Abstract
Loeffler endocarditis is a major cause of morbidity and mortality in patient with hypereosinophilia-associated syndromes. It is a rare disease that occurs due to eosinophilic inflammation of ...the endocardium with fibrosis and thrombus formation. We describe a case of Loeffler endocarditis with unusual clinical course.
61-year-old woman was admitted due to chest pain and elevated troponin I level. Her past medical history was remarkable for persistent asthma, rhinitis, and productive cough for almost 1 year. Laboratory test revealed hypereosinophilia. Further workup for parasitic diseases, allergy, malignancy, hematological causes were all negative Coronary angiography excluded coronary artery disease. Transthoracic echocardiography (TEE) showed endocardium thickening at the septal left ventricular wall, diastolic dysfunction (Grade II), and moderate pericardial effusion (Figure 1A). Cardiac magnetic resonance imaging (cMR) confirmed endocardial inflammation. The Loeffler endocarditis was diagnosed and corticosteroid treatment was started. After one year the patient was asymptomatic, TEE showed improvement of left ventricular diastolic function (Grade I) and complete regression of myocardial thickening (Figure 1B). Therefore, corticosteroids were slowly tapered. After 16 months she relapsed with severe chest pain, marked eosinophilia and elevated troponin I level. Left ventricular systolic function rapidly deteriorated to ejection fraction of 40%. Urgent cMR showed diffuse edomyocardial inflammation with apical thrombus formation. She was immediately treated with high doses of corticosteroid and heparin, which resulted in systolic function normalization and thrombus disappearance.
In conclusion, Loeffer endocarditis still represents diagnostic challenge, may have unpredictable clinical course and generally respond well to corticosteroid treatment in early stages.
Abstract P1685 Figure 1.
Abstract
Funding Acknowledgements
Type of funding sources: None.
Background
Myocardial fibrosis is a known prognostic factor in patients with systemic right ventricle (SRV). In these patients fixed ...myocardial perfusion defects are a common finding and are thought to represent areas of myocardial infarction and fibrosis. However, no study has yet correlated myocardial perfusion imaging findings with cardiac magnetic resonance (CMR) imaging, which is the imaging gold standard for detecting myocardial fibrosis.
Purpose
Our aim was to evaluate whether fixed myocardial perfusion defects in adult patients with SRV represent myocardial fibrosis.
Methods
Patients with SRV followed at our outpatient clinic for congenital heart disease were prospectively included. Myocardial perfusion was evaluated with a two-day stress/rest single-photon emission computed tomography (SPECT) protocol, focal myocardial fibrosis with late gadolinium enhancement (LGE) and diffuse myocardial fibrosis with T1 mapping by CMR. The 12-segment model of the right ventricle was used to report segments with myocardial perfusion defects and fibrosis (Figure 1).
Results
Fifteen patients with SRV (12 patients with transposition of the great arteries following atrial switch procedure and 3 patients with congenitally corrected transposition of the great arteries; 4 (26.7%) females; mean age 34.6 ± 10.0 years) were included. Myocardial perfusion defects were present in 14 patients (93%), with predominate fixed perfusion defects (73%) and less common reversible perfusion defects (27%). Fixed myocardial perfusion defects were most frequent in anterior RV segments (figure 1), with multiple segments affected in 11 patients (median number of affected segments – 2 segments). CMR was possible in 11 (73%) patients, others had a permanent pacemaker. LGE indicating focal myocardial fibrosis was detected in only 1 (9%) patient, while increased T1 values indicating diffuse myocardial fibrosis were present in 7 (64%) patients. There was no matching between areas of fixed myocardial perfusion defects and focal myocardial fibrosis in individual patients.
Conclusions
In our study, fixed myocardial perfusion defects detected on SPECT in patients with SRV did not represent areas of focal myocardial fibrosis on CMR. Other causes than scar may explain the frequently reported fixed perfusion defects, such as SRV anatomy with anterior position of the outflow tract and aorta, SRV morphology with variable degree of wall thickness and hypertrophy that influences tracer accumulation and image quality, or difficulties due to complex image acquisition and interpretation. To improve the diagnostic accuracy, the use of fused imaging modalities (SPECT-CT or PET-CT) is recommended in patients with SRV.
Figure 1. Bull`s eye 12-segment plots of the right ventricle (RV) representing the number of segments with fixed myocardial perfusion defects detected by SPECT (1A) and LGE by CMR (1B) in patients with SRV. ANT – anterior, FW – free wall, INF – inferior, SEP – septal wall of RV.
Abstract Figure.
The prospective, multicentre EURECA registry assessed the use of imaging and adoption of the European Society of Cardiology (ESC) Guidelines (GL) in patients with chronic coronary syndromes (CCS).
...Between May 2019 and March 2020, 5156 patients were recruited in 73 centres from 24 ESC member countries. The adoption of GL recommendations was evaluated according to clinical presentation and pre-test probability (PTP) of obstructive coronary artery disease (CAD).
The mean age of the population was 64 ± 11 years, 60% of patients were males, 42% had PTP >15%, 27% had previous CAD, and ejection fraction was <50% in 5%. Exercise ECG was performed in 32% of patients, stress imaging as the first choice in 40%, and computed tomography coronary angiography (CTCA) in 22%. Invasive coronary angiography (ICA) was the first or downstream test in 17% and 11%, respectively. Obstructive CAD was documented in 24% of patients, inducible ischaemia in 19%, and 13% of patients underwent revascularization. In 44% of patients, the overall diagnostic process did not adopt the GL. In these patients, referral to stress imaging (21% vs. 58%; P < 0.001) or CTCA (17% vs. 30%; P < 0.001) was less frequent, while exercise ECG (43% vs. 22%; P < 0.001) and ICA (48% vs. 15%; P < 0.001) were more frequently performed. The adoption of GL was associated with fewer ICA, higher proportion of diagnosis of obstructive CAD (60% vs. 39%, P < 0.001) and revascularization (54% vs. 37%, P < 0.001), higher quality of life, fewer additional testing, and longer times to late revascularization.
In patients with CCS, current clinical practice does not adopt GL recommendations on the use of diagnostic tests in a significant proportion of patients. When the diagnostic approach adopts GL recommendations, invasive procedures are less frequently used and the diagnostic yield and therapeutic utility are superior.
Abstract
Background
Cardiac masses are rare entities and often diagnostically challenging. When incidentally found multimodality diagnostic approach is warranted for further characterization and ...evaluation of its contribution to the patient’s symptoms.
Case report
62-year-old male, smoker, with a history of non-Hodgkin lymphoma treated with radiation and chemotherapy 10 years ago, was admitted due to subacute myocardial infarction. Coronary angiogram showed subacute occlusion of partly collateralized RCA, subsequently treated with dilatation and stenting. Echocardiography revealed signs of dilated cardiomyopathy with severely reduced left ventricular ejection fraction (EF 20%), that could be due to chemotherapy-related cardiotoxicity. On admission CTA was performed to exclude aortic dissection since the patient presented with very severe chest pain radiating to the back. It showed a heterogeneous formation within the right ventricle (RV) extending along the interventricular septum from the apex towards the tricuspid annulus, appearing to involve interventricular septum as well. According to the imaging characteristics the mass was suspected to be a liposarcoma (Figure A). With contrast echocardiography hypoechogenic formation in the RV on parasternal long-axis view could be visualized (Figure B). Due to previous history of malignancy PET scan was performed that revealed hypometabolic RV mass, suggesting a benign tumor, without any sign of metastasis. We decided for percutaneous biopsy that was done under intracardiac echocardiography (ICE) guidance using the view from the RV towards RV outflow tract (Figure C). Histology revealed proliferation of mature adipocytes, either orthotopic or multiplied (Figure D). To exclude well-differentiated liposarcoma FISH (fluorescent in situ hybridization) with amplification of MDM2 gene was undertaken, confirming benign lesion, most probably cardiac lipoma. It was concluded that the mass was an incidental finding, not related to the patient’s symptoms and not causing any functional disturbances; therefore surgical therapy could be avoided.
Conclusion
While echocardiography remains the first-line imaging modality, multimodality diagnostic approach is mandatory for evaluation and treatment decision of a newly-discovered cardiac mass. Histology provides definitive diagnosis and ICE could be helpful for guiding percutaneous biopsy, thus avoiding invasive open-heart procedures.
Figure. Cardiac CTA (A), contrast echocardiography (B), intracardiac echocardiography (C) and histologic specimen (D) of the right ventricular mass (arrows).
Abstract P1344 Figure.
Introduction: Type 1 diabetes specific autoantibodies are glutamic acid decarboxylase (GAD65), protein tyrosine phosphatase-like islet antigen 2 (IA2), insulin (IAA), and Zinc transporter 8 protein ...(ZnT8). The aim of our study was to determine if the presence of ZnT8 antibodies(Ab) was predictive of clinical presentation at diagnosis or subsequent disease course.
Methods: Between January 2003 and May 2019, 105 patients aged ≤ 21 years with a clinical diagnosis of T1DM had at least 1 diabetes autoantibody measured. For subjects who had less than 4 autoantibodies analyzed initially, residual serum sample was retrieved, and remaining autoantibodies were analyzed. Retrospective chart review was completed. At diagnosis, we evaluated BMI z-score, HbA1c, and the presence and severity of DKA. Complications recorded post diagnosis included episodes of DKA, diagnosis of autoimmune (AI) disease, and the presence of vascular complications.
Results: Of the 105 patients, 71 were ZnT8(+) (68%). When comparing ZnT8Ab(+) to (-) patients at diagnosis, there was no difference in age (p=0.94), BMI z-score (p=0.83) or presence of DKA (p=0.26). There was no difference in duration of follow up between ZnT8 Ab (+) and (-) groups (p=0.54). Over follow-up, (0.05-15.7 years), there was no difference in rates of DKA episodes (p=0.71). There were no macrovascular complications recorded and no difference in microvascular complications between the 2 groups (p=0.14). There were more AI conditions in the ZnT8Ab(+) group, 77% compared to 23% in ZnT8Ab(-), but this did not reach statistical significance (p=0.12).
Conclusions: Our study, unlike others, suggests that the presence of ZnT8 Ab does not result in a difference in disease course at presentation. This study adds to the literature as it has follow up on patients. Over up to 15 years of follow-up, there is no difference in complications between those with and without ZnT8 Ab’s. This study should be validated with larger numbers and longer follow up.
Disclosure
A. R. Dahl: None. S. Jenkins: None. J. Zbacnik: None. J. Foster: None. S. Pittock: None.
Purpose
To evaluate the different degrees of residual structure in the unfolded state of interferon-τ using chemical denaturation as a function of temperature by both urea and guanidinium ...hydrochloride.
Methods
Asymmetrical flow field-flow fractionation (AF4) using both UV and multi-angle laser light scattering (MALLS). Flow Microscopy. All subvisible particle imaging measurements were made using a FlowCAM flow imaging system.
Results
The two different denaturants provided different estimates of the conformational stability of the protein when extrapolated back to zero denaturant concentration. This suggests that urea and guanidinium hydrochloride (GnHCl) produce different degrees of residual structure in the unfolded state of interferon-τ. The differences were most pronounced at low temperature, suggesting that the residual structure in the denatured state is progressively lost when samples are heated above 25°C. The extent of expansion in the unfolded states was estimated from the m-values and was also measured using AF4. In contrast, the overall size of interferon-τ was determined by AF4 to decrease in the presence of histidine, which is known to bind to the native state, thereby providing conformational stabilization. Addition of histidine as the buffer resulted in formation of fewer subvisible particles over time at 50°C. Finally, the thermal aggregation was monitored using AF4 and the rate constants were found to be comparable to those determined previously by SEC and DLS. The thermal aggregation appears to be consistent with a nucleation-dependent mechanism with a critical nucleus size of 4 ± 1.
Conclusion
Chemical denaturation of interferon-τ by urea or GnHCl produces differing amounts of residual structure in the denatured state, leading to differing estimates of conformational stability. AF4 was used to determine changes in size, both upon ligand binding as well as upon denaturation with GnHCl. Histidine appears to be the preferred buffer for interferon-τ, as shown by slower formation of soluble aggregates and reduced levels of subvisible particles when heated at 50°C.
Purpose To evaluate the different degrees of residual structure in the unfolded state of interferon-tau using chemical denaturation as a function of temperature by both urea and guanidinium ...hydrochloride. Methods Asymmetrical flow field-flow fractionation (AF4) using both UV and multi-angle laser light scattering (MALLS). Flow Microscopy. All subvisible particle imaging measurements were made using a FlowCAM flow imaging system. Results The two different denaturants provided different estimates of the conformational stability of the protein when extrapolated back to zero denaturant concentration. This suggests that urea and guanidinium hydrochloride (GnHCl) produce different degrees of residual structure in the unfolded state of interferon-tau. The differences were most pronounced at low temperature, suggesting that the residual structure in the denatured state is progressively lost when samples are heated above 25°C. The extent of expansion in the unfolded states was estimated from the m-values and was also measured using AF4. In contrast, the overall size of interferon-tau was determined by AF4 to decrease in the presence of histidine, which is known to bind to the native state, thereby providing conformational stabilization. Addition of histidine as the buffer resulted in formation of fewer subvisible particles over time at 50°C. Finally, the thermal aggregation was monitored using AF4 and the rate constants were found to be comparable to those determined previously by SEC and DLS. The thermal aggregation appears to be consistent with a nucleation-dependent mechanism with a critical nucleus size of 4 ± 1. Conclusion Chemical denaturation of interferon-tau by urea or GnHCl produces differing amounts of residual structure in the denatured state, leading to differing estimates of conformational stability. AF4 was used to determine changes in size, both upon ligand binding as well as upon denaturation with GnHCl. Histidine appears to be the preferred buffer for interferon-tau, as shown by slower formation of soluble aggregates and reduced levels of subvisible particles when heated at 50°C.
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