Abstract We report the case of a 48–year–old female with a special history of ischemic heart disease. It started in 2021 with an acute anterior ST myocardial infarction: the coronary angiography showed subocclusive stenosis at the first tract of the left anterior descending (LAD), without other coronary stenoses. The patient was treated with primary PCI + DES; the echocardiogram showed EF 30% and akinesia of apex, intermediate segments of anterior wall and interventricular septum. During the same hospitalization the patient experienced recurrence of angina with transient ST elevation in the anterior and inferior leads; a control coronary angiography was unchanged. She was started on therapy with nitrates and diltiazem up to the tolerated dose, statin as well as antiplatelet and anticoagulant therapy due to apical thrombus. She underwent ICD implantation in primary prevention in January 2022. During 2022, there were several accesses to the ED for vasospastic angina despite medical therapy at the maximum tolerated dose. In October 2023 the patient experienced an acute chest pain followed by cardiac arrest, pulseless electrical activity and acute cardiogenic shock. After resuscitation procedures, she underwent an emergency coronary angiography which showed excellent result of the previous PCI, as well as stenosis of the distal edge of the stent resolving after injection of i.c. nitrates. No arrhythmic events at ICD recording and continuous electrocardiographic monitoring in ICU were observed. Cardiac MRI showed areas of subendocardial LGE involving the apex, anterior, anterolateral and septal segments due to ischemic scar; we also observed areas of edema and LGE with an ischemic pattern at the infero–septal and inferior wall in toto, suggesting recent ischemia; EF was 31%. During hospitalization the patient experienced episodes of typical angina with poor response to medical therapy at the maximum tolerated dose. The current clinical case most likely points to acute myocardial infarction due to vasospasm of the right coronary artery with acute pump failure resulting in cardiac arrest and no specific recommendations exist in recent cardiologic guidelines on the strategy to adopt for this acute clinical setting. In the hypothesis of a vasospastic pathogenesis of the problem, counseling for modulation of the stellate ganglion was requested.

Abstract We report the case of a 51 year old male, smoker of about 20 cigarettes/day and with history of uninvestigated headache. In March 2023 he arrived in the Emergency Department for oppressive retrosternal chest pain radiating to the left arm; the electrocardiogram (ECG) showed sinus rhythm, no acute ST segment changes; HS troponin T was elevated. Transthoracic echocardiogram showed 55% EF, no kinesis defects, no major valvulopathy, neither pericardial effusion. The patient was admitted to the ICU with a diagnosis of NSTE myocardial infarction. Despite on admission HS troponin T HS was further increased and ECG evolved with inferior and lateral subepicardial ischemia, coronary angiogram showed normal coronary arteries, with an area of haziness in the right coronary artery. To investigate the etiology of myocardial damage the patient underwent cardiac MRI, that showed a left ventricle with normal size and mildly reduced systolic function; inferior apical and septal segment were akinetic; right ventricle was normal with preserved systolic function; edema and fibrosis with ischemic pattern was seen in the inferior apical and septal segment. Then we looked for embolic sources: continuous ECG monitoring excluded supraventricular arrhythmias. We performed echocardiogram that raised suspicion of a patent foramen ovale (PFO) confirmed on transesophageal contrast echocardiogram. Brain MRI showed no obvious changes compatible with recent ischemic lesions; few millimeters focal hyperintense areas in T2 and FLAIR, of gliotic nonspecific significance were reported in the frontal subcortical white matter bilaterally, left parietal and in the right corona radiata. We concluded for diagnosis of paradoxical coronary embolism and the patient was treated with percutaneous closure of PFO: by right femoral vein route and under intracardiac ultrasound guidance, closure of PFO was performed with Amplatzer No. 25 with excellent fluoroscopic and final ultrasound result. The broad spectrum of the possible cause of myocardial injury in MINOCA makes defining the extact underlying etiology challenging, but correctly knowing the culprit mechanism improves therapies and outcome; paradoxical coronary embolism due to a patent foramen ovale is rarely diagnosed; in our clinical case integrated multimodality imaging diagnostic work up, proved highly effective in identifying the cause of the myocardial damage and allowed the appropriate treatment.

Seven-hundred and nine patients with thalassemia major who performed a baseline and a 1st follow-up CMR scan after 18 months were followed prospectively in order to evaluate the predictive value of changes in CMR parameters (myocardial iron, biventricular function, and replacement myocardial fibrosis) for cardiac complications. During a mean follow-up of 89.4±33.3 months, cardiac events (heart failure, arrhythmias, and pulmonary hypertension) were recorded in 7.1% of patients. In the univariate Cox regression analysis, cardiac iron clearance and replacement myocardial fibrosis were identified as univariate prognosticators but in the multivariate analysis only myocardial fibrosis remained an independent predictor factor.

The complexity of cardiovascular diseases has led to an extensive use of technological instruments and the development of multimodality imaging. This extensive use of different cardiovascular imaging tests in the same patient has increased costs and waiting times. The concept of appropriateness has changed over time. Appropriateness criteria address the need for specific cardiovascular imaging tests in well-defined clinical scenarios, and define the kind of cardiovascular imaging that is appropriated for each clinical scenario in different stages of the disease. The concept of appropriateness criteria has replaced the old idea of appropriate use criteria and reflects the increasing effort of the international Scientific Societies to create and review in a critical way the management of diagnostic tests used by clinicians. The aim of this Italian consensus document is to address the use of multimodality imaging in the diagnosis and management of the major cardiovascular clinical scenarios, taking into consideration not only the international guidelines and scientific documents already published, but also the reality of Italian laws as well as the various professional profiles involved in patient management and availability of technological diagnostic instruments.

18. Le patologie cardiovascolari, sempre più complesse e diffuse, hanno portato allo sviluppo di nuove e performanti tecniche di imaging cardiovascolare. Purtroppo, il numero di procedure di imaging sta aumentando in maniera esponenziale portando ad un incremento dei costi e delle liste d’attesa. Da qui uno dei razionali alla base della necessità di definire l’utilizzo appropriato dell’imaging cardiovascolare. Il concetto di appropriatezza applicato alla cura della salute mette sul piatto della bilancia il rischio e il beneficio di un trattamento, di un test diagnostico o di una procedura nell’ambito delle risorse disponibili per un determinato paziente con specifiche caratteristiche. I criteri di appropriatezza dovrebbero fornire lo strumento adatto a coadiuvare il clinico nel giudicare se un paziente è un ragionevole candidato per eseguire un determinato trattamento, test diagnostico o procedura. Il concetto di appropriatezza successivamente è stato sostituito da quello dell’uso appropriato dei criteri (di appropriatezza) che rappresenta il crescente sforzo delle Società Scientifiche di creare, rivedere e sistematizzare in modo critico ed organico tutte quelle situazioni cliniche nelle quali i test diagnostici e le procedure sono utilizzate dai medici curanti per i pazienti con patologie cardiovascolari.Questo documento di consenso delle maggiori Società Scientifiche italiane è nato tenendo conto non solo delle linee guida internazionali e dei documenti scientifici pubblicati, ma anche della realtà clinica e legislativa italiana oltre che dei vari profili professionali coinvolti nell’iter gestionale dei pazienti.

Abstract Introduction The MIOT (Myocardial Iron Overload in Thalassemia) Network was a network of thalassemia and CMR centers built in 2006 in order to assure homogeneous and standardized cardiac iron overload assessment for a significant number of patients. Purpose We describe the impact of this ten-year Network on cardiac iron, complications and deaths in patients with thalassemia major (TM). Methods 1746 TM patients (911 F; age 31.17±9.09 yrs) were enrolled in the MIOT Network. Myocardial iron overload (MIO) was quantified by the multislice multiecho T2* technique. Biventricular function was quantified by cine images. Results 1392 TM patients performed an end-of-study CMR. At the last CMR significantly higher global heart T2* values (35.44±10.69 ms vs 29.16±12.02 ms; P<0.0001) and a significant lower number of patients with global heart T2*<20 ms (26.3% vs 12.0%; P<0.0001) were detected. Four patterns of MIO were identified: no MIO (all segments with T2*≥20 ms), heterogeneous MIO and global heart T2*≥20 ms, heterogeneous MIO and global heart T2*<20 ms, and homogeneous MIO (all T2*<20 ms). At the last CMR a significant higher frequency of patients with no MIO and a significant lower frequency for the other three patterns indicating MIO were found (Figure 1). In patients with global heart T2*<20 ms a significant increase in left ventricular ejection fraction (EF) (difference: 3.2±8.5%, P<0.0001) as well as in right ventricular EF (difference: 1.2±8.9%, P=0.002) were detected. Based on CMR results the 75% of the patients changed the chelation therapy. At the last CMR the percentage of patients with an excellent/good compliance was significantly higher (94.8% vs 92.2%%; P<0.0001). The complete history of cardiac complications-CC (heart failure, arrhythmias, pulmonary hypertension, myocardial infarction, angina, myo/pericarditis, peripheral vascular disease) was present for 1062 patients. Out of the 1001 patients with resolved CC or without CC before the enrolment in the project, the 6.6% had a CC before the enrolment in the project. During the study, the frequency of CC was 4.4%, significantly lower (P=0.023). In particular, the frequency of heart failure (HF) was significantly lower (3.5% vs 0.8%, P<0.0001). Forty-six patients died during the study. HF continues to be the leading cause of death (30.4% of all causes), but there was a consistent decline in HF mortality rate, that was 60.2% in an Italian study dated 2004. No patients died for arrhythmias while cancer was the second leading cause of death. Conclusion Over a period of 10 years, the continuous monitoring of cardiac iron levels and a tailored chelation therapy allowed a reduction of MIO in the 70% of patients, with consequent improvement of cardiac function and reduction of cardiac complications and mortality from MIO-related HF. So, a national networking was effective in improving the care and reducing cardiac outcomes of TM patients.

In non transfusion dependent thalassemia (NTDT) the lack of a clear genotype-phenotype relationship complicates the already complex and extensive scenario in clinical practice. Our aim was to detect if the presence of a β°/β° homozygous genotype was associated to different rate of cardiac findings by Cardiovascular Magnetic Resonance (CMR) and cardiac complications. We considered 81 patients with thalassemia intermedia never transfused o who received occasional transfusions (37.7±11.4 years, 39 females) consecutively enrolled in the Myocardial Iron Overload in Thalassemia project. CMR 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 cardiac complications were classified according to international guidelines. Two groups of patients were identified: non homozygous β°/β° genotype (N=61) and homozygous β°/β° genotype (N=20.) No significant differences for sex and age were found between the groups. Patients with homozygous β°/β° genotype had lower mean haemoglobin levels (8.6±1.1g/dl vs 9.2±1.2 g/dl) but the difference did not reach the statistical significance (P=0.060). No patient showed cardiac iron and global heart T2* values were comparable between the two groups. Left atrial area index, left ventricular (LV) end-diastolic, end-systolic and stroke volume indexes, LV mass index, right ventricular end-diastolic and end-systolic volume indexes were significantly higher in the homozygous β°/β° group (see Table). Frequencies of heart failure and arrhythmias were comparable between the groups. Non-β0/β0 homozygous genotype β0/β0 homozygous genotype P Left Atrial Area (cm2/m2) 13.87±2.59 16.63±2.59 0.001 LV EDVI (ml/m2) 94.88±15.59 112.94±21.52 0.003 LV ESVI (ml/m2) 35.22±9.55 41.88±9.70 0.018 LV SVI (ml/m2) 61.98±12.57 69.81±11.20 0.029 LV mass index (g/m2) 61.26±10.15 68.63±15.89 0.030 RV EDVI (ml/m2) 91.27±23.50 107.53±23.87 0.019 RV ESVI (ml/m2) 33.93±17.55 40.81±15.20 0.043 Heart remodelling related to a high cardiac output state cardiomyopathy was more pronounced in patients with homozygous β°/β° genotype. These data can support the knowledge of different phenotypic groups in the management of NTDT patients.

Abstract Background Some preliminary data have postulated a correlation between pancreatic iron overload and heart iron and function in thalassemia major (TM) patients. Purpose In the present multicenter study we explored systematically the link between pancreatic iron and heart disease in a large cohort of TM patients. Methods We considered 880 TM patients (467 M, mean age 37.83±10.05 years) enrolled in the E-MIOT (Extension-Myocardial Iron Overload in Thalassemia) project. T2* measurements were performed over pancreatic head, body and tail and global value was the mean. Myocardial iron overload (MIO) was quantified using a T2* segmental approach. Biventricular function parameters were assessed by cine images. Late gadolinium enhancement (LGE) images were acquired to detect myocardial fibrosis. Results A significant correlation between pancreatic and cardiac iron was reconfirmed in this more numerous population and a normal pancreas T2* showed negative predictive value of 100% for cardiac iron. LGE sequences were acquired in 273 TM patients and 84 (30.77%) of them showed macroscopic myocardial fibrosis. Global pancreas T2* values were significantly lower in patients with fibrosis (7.38±6.19 ms vs 11.91±9.79 ms; P<0.0001). Sixty-four patients had at least one cardiac complication (arrhythmias, heart failure, pulmonary hyperthension, vascular disease). Patients with cardiac complications showed a significant lower global pancreas T2* (7.83±5.61 ms vs 12.76±10.34 ms; P=0.024). Specifically, global pancreas T2* values were significantly lower in patients with heart failure as well as in patients with arrhythmias and all patients with one of these two diseases had a pathologic global pancreas T2* value (see Figure). Conclusion Pancreatic iron is a strong predictor not only for cardiac iron, but also for cardiac complications supporting a more profound link between pancreatic iron and heart disease in TM.

Aims. The aim of this prospective and multicentre study was toassess the predictive value of traditional and non traditional cardiovascular risk factors (CRF) and Cardiovascular Magnetic Resonance (CMR) parameters for vascular events (VE) and cardiac complications (CC) in thalassemia major (TM) patients.

Abstract Introduction: Cardiovascular Magnetic Resonance (CMR) has an established role in managing and predicting prognosis of patients with Thalassemia Major (TM). Thalassemia Intermedia (TI) is a milder variant of beta-thalassemia showing a different clinical and prognostic profile; pulmonary hypertension (PH) is a more common complication in TI patients. We prospectively determined the predictive value of CMR parameters, including measurement of right ventricular mass, for cardiac complications in TI. Methods: We considered 342 TI patients enrolled in the Myocardial Iron Overload in Thalassemia network; about half of them (178/302, 58.9%) were transfusion-dependent. Myocardial and liver iron overload were measured by T2* multiecho technique. Atrial dimensions, left and right ventricular mass and systolic function were quantified by cine images. Late gadolinium enhancement (LGE) images were acquired to detect myocardial fibrosis. Results: Twenty-three patients were excluded because a cardiac complication was present at the time of first CMR, so we prospectively followed 319 patients. All 319 patients were white, with a mean age at time of their first scan of 38.02±11.69 years and 165 (51.7%) of them were females. Mean follow-up time was 52.24±24.87 months (median 54.64 months). Cardiac events were recorded in 22 patients (6.9%): heart failure (HF) in 1 patient, arrhythmias in 12 patients, pulmonary hypertension (PH) in 7 patients and myocardial infarction (MI) in 2 patients. Due to the low number of events, only arrhythmias, PH and cardiac complications globally considered were taken as cardiac outcomes for univariate and multivariate analysis. In the multivariate analysis RV hypertrophy was the only independent predictive factor for arrhythmias (HR=33.83, 95% CI=6.07-188.74, P<0.0001) and PH (HR=73.33, 95% CI=10.00-537.57, P<0.0001). When cardiac complications were considered all together, RV hypertrophy (HR=24.12, 95% CI=5.09-114.12, P<0.0001) and myocardial fibrosis by LGE (HR=6.59, 95% CI=1.33-32.67, P=0.021) were independent prognostic factors in the multivariate analysis. The Figures display the Kaplan-Meier curves showing the impact of the independent predictive factors on each outcome. Conclusions: For the first time we studied the prognostic value of right ventricular mass as part of multiparametric CMR imaging in a population of TI patients. RV hypertrophy identified patients at high risk for arrhythmias and PH. Both RV hypertrophy and fibrosis detected by LGE were independent predictive factor for cardiac complications. Measurement of RV mass should be part of the multi-parametric CMR study of patient with thalassemia intermedia. Figure 1. Figure 1. Disclosures Pepe: ApoPharma Inc: Speakers Bureau; Novartis: Speakers Bureau; Chiesi: Speakers Bureau.

AIMSPatients with acute coronary syndromes (ACSs) who are managed without coronary revascularization represent a mixed and understudied population that seems to receive suboptimal pharmacological treatment.METHODS AND RESULTSWe assessed patterns of antithrombotic therapies employed during the hospitalization and in-hospital clinical events of medically managed patients with ACS enrolled in the prospective, multicentre, nationwide EYESHOT (EmploYEd antithrombotic therapies in patients with acute coronary Syndromes HOspitalized in iTalian cardiac care units) registry. Among the 2585 consecutive ACS patients enrolled in EYESHOT, 783 (30.3%) did not receive any revascularization during hospital admission. Of these, 478 (61.0%) underwent coronary angiography (CA), whereas 305 (39.0%) did not. The median GRACE and CRUSADE risk scores were significantly higher among patients who did not undergo CA compared with those who did (180 vs. 145, P < 0.0001 and 50 vs. 33, P < 0.0001, respectively). Antithrombotic therapies employed during hospitalization significantly differ between patients who received CA and those who did not with unfractioned heparin and novel P2Y12 inhibitors more frequently used in the first group, and low-molecular-weight heparins and clopidogrel in the latter group. During the index hospitalization, patients who did not receive CA presented a higher incidence of ischaemic cerebrovascular events and of mortality compared with those who underwent CA (1.6 vs. 0.2%, P = 0.04 and 7.9 vs. 2.7%, P = 0.0009, respectively).CONCLUSIONAlmost one-third of ACS patients are managed without revascularization during the index hospitalization. In this population, a lower use of recommended antiplatelet therapy and worse clinical outcome were observed in those who did not undergo CA when compared with those who did.CLINICAL TRIAL REGISTRATIONUnique identifier: NCT02015624, http://www.clinicaltrials.gov.