Endomyocardial Fibrosis Associated With Hypereosinophilic Syndrome: Diagnostic and Management Insights From a Case Report.

Background: Hospitalised COVID-19 patients frequently have acute myocardial injury with elevated troponin levels Underlying aetiologies are ill-defined We use

An 18-year-old Venezuelan woman, with a history of idiopathic restrictive cardiomyopathy, presented at our hospital with ongoing dyspnea, palpitations, and lightheadedness. A medical evaluation revealed heart failure, pulmonary hypertension, and heparin-induced thrombocytopenia. Cardiac catheterization revealed normal coronary arteries. The left ventriculogram demonstrated late filling of the apex, which was almost obliterated by prominent trabeculations. Moderate mitral regurgitation with significant enlargement of the left atrium was observed. The ejection fraction was 44%, without regional wall motion abnormalities (Figure 1). Figure 1. End-systolic (A) and end-diastolic (B) frames of the left ventriculography show partial obliteration of the cavity …

759 Prognostic Value of Stress Perfusion Cardiac Magnetic Resonance Imaging in Obese Patients

A 48-year-old man, with only a history of mild systemic hypertension, was initially evaluated after presenting with symptoms of exertional dyspnea occurring predominantly with inclines. At that time, an abnormal 12-lead ECG was obtained demonstrating left ventricular hypertrophy by conventional voltage criteria, prompting additional testing with a 2-dimensional echocardiogram that showed normal systolic function (ejection fraction=65%), with 14-mm ventricular septal thickness and 12 mm in the posterolateral wall, and mild systolic anterior motion (SAM) of the mitral valve (bend of anterior leaflet into outflow tract without septal contact). A stress nuclear stress test showed no myocardial ischemia at rest or at peak exercise with a normal blood pressure response and no arrhythmias or ST-T changes during exercise or in recovery. The patient was prescribed a β-blocker for treatment of systemic hypertension. During the next 2 years, the patient developed more limiting exertional symptoms with routine activities. β-Blocker dosage was increased, and a repeat echocardiogram demonstrated similar findings to the initial study, borderline left ventricular (LV) wall thickness despite well-controlled blood pressure. The abnormal ECG, and mild SAM at rest, raised consideration for a diagnosis of hypertrophic cardiomyopathy (HCM) and management for limiting heart failure symptoms. HCM is often suspected in a patient based on the presence of cardiovascular symptoms, detection of abnormal ECG, systolic ejection murmur on routine examination, or as part of pedigree screening.1,2 Abnormalities on ECG are present in >90% of patients with HCM, although no specific ECG pattern is pathognomonic.1 Clinical diagnosis of HCM can reliably be made in the majority of patients with 2-dimensional transthoracic echocardiography by imaging increased LV wall thickness (≥15 mm) with a nondilated cavity in the absence of any disease known to cause LV hypertrophy of that magnitude (ie, systemic hypertension or aortic stenosis).1–5 In …

Cardiac Magnetic Resonance Imaging Detects Long-Term Fibrotic Changes in Patients with Primary Eosinophilic Disorders: A Cross-Sectional Study

Background Compelling evidence indicates that women with coronary artery disease (CAD) experience worse outcomes than men due to a lack of early diagnosis and management. Numerous clinical studies have shown that stress cardiovascular magnetic resonance (CMR) detects evidence of myocardial ischemia and infarction at high accuracy. However, long-term prognosis data are limited. Purpose The aim of this study was to test the hypothesis that stress perfusion CMR imaging can provide robust prognostic value in women presenting with suspected ischemia, to the same extent as in men. Material Consecutive patients referred for vasodilator stress perfusion CMR with dipyridamole were followed for the occurrence of major adverse cardiovascular events (MACE) defined as cardiovascular death or non-fatal myocardial infarction (MI). The secondary endpoint was cardiovascular death. The safety of the CMR was assessed by clinical monitoring for 1 hour after the end of the CMR. Univariable and multivariable Cox regressions for MACE were performed to determine the prognostic value of inducible ischemia or late gadolinium enhancement (LGE) by CMR in each sex. Results Of 3436 patients referred for stress CMR in a single French center, 3322 (97%) completed the CMR protocol (59.9±11.8 years, 57% men), and among those 3033 (91%) completed the follow-up (median follow-up 5.4±0.2 years). Reasons for failure to complete CMR included renal failure (n=29), claustrophobia (n=26), poor gating (n=22), intolerance to stress agent (n=19) and declining participation (n=18). Stress CMR was well tolerated without occurrence of death or severe disabling adverse event. Using Kaplan-Meier analysis, the presence of inducible myocardial ischemia identified the occurrence of MACE for both women (hazard ratio HR 2.36; 95% confidence interval CI: 1.54–3.62; p<0.001) and men (HR 3.57; 95% confidence interval CI: 2.75–4.64; p<0.001) (Figure). Moreover, inducible ischemia was associated with cardiovascular death for both women (hazard ratio HR 1.92; 95% confidence interval CI: 1.12–2.74; p=0.04) and men (HR 2.71; 95% confidence interval CI: 1.98–4.41; p<0.001). In a multivariable stepwise Cox regression including clinical characteristics and CMR, presence of inducible ischemia was an independent predictor of a higher incidence of MACE for both women (hazard ratio HR 1.85; 95% confidence interval CI: 1.18–2.92; p=0.008) and men (HR 3.55; 95% confidence interval CI: 2.73–4.63; p<0.001). Moreover, inducible ischemia was associated with cardiovascular death for men (HR 1.99; 95% confidence interval CI: 1.65–3.01; p<0.01) but not for women (p=0.11). Conclusion Stress CMR is feasible, safe and has a good discriminative prognostic value to predict the occurrence of MACE in patients of either sex presenting with inducible ischemia. However, inducible ischemia is an independent predictor of a higher incidence of CV mortality only in men. Kaplan-Meier curves for MACE in each sex Funding Acknowledgement Type of funding source: None

Funding Acknowledgements Type of funding sources: None. BACKGROUND Compelling evidence indicates that women with coronary artery disease (CAD) experience worse outcomes than men due to a lack of early diagnosis and management. Numerous clinical studies have shown that stress cardiovascular magnetic resonance (CMR) detects evidence of myocardial ischemia and infarction at high accuracy. However, long-term prognosis data are limited. PURPOSE The aim of this study was to test the hypothesis that stress perfusion CMR imaging can provide robust prognostic value in women presenting with suspected ischemia, to the same extent as in men. METHODS Consecutive patients referred for vasodilator stress perfusion CMR with dipyridamole were followed for the occurrence of major adverse cardiovascular events (MACE) defined as cardiovascular death or non-fatal myocardial infarction (MI). The secondary endpoint was cardiovascular death. The safety of the CMR was assessed by clinical monitoring for 1 hour after the end of the CMR. Univariable and multivariable Cox regressions for MACE were performed to determine the prognostic value of inducible ischemia or late gadolinium enhancement (LGE) by CMR in each sex. RESULTS Of 3436 patients referred for stress CMR in a single French center, 3322 (97%) completed the CMR protocol (59.9 ± 11.8 years, 57% men), and among those 3033 (91%) completed the follow-up (median follow-up 5.4 ± 0.2 years). Reasons for failure to complete CMR included renal failure (n = 29), claustrophobia (n = 26), poor gating (n = 22), intolerance to stress agent (n = 19) and declining participation (n = 18). Stress CMR was well tolerated without occurrence of death or severe disabling adverse event. Using Kaplan-Meier analysis, the presence of inducible myocardial ischemia identified the occurrence of MACE for both women (hazard ratio HR 2.36 ; 95% confidence interval CI: 1.54–3.62; p < 0.001) and men (HR 3.57 ; 95% confidence interval CI: 2.75 – 4.64; p < 0.001) (Figure). Moreover, inducible ischemia was associated with cardiovascular death for both women (hazard ratio HR 1.92; 95% confidence interval CI: 1.12 – 2.74; p = 0.04) and men (HR 2.71 ; 95% confidence interval CI: 1.98 – 4.41; p < 0.001). In a multivariable stepwise Cox regression including clinical characteristics and CMR, presence of inducible ischemia was an independent predictor of a higher incidence of MACE for both women (hazard ratio HR 1.85 ; 95% confidence interval CI: 1.18 – 2.92; p = 0.008) and men (HR 3.55 ; 95% confidence interval CI: 2.73 – 4.63; p < 0.001). Moreover, inducible ischemia was associated with cardiovascular death for men (HR 1.99; 95% confidence interval CI: 1.65 – 3.01; p < 0.01) but not for women (p = 0.11). CONCLUSION Stress CMR is feasible, safe and has a good discriminative prognostic value to predict the occurrence of MACE in patients of either sex presenting with inducible ischemia. However, inducible ischemia is an independent predictor of a higher incidence of CV mortality only in men. Abstract Figure.

Gadolinium-enhanced cardiovascular magnetic resonance for the detection and characterization of Loeffler endocarditis in patients with hypereosinophilic syndrome

Funding Acknowledgements Type of funding sources: Other. Main funding source(s): This study was financed by the Ministry of Innovation and Technology NRDI Office within the framework of the Artificial Intelligence National Laboratory Program. LS is supported by the EACVI Research Grant 2021. Introduction Cardiac adaptation due to regular and intense exercise is a well-known phenomenon. Cardiac magnetic resonance (CMR) imaging is a well suited, highly reproducible technique that has a vital role in differentiating physiological adaptation and pathological alterations. Native T1 and T2 mapping enable the quantitative assessment of tissue characteristics without the administration of contrast material. These techniques are increasingly used in studies aiming to consider subtle differences. However, the sex-and training-dependence of native T1 and T2 mapping values remains incompletely understood. Purpose We aimed to describe the differences in native T1 and T2 mapping among healthy athletes and less active individuals. Methods We enrolled healthy elite athletes (n=88, 56 male, 25±5 years) and healthy volunteers (n=82, 46 male, 25±3 years) to undergo CMR examinations at our Centre. Healthy elite athletes performed high sports activity levels (>10 hours/week) and competed nationally or internationally. Sex- and age-matched healthy volunteers engaged in ≤6 hours/week of sports activity. Standardized CMR protocol included short- and long-axis cine images covering the entire left (LV) and right (RV) ventricle and native T1 and T2 mapping in basal, midventricular and apical slices. Results Athletes had consistently higher LV and RV volumes and mass indexes compared to healthy volunteers (p<.001 for all). Native T1 mapping was lower in athletes than in the control group (T1: 954±24 ms vs 970±23 ms; p <.001). T1 mapping showed a moderately strong negative correlation to markers of cardiac adaptation, including LV mass, end-diastolic volume and stroke volume indexes (p<.001 for all). Moreover, we found a negative correlation between native T1 and training hours (Rho: -0.302; p<.001). On the other hand, native T2 mapping showed no difference between athletes and less active controls. Furthermore, T2 correlated with LV shape features but not with training hours. We found that mapping values differed between sexes, both in the athletic and control groups. Females showed slightly higher values compared to their male counterparts (T2: 46±2 vs 43±2; p<.001). Finally, native T1 mapping was associated with training hours and sex in our multiple linear regression model, adjusted for age, resting heart rate, body mass index, body surface area and LVM (p<.001). While T2 mapping was associated only with sex considering the same covariates. Conclusion Our study demonstrates the importance of sex-matched controls in CMR studies evaluating mapping parameters. Moreover, the consideration of exercise load seems paramount in the case of T1 mapping.

This is a 72 year-old man known for idiopathic HES with endomyocardial fibrosis and moderate aortic regurgitation, treated with corticosteroids and antithrombotics. Two months prior to admission, he develops progressive exertional dyspnea reaching NYHA 3 class, with concomitant peripheral edema and rise in NT-proBNP. Cardiac transthoracic work-up shows a preserved left ventricular ejection fraction, but more marked dilatation with decrease in left ventricular volume due to a large echodense mass in the apex. The transmitral flow is restrictive, and the aortic regurgitation moderate. In view of this rapid evolution, resection of the left ventricular mass with concomitant aortic valve replacement is performed. Pathology confirmed eosinophilic infiltration. First described in 1975, hypereosinophilic syndrome (HES) is a rare pathology that is defined as an absolute eosinophil count (>1,5 G/L) in the peripheral blood with eosinophil-mediated organ damage and /or dysfunction. We know three mains categories; primary (or neoplastic), secondary (or reactive) and idiopathic. Cardiac involvement is uncertain and cardiac injury does not clearly correlate with degree of peripheral eosinophilia. Heart tissue damage evolves through three stages (necrotic, intermediate and fibrotic). Endomyocardial fibrosis and eosinophilic myocarditis, also known as "Loeffler’s endocarditis", are major causes of morbidity and mortality among patients with HES. Echocardiography plays a crucial role in initial diagnosis of endomyocardial fibrosis, displaying myocardial wall thickening, signs of restriction (mitral inflow with large E wave and small A wave; E/A ratio greater than 2, short deceleration time less than 150 ms) and intracardiac thrombus (fixed mass embedding left ventricular apex). Valve regurgitation is another possible complication due to entrapment of the chordae tendinae and/or leaflets during the fibrotic stage of this disease. Cardiac MRI is another important diagnostic tool for precising cardiac involvement. Despite of steadily improving noninvasive diagnostic imaging methods, endomyocardial biosy still remains the gold standard exam and ultrasounds work-up is crucial for follow-up. In conclusion, endomyocardial fibrosis is a rare cause of restrictive cardiomyopathy characterized by echocardiographic sign of elevated filling pressure, progressive endomyocardial thickening, valve regurgitation and possible intracardiac thrombus formation. This case underlines the importance of echocardiography in initial diagnosis and regular follow-up of this type of patients, in order to adapt medical treatment and monitor hemodynamic evolution of the restrictive physiology and of valvular damage. Abstract P703 Figure. HES-3

Purpose: The hypereosinophilic syndromes (HES) are rare diseases characterized by blood hypereosinophilia and eosinophil-related organ damage, with the Churg-Strauss syndrome as the vasculitic subtype. Cardiac involvement, reported in 20-50% of patients in earlier series, carries poor prognosis and justifies aggressive immunosuppressive therapy. Cardiac magnetic resonance imaging (MRI) is the reference method for assessment of left ventricular (LV) function and allows evaluation of myocardial inflammation with consecutive oedema or myocardial fibrosis. We therefore sought to evaluate the frequency and patterns of cardiac manifestation of HES using cardiac MRI. Methods: 37 patients with definite HES were referred to our department for cardiac MRI because of possible cardiac involvement according to clinical, echocardiography and laboratory findings. MRI analysis included cine sequences for the assessment of LV function. T2-weighted imaging was conducted for assessment of myocardial oedema. Gadolinium late enhancement (LE) imaging was conducted for detection of fibrosis. Results: Of the 37 patients 14 were male. The mean age was 51.9±2.4 years. 20 patients also underwent coronary angiography, and only one patient had a significant stenosis of a marginal branch. Four patients (11%) had a cardiac MRI scan without pathological findings; three patients (8%) a non-circumferential PE of ≤ 5mm and an otherwise normal MRI. In 12 patients a PE ranging from 4 to 30mm was associated with other pathological findings. Quantitative analysis of LV function revealed a mean ejection fraction (EF) of 51.9±2.3%, EF was reduced (<55%) in 20 patients (54%). 7 patients (19%) showed hyperintense areas in T2-weighted images indicative of myocardial oedema. LE of the myocardium was observed in 23 patients (62%), all demonstrating a non-ischemic pattern of LE or multiple small endocardial LE zones not compatible with myocardial infarction due to coronary artery disease. Lastly, 4 patients (11%) showed the typical pattern of endomyocadial fibrosis with oedema and contrast enhancement of the myocardial/endocardial border zone and thrombotic material at the left and right ventricular apex. Conclusions: Cardiac MRI revealed pathologies in 90% of the study cohort. Yet, the pattern of cardiac manifestations varies significantly. More than half of the patients showed cardiomyopathy with reduced systolic LV function. Non-ischemic LE was present in the majority of patients and combined in one third of cases with myocardial oedema indicating acute inflammation. The unique pattern of endomyocardial fibrosis was observed in 11% of the patients.

To determine the utility of cardiac magnetic resonance (MR) T1 mapping for quantification of diffuse myocardial fibrosis compared with the standard of endomyocardial biopsy. This HIPAA-compliant study was approved by the institutional review board. Cardiomyopathy patients were retrospectively identified who had undergone endomyocardial biopsy and cardiac MR at one institution during a 5-year period. Forty-seven patients (53% male; mean age, 46.8 years) had undergone diagnostic cardiac MR and endomyocardial biopsy. Thirteen healthy volunteers (54% male; mean age, 38.1 years) underwent cardiac MR as a reference. Myocardial T1 mapping was performed 10.7 minutes ± 2.7 (standard deviation) after bolus injection of 0.2 mmol/kg gadolinium chelate by using an inversion-recovery Look-Locker sequence on a 1.5-T MR imager. Late gadolinium enhancement was assessed by using gradient-echo inversion-recovery sequences. Cardiac MR results were the consensus of two radiologists who were blinded to histopathologic findings. Endomyocardial biopsy fibrosis was quantitatively measured by using automated image analysis software with digital images of specimens stained with Masson trichrome. Histopathologic findings were reported by two pathologists blinded to cardiac MR findings. Statistical analyses included Mann-Whitney U test, analysis of variance, and linear regression. Median myocardial fibrosis was 8.5% (interquartile range, 5.7-14.4). T1 times were greater in control subjects than in patients without and in patients with evident late gadolinium enhancement (466 msec ± 14, 406 msec ± 59, and 303 msec ± 53, respectively; P < .001). T1 time and histologic fibrosis were inversely correlated (r = -0.57; 95% confidence interval: -0.74, -0.34; P < .0001). The area under the curve for myocardial T1 time to detect fibrosis of greater than 5% was 0.84 at a cutoff of 383 msec. Cardiac MR with T1 mapping can provide noninvasive evidence of diffuse myocardial fibrosis in patients referred for evaluation of cardiomyopathy.

BackgroundT1 and T2 cardiac magnetic resonance (CMR) mapping methods have shown promise for infarct characterisation in patients with acute ST-elevation myocardial infarction (STEMI). Non-ST elevation MI is typically a sub-acute...

Myocardial tissue characterization by cardiac magnetic resonance imaging using T1 mapping predicts ventricular arrhythmia in ischemic and non-ischemic cardiomyopathy patients with implantable cardioverter-defibrillators.

The restrictive cardiomyopathies constitute a heterogeneous group of myocardial diseases with a different pathogenesis and overlapping clinical presentations. Diagnosing them frequently poses a challenge. Echocardiography, electrocardiograms and laboratory tests may show non-specific changes. In this context, cardiac magnetic resonance (CMR) may play a crucial role in defining the diagnosis and guiding treatments, by offering a robust myocardial characterization based on the inherent magnetic properties of abnormal tissues, thus limiting the use of endomyocardial biopsy. In this review article, we explore the role of CMR in the assessment of a wide range of myocardial diseases causing restrictive patterns, from iron overload to cardiac amyloidosis, endomyocardial fibrosis or radiation-induced heart disease. Here, we emphasize the incremental value of novel relaxometric techniques such as T1 and T2 mapping, which may recognize different storage diseases based on the intrinsic magnetic properties of the accumulating metabolites, with or without the use of gadolinium-based contrast agents. We illustrate the importance of these CMR techniques and their great support when contrast media administration is contraindicated. Finally, we describe the useful role of cardiac computed tomography for diagnosis and management of restrictive cardiomyopathies when CMR is contraindicated.