This study analyses various chicken cuts (breast, leg, and thigh) in terms of ten biocompounds: nicotinamide, anserine, carnosine, malondialdehyde, and biogenic amines (putrescine, cadaverine, histamine, tyramine, spermidine, and spermine). The analysis is conducted on refrigerated chicken meat cuts using three different packaging solutions: modified atmosphere packaging (MAP), vacuum skin packaging (SKIN), and permeable O2 plastic film (STRETCH). Our results show that nicotinamide was the most discriminant compound followed for cuts and time. Remarkably, its concentration decreases over time, unaffected by any of the packaging solutions. The collective contribution of all the examined biocompounds is highlighted through multivariate statistical analysis, utilizing partial least square discriminant analysis (PLS-DA) and the support vector machine (SVM) algorithm. Both machine learning models demonstrate high classification accuracy: for cut, storage time, and packaging, PLS-DA yields classification accuracy rates of 87%, 85%, and 70%, respectively. SVM achieves even higher accuracy rates of 97%, 99%, and 94% for cut, storage time, and packaging, respectively. These results underscore the importance of considering the combined influence of all the biocompounds investigated in this study for a finer classification of chicken meat cuts and packaging. Furthermore, our findings highlight the efficacy of SVM as a discriminant multivariate approach in food classification.

In this work, the chemical composition and the antioxidant evaluation of the inflorescences from 12 Cannabis sativa L. monoecious cultivars (Carmagnola Lemon CL, Ferimon F, Gran Sasso Kush GSK, Antal A, Carmagnola C, Kompolti K, Futura 75 F75, Villanova V, Tiborzallasi T, Finola FL, Kc Virtus KV and Pineapple P) cultivated at the same condition, were investigated. GC-MS analysis was carried out to evaluate the volatile fraction, while HPLC-MS/MS was used for cannabinoids and polyphenolic compounds. The evaluation of antioxidant activity was carried out using ABTS(*+), Trolox equivalence antioxidant capacity (TEAC), ferric reducing antioxidant property (FRAP) and 2,2-diphenyl-1-picrylhydrazyl (DPPH*) assays in vitro. The obtained data, demonstrated that each cultivar has a characteristic chemical profile, with highest antioxidant capacity for CL, F75, GSK and F. Based on the in vitro antioxidant activity the plant extracts can be considered as promising candidates for different applications in food field.

Lupin alkaloids (LAs) represent a class of toxic secondary metabolites in plants, in particular in Lupinus spp.; they are produced as a defense mechanism due to their strong bitter taste and are very dangerous for human and animals. In this work, a sensitive and reliable high performance liquid chromatography—tandem mass spectrometry (HPLC-MS/MS) analytical method for the identification and quantification of thirteen lupin alkaloids was developed and validated according to FDA guidelines. Efficient extraction and clean-up steps, carried out by solid-phase extraction, were finely tuned on the basis of the characteristics of the analytes and lupin samples, providing good selectivity with minimized matrix interference. The effectiveness of the method was proven by the satisfactory recovery values obtained for most of the analytes and a matrix effect ≤23% for all tested levels. In addition, a sensitive and reliable determination of the target compounds was obtained; LOQs were between 1 and 25 µg Kg−1, i.e., below the requested maximum levels (<200 mg Kg−1). The method was applied to evaluate the LAs profile in different batches of raw L. albus L. samples, varying in size and across farming treatments.

Autoimmune rheumatic diseases (ARDs) involve multiple organs including the heart and vasculature. Despite novel treatments, patients with ARDs still experience a reduced life expectancy, partly caused by the higher prevalence of cardiovascular disease (CVD). This includes CV inflammation, rhythm disturbances, perfusion abnormalities (ischaemia/infarction), dysregulation of vasoreactivity, myocardial fibrosis, coagulation abnormalities, pulmonary hypertension, valvular disease, and side-effects of immunomodulatory therapy. Currently, the evaluation of CV involvement in patients with ARDs is based on the assessment of cardiac symptoms, coupled with electrocardiography, blood testing, and echocardiography. However, CVD may not become overt until late in the course of the disease, thus potentially limiting the therapeutic window for intervention. More recently, cardiovascular magnetic resonance (CMR) has allowed for the early identification of pathophysiologic structural/functional alterations that take place before the onset of clinically overt CVD. CMR allows for detailed evaluation of biventricular function together with tissue characterization of vessels/myocardium in the same examination, yielding a reliable assessment of disease activity that might not be mirrored by blood biomarkers and other imaging modalities. Therefore, CMR provides diagnostic information that enables timely clinical decision-making and facilitates the tailoring of treatment to individual patients. Here we review the role of CMR in the early and accurate diagnosis of CVD in patients with ARDs compared with other non-invasive imaging modalities. Furthermore, we present a consensus-based decision algorithm for when a CMR study could be considered in patients with ARDs, together with a standardized study protocol. Lastly, we discuss the clinical implications of findings from a CMR examination.

Polyphenols (PCs) are a numerous class of bioactive molecules and are known for their antioxidant activity. In this work, the potential of the quadrupole/linear ion trap hybrid mass spectrometer (LIT-QqQ) was exploited to develop a semi-untargeted method for the identification of polyphenols in different food matrices: green coffee, Crocus sativus L. (saffron) and Humulus lupulus L. (hop). Several conjugate forms of flavonoids and hydroxycinnamic acid were detected using neutral loss (NL) as a survey scan coupled with dependent scans with enhanced product ion (EPI) based on information-dependent acquisition (IDA) criteria. The presented approach is focused on a specific class of molecules and provides comprehensive information on the different conjugation models that are related to specific base molecules, thus allowing a quick and effective identification of all possible combinations, such as mono-, di-, or tri-glycosylation or another type of conjugation such as quinic acid esters.

Type of funding sources: Private company. Main funding source(s): The E-MIOT project receives “no-profit support” from industrial sponsorships (Chiesi Farmaceutici S.p.A. and ApoPharma Inc. and Bayer). Background. The T2* cardiovascular magnetic resonance (CMR) is the gold standard for the non invasive detection of myocardial iron overload (MIO). The native myocardial T1 mapping has been proposed as a complementary tool, thanks to its higher sensitivity in presence of small amounts of iron, but no data are available in literature about its clinical impact. To explore the clinical impact of T1 mapping for detecting cardiac complications in thalassemia major (TM). Methods. We considered 146 TM patients (87 females, 38.7 ± 11.1 years) consecutively enrolled in the Extension-Myocardial Iron Overload in Thalassemia Network. Three parallel short-axis slices of the left ventricle (LV) were acquired with the Modified Look-Locker Inversion recovery (MOLLI) sequence. The native T1 values in all 16 myocardial segments were obtained and the global value was the mean. Results. Twenty-one patients had an history of cardiac complications: 11 heart failure,  8 arrhythmias (7 supraventricular and 1 ventricular), and 2 pulmonary hyperthension. Patients with cardiac complications had significantly lower global heart T1 values (879.3 ± 121.9 ms vs 963.2 ± 98.5 ms; P < 0.0001) (Figure) but comparable T2* values (33.32 ± 11.66 ms vs 37.17 ± 9.15 ms; P = 0.116). Cardiac complications were more frequent in the group of patients with reduced global heart T1 value (<928 ms for males and <989 ms for females) compared to the group with normal global heart T1 value (71.4% vs 39.5%; P = 0.009). Odds ratio (OR) for cardiac complications was 3.8 (95%CI = 1.3-10.9) for patients with reduced global heart T1 value versus patients with normal global heart T1 value. We found out a significant association between decreased native global heart T1 values and a history of cardiac complications, suggesting that an early detection of myocardial iron  burden by  native T1 can support the clinicians in modifing chelation therapy earlier. Abstract Figure.

In this work two different acquisition approaches were used for the quantification and/or tentative identification of phenolic compounds (PCs) in plant matrices by HPLC-MS/MS. A targeted approach, based on MRM acquisition mode, was used for the identification and quantification of a list of target analytes by comparison with standards; a semi-targeted approach was also developed by the precursor ion scan and neutral loss for the tentative identification of compounds not included in the target list. Analysis of phenolic content in three different plant matrices (curry leaves, hemp and blueberry) was carried out. The extraction and clean-up steps were set up according to the characteristics of the sample allowing to minimize the interfering compounds present in such complex matrices, as proved by the low matrix effect obtained (<16%) and recovery values ranging from 45% to 98% for all the analytes. This approach provided a sensitive and robust quantitative analysis of the target compounds with LOQs between 0.0002 and 0.05 ng mg-1, which allowed the identification and quantification of several hydroxycinnamic and hydroxybenzoic acids, in addition to numerous flavonoids in all three matrices. Furthermore, different moieties were considered as neutral losses or as precursor ions in semi-targeted MS/MS approach, providing the putative identification of different glycosylated forms of flavonoids, such as luteolin-galactoside and diosmin in all three matrices, while apigenin-glucuronide was detected in hemp and quercetin-glucuronide in blueberry. A further study was carried out by MS3, allowing the discrimination of compounds with similar aglycones, such as luteolin and kaempferol.

Abstract Funding Acknowledgements Type of funding sources: Private company. Main funding source(s): The MIOT project receives “no-profit support” from industrial sponsorships (Chiesi Farmaceutici S.p.A., ApoPharma Inc.). Background. Cardiac function indices in patients with hemoglobinopathies are different from those in healthy population, mainly due to chronic anemia. Normal reference values specific for SCD patients are not available by CMR. Aim. We aimed to define the normal cut-off value in SCD patients for bi-atrial and biventricular cardiac magnetic resonance (CMR) parameters. Methods. We considered forty-eight adult SCD patients with no known risk factors or cardiac disease, normal electrocardiogram, no macroscopic myocardial fibrosis, and all cardiac segments with T2*≥20 ms, consecutively enrolled in the MIOT network (Myocardial iron overload in thalassemia). SCD patients were compared with ninety-six healthy controls and 96 thalassemia major (TM) patients without cardiac damage, both matched for age and gender. Nine pediatric SCD patients were also analysed in comparison with 9 TM patients and 9 healthy subjects matched for age and gender. Cine images were acquired to quantify biventricular function parameters: LV and RV end-diastolic volume (EDV), end-systolic volume (ESV) and stroke volume (SV) were normalized for body surface area (EDVI, ESVI, SVI), as well as biventricular mass and atrial areas. Myocardial iron overload was assessed by segmental T2* technique. Late gadolinium enhancement (LGE) images were acquired for evaluation of macroscopic myocardial fibrosis. Results. In all three groups males showed higher biventricular volumes and mass indexes than females. SCD male patients had significantly higher LVEDVI (p < 0.0001), LVESVI (p = 0.010), LVSVI (p = 0.003), cardiac index (p = 0.002), LV and RV mass index (p = 0.008 and p = 0.001, respectively) and left and right atrial areas (p < 0.001 and p = 0.011) than healthy subjects. No significant differences were found in RVEDVI, EVESVI and biventricular EF. Compared to healthy volunteers, females with SCD showed a larger LVEDVI (p = 0.020), LVSVI (p = 0.039), RV mass index (p = 0.002) and left atrial area (p = 0.008). SCD and TM patients showed comparable values of bi-atrial and biventricular volumes and function. When compared to TM, SCD patients showed a larger LV (p < 0.001) and RV mass index (p = 0.001) in male group and a larger RV mass index (p = 0.001) in female group. Table 1 shows the cut-offs for bi-atrial and biventricular MR parameters for adult SCD patients by gender. No significant differences in MR parameters were found among the pediatric groups. Conclusions. Normal reference ranges of bi-atrial and biventricular MR parameters for adult males and females SCD patients were established. The use of these reference values will prevent possible misdiagnosis of cardiomyopathy in patients with SCD.

Type of funding sources: None. Background. Native T1 values are usually assessed in the end-diastole to minimize motion artifacts while the systolic data acquisition offers the advantage of a thicker myocardium, with reduced partial-volume effects. Higher myocardial T1 values have been detected in diastole at both 1.5T and 3T but the dependence of this difference on myocardial segments or gender has not been fully explored. Aim. We provided a systematic comparison of myocardial native T1 values in diastole and systole, by considering separately myocardial segments and dividing males and females. Methods. Sixty-one healthy subjects (46.0 ± 14.1 years, 32 males) underwent CMR at 1.5T (Signa Artist; GE Healthcare). Three short-axis slices of the left ventricle acquired in diastole and systole using a Modified Look–Locker Inversion Recovery sequence. Image analysis was performed with a commercially available software package. T1 value was assessed in all 16 myocardial segments and global value was the mean. Results. Table 1 shows the comparison between T1 values calculated from maps obtained in diastole and systole. Systolic T1 values were significantly lower in the basal anterolateral segment, in all medium segments except for the medium inferior segment, and in all apical segments. The percentage difference between diastolic and systolic T1 values was considered to compensate for the higher T1 values in females, and a significantly higher value was detected in females for the majority of medium segments, for all apical segments, and for the global value. Conclusion. The diastolic-systolic discrepancy was more pronounced for the females and at the apical level, supporting the hypothesis that, besides the physiologic variations in myocardial blood volume during the cardiac cycle, the partial volume-effect may be a strong additional contributing factor. Native T1 values should be obtained always in the same cardiac phase to avoid a potential bias in the discrimination between healthy and pathologically affected myocardium.

Abstract Funding Acknowledgements Type of funding sources: Private company. Main funding source(s): The E-MIOT project receives “no-profit support” from industrial sponsorships (Chiesi Farmaceutici S.p.A. and Bayer). Background The T2* magnetic resonance imaging (MRI) technique for the noninvasive quantification of iron overload has significantly improved the survival of patients with hemoglobinopathies by tailoring the chelation therapy. In Italy, the E-MIOT (Extension-Myocardial Iron Overload in Thalassemia) Network, a collaborative project among MRI and hematological centers, has assured high-quality quantification of iron in vital organs such as heart, liver, and pancreas. The COVID-19 pandemic has disrupted healthcare services around the world, also leading to postpone or delete deferable diagnostic evaluations. Aim We evaluated the impact of the COVID-19 pandemic on MRI services for iron overload quantification in Italy. Methods The activity of the MRI centers of the E-MIOT Network in the year 2020 was compared to the activity in the same months of 2019. A specific survey was filled out by the MRI operators to evaluate if the availability of MRI slots for patients with hemoglobinothies was reduced and the reasons. Results In comparison with the year 2019, in 2020 there was a significant reduction in the number of T2* MRIs performed (350 vs 656; P < 0.0001). The comparison month by month between the two years highlighted a marked decline (86.9%) in the four-month period March-June 2020, a reduction in the gap between the two years in the three-month period July-September, and a new decline (41.4%) in the three-month period October-December (Figure 1). No patient with hemoglobinopathy could undergo an MRI scan during the Italian lockdown (9 March-10 May 2020). Figure 2 shows the percentage of decline (year 2020 vs 2019) in the number of T2*MRI scans for each MRI center. If no decline or an increase were present, the vertical axis was set at 0. All centers experienced a significant drop in the number of the T2* MRIs in the four-month period March-June (from 75 to 100%). In the three-month period July-September only the centers of Pisa and Taranto dropped the number of T2* MRIs due to the rescheduling of the other MRI appointments deleted during the lockdown. In the three-month period October-December a reduction of the T2* MRI scans was experienced by all centers, except for Campobasso. In the centers of Ferrara and Lamezia Terme the reduction was the consequence of the general reduction in the number of the total MRIs scheduled per day for the sanitation procedures. In the other centers, the availability for T2* MRI scans was unchanged in comparison to 2019, but the patients refused the MRI follow up for fear of getting sick of COVID. Conclusion The COVID-19 pandemic is having a strong negative impact on the quantification of iron overload by MRI, which may seriously worsen the prognosis of patients with hemoglobinopathies. Strategies to ensure proved lifesaving MRI exam and to reassure patients about the health safety of the hospitals are recommended. Abstract Figure 1

The aim of the present study was to assess the cultivable microbiota of “mothers” of Vino cotto collected from production of different years 1890, 1895, 1920, 1975, 2008. A total of 73 yeasts and 81 bacteria were isolated. Starmerella lactis-condensi, Starmerella bacillaris, Hanseniaspora uvarum, Saccharomyces cerevisiae, Hanseniaspora guillermondi and Metschnikowia pulcherrima were identified. Bacteria isolates belonged to lactic acid bacteria (Lactiplantibacillus plantarum and Pediococcus pentosaceus) and acetic acid bacteria (Gluconobacter oxydans). Remarkable biodiversity was observed for Starm. bacillaris, as well as L. plantarum and G. oxydans. Organic acids and volatile compounds were also determined. Malic and succinic acids were the main ones with values ranging from 8.49 g/L to 11.76 g/L and from 4.15 g/L to 7.73 g/L respectively, while citric acid was present at low concentrations (<0.2 g/L) in all samples. Esters and higher alcohols were the main volatile compounds detected followed by alkanes. This study permits to better understand the microbial communities associated to this product and could be considered a starting point for the definition of tailored starter cultures to improve the quality of Vino cotto preserving its typical traits.

Abstract Funding Acknowledgements Type of funding sources: Private company. Main funding source(s): The MIOT project receives “no-profit support” from industrial sponsorships (Chiesi Farmaceutici S.p.A., ApoPharma Inc.). Background. Sickle β-thalassemia (Sβ-thal) is a hereditary hemoglobinopathy resulting from the combined heterozygosity for sickle cell and β-thalassemia genes. Cardiac involvement in Sβ-thal patients has been poorly investigated. Aim. We aimed to evaluate myocardial iron overload and cardiac function by cardiovascular magnetic resonance (CMR) in patients with Sβ-thal. Methods. One hundred and eleven Sβ-thal patients consecutively enrolled in the Myocardial Iron Overload in Thalassemia (MIOT) network were studied and compared with 46 sickle cell disease (SCD) patients. Biatrial and biventricular function CMR parameters of Sβ-thal patients were compared with those of 111 healthy volunteers, matched by gender and age. Myocardial iron overload (MIO) was assessed by T2* technique. Cine images were acquired to quantify biventricular function. Macroscopic myocardial fibrosis was evaluated by late gadolinium enhancement (LGE) technique. Results. In Sβ-thal and SCD patients morphological and functional MR parameters were not significantly different, except for left atrial area and SVI (p = 0.023 and p = 0.048, respectively) that were significantly higher in SCD patients. No significant differences between the two groups were found in terms of myocardial iron overload and macroscopic myocardial fibrosis. When compared to healthy subjects, Sβ-thal patients showed significantly higher bi-atrial and biventricular parameters except for LVEF that was significantly lower (Fig.1). Conclusions. The CMR analysis confirmed that Sβ-thal and SCD patients are phenotypically similar. Since Sβ-thal patients showed markedly different morphological and functional indices from healthy subjects, it would be useful to identify Sβ-thal/SCD-specific bi-atrial and biventricular reference values.

Type of funding sources: None. Systemic sclerosis (SSc) is a connective tissue disease characterized by diffuse vascular lesions and fibrosis, also affecting the heart. Cardiovascular magnetic resonance (CMR) can detect replacement myocardial fibrosis by late gadolinium enhancement (LGE) and interstitial myocardial fibrosis/edema by T1 mapping techniques. To evaluate the prevalence of cardiac involvement by native T1 mapping and its correlation with clinical and CMR parameters in SSc patients. Fifty-one consecutive SSc patients (mean age 51.8 ± 13.7 years, 42 females) and 51 healthy subjects matched for age and sex underwent clinical, bio-humoral assessment, and CMR at 1.5T (Signa Artist, GE Healthcare ). The imaging protocol included: cine, T1 mapping by MOLLI, T2 mapping by multi-echo fast-spin-echo sequence, LGE, and STIR T2-weighted sequences. Native T1 and T2 values were assessed in all 16 myocardial segments and the global value was the mean. Results. Global native T1 values were significantly higher in SSc patients than in healthy subjects (1076.4 ± 50.7 vs 1033.3 ± 31.9 ms; P < 0.0001). As in healthy subjects, in patients native T1 values were significantly lower in males than in females (1033.4 ± 38.3 vs 1085.6 ± 48.6 ms; P = 0.004) and inversely correlated with age (R=-0415; P = 0.002). Twenty-three (45.1%) patients had an increased global heart T1 value (>1060 ms in males and >1085 ms in females). Of them, 14 patients (60.9 %) showed positive LGE. Frequency of cardiovascular risk factors, indices of disease activity and chronicity, biochemical parameters, and cardio-active therapy were comparable between patients with normal and elevated T1. Compared to patients with normal T1 value, patients with elevated T1 had significantly higher left ventricular (LV) end-diastolic volume index (76.8 ± 13.3 vs 69.2 ± 11.8, P = 0.050), LV stroke volume index (49.7 ± 6.4 vs 44.4 ± 6.9 ml/m2; P = 0.010), LV cardiac output (3.6 ± 0.5 vs 3.0 ± 0.6 l/min /m2; P < 0.0001), and global heart T2 values (60.1 ± 3.6 vs 55.7 ± 3.1 ms; P < 0.0001). Replacement myocardial fibrosis was detected in 24 (47.1%) patients and they showed significantly higher global heart native T1 values (Figure 1A). Positive T2-weighted images for myocardial oedema were found in 5 (9.8%) patients, all with increased global heart native T1 value. Patients with oedema had significantly higher native global heart T1 values (Figure 1B). Elevated native T1 values measured by CMR are frequent in SSc patients and they are associated with inflammation, replacement fibrosis, and increased LV dimension. CMR T1 mapping seems to be a sensitive parameter to include in the routine clinical assessment of SSc patients for detecting earlier pejorative cardiac involvement, although prospective data are recommended. Abstract Figure.

Abstract Introduction Cardiac involvement in systemic sclerosis (SSc) accounts for 26–36% of deaths. This most frequently manifests as ventricular rhythm disturbances (VRDs), eventually culminating in sudden cardiac death. However, no specific guidelines exist for implantation of cardioverter defibrillators (ICD) in SSc patients. Parametric cardiovascular magnetic resonance (CMR) indices of myocardial oedema and fibrosis like native T1/T2 mapping have been shown to be associated with prognosis in SSc patients with acute cardiac events and normal echocardiograms. However, their relationship with arrhythmogenicity per se has not been previously investigated in SSc. Objectives To investigate the relationship between parametric CMR indices and arrhythmogenicity in SSc patients. Methods 84 consecutive SSc patients (80% diffuse-cutaneous SSc) from eight European centers presenting with cardiac symptoms were examined using a 1.5 T CMR system. 24h Holter recordings were obtained within a month of the CMR scan. The presence of VRDs was defined as any type of premature ventricular contraction (PVC) in couples, triplets, bigeminism, trigeminism, quadrigeminism and non-sustained ventricular tachycardia, as well as having >30 PVCs per hour. Logistic regression analysis was used to evaluate the relationship between VRD occurrence and native T1/T2 mapping as well as myocardial extracellular volume fraction (ECV). Results Mean age in the cohort was 55 (13) years and 78 (93%) patients were female. Of these, 67 (80%) experienced at least one type of VRDs. Each 10 ms increase of native T1-mapping was associated with a higher occurrence of VRDs [odds ratio (95% confidence interval): 1.21 (1.08–1.36), p=0.001]. Similarly, a 1% increase in ECV conferred an increased probability of experiencing VRDs [1.25 (1.01–1.53), p=0.037]. Lastly, a 1ms unit increase in T2-mapping also led to increased probability of having experienced VRDs [1.09 (1.01–1.19), p=0.035]. Conclusion Parametric CMR indices are associated with arrhythmogenicity in SSc patients with cardiac symptoms and should be investigated further in larger studies for their clinical utility in selecting high-risk SSc patients for ICD implantation. Funding Acknowledgement Type of funding source: None

Background:Cardiac involvement in systemic sclerosis (SSc) accounts for 26-36% of deaths. This most frequently manifests as ventricular rhythm disturbances (VRDs), eventually culminating in sudden cardiac death. However, no specific guidelines exist for implantation of cardioverter defibrillators (ICD) in SSc patients. Parametric cardiovascular magnetic resonance (CMR) indices of myocardial oedema and fibrosis like native T1/T2 mapping have been shown to be associated with prognosis in SSc patients with acute cardiac events and normal echocardiograms. However, their relationship with arrhythmogenicity per se has not been previously investigated in SSc.Objectives:To investigate the relationship between parametric CMR indices and arrhythmogenicity in SSc patients.Methods:84 consecutive SSc patients (80% diffuse-cutaneous SSc) from eight European centers presenting with cardiac symptoms were examined using a 1.5 T CMR system. 24h Holter recordings were obtained within a month of the CMR scan. The presence of VRDs was defined as any type of premature ventricular contraction (PVC) in couples, triplets, bigeminism, trigeminism, quadrigeminism and non-sustained ventricular tachycardia, as well as having >30 PVCs per hour. Logistic regression analysis was used to evaluate the relationship between VRD occurrence and native T1/T2 mapping as well as myocardial extracellular volume fraction (ECV).Results:Mean age in the cohort was 55 (13) years and 78 (93%) patients were female. Of these, 67 (80%) experienced at least one type of VRDs. Each 10 ms increase of native T1-mapping was associated with a higher occurrence of VRDs [odds ratio (95% confidence interval): 1.21 (1.08-1.36), p=0.001]. Similarly, a 1% increase in ECV conferred an increased probability of experiencing VRDs [1.25 (1.01-1.53), p=0.037]. Lastly, a 1ms unit increase in T2-mapping also led to increased probability of having experienced VRDs [1.09 (1.01-1.19), p=0.035].Conclusion:Parametric CMR indices are associated with arrhythmogenicity in SSc patients with cardiac symptoms and should be investigated further in larger studies for their clinical utility in selecting high-risk SSc patients for ICD implantation.Disclosure of Interests:Sophie I. Mavrogeni: None declared, Luna Gargani: None declared, Alessia Pepe: None declared, Lorenzo Monti: None declared, George Markousis-Mavrogenis: None declared, Maria De Santis: None declared, Antonella Meloni: None declared, Loukia Koutsogeorgopoulou: None declared, Georgia Karabela: None declared, Efthymios Stavropoulos: None declared, Gkikas Katsifis Grant/research support from: UCB Pharma, Janssen, Abbvie, Novartis, MSD, Aenorasis, Genesis Pharma, Pfizer, Roche, Consultant of: UCB Pharma, Janssen, Abbvie, Novartis, MSD, Aenorasis, Genesis Pharma, Pfizer, Roche, Speakers bureau: UCB Pharma, Janssen, Abbvie, Novartis, MSD, Aenorasis, Genesis Pharma, Pfizer, Roche, Konstantinos Bratis: None declared, Silvia Bellando Randone: None declared, Serena Guiducci: None declared, Cosimo Bruni: None declared, Alberto Moggi-Pignone: None declared, Theodoros Dimitroulas: None declared, Paraskevi Voulgari: None declared, Genovefa Kolovou: None declared, Vasiliki-Kalliopi Bournia Grant/research support from: Travel Grant from Boehringer Ingelheim, Monica Mukherjee: None declared, Joao Lima: None declared, George D. Kitas: None declared, Petros Sfikakis Grant/research support from: Grant/research support from Abvie, Novartis, MSD, Actelion, Amgen, Pfizer, Janssen Pharmaceutical, UCB, Marco Matucci-Cerinic Grant/research support from: Actelion, MSD, Bristol-Myers Squibb, Speakers bureau: Acetelion, Lilly, Boehringer Ingelheim

Abstract Background Malignant arrhythmias due to cardiac involvement are a frequent cause of death in systemic sclerosis (SSc). Cardiac involvement, which is linked to both ischaemic and non-ischaemic fibrotic deposition, is often subclinical. Cardiovascular magnetic resonance (CMR) is the non-invasive gold standard for myocardial tissue characterization and can detect macroscopic myocardial fibrosis through late Gadolinium enhancement (LGE). Aim To evaluate the role of LGE to predict malignant arrhythmias requiring implantable cardioverter defibrillator (ICD) in SSc. Methods 289 SSc patients underwent a thorough clinical evaluation and CMR exam using a 1.5 T scanner. Biventricular function parameter by SSFP cine images, oedema by STIR T2 images, and macroscopic fibrosis by LGE were assessed. Patients were followed-up and malignant ventricular arrhythmias requiring ICD implantation was considered as event. Results Out of 289 patients, 111 (38.4%) showed LGE and 83/111 (28.7% of the total population) showed a non-junctional distribution. During the follow-up (45±27 months), 10 patients needed ICD after malignant ventricular arrhythmias (7 patients with LGE, 3 patients without LGE). CMR predictors of cardiac events by univariate analysis were left and right ventricular ejection fractions, indexed right atrial area and non-junctional LGE. At multivariate analysis, macroscopic myocardial fibrosis detected by LGE was an independent predictor (hazard ratio 5.4; 95% C.I. 1.1–28.8, p<0.05; see figure for Kaplan-Meier curves). Conclusions Presence of ventricular LGE at CMR may represent an independent predictor for further malignant ventricular arrhythmias requiring ICD implantation in SSc patients. Kaplan-Meier curves Funding Acknowledgement Type of funding source: None

Background: Transfusion-dependent-thalassemia (TDT) is the most severe clinical presentation of β-thalassemia. It is characterized by extremely diverse clinical manifestations, further complicated by the inclusion in TDT group of the patients with TI patients who started regular transfusion in adult age. Determination of factors causing such a diverse clinical presentation has clinical significance. Aims: Our study aimed to investigate if the presence of a β°/β° homozygous genotype was associated to increased iron overload and rate of complications. Methods: We considered 747 TDT patients enrolled in the Myocardial Iron Overload in Thalassemia (MIOT) project. Magnetic Resonance Imaging (MRI) was used to quantify iron overload (T2∗ technique), biventricular morphological and functional parameters (cine sequences), and the presence of myocardial fibrosis (late gadolinium enhancement-LGE technique). All complications were classified according to international guidelines. Results: Two groups of patients were identified: non homozygous β°/β° genotype (N = 493) and homozygous β°/ β° genotype (N = 254). No significant differences for sex was found between the groups while the homozygous β°/β° group was significantly older (30.8 ± 8.9 yrs vs 29.1 ± 9.4 yrs; P = 0.009) and showed a significant higher frequency of transfusions in the past 12 months (41.7 ± 11.8 vs 37.4 ± 11.2; P < 0.0001). Mean haemoglobin levels, serum ferritin levels, liver transaminases and MRI liver iron concentration (LIC) values were comparable between the groups. Patients with homozygous β°/β° genotype had a significant higher frequency of liver fibrosis, also adjusting for age (12.4% vs 3.2%; P = 0.015). The homozygous β°/β° group showed significantly lower global heart T2∗ values (26.6 ± 13.0 ms vs 30.1 ± 11.6 ms; P = 0.001) and a higher number of patients with a global heart T2∗ value<20 ms (33.1% vs 21.3%; P < 0.0001). Left ventricular (LV) stroke volume index was significantly lower in the homozygous β°/β° group, also adjusting for age and number of transfusions (52.1 ± 10.1 ml/m2 vs 54.4 ± 11.7 ml/m2; P = 0.001). No difference between groups was detected in terms of frequency of diabetes, osteoporosis, hypogonadism, and hypoparathyroidism while frequency of hypothyroidism was significantly higher in the homozygous β°/β° group, also adjusting for age (23.4% vs 13.5%, P = 0.004) Frequency of heart failure was comparable between groups while the frequency of arrhythmias was significantly higher in the homozygous β°/β° group (7.8% vs 1.9%; P = 0.001). Summary/Conclusion: Patients with β°/β° homozygous genotype had more cardiac iron and showed an higher frequency of liver fibrosis, hypothyroidism, and arrhythmias. These data support the knowledge of different phenotypic groups in the management of TDT patients.

Background:Patients with non‐transfusion dependent thalassaemia (NTDT) can be placed on regular transfusional therapy either for the prevention or for the management of different complications.Aims:We retrospectively evaluated the effects of blood transfusions (BT) in the real‐life and quite extensive context of the Myocardial Iron Overload in Thalassaemia (MIOT) network where most of biochemical, cardiac, hepatic parameters and morbidities typical of the NTDT patients could be analysed and compared in a chronologically manner.Methods:147 NTDT patients (46.49 ± 12.27 years; 92 females) who started regular BT at the mean age of 21.84 ± 17.85 years were considered. Magnetic Resonance Imaging (MRI) was used to quantify iron overload (T2∗ technique) and biventricular morphological and functional parameters (cine sequences).Results:For the 40% of the patients there were more than two indications for the transition to regular BT, with anemia being present in the 92.5% of the cases. The patients reached a pre‐transfusion hemoglobin of 9.64 ± 0.72 g/dl.The percentage of chelated patients increased significantly after starting regular BT (52.5% vs 91.7%; P < 0.00001).Table 1 shows the changes (6 months before vs 6 months after starting regular BT) in the haematochemical and iron‐overload parameters. A down‐regulation of all erythropoietic and/or haemolysis indices and a drop‐in platelets and white blood cell count were detected. No changes in serum ferritin, MRI liver iron concentration (LIC) and global heart T2∗ values were detected.There was a significant decrease of the left ventricular end‐diastolic volume index (101.58 ± 19.67 ml/m2 vs 91.65 ± 20.99; P = 0.050).After the start of regular BT, patients were followed‐up for 24.08 ± 15.62 years. There was a significant increase in the frequency of endocrinopathies (diabetes, hypogonadism, hypothyroidism and osteopenia), bone pain, infections, and alloimunization, but their prevalence remained lower than 20%.A positive impact on patients’ health‐related quality of life was detected, with the sense of well‐being passing from 16.4% to 73.0% (P < 0.0001).Summary/Conclusion:Following the use of regular BT and chelation therapy, only a mild increase in most of the complications evaluated in the analysis was recorded; we cannot exclude that it could be ascribable to aging per se, as it happens for general population. No increase in iron overload parameters was detected and conversely, we recorded a positive cardiac remodelling. Therefore, in the Italian context, the decision to regularly transfuse patients with NTDT, despite sometimes complex and problematic, may represent a way to prevent or slow down the natural progression of the disease and improve the patients overall quality of life.image