Evidence Of Late Gadolinium Enhancement Research Articles

Worse outcomes in patients with cardiac sarcoidosis and involvement of the right ventricle in cardiac magnetic resonance

Abstract Background Cardiac sarcoidosis (CS) can manifest with variety of unspecific symptoms. It can mimic all types of cardiomyopathies but most often it resembles dilated cardiomyopathy. Involvement of right ventricle (RV) is rare and may lead to a false diagnosis of arrhythmogenic right ventricular cardiomyopathy and delay of the immunosuppressive therapy. Purpose The aim of this research was to study the characteristics and the outcomes of this peculiar subtype of patients. Methods We analyzed 112 patients diagnosed with CS after elaborate work-up with cardiac magnetic resonance (CMR), FDG PET and endomyocardial biopsy (EMB). All patients underwent follow-up for occurrence of combined endpoint of death, LV assist device implantation or heart transplantation. Patients with evidence of late gadolinium enhancement (LGE) involving RV (RV group) were identified and compared to the rest (DCM group) for baseline clinical characteristics, arhythmic outcomes and combined endpoint. Results Involvement of the RV in CMR was observed in 19 (17%) patients. Patients in the RV group were more frequently male: 84% vs. 57%, P = 0.032. EMB confirmed CS in 63% vs. 19.4% in the DCM group; P= 0.0001. LV ejection fraction in the RV group was comparable to the DCM group: 42±14% vs. 45±14%; P = 0.3. Furthermore, in the RV group the rates of sustained ventricular tachycardia were significantly higher than the DCM: 68% vs. 34%; P = 0.009. Long-term immunosuppressive therapy was started in 74% in RV and in 70% of the DCM group; P=0.77. Despite this, during follow-up, the rate of death, LVAD implantation or heart transplantation was significantly higher in the RV than in the DCM group; 26.3% vs. 7.5%; P = 0.03. In binary logistic analysis, the OR for the combined endpoint was 4.4; 95% CI 1.2-15.7. Conclusions Patients with CS and RV involvement in CMR have worse outcomes as compared to patients presenting as DCM, despite the comparable LV EF and immunosuppressive therapy.

Abstract 17652: Association Between Ventricular Arrhythmias and Myocardial Fibrosis in Patients With Chagas Disease Without Known Cardiomyopathy

Introduction: Chagas disease (CD) is an important cause of cardiomyopathy, arrhythmias and sudden death. The presence of myocardial fibrosis detected by late gadolinium enhancement (LGE) on cardiac magnetic resonance imaging (CMR) in these patients has been correlated with an increased prevalence of ventricular arrhythmias. The incidence of ventricular arrhythmias in patients with CD without established cardiomyopathy has not been well described. Hypothesis: We aim to evaluate if there is an association between LGE on CMR and incidence of ventricular arrhythmias in patients with CD but without cardiomyopathy. Methods: Patients treated at a Chagas disease center of excellence were retrospectively analyzed for ambulatory rhythm monitoring (Holter and/or 14-day patch monitor) and CMR data. Significant ventricular arrhythmias were defined as either a PVC burden >/=1% or non-sustained ventricular tachycardia (NSVT) ≥4 beats. A Fisher exact test was used to compare categorical variables. Results: There were 191 patients treated between 12/2014 and 8/2022 available for analysis. Of these, 46 patients with CD and no prior history of cardiomyopathy received both ambulatory rhythm monitoring and CMR. There were 16 male and 30 female patients with average age of 52.2 ± 11.5 years, and LV ejection fraction of 58.8% ± 14.2%. Of the 46 patients, 15 (32.6%) were found to have LGE on cMRI while 31 (67.4%) had no evidence of LGE. Within the patients with LGE, 8/15 (53.3%) were found to have significant ventricular arrhythmias. Of the patients without LGE, only 7/31 (22.6%) were determined to have significant ventricular arrhythmias (p = 0.038). Conclusions: These data suggest that for patients with CD without cardiomyopathy, the presence of LGE on CMR is independently associated with incident ventricular arrhythmias detected on ambulatory rhythm monitoring. Whether these findings indicate increased risk of sudden cardiac death requires further study.

Clinical characteristics and one-year all-cause mortality outcomes in Africans with dilated cardiomyopathy

AimsDilated cardiomyopathy (DCM) is a common cause of heart failure in sub-Saharan Africa (SSA). The affected individuals present with new-onset heart failure with reduced ejection fraction and no identifiable primary or secondary aetiology. We aim to describe the clinical characteristics of participants with heart failure of unknown origin. MethodsWe screened 161 participants with heart failure of unknown origin and prospectively excluded primary and secondary causes of DCM. All study participants were subjected to laboratory biochemical testing, echocardiography, cardiovascular magnetic resonance (CMR) imaging and invasive coronary angiography. ResultsThe study comprised 93 participants with a mean age of 47.5 SD 13.1 years. Forty-six (56.1%) participants had evidence of late gadolinium enhancement (LGE) on imaging, and LGE was visualised in the mid wall in 28 (61.0%) of these participants. After a median duration of 13.4 months [interquartile range (IQR): 8.8–28.9 months], 18 (19%) participants died. Non-survivors had a higher median left atrial volume index (44.9 mL/m2 (IQR: 34.4–58.7) compared to survivors [32.9 mL/m2 (IQR: 24.5–47.0), p = 0.017)]. The rate of all-cause rehospitalisation was 29.3%, of which 17 of the 22 re-hospitalisations were heart failure related. ConclusionDilated cardiomyopathy in Africans primarily affects young males. In our cohort, this disease was associated with an all-cause mortality of 19% in one year. In SSA, large multicenter studies are required to investigate this disease's pathogenesis and outcomes.

The pivotal role of ECG in cardiomyopathies.

Cardiomyopathies are a heterogeneous group of pathologies characterized by structural and functional alterations of the heart. Recent technological advances in cardiovascular imaging offer an opportunity for deep phenotypic and etiological definition. Electrocardiogram (ECG) is the first-line diagnostic tool in the evaluation of both asymptomatic and symptomatic individuals. Some electrocardiographic signs are pathognomonic or fall within validated diagnostic criteria of individual cardiomyopathy such as the inverted T waves in right precordial leads (V1-V3) or beyond in individuals with complete pubertal development in the absence of complete right bundle branch block for the diagnosis of arrhythmogenic cardiomyopathy of the right ventricle (ARVC) or the presence of low voltages typically seen in more than 60% of patients with amyloidosis. Most other electrocardiographic findings such as the presence of depolarization changes including QRS fragmentation, the presence of epsilon wave, the presence of reduced or increased voltages as well as alterations in the repolarization phase including the negative T waves in the lateral leads, or the profound inversion of the T waves or downsloping of the ST tract are more non-specific signs which can however raise the clinical suspicion of cardiomyopathy in order to initiate a diagnostic procedure especially using imaging techniques for diagnostic confirmation. Such electrocardiographic alterations not only have a counterpart in imaging investigations such as evidence of late gadolinium enhancement on magnetic resonance imaging, but may also have an important prognostic value once a definite diagnosis has been made. In addition, the presence of electrical stimulus conduction disturbances or advanced atrioventricular blocks that can be seen especially in conditions such as cardiac amyloidosis or sarcoidosis, or the presence of left bundle branch block or posterior fascicular block in dilated or arrhythmogenic left ventricular cardiomyopathies are recognized as a possible expression of advanced pathology. Similarly, the presence of ventricular arrhythmias with typical patterns such as non-sustained or sustained ventricular tachycardia of LBBB morphology in ARVC or non-sustained or sustained ventricular tachycardia with an RBBB morphology (excluding the "fascicular pattern") in arrhythmogenic left ventricle cardiomyopathy could have a significant impact on the course of each disease. It is therefore clear that a learned and careful interpretation of ECG features can raise suspicion of the presence of a cardiomyopathy, identify diagnostic "red flags" useful for orienting the diagnosis toward specific forms, and provide useful tools for risk stratification. The purpose of this review is to emphasize the important role of the ECG in the diagnostic workup, describing the main ECG findings of different cardiomyopathies.

Cardiovascular Magnetic Resonance Imaging Findings in Africans with Idiopathic Dilated Cardiomyopathy

In sub-Saharan Africa, idiopathic dilated cardiomyopathy (IDCM) is a common yet poorly investigated cause of heart failure. Cardiovascular magnetic resonance (CMR) imaging is the gold standard for tissue characterisation and volumetric quantification. In this paper, we present CMR findings obtained from a cohort of patients with IDCM in Southern Africa suspected of having a genetic cause of cardiomyopathy. A total of 78 IDCM study participants were referred for CMR imaging. The participants had a median left ventricular ejection fraction of 24% [interquartile range, (IQR): 18-34]. Late gadolinium enhancement (LGE) was visualised in 43 (55.1%) participants and localised in the midwall in 28 (65.0%) participants. At the time of enrolment into the study, non-survivors had a higher median left ventricular end diastolic wall mass index of 89.4 g/m2 (IQR: 74.5-100.6) vs. 73.6 g/m2 (IQR: 51.9-84.7), p = 0.025 and a higher median right ventricular end-systolic volume index of 86 mL/m2 (IQR:74-105) vs. 41 mL/m2 (IQR: 30-71), p < 0.001. After one year, 14 participants (17.9%) died. The hazard ratio for the risk of death in patients with evidence of LGE from CMR imaging was 0.435 (95% CI: 0.259-0.731; p = 0.002). Midwall enhancement was the most common pattern, visualised in 65% of participants. Prospective, adequately powered, and multi-centre studies across sub-Saharan Africa are required to determine the prognostic significance of CMR imaging parameters such as late gadolinium enhancement, extracellular volume fraction, and strain patterns in an African IDCM cohort.

Impact of wideband cardiac magnetic resonance on diagnosis, decision-making and outcomes in patients with implantable cardioverter defibrillators.

Although myocardial scar assessment using late gadolinium enhancement (LGE) cardiac magnetic resonance (CMR) imaging is frequently indicated for patients with implantable cardioverter defibrillators (ICDs), metal artefact can degrade image quality. With the new wideband technique designed to mitigate device related artefact, CMR is increasingly used in this population. However, the common clinical indications for CMR referral and impact on clinical decision-making and prognosis are not well defined. Our study was designed to address these knowledge gaps. One hundred seventy-nine consecutive patients with an ICD (age 59 ± 13 years, 75% male) underwent CMR using cine and wideband pulse sequences for LGE imaging. Electronic medical records were reviewed to determine the reason for CMR referral, whether there was a change in clinical decision-making, and occurrence of major adverse cardiac events (MACEs). Referral indication was the most common evaluation of ventricular tachycardia (VT) substrate (n = 114, 64%), followed by cardiomyopathy (n = 53, 30%). Overall, CMR resulted in a new or changed diagnosis in 64 (36%) patients and impacted clinical management in 51 (28%). The effect on management change was highest in patients presenting with VT. A total of 77 patients (43%) experienced MACE during the follow-up period (median 1.7 years), including 65 in patients with evidence of LGE. Kaplan-Meier analysis showed that ICD patients with LGE had worse outcomes than those without LGE (P = 0.006). The clinical yield from LGE CMR is high and provides management changing and meaningful prognostic information in a significant proportion of patients with ICDs.

COVID-19 Vaccine-Related Myocardial and Pericardial Inflammation.

To review myocarditis and pericarditis developing after COVID-19 vaccinations and identify the management strategies. COVID-19 mRNA vaccines are safe and effective. Systemic side effects of the vaccines are usually mild and transient. The incidence of acute myocarditis/pericarditis following COVID-19 vaccination is extremely low and ranges 2-20 per 100,000. The absolute number of myocarditis events is 1-10 per million after COVID-19 vaccination as compared to 40 per million after a COVID-19 infection. Higher rates are reported for pericarditis and myocarditis in COVID-19 infection as compared to COVID-19 vaccines. COVID-19 vaccine-related inflammatory heart conditions are transient and self-limiting in most cases. Patients present with chest pain, shortness of breath, and fever. Most patients have elevated cardiac enzymes and diffuse ST-segment elevation on electrocardiogram. Presence of myocardial edema on T2 mapping and evidence of late gadolinium enhancement on cardiac magnetic resonance imaging are also helpful additional findings. Patients were treated with non-steroidal anti-inflammatory drugs and colchicine with corticosteroids reserved for refractory cases. At least 3-6months of exercise abstinence is recommended in athletes diagnosed with vaccine-related myocarditis. COVID-19 vaccination is recommended in all age groups for the overall benefits of preventing hospitalizations and severe COVID-19 infection sequela.

Cardiac Magnetic Resonance Imaging-Based Screening for Cardiac Sarcoidosis in Patients With Atrioventricular Block Requiring Temporary Pacing.

BackgroundSome myocardial diseases, such as cardiac sarcoidosis, predispose to complete atrioventricular block. The European Society of Cardiology Guidelines on cardiac pacing in 2021 recommend myocardial disease screening in patients with conduction disorder requiring pacemaker with multimodality imaging, including cardiac magnetic resonance (CMR) imaging. The ability of CMR imaging to detect myocardial disease in patients with a temporary pacing wire is not well documented.Methods and ResultsOur myocardial disease screening protocol is based on using an active fixation pacing lead connected to a reusable extracorporeal pacing generator (temporary permanent pacemaker) as a bridge to a permanent pacemaker. From 2011 to 2019, we identified 17 patients from our CMR database who underwent CMR imaging with a temporary permanent pacemaker for atrioventricular block. We analyzed their clinical presentations, CMR data, and pacemaker therapy. All CMRs were performed without adverse events. Pacing leads induced minor artifacts to the septal myocardial segments. The extent of late gadolinium enhancement in CMR imaging was used to screen patients for the presence of myocardial disease. Patients with evidence of late gadolinium enhancement underwent endomyocardial biopsy. If considered clinically indicated, also 18‐F‐fluorodeoxyglucose positron emission tomography and extracardiac tissue biopsy were performed if sarcoidosis was suspected. Eventually, 8 of 17 patients (47.1%) were diagnosed with histologically confirmed granulomatous inflammatory cardiac disease. Importantly, only 1 had a previously diagnosed extracardiac sarcoidosis at the time of presentation with high‐degree atrioventricular block.ConclusionsCMR imaging with temporary permanent pacemaker protocol is an effective and safe early screening tool for myocardial disease in patients presenting with atrioventricular block requiring immediate, continuous pacing for bradycardia.

Abstract 13478: Cardiac Magnetic Resonance Imaging Identifies Cardiac Disease in Limb-Girdle Muscular Dystrophy in Previously Low Risk Mutations

Introduction: Limb-Girdle Muscular Dystrophies (LGMD) are an inheritable group of genetic disorders primarily involving progressive weakening and atrophy of the pelvic or shoulder girdle musculature with potential cardiac dysfunction. Prior reports stratified frequency of cardiac disease based on genetic mutations. The general incidence of cardiac muscle involvement in LGMD is variable depending on genetic factors and carry important indications for clinical management. Cardiac magnetic resonance imaging (CMR) is increasingly used in dystrophinopathies and determining management. There is limited CMR data in adult LGMD patients and fewer reports in younger patients. Hypothesis: Therefore, we sought to determine the extent of cardiac involvement based on genetic risk of cardiomyopathy. Methods: This is a retrospective review of LGMD patients followed at our institution who underwent CMR assessment. Patients stratified into Group A (high incidence of cardiomyopathy; LGMDs 1B, 2E, 2F and 2I) and Group B (rare or no reported cardiomyopathy; LGMDs 1A, 1C, 2A, 2B, 2C, 2D, 2G, 2H, 2J) with CMR studies were assessed for presence of cardiac scar defined as late gadolinium enhancement (LGE) and LVEF for each subject. Results: 41 subjects were identified with average age of 21.3±11.5 years (range 6-50) with normal LVEF 58±12.1% and 12/41 (29.3%) with LGE with the youngest patient age 12.4 years. Group A included 14/41 (34%) and 27/41 (66%) were in Group B. Group A had higher incidence of LGE 6/14(42.9%) with preserved LVEF of 58.2±11% compared to Group B with 6/27 (22.2%) with no difference in LVEF of 55.7±16.4% (p = 0.5). When stratified by LGE status, the LGE-positive group was older at 26.4±11.9 years and had preserved LVEF of 61.9±11.1% versus LGE-negative group of 19.1±10.9 years (p = 0.03) with abnormal LVEF of 49.4±9.5% (p =0.0008). Conclusions: This is the largest study of CMR in LGMD patients, confirming presence of cardiac disease by LGE in the high risk group. Surprisingly, nearly 25% of the low risk mutations have evidence of LGE. Abnormal LGE predicted abnormalities in LVEF in both Group A and Group B. Management of LGMD patients should include CMR even in low risk population. Ongoing data collection may reveal disease cluster in previous low risk groups.

Persistence of Late Gadolinium Enhancement on Follow-Up CMR Imaging in Children with Acute Myocarditis.

Cardiac magnetic resonance (CMR) imaging is the gold standard non-invasive test for the diagnosis of acute myocarditis. Late gadolinium enhancement (LGE) on CMR imaging signifies myocardial fibrosis or scar but information on long-termfollow-up in pediatric patients is limited. The aim of this study is to evaluate the persistence of LGE in follow-up CMR imaging and highlight the need for serially following LGE after acute myocarditis in children. A retrospective review of the CMR images in pediatric patients less than 18years of age at Children's Memorial Hermann Hospital was performed from October 31, 2010, to December 31, 2017. The ages of the patients included in the study ranged from 10 to 17years. Out of 34 subjects, 12 subjects had a follow-up CMR imaging after the initial CMR imaging. Cardiac enzymes and inflammatory markers were elevated in all patients on initial presentation with normalization seen for all patients on follow-up testing. Baseline CMR imaging demonstrated depressed function in 50% of patients (6/12) with recovery of biventricular function seen in all except for one patient. LGE was detected in 10 out of 12 patients on baseline CMR and persisted in all on follow--up CMR imaging. Two patients did not have LGE at baseline CMR and had no evidence of LGE at follow-up CMR. Despite resolution of acute myocardial inflammation, LGE persisted in all of our study subjects. There is neither an agreement on time interval to perform follow-up CMR, nor on duration of follow-up. We recommend large-scale prospective CMR study for long-term follow-up evaluation of children after acute myocarditis.

Duchenne and Becker muscular dystrophy carriers: Evidence of cardiomyopathy by exercise and cardiac MRI testing

BackgroundVaried detection methods have resulted in conflicting reports on the prevalence of cardiac disease in Duchenne and Becker muscular dystrophy carriers (MDC). MethodsWe performed a prospective cohort study of 77 genetically-confirmed MDC mothers, 22 non-carrier mothers, and 25 controls. All participants underwent Cardiopulmonary Exercise Testing (CPET) and Cardiac Magnetic Resonance imaging (CMR). Results25% of carriers had ventricular ectopy in recovery of exercise (RecVE) as compared to 1 non-carrier and no controls (p = .003). No difference in age or maximal oxygen consumption was noted. 11 carriers had abnormal (<55%) left ventricular ejection fraction by CMR. Evidence of late gadolinium enhancement (LGE) was noted in 48% of MDC, 1 non-carrier patient and no control subjects (p < .0001). Subset analysis of LGE+ and LGE- subjects revealed differences in age (44.1 v 38.6 yrs.; p = .005), presence of RecVE, (38.9% v 10.5%, p = .004), and high serum creatine kinase (CK) (> 289 U/l; 52.8% v 31.6%, p = .065). ConclusionWe describe the prevalence of disease using CPET and CMR in genetically-proven MDC. 49% of carriers had fibrosis, opposed to 5% of non-carriers, highlighting the importance of genetic testing in this population. Despite cardiomyopathy, functional assessment by treadmill was normal, illustrating the discrepancy in cardiac and skeletal muscle impacts. Age, RecVE and serum CK appear to have an important role in predicting cardiomyopathy. Serum CK levels suggest that a systemic higher global disease severity and not tissue heterogeneity may be the etiology for greater cardiac disease and relatively spared skeletal muscle disease in this population.Clinical Trial Registrationhttps://clinicaltrials.gov/ct2/show/NCT02972580?term=mendell&cond=Duchenne+Muscular+Dystrophy&rank=5; ClinicalTrials.gov Identifier: NCT02972580

P89 Role of inflammation as a risk factor for atrial fibrillation in rheumatic mitral stenosis and its correlation with atrial late gadolinium enhancement (LGE) on cardiac MRI

Abstract Funding Acknowledgements ICMR Aims Association of atrial fibrillation (AF) is a common cause of morbidity and mortality in patients with rheumatic heart disease (RHD). Association of Atrial Fibrillation with inflammation is well delineated in nonvalvular AF as compared to Rheumatic AF. This study evaluated the presence of atrial inflammation, in patients with AF and rheumatic mitral stenosis (MS), using cardiac MRI and its correlation with inflammatory markers. Methods and Results Twenty RHD patients with predominant mitral stenosis were recruited for the study. Ten patients had persistent AF, three had paroxysmal AF and seven were in sinus rhythm. Patients with AF had lower mitral valve areas. Cardiac MRI showed evidence of late gadolinium enhancement (LGE) in the atrial wall in 61.54% (8/13) of patients with AF as compared with only 20% (1/5; p &amp;lt; 0.05) of patients in sinus rhythm. Mean levels of IL-6 were also significantly higher in patients with AF (10.62 ± 5.92 pg/mL versus 4.37 ± 2.68 pg/mL; P= 0.017). IL6 with a cutoff of ≥ 6.5 pg/mL was associated with a sensitivity of 76.92 % and specificity of 71.43 % for prediction of AF. There was also significant association between high IL-6 levels and LGE on MRI (13.13 ± 5.75 pg/mL vs 5.29 ± 3.23pg/mL; P &amp;lt; 0.05) Conclusion Patients with MS who developed AF show evidence of atrial inflammatory sequelae in the form of fibrosis as suggested by LGE on cardiac MRI. Systemic inflammation in the form of elevated IL-6 levels correlated significantly with atrial fibrosis and AF. Table 1: MS patients with or without AF Clinical Features Rhythm P-Value NSR (n = 7) AF (n = 13) Mean Valve Area (cm2) 1.28 ± 0.35 0.83 ± 0.26 &amp;lt; 0.01 Mean Diastolic Gradient (mmHg) 6.29 ± 2.2 11.92 ± 5.65 &amp;lt;0.01 Wilkin Score 7 ± 0.816 8.46 ± 1.66 0.04 LVEF % (non indexed) 59.57 ± 1.13 58.77 ± 2.89 0.49 LA Volume with MRI (ml) 127.50 ± 51.32 284.33 ± 133.12 &amp;lt;0.01 Inflammatory Markers hs-CRP (mg/L) 5.59 ± 3.89 3.94 ± 3.47 0.34 IL6 (pg/mL) 4.37 ± 2.68 10.62 ± 5.92 0.017 sCD-40L (ng/mL) 3.72 ± 3.73 4.77 ± 2.80 0.48 Atrial LGE 0 (0%) 8 (61.5%) 0.04 Abstract P89 Figure. Image 1: Cardiac MRI showing LGE

P1707 Strain imaging - resolving the diagnostic conundrum of cardiac amyloidosis

Abstract Introduction Cardiac amyloidosis is a common cardiac condition and is still significantly underdiagnosed. Autopsy studies revealed that myocardial tranthyretin amyloid deposition is found in up to 30% of patients with HFpEF (heart failure with preserved ejection fraction). A study in 2015 reported that 13% of patients hospitalised due to HFpEF (older than 60 years old and with left ventricular hypertrophy - LVH) had moderate to severe uptake on the Technetium DPD scintigraphy. Strain imaging through Echocardiography can be a useful diagnostic tool and can provide valuable clues towards the aetiology of LVH. Relative "apical sparing" pattern of longitudinal strain has been reported to have good sensitivity and specificity in differentiating patients with cardiac amyloidosis from controls. Case A 66 year old man presented with progressive breathlessness and peripheral oedema. His past medical history included peripheral neuropathy, bilateral decompression procedures for carpal tunnel syndrome and IgM Monoclonal Gammopathy of Undetermined Significance (MGUS). His B-type natriuretic peptide levels were elevated and his ECG showed normal sinus rhythm, RsR" pattern on the anterior leads and no evidence of LVH. The transthoracic echocardiogram showed moderate concentric LVH, normal left ventricular systolic function and mildly impaired left ventricular diastolic function. No evidence of pericardial effusion. Urine protein:creatinine ratio findings were consistent with nephrotic range proteinuria. He had a cardiac MRI that confirmed normal biventricular wall motion and systolic function and a moderate increase in the wall thickness of both ventricles, but showed no evidence of late gadolinium enhancement. At this point, we repeated his transthoracic echocardiogram with the use of strain imaging and it revealed a pattern of apical sparing suggestive of cardiac amyloidosis. After collaboration with the Haematology team, a bone marrow biopsy was performed that showed that the MGUS had progressed to IgM multiple myeloma. There was however no evidence of amyloid deposits. He was subsequently referred to the national amyloidosis centre (as per family request), where a SAP (serum amyloid P) scan showed renal and splenic amyloid deposits confirming Light-chain Amyloidosis. To exclude the possibility of TTR Amyloidosis, he also had a 99mTc-DPD scintigraphy which did not detect any amyloid deposits. He was treated under the care of the Haematology team with Velcade/Cyclophosphamide/Dexamethasone chemotherapy. Conclusion In patients with a clinical suspicion of cardiac amyloidosis, Echocardiography with strain imaging can be very useful and should be performed routinely. Furthermore, in patients with high clinical suspicion of cardiac amyloidosis, diagnostic investigations should persist even if the initial workup does not yield specific findings. Abstract P1707 Figure. Strain imaging findings

P5278Myocardial abnormalities in people living with Human Immunodeficiency Virus (PLWH) detected using cardiovascular magnetic resonance (CMR)

Abstract Background Antiretroviral therapy (ART) has dramatically improved the prognosis in PLWH with survival nearing that of HIV negative people. PLWH may develop significant comorbidities as they age including cardiovascular disease (CVD) such as myocardial infarction, sudden cardiac death, heart failure, as well as subclinical evidence of myocardial inflammation. Overall, ART treated patients are at an increased CVD risk with some studies quoting a 2.2 fold relative risk. Aim To assess the incidence of functional, structural and tissue characterisation changes in PLWH using CMR with multiparametric mapping Methods 39 PLWH (34 men, mean age 55.8±10.9, mean duration of HIV 17.8±9.29 years) and 29 healthy volunteers (20 men, mean age 45.1±8.3) underwent CMR with late gadolinium enhancement (LGE) imaging, T1 and T2 mapping. Results Of PLWH, only 7 scans (18%) were normal. LV ejection fraction was significantly lower and LV mass significantly higher compared to controls. Native T1, a marker of diffuse fibrosis or increased myocardial water content was no different between the groups. T2, a more specific marker of myocardial oedema, was elevated in PLWH. Sixteen PLWH (41%) had evidence of LGE including 8 with an ischaemic pattern (7 sub-endocardial and one transmural) and 8 with a non-ischaemic pattern (5 with mid-wall enhancement and 3 with RV insertion point LGE. No controls had evidence of LGE. Student t-Test: PLWH versus controls PLWH (n=39) Control (n=29) P-value LVEF (%) 59±15 67±5 &lt;0.01 iLVM 79±24 56±13 &lt;0.01 Native T1 (ms) 1021±52 1001±41 0.116 T2 (ms) 48±2 46±2 &lt;0.05 LGE 16 (41%) 0 (0%) &lt;0.001 LVEF (%) = Left Ventricular Ejection Fraction; iLVM = Indexed Left Ventricular Mass (g/m2); LGE = Late Gadolinium Enhancement. Conclusion This study identifies a number of cardiac changes associated with HIV and prolonged treatment with ART. The elevated LV mass may be associated with hypertension, commonly found in PLWH. The elevated myocardial T2 compared to controls may be due to chronic inflammation associated to prolonged HIV exposure. There was no evidence of diffuse fibrosis but focal areas of non-ischaemic scar were a common finding which may relate to previous myocarditis or HIV-related cardiomyopathy. A fifth of PLWH also had evidence of previous myocardial infarction. We propose to image asymptomatic PLWH to further classify, diagnose and treat a vulnerable group of patients who are now described as having a normal life expectancy.

Cardiovascular Magnetic Resonance Imaging Evidence of Edema in Chronic Chagasic Cardiomyopathy

The persistence of inflammatory processes in the myocardium in varying degrees of chronic Chagas heart disease has been poorly investigated. We hypothesized that edema could occur in patients with chronic chagasic cardiomyopathy and corresponds to the persistence of inflammatory processes in the myocardium. Eighty-two Chagas disease (CD) seropositive patients (64.6% females; age = 58.9 ± 9.9) without ischemic heart disease or conditions that cause myocardial fibrosis and dilation were considered. Late gadolinium enhancement (LGE) and T2-weighted magnetic resonance imaging of edema were obtained and represented using a 17-segment model. Patients were divided into three clinical groups according to the left ventricular (LV) ejection fraction (EF) as G1 (EF > 60%; n=37), G2 (35% > EF < 60%; n=33), and G3 (EF < 35%; n=12). Comparisons were performed by the Fisher or ANOVA tests. Bonferroni post hoc, Spearman correlation, and multiple correspondence analyses were also performed. Edema was observed in 8 (9.8%) patients; 2 (5.4%) of G1, 4 (12.1%) of G2, and 2 (16.7%) of G3. It was observed at the basal inferolateral segment in 7 (87.5%) cases. LGE was observed in 48 (58.5%) patients; 16 (43.2%) of G1, 21 (63.6%) of G2, and 11 (91.7%) of G3 (p < 0.05). It was observed in the basal inferior/inferolateral/anterolateral segments in 35 (72.9%) patients and in the apical anterior/inferior/lateral and apex segments in 21 (43.7%), with midwall (85.4%; n=41), subendocardial (56.3%; n=27), subepicardial (54.2%; n=26), transmural (31.2%; n=15), and RV (1.2%; n=1) distribution. Subendocardial lesions were observed only in patients with LVEF < 35%. There was no involvement of the mid-inferolateral/anterolateral segments with an LVEF > 35% (p < 0.05). Deteriorations of the LV and RV systolic functions were positively correlated (rs=0.69; p < 0.05) without evidence of LGE in the RV. Edema can be found in patients with chagasic cardiomyopathy in the chronic stage. In later stages of cardiac dilation with low LVEF, the LGE pattern involves subendocardium and mid locations. Deteriorations of RV and LV are positively correlated without evidence of fibrosis in the RV.

Agreement between gadolinium-enhanced cardiac magnetic resonance and electro-anatomical maps in patients with non-ischaemic dilated cardiomyopathy and ventricular arrhythmias.

We sought to investigate the overlap between late gadolinium enhancement (LGE) in cardiac magnetic resonance (CMR) and electro-anatomical maps (EAM) of patients with non-ischaemic dilated cardiomyopathy (NIDCM) and how it relates with the outcomes after catheter ablation of ventricular arrhythmias (VA). We identified 50 patients with NIDCM who received CMR and ablation for VA. Late gadolinium enhancement was detected in 16 (32%) patients, mostly in those presenting with sustained ventricular tachycardia (VT): 15 patients. Low-voltage areas (<1.5 mV) were observed in 23 (46%) cases; in 7 (14%) cases without evidence of LGE. Using a threshold of 1.5 mV, a good and partially good agreement between the bipolar EAM and LGE-CMR was observed in only 4 (8%) and 9 (18%) patients, respectively. With further adjustments of EAM to match the LGE, we defined new cut-off limits of median 1.5 and 5 mV for bipolar and unipolar maps, respectively. Most VT exits (12 out of 16 patients) were found in areas with LGE. VT exits were found in segments without LGE in two patients with VT recurrence as well as in two patients without recurrence, P = 0.77. In patients with VT recurrence, the LGE volume was significantly larger than in those without recurrence: 12% ± 5.8% vs. 6.9% ± 3.4%; P = 0.049. In NIDCM, the agreement between LGE and bipolar EAM was fairly poor but can be improved with adjustment of the thresholds for EAM according to the amount of LGE. The outcomes were related to the volume of LGE.

The Role of Cardiac Magnetic Resonance Imaging in Severe Anorexia Nervosa.

ObjectiveAnorexia nervosa (AN) patients are at an increased risk of developing cardiac complications including bradyarrhythmias, systolic dysfunction, pericardial effusions, and sudden cardiac death. Although previous echocardiographic studies in AN patients have demonstrated a reduction in overall left ventricular (LV) mass, systolic dysfunction, and silent pericardial effusions, little is known about the role of cardiac magnetic resonance imaging (CMR) in assessing this patient population. The objective of this study was to assess cardiac indices and the presence of myocardial fibrosis in AN patients.MethodsBetween 2014 and 2015, a cross-sectional pilot study of 16 female patients who met the Diagnostic and Statistics Manual of Mental Disorders, fifth edition (DSM-5) criteria for AN was conducted at a single tertiary care center. Baseline characteristics including age, weight, food restriction behavior, over-exercise, self-induced vomiting, and laxative abuse were collected in the study population. Electrocardiography, transthoracic echocardiography (TTE), and CMR were performed.ResultsThe mean age was 17 years (range: 13-22 years). There were no conduction abnormalities as the average PR interval was 152 ms (range: 130-190 ms) and QTc was 413 ms (range: 360-450 ms). Using TTE, the left ventricular ejection fraction (LVEF) was 54 ± 4% with a lower LV mass/body surface area (BSA) of 56 ± 7g/m2 in AN patients as compared to controls. Using CMR, both the mean LVEF of 52 ± 9% and LV mass/BSA of 45 ± 4g/m2 were lower in AN patients as compared to controls. Using CMR, both right ventricular ejection fraction (RVEF) of 50 ± 10% and a right ventricular (RV) mass/BSA of 18 ± 3g/m2 were smaller in AN patients as compared to controls. There was no evidence of late gadolinium enhancement (LGE) in the study population.ConclusionsYoung patients with AN have lower cardiac mass and volumes with no evidence of myocardial fibrosis.

Late Gadolinium Enhancement in Patients With Hypertrophic Cardiomyopathy and Preserved Systolic Function

BackgroundA high proportion of patients with hypertrophic cardiomyopathy (HCM) have evidence of late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR). ObjectivesThis study sought to assess the incremental prognostic utility of LGE in patients with HCM. MethodsWe studied 1,423 consecutive low-/intermediate-risk patients with HCM (age ≥18 years) with preserved left ventricular (LV) ejection fraction (mean age 66 ± 14 years, 60% men) who underwent transthoracic echocardiography (TTE) (including dimensions and LV outflow tract gradients) and CMR (including LGE as a % of LV mass) at our center between January 2008 and December 2015. The primary composite endpoint was sudden cardiac death (SCD) and appropriate implantable cardioverter-defibrillator discharge. The percent 5-year SCD risk score was calculated. ResultsThe mean 5-year SCD risk score was 2.3 ± 2.0. Mean maximal LV outflow tract gradient (TTE) was 70 ± 55 mm Hg (median 74 mm Hg [interquartile range (IQR): 10 to 67 mm Hg]); indexed LV mass and LGE (both on CMR) were 91 ± 10 g/m2 and 8.4 ± 12% (IQR: 0% to 19%); 50% had LGE on CMR. Of these, 458 were nonobstructive and 965 were obstructive (of which 686 were underwent myectomy). At 4.7 ± 2.0 years of follow-up, 60 (4%) met the composite endpoint. On quadratic spline analysis, LGE ≥15% was associated with increased risk of composite events. In the obstructive subgroup, on competing risk regression analysis, ≥15% LGE (subhazard ratio: 3.04 [95% confidence interval: 1.48 to 6.10]) was associated with a higher rate and myectomy (subhazard ratio: 0.44 [95% confidence interval: 0.20 to 0.76]) was associated with a lower rate of composite endpoints (both p < 0.01). Similarly, sequential addition of LGE ≥15% and myectomy to % 5-year SCD risk score improved the log likelihood ratios from −227.85 to −219.14 (chi-square 17) and to −215.14 (chi-square 8; both p < 0.01). Association of %LGE with composite events was similar even in myectomy and nonobstructive subgroups. ConclusionsIn low-/intermediate-risk adult patients with HCM (obstructive, myectomy, and nonobstructive subgroups) with preserved systolic function, %LGE was significantly associated with a higher rate of composite endpoint, providing incremental prognostic utility.

Ischaemic Colitis Associated with Adrenergic Acute Cardiomyopathy: A discovery mode of pheochromocytoma

Pheochromocytoma is usually diagnosed in patients with resistant high blood pressure or the classical triad of symptoms including palpitations, sweats and headache. To our knowledge, we describe here the first case of concomitant bifocal complications of pheochromocytoma with a concomitant ischaemic colitis and Takotsubo-like cardiomyopathy. A 65-year-old man with a history of thyroid surgery 20 years ago, dyslipidaemia and smoking was admitted for syncope. Troponine Ic level rose to 3.72 ng/ mL and transthoracic echocardiography revealed severe left ventricular (LV) systolic dysfunction with large akinesia involving midventricular segments. Coronary angiogram was normal and cardiac magnetic resonance (CMR) confirmed the decreased LV ejection fraction (20%) with no evidence of late-gadolinium enhancement (Figure 1), compatible with a median form of Takotsubo. Surprisingly, on the second day of hospitalisation, he presented diarrhoea, abdominal pain and fever (40 8C). An abdominal CT scan suspected sigmoid colitis and revealed a voluminous left adrenal mass. Rectosigmoidoscopy confirmed sigmoid bleeding colitis and all bacteriologic samples remained sterile. Evolution was marked with resolution of digestive symptoms in four days with fasting and symptomatic treatment and recovery of LV systolic function in five days. Given this adrenal mass, pheochromocytoma screening was undertaken and plasmatic catecholamine and chromogranine A showed abnormally high levels. Once adequate aand b-adrenergic receptor blockages were obtained, the patient underwent resection of this left adrenal mass (30 day). Anatomical pathology (histology and immunology) confirmed the suspected diagnosis of pheochromocytoma.

Increased myocardial native T1 and extracellular volume in patients with Duchenne muscular dystrophy.

BackgroundDuchenne muscular dystrophy (DMD) cardiomyopathy is a progressive disease for which there is no cure. Disease-specific therapies are needed that can be initiated before irreversible myocardial damage ensues. In order to evaluate therapeutic efficacy, surrogate endpoints other than ejection fraction must be found. The hypothesis of this study is that T1 and extracellular volume fraction (ECV) mapping using cardiovascular magnetic resonance (CMR) can detect diffuse extracellular matrix expansion in DMD patients with normal left ventricular ejection fraction (LVEF) and without myocardial late gadolinium enhancement (LGE).MethodsThirty-one DMD and 11 healthy control participants were prospectively enrolled. CMR using a modified Look-Locker (MOLLI) sequence was performed in all participants before and after contrast administration. T1 and ECV maps of the mid left ventricular myocardium were generated and regions of interest were contoured using the standard 6-segment AHA model. Global and segmental values were compared between DMD and controls using a Wilcoxon rank-sum test.ResultsThe DMD participants had significantly higher mean native T1 compared with controls (1045 ms vs 988 ms, p = 0.001). DMD participants with normal LVEF and without evidence of LGE also demonstrated elevated mean native T1 (1039 ms vs 988 ms, p = 0.002, and 1038 ms vs 988 ms, p = 0.011). DMD participants had a significantly greater mean ECV than controls (0.31 vs 0.24, p < 0.001), even in the settings of normal LVEF (0.28 vs 0.24, p < 0.001) and negative LGE (0.29 vs 0.24, p = 0.001).ConclusionsDMD participants have elevated LV myocardial native T1 and ECV, even in the setting of normal LVEF and in the absence of LGE. T1 and ECV mapping in DMD have potential to serve as surrogate cardiomyopathy outcome measures for clinical trials.Electronic supplementary materialThe online version of this article (doi:10.1186/s12968-016-0224-7) contains supplementary material, which is available to authorized users.