Patchy Late Gadolinium Enhancement Research Articles

Abstract 18997: Management of Immune Checkpoint Inhibitor Mediated Myocarditis

Clinical Presentation: A 52-year-old female with metastatic Stage IVB (T4N3M1c) lung adenocarcinoma presented with worsening dyspnea. She had just completed her 3 rd round of carboplatin/pemetrexed/pembrolizumab/MK-5890 chemotherapy. Upon examination she was noted to be hypertensive and tachycardic with an elevated high-sensitivity troponin T. ECG showed sinus tachycardia, left axis deviation, and flattened T waves in lead V5 and V6. In the ED, she subsequently developed supraventricular tachycardia and was admitted to the ICU. TTE demonstrated moderately reduced EF of 30-35%. TTE also identified hypokinesis in the basal to mid anterior and lateral walls and apical akinesis. Initial CMR showed an EF of 31% with akinesis of the mid and apical anterior, septal and inferior wall. Given the clinical and imaging findings, she was diagnosed with myopericarditis secondary to pembrolizumab and started on 1 mg/kg methylprednisolone taper for 60 days. Repeat CMR after completion of steroid taper showed evidence of weighted T2 edema in myocardium and pericardium. Late gadolinium enhancement (LGE) in inferior and lateral epicardium and adjacent pericardium as well as patchy LGE throughout the myocardium with significant EF improvement (57%) (Figure 1A-B). In our patient, multi-modality imaging was used to confirm the diagnosis and monitor treatment response. Discussion: Immune checkpoint inhibitors (ICI) are a novel class of immunotherapies used in certain malignancies. Initial treatment of myocarditis consists of cessation of further ICI and administration of corticosteroids. Additionally, symptomatic myocarditis treatment should be started. Reinitiating ICI therapy should ultimately be done with shared decision making.

Septal Midwall Late Gadolinium Enhancement in Ischemic Cardiomyopathy and Nonischemic Dilated Cardiomyopathy—Characteristics and Prognosis

Septal midwall late gadolinium enhancement (LGE) is a characteristic finding on cardiac magnetic resonance imaging (CMR) in nonischemic dilated cardiomyopathy (DCM) and is associated with adverse events. Its significance in ischemic cardiomyopathy (ICM) is unknown. With this multicenter observational study, we aimed to study the characteristics of septal midwall LGE and evaluate its prognostic value in ICM. A total of 1,084 patients with an impaired left ventricular (LV) ejection fraction (<50%) on LGE-CMR, either because of ICM (53%) or DCM, were included retrospectively. Septal midwall LGE was defined as midmyocardial stripe-like or patchy LGE in septal segments and was present in 10% of patients with ICM compared with 34% of patients with DCM (p<0.001). It was significantly associated with larger LV volumes and lower LV ejection fraction, irrespective of etiology. The primary endpoint was all-cause mortality and secondary endpoint was ventricular arrhythmias (VAs), including resuscitated cardiac arrest, sustained VA, and appropriate implantable cardioverter-defibrillator (ICD) therapy. During a median follow-up of 2.7years, we found a significant association between septal midwall LGE and mortality in patients with DCM (hazard ratio [HR] 1.92, p=0.03), but not in patients with ICM (HR 1.35, p=0.39). Risk of VAs was significantly higher in patients with septal midwall LGE on CMR, both in DCM (HR 2.80, p<0.01) and in ICM (HR 2.70, p<0.01). In conclusion, septal midwall LGE, typically seen in DCM, was also present in 10% of patients with ICM and was associated with increased LV dilation and worse function, irrespective of etiology. When present, septal midwall LGE was associated with adverse outcome.

Use of Cardiac Magnetic Resonance Imaging to Distinguish Between Acute Myocarditis and Takotsubo Cardiomyopathy

Use of Cardiac Magnetic Resonance Imaging to Distinguish Between Acute Myocarditis and Takotsubo Cardiomyopathy

Abstract 14106: Prevalence and Respective Predictive Value of Late Gadolinium Enhancement Phenotypes in Patients Referred for Suspected Infiltrative Cardiomyopathy

Introduction: The respective value of different late gadolinium enhancement (LGE) phenotypes in patients with suspected infiltrative cardiomyopathy remains uncertain. Methods: In a large cohort of patients referred for suspected infiltrative cardiomyopathy, we evaluated respective associations between the dominant LGE phenotype and the composite endpoint of heart failure hospitalization, survived cardiac arrest or death. Kaplan-Meier survival curves were executed for six pre-defined LGE phenotypes. Multivariable analysis was performed to assess their independent association with the composite outcome, adjusted for relevant confounders. Final diagnosis was adjudicated using standardized disease-specific criteria. Results: 588 patients (68% male, median age 70 years, mean LVEF 54.7±12.7%) were studied. Of these patients, 139 (24%) were confirmed to have cardiac amyloidosis (CA) (81 [58%] transthyretin, 54 [39%] light chain and 4 [3%] undifferentiated) by objective non-CMR diagnostic criteria. Any LGE was present in 394 patients (67%) with the dominant phenotype being diffuse in 137 (23%), mid-wall patchy in 118 (20%), ischemic in 55 (9.4%), subepicardial in 18 (3.1%), mid-wall striae in 8 (1.4%), and RV insertion site in 58 (9.9%). The sensitivity and specificity of diffuse LGE for CA was 86% and 96%, respectively. Over a median 855 (IQR 372, 1542) days, 239 (41%) experienced the primary outcome. Unadjusted survival curves for each LGE phenotype are shown in the Figure. Following adjustment, diffuse (HR 2.30, p&lt;0.001), mid-wall patchy (HR 1.52, p=0.040) and ischemic LGE (HR 1.78, p=0.023) remained independently associated (Figure). Conclusions: In patients referred for suspected infiltrative cardiomyopathy, diffuse, mid-wall patchy and ischemic patterns all showed independent prognostic value. We confirm that diffuse LGE pattern reliably identifies CA patients with high sensitivity and specificity while delivering strong prognostic value.

Abstract 13713: Myocarditis and Coronary Vasospasm in a Patient With Antiphospholipid Syndrome and MINOCA

Background : The prevalence of MINOCA in patients with antiphospholipid syndrome (APLS) is estimated to be 20%. Frequent causes in this population include coronary vasospasm, microcirculatory dysfunction and takotsubo cardiomyopathy. Objective : This case reveals myocarditis and coronary vasospasm as newly described complications of APLS associated MINOCA and illustrates the use of CMR to differentiate ischemic injury from myocarditis in this population. Case : A 40-year-old female presented with two-days of left-sided, substernal, pleuritic, and radiating chest pain associated with dyspnea. Admission ECG showed 0.5mm ST elevations in the inferior leads with ST depression in aVL. She had an initial high sensitivity troponin of 1531 ng/L, NT-proBNP of 18,357 pg/mL, baseline creatinine of 1.53 mg/dL, platelets of 95,000/μL, hemoglobin of 8.4 g/dL, and a subtherapeutic INR of 1.1 on warfarin. An emergent catheterization showed no coronary artery disease or evidence of SCAD. TTE showed hypokinesis of inferior and lateral walls with an LVEF of 45% and CTA chest ruled out pulmonary embolism. The patient’s admission was complicated by rising troponins to a peak of 9790 ng/L, oliguric renal failure requiring continuous renal replacement therapy, and fever. Patient was medically managed for presumed myocardial infarction and repeat TTE two days after catheterization showed a recovered LVEF of 59%. CMR showed a transmural infarct of the basal and inferior walls with signs of microvascular obstruction and diffuse patchy late gadolinium enhancement of the LV midwall, consistent with myocarditis. An infectious workup for myocarditis was negative. Patient was discharged home on warfarin, aspirin, and amlodipine. Conclusion : MINOCA is more common in patients with APLS, although coronary vasospasm and myocarditis have not been described in the same patient. CMR is useful to differentiate the etiology of ischemic injury following successful diagnosis of MINOCA.

Cardiovascular Magnetic Resonance Imaging Patterns in Rare Cardiovascular Diseases

Rare cardiovascular diseases (RCDs) have low incidence but major clinical impact. RCDs' classification includes Class I-systemic circulation, Class II-pulmonary circulation, Class III-cardiomyopathies, Class IV-congenital cardiovascular diseases (CVD), Class V-cardiac tumors and CVD in malignancy, Class VI-cardiac arrhythmogenic disorders, Class VII-CVD in pregnancy, Class VIII-unclassified rare CVD. Cardiovascular Magnetic Resonance (CMR) is useful in the diagnosis/management of RCDs, as it performs angiography, function, perfusion, and tissue characterization in the same examination. Edema expressed as a high signal in STIRT2 or increased T2 mapping is common in acute/active inflammatory states. Diffuse subendocardial fibrosis, expressed as diffuse late gadolinium enhancement (LGE), is characteristic of microvascular disease as in systemic sclerosis, small vessel vasculitis, cardiac amyloidosis, and metabolic disorders. Replacement fibrosis, expressed as LGE, in the inferolateral wall of the left ventricle (LV) is typical of neuromuscular disorders. Patchy LGE with concurrent edema is typical of myocarditis, irrespective of the cause. Cardiac hypertrophy is characteristic in hypertrophic cardiomyopathy (HCM), cardiac amyloidosis (CA) and Anderson-Fabry Disease (AFD), but LGE is located in the IVS, subendocardium and lateral wall in HCM, CA and AFD, respectively. Native T1 mapping is increased in HCM and CA and reduced in AFD. Magnetic resonance angiography provides information on aortopathies, such as Marfan, Turner syndrome and Takayasu vasculitis. LGE in the right ventricle is the typical finding of ARVC, but it may involve LV, leading to the diagnosis of arrhythmogenic cardiomyopathy. Tissue changes in RCDs may be detected only through parametric imaging indices.

Improving 18F-FDG PET Imaging Using Canola Oil For Evaluation Of Cardiac Sarcoidosis

Objective The incidence and prevalence of cardiac sarcoidosis (CS) is increasing significantly, likely due to improved noninvasive diagnostic methods such as 18F-FDG PET imaging and a higher index of suspicion among clinicians. Cardiac PET for diagnosis of CS requires a strict low carbohydrate diet which even if adhered to can still lead to false positives. One protocol that has been explored with mixed results is consuming a beverage high in fat to improve myocardial suppression. Our objective was to evaluate if a new diet preparation protocol using canola oil can improve 18F-FDG PET imaging. Methods In patients undergoing evaluation for CS with 18F-FDG PET and inadequate myocardial suppression on a prior study, we administered 1 cup of canola oil (224 grams of fat) 1 hour before repeating the study in addition to our standard diet. The repeat study was compared to the prior to determine if there was improved myocardial suppression. Canola oil was specifically chosen as we considered it to be most palatable to drink in a large quantity in one sitting. Results Our study protocol showed that all four patients had an improvement in myocardial suppression (Figure 1). Patient A is a 60-year old man with CS who underwent a PET-CT 3 months after initiating treatment which showed increased myocardial FDG uptake. The repeat scan with the canola oil protocol demonstrated less FDG myocardial uptake with an SUV max of 5.9 (previously 11.3). Similarly, Patient B is a 63-year old man with CS who initiated treatment and had a PET scan at 3 months showing increased FDG uptake with the repeat scan using our protocol showing decreased myocardial FDG uptake with an SUV of 3.1 (previously 12.8). Patient C is a 50-year old man with pulmonary sarcoidosis whose initial PET scan showed diffuse FDG uptake (SUV 6.4) and repeat with the protocol showed minimal FDG uptake (SUV 3.0). Finally, Patient D is a 54-year old man with frequent PVCs and MRI showing patchy late gadolinium enhancement whose initial PET_CT showed patchy FDG uptake (SUV 7.5) and repeat scan with the protocol showed no evidence of sarcoidosis (SUV 1.3). Overall the only side effect was steatorrhea for up to one day after canola oil consumption. Conclusion In patients undergoing evaluation for CS with 18F-FDG PET who have inadequate myocardial suppression, canola oil may improve imaging characteristics and decrease false positives. The incidence and prevalence of cardiac sarcoidosis (CS) is increasing significantly, likely due to improved noninvasive diagnostic methods such as 18F-FDG PET imaging and a higher index of suspicion among clinicians. Cardiac PET for diagnosis of CS requires a strict low carbohydrate diet which even if adhered to can still lead to false positives. One protocol that has been explored with mixed results is consuming a beverage high in fat to improve myocardial suppression. Our objective was to evaluate if a new diet preparation protocol using canola oil can improve 18F-FDG PET imaging. In patients undergoing evaluation for CS with 18F-FDG PET and inadequate myocardial suppression on a prior study, we administered 1 cup of canola oil (224 grams of fat) 1 hour before repeating the study in addition to our standard diet. The repeat study was compared to the prior to determine if there was improved myocardial suppression. Canola oil was specifically chosen as we considered it to be most palatable to drink in a large quantity in one sitting. Our study protocol showed that all four patients had an improvement in myocardial suppression (Figure 1). Patient A is a 60-year old man with CS who underwent a PET-CT 3 months after initiating treatment which showed increased myocardial FDG uptake. The repeat scan with the canola oil protocol demonstrated less FDG myocardial uptake with an SUV max of 5.9 (previously 11.3). Similarly, Patient B is a 63-year old man with CS who initiated treatment and had a PET scan at 3 months showing increased FDG uptake with the repeat scan using our protocol showing decreased myocardial FDG uptake with an SUV of 3.1 (previously 12.8). Patient C is a 50-year old man with pulmonary sarcoidosis whose initial PET scan showed diffuse FDG uptake (SUV 6.4) and repeat with the protocol showed minimal FDG uptake (SUV 3.0). Finally, Patient D is a 54-year old man with frequent PVCs and MRI showing patchy late gadolinium enhancement whose initial PET_CT showed patchy FDG uptake (SUV 7.5) and repeat scan with the protocol showed no evidence of sarcoidosis (SUV 1.3). Overall the only side effect was steatorrhea for up to one day after canola oil consumption. In patients undergoing evaluation for CS with 18F-FDG PET who have inadequate myocardial suppression, canola oil may improve imaging characteristics and decrease false positives.

Late-gadolinium enhancement properties associated with atrial fibrillation rotors in patients with persistent atrial fibrillation.

A computational model demonstrated that atrial fibrillation (AF) rotors could be distributed in patchy late-gadolinium enhancement (LGE) areas and play an important role in AF drivers. However, this was not validated in humans. The purpose of this study was to evaluate the LGE properties of AF rotors in patients with persistent AF. A total of 287 segments in 15 patients with persistent AF (long-standing persistent AF in 9 patients) that underwent AF ablation were assessed. Non-passively activated areas (NPAs), where rotational activation (AF rotor) was frequently observed, were detected by the novel real-time phase mapping (ExTRa Mapping). The properties of the LGE areas were assessed using the LGE heterogeneity and the density which was evaluated by the entropy (LGE-entropy) and the volume ratio of the enhancement voxel (LGE-volume ratio), respectively. NPAs were found in 61 (21%) of 287 segments and were mostly found around the pulmonary vein antrum. A receiver operating characteristic curve analysis yielded an optimal cutoff value of 5.7% and 10% for the LGE-entropy and LGE-volume ratio, respectively. The incidence of NPAs was significantly higher at segments with an LGE-entropy of >5.7 and LGE-volume ratio of >10% than at the other segments (38 [30%] of 126 vs. 23 [14%] of 161 segments; p = .001). No NPAs were found at segments with an LGE-volume ratio of >50% regardless of the LGE-entropy. Of five patients with AF recurrence, NPAs outside the PV antrum were not ablated in three patients and the remaining NPAs were ablated, but their LGE-entropy and LGE-volume ratio were low. AF rotors are mostly distributed in relatively weak and much more heterogenous LGE areas.

Role of T1 mapping in identification of convalescent Takotsubo cardiomyopathy

Abstract Funding Acknowledgements Type of funding sources: None. Background Takotsubo cardiomyopathy remains an elusive entity to diagnose, especially with increasing time following the initial clinical event. Patients are often referred for cardiac MR, but when performed on a convalescent patient, the conventional CMR exam can be normal. However CMR elevated native T1 values on mapping may persist, aiding diagnosis. Purpose We sought to review a series of confirmed Takotsubo cases to evaluate which CMR features were most helpful in making a diagnosis. Methods We reviewed 2 years of CMR exams in our institution and identified 14 patients with a confirmed diagnosis of Takotsubo cardiomyopathy after a read of all clinical data by two consultant cardiologists. All of the 14 patients had a troponin positive cardiac event, unobstructed coronary arteries on invasive angiography and echocardiographic evidence of apical hypo/akinesia (or basal, in the non-apical variants) at the time of presentation. All CMR exams were performed at 1.5T. All included functional assessment, T1 mapping and late gadolinium enhancement(LGE). Some exams also included T2 mapping and STIR. Results All patients were female. The time interval between presentation and CMR exam varied from 24 hours to 2 years. 2 of the scans showed evidence of non-apical Takotsubo cardiomyopathy. At the time of CMR, only 7 patients(50%) had a residual regional wall motion abnormality, but 13 patients(93%) had a region of elevated native T1 (&amp;gt;1 myocardial segment). High signal was identified on STIR in 6(43%) and elevated native T2 in 10(71%). Of interest, we identified small areas of patchy LGE in 2 patients(14%). Conclusion Increased native T1 signal may be the only residual cardiac MRI marker of previous Takotsubo cardiomyopathy, persisting for months after the initial cardiac event, even after resolution of regional wall motion abnormalities and recovery of the left ventricular function. T1 mapping should be performed in CMR exams for Takotsubo cardiomyopathy to improve diagnostic yield. Abstract Figure. CMR in convalescent Takotsubo

EP11 A case of COVID-19 induced myocarditis in a healthcare-worker

EP11 A case of COVID-19 induced myocarditis in a healthcare-worker

P1113Patchy localisation of late gadolinium enhancement associated with ventricular arrhythmia in dilated cardiomyopathy

Abstract Funding Acknowledgements None Introduction Myocardial fibrosis detected using late gadolinium enhancement(LGE) on cardiac magnetic resonance(CMR) imaging holds prognostic value in dilated cardiomyopathy(DCM). Recent reports have demonstrated the localisation of LGE to be promising predictors of ventricular arrhythmic (VA).Aim: To determine the localisation of LGE associated with high risk of VA in DCM patients. Methods: Retrospective review of consecutive DCM patients(n = 85) implanted with an implantable cardioverter defibrillator(ICD) at a single tertiary centre between 2011-2018. All patients with insufficient follow-up data, cardiac channelopathies, primary valvular pathology and congenital heart disease were excluded from analysis(n = 11). Details of VA occurrence were obtained from medical and pacing notes. VA was defined as VA causing haemodynamic compromise or appropriate device therapy (anti-tachycardia pacing/shock). Localisation of LGE was defined as midwall, patchy, subepicardial or transmural. Left ventricular ejection fraction(LVEF) &amp;lt;35% was defined as severely impaired function. Results:74 DCM patients implanted with an ICD were identified for analysis; LGE was observed in 18(60%) VA and 29(66%) non-VA patients(p = 0.6). There was no observed difference in mean age for patients with and without LGE (68 ± 10 vs. 65 ± 10 years,p = 0.07). A significant difference was seen between localisation and VA (p = 0.04), with patchy LGE demonstrating a higher arrhythmic risk(p = 0.005). There was no association between LVEF and LGE(p = 0.2) however, a significant difference was seen in LVEF and arrhythmic risk, with a more severely impaired LV function seen in patients without VA(p = 0.01). Conclusion:This study has demonstrated a patchy LGE localisation to be strongly associated with ventricular arrhythmia in DCM. Whilst this is a valuable tool in risk stratification, a prospective study with a larger population is required to confirm the validity of this finding. Moreover, an additional method will need to be considered to identify high risk patients without LGE. Ventricular Arrhythmia (n = 30) No Ventricular Arrhythmia (n = 44) P Value Male(%) 20(67%) 24(55%) p = 0.29 Age(Mean ± SD) 65 ± 12 65 ± 10 p = 0.36 LGE Midwall 10(56%) 24(83%) p = 0.04 Subepicardial 1(5.5%) 2(7%) p = 0.85 Transmural 1(5.5%) 2(7%) p = 0.85 Patchy 6(33%) 1(3%) p = 0.005 LVEF &amp;lt;35% 23(77%) 42(95%) p = 0.01

Correlation between septal midwall late gadolinium enhancement on CMR and conduction delay on ECG in patients with nonischemic dilated cardiomyopathy

BackgroundSeptal midwall late gadolinium enhancement (LGE) on cardiac magnetic resonance imaging (CMR) is a characteristic finding in nonischemic dilated cardiomyopathy (DCM) and is associated with adverse cardiac events. QRS-prolongation in DCM is also frequently present and a predictor of arrhythmic events and mortality. Since the His-Purkinje fibres are located in the interventricular septum, QRS-prolongation may directly result from septal fibrosis, visualized by LGE. Our aim was to study the correlation of the presence and extent of septal midwall LGE and QRS-duration. MethodsDCM-patients with left ventricular (LV) dysfunction (LVEF < 50%) were included. LV volumes, systolic function and nonischemic septal midwall LGE, defined as patchy or stripe-like LGE in the septal segments, were quantified. QRS-duration on standard 12-lead ECG was measured. Results165 DCM-patients were included (62% male, mean age 59 ± 15 years) with a median LVEF of 36% [24–44]. Fifty-one patients (31%) demonstrated septal midwall LGE with a median extent of 8.1 gram [4.3–16.8]. Patients with midwall LGE had increased LV end-diastolic volumes (EDV) 248 mL [193–301] vs. 193 mL [160–239], p < 0.001) and lower LVEF (26% [18–35] vs. 40% [32–45], p < 0.001). Median QRS-duration was 110 ms [95–146] without a correlation to the presence nor extent of midwall LGE. QRS-duration was moderately correlated with LV-dilation and mass (respectively r = 0.35, p < 0.001 and r = 0.30, p < 0.001). ConclusionIn DCM-patients, QRS-prolongation and septal midwall LGE are frequently present and often co-exist. However, they are not correlated. This suggests that the assessment of LGE-CMR has complementary value to ECG evaluation in the clinical assessment and risk stratification of DCM-patients.

Work-up of an inflammatory cardiomyopathy

Abstract A 72-year-old woman was admitted for acute heart failure. The echocardiography revealed moderate depression of the left ventricular ejection fraction. Coronary disease was excluded by coronarography. Cardiac magnetic resonance showed predominantly left ventricular septal hypertrophy and severe depression of the left ventricular systolic function. There was also a bright, multifocal and patchy late gadolinium enhancement with subendocardial, mesocardial and subepicardial involvement, suggestive of sarcoidosis. Biochemical study, thoracic computed tomography and positron emission tomography were inconclusive for extra-cardiac sarcoidosis. Therefore, an endomyocardial biopsy was performed. The procedure was complicated by the development of complete atrioventricular block, requiring implantation of a cardiac resynchronization pacing device. A few days after device implantation, the patient developed fever. The echocardiography revealed extensive vegetations, and thus the diagnosis of a device-associated infective endocarditis was made. Even though antibiotic therapy was promptly started, the patient ended up dying. Biopsy results revealed lymphocytic myocarditis. This case is paradigmatic because it shows how the etiologic diagnosis of dilated cardiomyopathy can be challenging. Non-invasive diagnostic exams may not provide a definite diagnosis, requiring an endomyocardial biopsy. However, the benefits versus risks of such procedure must always be carefully weighted.

P726 Cardiac amyloidosis: unmasking the simulator

Abstract Introduction A non-invasive diagnosis of cardiac amyloidosis is a challenge, especially in cases of atypical phenotypic presentation. Differential diagnosis includes hypertrophic cardiomyopathy (HCM), hypertensive cardiomyopathy, and other infiltrative disorders. Multimodality imaging is essential to make a final diagnosis. Case We present the case of a 65 years old woman, with a personal history of resistant arterial hypertension and mild hypertensive cardiomyopathy. She was diagnosed 6 years earlier with multiple myeloma, treated with chemotherapy and allogeneic hematopoietic stem cell transplant, presenting with several relapses and in a stable situation at the moment of our first consult. The patient was referred for heart failure in context of acquired community pneumonia one month earlier. Transthoracic echocardiography showed severe asymmetric left ventricle (LV) hypertrophy (Image A), systolic anterior motion of the mitral valve and diastolic dysfunction suggestive of HCM, not present in the previous examination. Strain imaging of the LV showed a typical amyloid infiltration pattern, with lower longitudinal strain values in the base compared to the apical segments (Image D). Further characterization of the myocardial tissue established the diagnosis along with performing a cardiac magnetic nuclear imaging (MRI). It showed the presence of inferior septum severe LV hypertrophy along with extensive patchy late gadolinium enhancement (LGE) of the lateral wall involving the endocardium (Image C), with normal LV contractility. There was no pleural effusion, but a small pericardial effusion was seen (Image B). With the suspicion of infiltrative heart disease, probably amyloid with an atypical LGE pattern, an oral mucosal biopsy was performed confirming amyloid diagnosis (Images E1,E2). Heart failure treatment was continued, but clinical evolution was poor with the deceasement of the patient 3 years after diagnosis. Discussion This case represents an example of the variety of imaging patterns we can see in cardiac amyloidosis. Despite the classical pattern of cardiac amyloidosis, with concentric LV hypertrophy, up to 8% of cases may present with asymmetrical LV hypertrophy, mimicking HCM. LGE extension and pattern can shows this variability as well: global transmural or subendocardial LGE is the most common, but focal patterns (up to 6% of cases) are described. The complexity of the diagnosis in these cases require a clinical and multimodality image approach. Abstract P726 Figure. Images of the case

Intensity and Distribution of Patchy Late Gadolinium Enhancement in Left Atrium in Patients With Atrial Fibrillation.

Late gadolinium enhancement (LGE) cardiac magnetic resonance imaging (MRI) studies were performed on healthy individuals to establish signal intensity thresholds for reproducible left atrial (LA) patchy LGE detection. Using established criteria, differences in LA patchy LGE between healthy volunteers (HV) and patients with atrial fibrillation (AF) or hypertension were analyzed. Fifty-three patents with AF (mean age 56 years, 60% men), 25 patients with hypertension and no history of AF (mean age 54 years, 40% men), and 28 HV (mean age 50 years, 52% men) were enrolled in an observational, non-interventional, case-control prospective study. LA patchy LGE quantification was performed using LGE MRI (1.5 T scanner, voxel size 1.25x1.25x2.5 mm) and the custom-built software based on estimation of LA voxel image intensity ratio and comparison with threshold value obtained from HV data. Based on analysis of healthy individuals' data, the optimal threshold value for the left atrial patchy LGE quantification was determined at 1.38. Patients with AF had a higher extent of LA patchy LGE (9.1 [1.72; 18.58] %) than patients with hypertension (3.81 [0.57; 9.51] %) and HV (0.78 [0.05; 3.5] %). The predominant location of LA patchy LGE in AF was in the pulmonary vein ostia region, in hypertension - LA posterior wall, and in HV - lower part of LA posterior wall. In AF patients, the extent of LA patchy LGE correlated with LA end-diastolic volume (r=0.37) and LA ejection fraction (r=-0.4), in HV - with age (r=0.66) and LA end-diastolic volume (r=0.4). AF and hypertension are associated with higher extent and different location of LA patchy LGE compared to changes caused by natural aging. The extent of LA patchy enhancement correlates with LA dilatation.

4251Results from the Hungarian Cardiac Magnetic Resonance Registry of Structural Heart Disease and Aborted Sudden Cardiac Death in Athletes

Abstract Introduction Sudden cardiac death (SCD) is the most common cause of death in athletes occurring usually during intensive training. Cardiac magnetic resonance (CMR) has a crucial role in the detection of structural myocardial abnormalities. Aims Our aim was to investigate the etiology of SCD and to estimate the prevalence of myocardial structural heart diseases among Hungarian athletes using CMR. Methods Between January 2011 and January 2019 we performed CMR scans on 228 athletes (199 males, age: 29.1±13.2) with suspected structural myocardial disease. Twelve athletes were investigated after aborted sudden cardiac death and normal coronary angiography. Results CMR confirmed the diagnosis of structural myocardial disease in 62 athletes (26.2%) (28.8±9.1 years, 59 male): hypertrophic cardiomyopathy (HCM) in 14 cases (22.6%), arrhythmogenic right ventricular cardiomyopathy (ARVC) in 9 cases (14.5%), noncompaction (NCCMP) in 6 cases (9.7%) and dilated cardiomyopathy (DCM) in 5 cases (8.1%). Subendocardial late gadolinium enhancement (LGE), reflecting myocardial scar, was typical of previous myocardial infarction (post MI) in 3 cases (5.5%). Acute myocarditis was found in 2 cases (3.6%). Nonischaemic LGE pattern was found in 20 cases (32.2%): patchy subepi-midmyocardial LGE suggesting previous myocarditis in 8 athletes, and with aspecific pattern in 12 athletes. Athletes with nonischaemic LGE had normal clinical and laboratory parameters without wall motion abnormalities, in their cases further investigations ruled out systemic disease. One athlete was diagnosed with Fabry-disease, one with coronary artery abnormality (anomalous origin of the left main coronary artery from the right sinus of Valsalva), one athlete showed pheochromocytoma-related Tako-Tsubo cardiomyopathy (each 1.6%). Five athletes with confirmed structural heart disease were investigated after sustained ventricular tachycardia, seven athletes after aborted SCD: ARVC (n=6), aspecific LGE pattern (n=4), HCM (n=1) and pheochromocytoma-related Tako-Tsubo cardiomyopathy (n=1) were diagnosed. RVOT movie of an ARVC pts Conclusion In our national CMR registry the most common structural alteration was nonischaemic fibrosis, the most common cardiomyopathy was HCM, and the leading cause of SCD in Hungarian competitive athletes was ARVC. The national registers are highly important for a better understanding the etiology and the geographical differences of SCD in athletes. Acknowledgement/Funding Project no. NVKP_16-1-2016-0017 has been implemented with the support provided from the National Research, Development and Innovation Fund of Hungary

P902Danon disease: clinical features and outcomes

Abstract Background Danon Disease (DD) is a rare X-linked autophagic vacuolar myopathy, characterized by high penetrance and severe cardiomyopathy; cognitive, skeletal muscle and vision impairment may occur as well. Due to its rarity, clinical presentation and outcomes are still uncertain. Purpose To describe clinical features and outcomes of DD in female and male patients Methods Individuals and families from United Kingdom, Australia, and United States were recruited through via advertisements on Facebook groups related to DD. Participants completed a survey about symptoms and medical history and provided their medical records to the research team. Results A total of 44 patients (54.5% female) with positive genetic testing for DD were included. De novo mutations occurred in one out of four patients. Cardiomyopathy occurred in 86.3% of patients (18/24 females, 20/20 males) at a mean age of 7.3 years for males and 19.4 years for females (p=0.001). Females presented with either hypertrophic cardiomyopathy (HCM, 66.7%) or dilated cardiomyopathy (DCM, 8.3%) whereas males presented with HCM 90% of the time. 34.2% of patients were diagnosed with Wolff-Parkinson-White syndrome. Twelve patients (7 females, 5 males) underwent cardiac magnetic resonance (CMR) Out of the 9 cases, 8 (88.9%) exhibited extensive patchy late gadolinium-enhancement (LGE) in multiple segments of the left ventricle; 3 cases also had right ventricular LGE. Median cardiac mass index was 155 g/m2 (Q1-Q3: 70–237; v.n. 31–79 g/m2). Overall, 17 (38.6%) patients died or required or heart transplant (HTx). Median age at the time of death or HTx was 17 years and 42 years in males and females, respectively (p=0.025 by the log-rank test) Cognitive impairment, mainly described as learning disabilities, was diagnosed in 90.0% of males (18/20) and 79.2% (19/24) of females; intelligence quotient (IQ) measurement was reported in 8 patients (3 females, 5 males) and 7 of them showed IQ below the average. Symptomatic skeletal myopathy was present in 28 (63.3%) of patients, with a higher prevalence in males (85% vs. 45.8%; p&lt;0.01). Retinopathy was reported in 14 (31.2%) patients and occurred equally in both genders (p=0.34). Conclusions DD causes significant cardiac morbidity with the need for transplant at a young age; in 25% of cases DD is due to a de novo mutation. While in males DD is more frequently multisystemic with a more rapid clinical deterioration, in females the clinical presentation is variable. However, the presence of severe cases in females warrant the clinicians to screen for DD in both sexes with clinical manifestations or positive family history Acknowledgement/Funding Rocket Pharmaceuticals

The Natural History of Danon Disease: Lesson Learned from a Global Registry

Introduction Danon Disease (DD) is a rare X-linked autophagic vacuolar myopathy due to LAMP-2 mutation. DD is characterized by high penetrance, severe predominantly hypertrophic cardiomyopathy, cognitive and skeletal muscle as well as vision impairment. Due to its rarity, the natural history (NH) is still uncertain and delay in diagnosis is common. Methods Individuals and families from the United Kingdom, Australia, and North America completed a survey about symptoms and medical history and shared their medical records with the research team. Results A total of 46 patients (18 families, 43.5% males) with positive genetic testing for DD were included. The majority of patients were Caucasian (93.5%) and from the United States (80.4%). Among the 46 mutations, 23 were described as “pathogenic,” 10 as “likely pathogenic,” 3 as “variant of uncertain significance” and unknown in 10 cases. De novo mutations occurred in 30% of cases. Cardiomyopathy occurred in 85% of patients (19/26 females, 20/20 males) at a median age of 7.3 years for males and 19.4 years for females (p=0.001). Females presented with either hypertrophic cardiomyopathy (HCM, 69%) or dilated cardiomyopathy (DCM, 2.2%) whereas males presented with HCM 100% of the time. Left ventricular outflow tract (LVOT) obstruction was reported in 4.3% of patients. 34.2% of patients were diagnosed with Wolff-Parkinson-White syndrome. Twelve patients (7 females, 5 males) underwent cardiac magnetic resonance (CMR) Out of the 9 cases in whom contrast was used, 8 (89%) exhibited extensive patchy late gadolinium-enhancement (LGE) in multiple segments of the left ventricle; 3 cases also had right ventricular LGE. The median cardiac mass index was 155 g/m2 (Q1-Q3: 70-237; v.n. 31-79 g/m2). Cognitive impairment, mainly described as learning disabilities, was assessed in 90% of males (18/20) and 73.1% (19/26) of females. Symptomatic skeletal myopathy was present in 28 (60.1%) of patients, with a higher prevalence in males (85% vs 45.8%; p Conclusions DD causes significant cardiac morbidity with the need for Htx at a young age in both sexes. While in males DD is more frequently multisystemic with a more rapid clinical deterioration, females may present with isolated cardiac disease. These findings increase understanding of the natural history of DD and highlight the importance of a timely diagnosis regardless of sex.

The diagnostic performance of cardiac magnetic resonance in detection of myocardial involvement in AL amyloidosis

The non-invasive assessment of amyloid heart disease may be challenging. Cardiac magnetic resonance (CMR) represents a method of choice for assessment of left ventricular (LV) morphology and function, and it also provides a unique possibility to evaluate the presence of amyloid deposition by the late gadolinium enhancement (LGE) technique. However, so far, published studies have not been consistent in terms of described LGE patterns associated with amyloid cardiomyopathy. To compare echocardiographic and CMR assessment of LV morphology and function and to evaluate the presence and pattern of LGE in a population of patients with AL amyloid cardiomyopathy. Twenty-two consecutive patients with newly diagnosed AL amyloid cardiomyopathy and without contraindications to CMR were comprehensively examined by echocardiography and CMR. Echocardiography and CMR did not differ in the evaluation of interventricular septal thickness, LV end-diastolic diameter and ejection fraction. Significant differences were found between echocardiographic and CMR estimates of LV end-diastolic volume (P<0·01) and LV mass (P<0·001). Various global LGE patterns (transmural homogenous or heterogeneous, subendocardial) were present in 17 patients (77%), patchy LGE was observed in one case (4·5%) and suboptimal nulling of the myocardium was reported in two subjects (9%). Echocardiography significantly overestimates LV mass and underestimates LV volumes in patients with AL amyloid cardiomyopathy as compared to CMR. As it is present in more than three quarters of individuals with AL amyloid cardiomyopathy, any type of global LGE pattern may be considered as pathogenomic for amyloid heart disease.

Spectra and appearance of late gadolinium enhancement in magnetic resonance imaging of non-ischemic myocardial disease - from pattern to diagnosis

Objective: Value of late gadolinium enhancement (LGE) in diagnosis of ischemic disorders in cardiac magnetic resonance is well-known. The purpose of the present paper was to describe more details and characteristics of LGE patterns arising in several non-ischemic myocardial diseases. Methods: Cardiac MRI was performed in 1,905 patients at a 1.5-T MR system and showed LGE with 1,646/1,905 demonstrating LGE patterns or other diagnostic criteria (e.g. laboratory results, ECG, coronary angiography findings) regarded typical for ischemic heart disease. The remaining 259/1,905 patients revealed LGE related to non-ischemic myocardial disease. Two experts (radiologists and/or cardiologist) qualitatively described LGE pattern in consensus reading with regard to its site (left or right ventricle), extent (subendocardial, midmyocardial, subepicardial, transmural, pericardial), segment (according to the 17- segment model of left ventricle (LV)), distribution (streaky, patchy, punctual, spotty, crescent, diffuse), and intensity (weak, moderate, strong). Thereafter, results were correlated to final diagnosis based on synopsis of clinical features, laboratory results, ECG, biopsy, or other imaging procedures. Results: In 250/259 patients (96.5%) only the LV was affected by LGE, most frequently midmyocardial (166/259). In most cases this was due to peri-/myocarditis (especially if of weak-moderate intensity), while a weaker, streaky or spotty LGE basal or lateral pointed to HCM (39/166) and streaky and lateral to DCM (20/166). A midmyocardial, patchy LGE in segment 3 and 5 was caused by Fabry’s disease (6x). In case of a transmural, spotty or patchy pattern LGE pointed to HCM, if intensity was weak-moderate, or to peri-/myocarditis, when LGE was moderate-strong. In addition to that, subepicardial LGE in basal segments with patchy, streaky, or spotty distribution and moderate intensity was also due to peri-/myocarditis, while it was located midventricular, spotty and weak in TASH (transcoronary ablation of septum hypertrophy). Conclusions: Several LGE patterns point to certain non-ischemic myocardial diseases and thereby might facilitate and confine differential diagnosis and might be useful in biopsy planing.