococcus bacteraemia in IVDU population, often only identified by transesophageal imaging. We present a case of tricuspid valve endocarditis in an IVDU with secondary seeding on the Eustachian valve, and an accompanying literature review on this rare topic.

A 42-year-old man presented with acute onset substernal chest pain. His only cardiac risk factor was mild, medicated dyslipidaemia and he was a lifelong non-smoker. He had a healthy body mass index and exercised regularly. There was no history of previous thromboembolic events or known haematological disease. On arrival to the emergency department, he was haemodynamically stable. Electrocardiogram initially showed sinus rhythm with biphasic anterior lead T wave inversion that resolved before angiogram (Box 1). Cardiac high sensitivity troponins were positive, peaking at 2479 ng/L (reference range, < 20 ng/L), while the remaining laboratory tests, including white cell count and inflammatory markers, were unremarkable. Telemetry throughout admission did not identify arrhythmia. Coronary angiography to investigate the source of the troponin leak showed normal coronary arteries (Box 2). Subsequent cardiac magnetic resonance imaging scan revealed localised hypokinesia of mid-anteroseptum and apical cap with non-contiguous subendocardial late gadolinium enhancement in mid-septum and distal cap (Box 3), raising the possibility of embolic events. Transthoracic echocardiogram demonstrated regional wall motion abnormalities with mid-anteroseptal and apical hypokinesis, with an early positive agitated saline bubble study and evidence of right-to-left shunt suspicious of patent foramen ovale (PFO). Subsequent transoesophageal echocardiogram confirmed moderate sized PFO with shunt present both at rest and after Valsalva manoeuvre. Ultrasound of the lower limbs showed superficial saphenous vein thromboembolism (unprovoked) but no deep vein thrombosis. Remaining investigations, including a thrombophilia screen, were unremarkable. After discussion with the patient, he was commenced on anticoagulation for 2 months until he underwent successful percutaneous PFO closure, at which point he was transitioned to antiplatelet therapy.

Graphical abstract The investigators present a rare case of myofibrillar cardiomyopathy in an 18-year-old male patient in which echocardiography, cardiac magnetic resonance, and genetic testing played complementary roles. At the top, the parasternal long- and short-axis views of the heart document increased wall thickness and normal systolic function. Significant diastolic dysfunction was present. Cardiac magnetic resonance imaging (bottom) showed delayed enhancement in thickened segments and was not suggestive of cardiac amyloid or hypertrophic cardiomyopathy. Quadriceps muscle biopsy showed histopathology compatible with myofibrillar myopathy. Subsequent genetic testing confirmed a novel desmin gene mutation as the cause., Highlights • Restrictive cardiomyopathies pose a diagnostic challenge. • Echocardiography is the key initial investigation that allows assessment of cardiac morphology and function, but it also provides clues as to etiology. • Cardiac magnetic resonance and echocardiography have complementary roles in the diagnostic workup of cardiomyopathies. • Cardiac imaging in cardiomyopathies has two distinct roles: it allows assessment of cardiac morphology and function, and it provides clues about the etiology of cardiomyopathies.

Introduction A 67-year-old male presented with progressive exertional dyspnoea, night sweats and 25 kg of weight loss over 2 years. He was febrile, 38.0°C, hypotensive and tachycardiac. Pulsus paradoxus was present. His electrocardiogram showed electrical alternans. He had previously had acute myeloid leukaemia treated with three cycles of chemotherapy. Results The patient was found to have a 3.6 cm pericardial effusion with features consistent with tamponade and three cardiac masses (largest 10 × 9 × 5 cm) and mediastinal lymphadenopathy. He had 825 mL of pericardial fluid drained from which cytology was consistent with extramedullary leukaemia. A bone marrow aspirate and trephine was normal. Conclusion The findings suggested extramedullary recurrence of leukaemia in the pericardium, without bone marrow involvement.

A 79-year-old asymptomatic man was referred to our clinic for routine immigration screening. His past medical history was significant only for remote treated pulmonary tuberculosis and heavy tobacco use. Examination revealed oxygen saturations of 90%, blood pressure of 150/90 mmHg, and a prominent right ventricular heave. There was no digital clubbing. Chest X-ray demonstrated an enlarged cardiothoracic ratio with a prominent right heart border and left hilar enlargement (Figure 1A ). His electrocardiograph (ECG) showed atrial fibrillation, right axis deviation and anterior T-wave inversion consistent with right ventricular strain (Figure 1B).

Insulin resistance (IR) and inflammation are associated with an increased risk of cardiovascular disease and may contribute to obesity cardiomyopathy. The earliest sign of obesity cardiomyopathy is impaired left ventricular (LV) diastolic function, which may be evident in obese children and adolescents. However, the precise metabolic basis of the impaired LV diastolic function remains unknown. The aims of this study were to evaluate cardiac structure and LV diastolic function by tissue Doppler imaging in overweight and obese (OW) youth and to assess the relative individual contributions of adiposity, IR, and inflammation to alterations in cardiac structure and function. We studied 35 OW (body mass index standard deviation score 2.0±0.8; non-IR n=19, IR n=16) and 34 non-OW youth (body mass index standard deviation score 0.1±0.7). LV diastolic function was reduced in OW youth compared with non-OW controls, as indicated by lower peak myocardial relaxation velocities (p

A four month old baby experienced itchy reddish raised maculopapular lesions on face, limbs and trunk within 24 hours of immunization with booster dose of pentavalent vaccine. The lesions were not associated with fever, cough, vomiting, or insect bite. There was no similar family history with siblings .There was no history of similar reactions to the previous two doses of the same vaccine. On taking the prescribed medications the lesions subsided in 2-3 days with no recurrence at the time of follow-up.

Although not recommended in isolation, visual estimation of echocardiographic ejection fraction (EF) is widely applied to confirm quantitative EF. However, interobserver variability for EF estimation has been reported to be as high as 14%. The aim of this study was to determine whether self-directed education could improve the accuracy and interobserver variability of visual estimation of EF and whether a multireader estimate improves measurement precision.Thirty-one participants provided single-point EF estimates for 30 echocardiograms with a spectrum of EFs, image quality, and clinical contexts in patients undergoing cardiac magnetic resonance (CMR) within 48 hours. Participants received their own case-by-case variance from CMR EF, and the 10 cases with the largest reader variability were discussed along with corresponding CMR images. Self-directed learning was undertaken by side-by-side review of echocardiographic and CMR images. Two months later, 20 new cases were shown to the same 31 participants, using the same methodology.The baseline interobserver variability of ±0.120 improved to ±0.097 after the intervention. EF misclassification (defined as ±0.05 of CMR EF) was reduced from 56% to 47% (P.001), and the intervention also resulted in a decrease in the absolute difference between CMR and echocardiography for all cases and all readers (from 0.07 ± 0.01 to 0.06 ± 0.01, P = .0001). This improvement was most prominent for the readers with lower baseline accuracy. A combined physician-sonographer EF estimate improved the precision of EF determination by 25% compared with individual reads.In readers with varying levels of experience, a simple, mostly self-directed intervention modestly decreased interobserver variability and improved the accuracy of EF measurements. Combined physician-sonographer EF reporting improved the precision of EF estimates.

Background Although impaired left ventricular (LV) filling in constrictive pericarditis (CP) is attributable to external constraints by a tethered pericardium, impaired left atrial (LA) function can further impair LV filling. Previous studies focused on the impact of a tethered pericardium on LV diastolic behavior, but its impact on LA function has been largely overlooked. The objectives of this study were to evaluate LA mechanics in CP and to assess the impact of pericardiectomy on LA mechanics. Methods A total of 52 patients with CP (mean age, 57 ± 12 years) and 19 control subjects were studied retrospectively. All patients with CP underwent echocardiography before (median, 12 days; interquartile range, 5–34 days) and after pericardiectomy (median, 20 days; interquartile range, 5–64 days). Global LA longitudinal strain (e) was calculated, which included peak negative e (e negative ), peak positive e (e positive ), and the sum of those values, total LA e (e total ), using speckle-tracking echocardiography with Velocity Vector Imaging. The regional difference of LA e between the septal and lateral walls was assessed before and after the procedure. Results Patients with CP showed depressed global LA e negative , LA e total , and LA e positive compared with controls. LA contractile (global LA e negative ) and reservoir functions (global LA e total ) showed significant increases after pericardiectomy. Regional analysis revealed that the improvement in LA function after surgery was more apparent in lateral segments, while the regional function of septal walls was depressed after surgery. Conclusions Patients with CP have impaired LA mechanics, presumably because of the constrictive tethering process involving the left atrium. Speckle-tracking echocardiography showed consistent results of changes in LA mechanics with conventional echocardiographic parameters early after the procedure. Regional e analysis aided in recognition of the impact of constrictive tethering and pericardiectomy on LA function.

A 39-year-old female from the Philippines was referred to our cardiology service following work-up by her local medical practitioner for atypical chest pain. Computed tomography coronary angiogram (CTCA) was negative for coronary artery disease but suspicious for an infiltrative cardiomyopathy, with increased left ventricular wall thickness and myocardial hypoattenuation streaks of −25HU ( Panels A and B ). Transthoracic echocardiography (TTE) revealed an increased basal biventricular wall thickness (RV-free wall 10 mm) with hyper-echogenicity ( Panels C and D ). Cardiovascular magnetic resonance (CMR) steady-state-free precession …

Background Transcatheter valve interventions rely on imaging for patient selection, preprocedural planning, and intraprocedural guidance. Objective We explored the use of prospective electrocardiogram (ECG)–triggered 4-dimensional (4-D) CT imaging in patients evaluated for transcatheter aortic valve replacement (TAVR). Methods A total of 47 consecutive patients underwent 128-slice dual-source CT with wide-window dose-modulated prospective ECG-triggered, axial acquisition of the aortic root, reconstructed during diastolic and systolic cardiac phases. Image quality was evaluated, aortic root and left ventricular (LV) geometry and function were analyzed, and radiation exposure was estimated. Results Image quality was generally good, with 41 of 47 (87%) patients scored as good or excellent. The mean aortic valve area was 0.93 ± 0.24 cm 2 . Mean LV ejection fraction was 56.8% ± 16.4%, and mean LV mass was 130.4 ± 43.8 g. The minor diameter of the annulus was larger in systole (systole, 2.29 ± 0.24 cm; diastole, 2.14 ± 0.25 cm; P = 0.006), but the mean and major diameters did not vary significantly between systole and diastole. The mean estimated effective dose was 5.9 ± 2.4 mSv. Conclusion Multiphase, prospective ECG-triggered axial image acquisition is a lower dose acquisition technique for 4-D aortic root imaging in patients being considered for TAVR.

Current guidelines recommend a multiparametric echocardiographic assessment of aortic regurgitation (AR). However, the absence of a hierarchical weighting of discordant parameters could cause interobserver variability. In the present study, we sought to define and improve the interobserver variability of AR assessment. Seventeen level 3 readers graded 20 randomly selected patients with AR. The readers also provided a usefulness score for each parameter, depending on its influence on their decision of the AR severity grade. A consensus strategy was subsequently formulated and validated against cardiac magnetic resonance imaging in a separate group of 80 patients. The readers were updated with the consensus document and recalibrated using the same cases. Agreement was statistically assessed using Randolph's free-marginal multirater kappa. At baseline, no uniform approach was used to combine the individual parameters, contributing to the interobserver variability (overall kappa 0.5). A consensus strategy to categorize AR severity was developed in which the left ventricular volume took precedence over the other parameters and was used to differentiate chronic severe AR from less severe categories. Recalibration of the readers using this consensus strategy improved concordance (kappa increased to 0.7). The new strategy also improved the accuracy relative to cardiac magnetic resonance imaging, as evidenced by full agreement on severe AR between the consensus document-based grading and AR severity defined by cardiac magnetic resonance imaging in the separate validation group of 80 patients. In conclusion, grading of chronic AR using a multiparametric approach has suboptimal consistency between readers and a left ventricular volume-based consensus document improved concordance and accuracy.

An increasing incidence of constrictive pericarditis (CP) after open heart surgery and the availability of highly effective CP treatments continue to pique investigator interest in CP. Differentiating between CP and restrictive cardiomyopathy (RCM) remains clinically challenging and has been

Objective To optimise an echocardiographic estimation of pulmonary vascular resistance (PVR e ) for diagnosis and follow-up of pulmonary hypertension (PHT). Design Cross-sectional study. Setting Tertiary referral centre. Patients Patients undergoing right heart catheterisation and echocardiography for assessment of suspected PHT. Methods PVR e ([tricuspid regurgitation velocity ×10/(right ventricular outflow tract velocity-time integral+0.16) and invasive PVR i ((mean pulmonary artery systolic pressure-wedge pressure)/cardiac output) were compared in 72 patients. Other echo data included right ventricular systolic pressure (RVSP), estimated right atrial pressure, and E/e9 ratio. Difference between PVR e and PVR i at various levels of PVR was sought using Bland–Altman analysis. Corrected PVR c ((RVSP−E/e9)/RVOT VTI ) (RVOT, RV outflow time; VTI, velocity time integral) was developed in the training group and tested in a separate validation group of 42 patients with established PHT. Results PVR e >2.0 had high sensitivity (93%) and specificity (91%) for recognition of PVR i >2.0, and PVR c provided similar sensitivities and specificities. PVR e and PVR i correlated well (r=0.77, p e underestimated marked elevation of PVR i —a trend avoided by PVR c . PVR c and PVR e were tested against PVR i in a separate validation group (n=42). The mean difference between PVR e and PVR i exceeded that between PVR c and PVR i (2.8±2.7 vs 0.8±3.0 Wood units; p i by at least one SD occurred in 10 patients over 6 months; this was detected in one patient by PVR e and eight patients by PVR c (p=0.002). Conclusion PVR e distinguishes normal from abnormal PVR i but underestimates high PVR i . PVR c identifies the severity of PHT and may be used to assess treatment response.

Background: Obesity is a common clinical problem that can complicate investigation of coronary artery disease (CAD). Knowledge-based iterative model reconstruction (IMR) represents a novel reconstruction technique in coronary computed tomography angiography (CCTA) with reduced radiation and improved image quality. The purpose of this study is to assess the feasibility of IMR-CCTA in patients with a Body Mass Index (BMI) ≥ 30 kg/m2. Methods: We evaluated 407 symptomatic patients between January 2015 and March 2017 undergoing CCTA on a Philips iCT 256 slice scanner with an IMR algorithm. An experienced interpreter evaluated for non-diagnostic segments, severity (>50% stenosis) and plaque morphology in a standard 16-segment model. Diagnostic scans were determined by need for additional functional or anatomical assessment due to non-definite segment severity. Logistic regression was used for data analysis. Results: Diagnostic scans were comparable between both BMI groups (89%/88%). Subgroup analysis (BMI≥30) revealed improved diagnostic ability with lower age 40) but with increased radiation dose. Conclusion: CCTA represents an excellent modality to investigate CAD, and a knowledge-based IMR algorithm demonstrates high diagnostic ability in patients with increased BMI (≥ 30). Feasibility of diagnostic scans is also high in morbidly obese patients (BMI>40) but with increased radiation dose.

Pericardiectomy is an effective intervention for constrictive pericarditis. Speckle-tracking echocardiography can provide quantitative information not only about longitudinal strain (LS) but about longitudinal displacement (LD) and septal-to-lateral rotational displacement (SLRD). The aim of this study was to investigate whether pericardiectomy improves myocardial mechanics using speckle-tracking analysis.Eighty-three patients with constrictive pericarditis who underwent echocardiography were retrospectively assessed (mean age, 58 ± 12 years; 72 men; 50 idiopathic, 20 postoperative, four viral, three radiation, and six others) and compared with 20 healthy volunteers. LD and SLRD were measured from the apical four-chamber view and global LS from three apical views.LD was less in the constrictive pericarditis group compared with control subjects (P.001). Only lateral LS was significantly less than that of control subjects (P.001), but septal LS was similar (P = .48). In pre- and post-pericardial surgery comparisons (n = 27), values of septal and lateral LD were almost identical (mean, 13.6 ± 4.7 vs 13.3 ± 5.4 mm; P = .70) before pericardiectomy, but septal LD decreased (mean, 9.3 ± 3.5 mm; P.001) and lateral LD increased (mean, 16.8 ± 4.7 mm; P = .0106) after the surgery, even though the difference in LS between the septal and lateral walls decreased (from 5.6 ± 5.3% to 2.5 ± 4.2%, P = .008). Systolic whole-heart swinging motion significantly increased to a counterclockwise direction after surgery (mean SLRD, -0.8 ± 3.3° vs 2.1 ± 3.0°; P = .001). Although the change in SLRD after pericardiectomy was not different between patients with decreases and increases in New York Heart Association class, SLRD change was significantly greater in patients who received fewer diuretics after surgery (mean, 4.00 ± 0.91 vs 0.27 ± 1.47; P = .027).After surgical removal of the pericardium, LD of the septal and lateral walls became significantly different, and counterclockwise SLRD increased, reflecting loss of pericardial support.

Polycomb Repressive Complexes 1 and 2 (PRC1 and 2) play a critical role in the epigenetic regulation of transcription during cellular differentiation, stem cell pluripotency, and neoplastic progression. Here we show that the Polycomb Group protein EED, a core component of PRC2, physically interacts with and functions as part of PRC1. Components of PRC1 and PRC2 compete for EED binding. EED functions to recruit PRC1 to H3K27me3 loci and enhances PRC1 mediated H2A ubiquitin E3 ligase activity. Taken together, we suggest an integral role for EED as an epigenetic exchange factor coordinating the activities of PRC1 and 2.

A 25-year-old man with no significant medical history was found to have a continuous murmur along the left sternal border on routine physical examination performed in preparation for running a marathon. Echocardiography showed a dilated left ventricle with normal function and continuous high-velocity flow in an aberrant vessel in close vicinity to the abdominal aorta (Movie I in the online-only Data Supplement). Chest x-ray (posterior-anterior and lateral projections, Figure 1) disclosed conglomeration of tubular densities in a scimitar pattern in the right lower lobe suggestive of anomalous pulmonary venous return of right lower lobe (scimitar syndrome; anomalous drainage of right lung pulmonary veins into the inferior vena cava). Cardiac magnetic resonance imaging (MRI) not only demonstrated normal drainage of all 4 pulmonary veins into the left atrium but also revealed an aberrant vessel (Movie II in the online-only Data Supplement) extending between the abdominal aorta and the right inferior pulmonary vein with an elevated systemic-to-pulmonary flow ratio of 1.8, consistent with a large left-to-left shunt. Cardiac computed tomography (CT) confirmed the presence of a large fistulous connection between the abdominal aorta and the right inferior …

Background— The aim of our study was to compare myocardial mechanics of constrictive pericarditis (CP) with restrictive cardiomyopathy (RCM), or healthy controls; to assess the impact of pericardial thickening detected by cardiac magnetic resonance on regional myocardial mechanics in CP; and to quantitate the effect of pericardiectomy on myocardial mechanics in CP. Methods and Results— Myocardial mechanics were evaluated by 2-dimensional speckle tracking in 52 consecutive patients with CP who underwent cardiac magnetic resonance examination before pericardiectomy, 35 patients with RCM, and 26 control subjects. CP patients had selectively depressed left ventricular (LV) anterolateral wall strain (LWS) and right ventricular (RV) free wall longitudinal systolic strain (FWS) but preserved LV septal wall systolic strain (SWS). In a comparison of RCM and normals, CP patients had significantly lower regional longitudinal systolic strain ratios (CP versus RCM and normal; LVLWS/LVSWS: 0.8±0.2 versus 1.1±0.2 and 1.0±0.2; PP =0.011). There was a significant inverse correlation between pericardial thickness and respective ventricular strains ( P =0.001). Pericardiectomy resulted in the improvement of the depressed LVLWS/LVSWS (0.83±0.18–0.95±0.12; P Conclusions— Regional longitudinal systolic strain ratios are robust novel diagnostic tools for CP. Regional myocardial mechanics inversely correlates with adjacent pericardial segment thickness detected by cardiac magnetic resonance, and pericardiectomy leads to systolic strain improvement, which is more pronounced in right ventricular and LV free walls.