In the past decade, cardiac magnetic resonance (CMR) has undergone remarkable progress, emerging as a pivotal tool in various cardiological scenarios. Its capacity for tissue characterization, both with and without contrast agents, makes CMR the perfect tool to study the substrate of arrhythmia. This review highlights the potential role of CMR in electrophysiology (EP) and its role in the ablation of atrial and ventricular arrhythmias. First, we will discuss the key aspects of ventricular arrhythmia ablation, while in the second part, we will review how CMR is changing the ablation of atrial arrhythmias. The potentiality of CMR in the pre-procedural, intra-procedural, and post-ablation assessment will be reviewed. In particular, CMR is capable of visualizing fibrosis and building 3D reconstruction. Furthermore, it is possible to merge a 3D-rendered shell of the heart into the EP room to guide radiation-free ablation through active or passive tracking. Finally, the accuracy of CMR in depicting ablation lesions and its ability to predict arrhythmia relapses will be discussed.

Heart failure (HF) is a chronic and progressive disease that often progresses to an advanced stage where conventional therapy is insufficient to relieve patients' symptoms. Despite the availability of advanced therapies such as mechanical circulatory support or heart transplantation, the complexity of defining advanced HF, which requires multiple parameters and multimodality assessment, often leads to delays in referral to dedicated specialists with the result of a worsening prognosis. In this review, we aim to explore the role of cardiac magnetic resonance (CMR) in advanced HF by showing how CMR is useful at every step in managing these patients: from diagnosis to prognostic stratification, hemodynamic evaluation, follow-up and advanced therapies such as heart transplantation. The technical challenges of scanning advanced HF patients, which often require troubleshooting of intracardiac devices and dedicated scans, will be also discussed.

The application of cardiac magnetic resonance (CMR) imaging in clinical practice has grown due to technological advancements and expanded clinical indications, highlighting its superior capabilities when compared to echocardiography for the assessment of myocardial tissue. Similarly, the utilization of implantable cardiac electronic devices (CIEDs) has significantly increased in cardiac arrhythmia management, and the requirements of CMR examinations in patients with CIEDs has become more common. However, this type of exam often presents challenges due to safety concerns and image artifacts. Until a few years ago, the presence of CIED was considered an absolute contraindication to CMR. To address these challenges, various technical improvements in CIED technology, like the reduction of the ferromagnetic components, and in CMR examinations, such as the introduction of new sequences, have been developed. Moreover, a rigorous protocol involving multidisciplinary collaboration is recommended for safe CMR examinations in patients with CIEDs, emphasizing risk assessment, careful monitoring during CMR, and post-scan device evaluation. Alternative methods to CMR, such as computed tomography coronary angiography with tissue characterization techniques like dual-energy and photon-counting, offer alternative potential solutions, although their diagnostic accuracy and availability do limit their use. Despite technological advancements, close collaboration and specialized staff training remain crucial for obtaining safe diagnostic CMR images in patients with CIEDs, thus justifying the presence of specialized centers that are equipped to handle these type of exams.

In the last decade, artificial intelligence (AI) has influenced the field of cardiac computed tomography (CT), with its scope further enhanced by advanced methodologies such as machine learning (ML) and deep learning (DL). The AI-driven techniques leverage large datasets to develop and train algorithms capable of making precise evaluations and predictions. The realm of cardiac CT is expanding day by day and multiple tools are offered to answer different questions. Coronary artery calcium score (CACS) and CT angiography (CTA) provide high-resolution images that facilitate the detailed anatomical evaluation of coronary plaque burden. New tools such as myocardial CT perfusion (CTP) and fractional flow reserve (FFR CT ) have been developed to add a functional evaluation of the stenosis. Moreover, epicardial adipose tissue (EAT) is gaining interest as its role in coronary artery plaque development has been deepened. Seen the great added value of these tools, the demand for new exams has increased such as the burden on imagers. Due to its ability to fast compute multiple data, AI can be helpful in both the acquisition and post-processing phases. AI can possibly reduce radiation dose, increase image quality, and shorten image analysis time. Moreover, different types of data can be used for risk assessment and patient risk stratification. Recently, the focus of the scientific community on AI has led to numerous studies, especially on CACS and CTA. This narrative review concentrates on AI's role in the post-processing of CACS, CTA, FFR CT , CTP, and EAT, discussing both current capabilities and future directions in the field of cardiac imaging.

Background:Left atrial appendage aneurysm (LAAA) is a rare condition mostly due to congenital malformations or secondary causes (i.e. mitral regurgitation). Case summary:We present a case of a 47-year-old male with a history of atrial fibrillation treated with propafenone presented to our emergency department for palpitation and epigastric pain. The electrocardiogram showed atrial fibrillation at high ventricular rate and a new-onset left bundle branch block. Urgent coronary angiogram excluded coronary artery disease. Echocardiography and cardiac magnetic resonance revealed a giant LAAA. The electrocardiogram alterations were deemed secondary to aberrancy and treatment with class IC antiarrhythmic. The patient was discussed in the heart team, and considering his will to avoid surgery, he was managed conservatively with closed follow-up, anticoagulant and antiarrhythmic therapy, and internal loop recorder. At 1-year follow-up, he showed asymptomatic and without arrhythmias. Discussion:Few cases are described in the literature; therefore, there is uncertainty in treatment and prognosis. Diagnosis is achieved with multimodality imaging. Treatment can be surgical with aneurysmectomy or conservative with regular follow-up by imaging examinations and pharmacological therapy aimed to prevent complications such as thrombosis and arrhythmias. Since high-quality scientific data are lacking, shared decision-making is essential for the management of patients affected by LAAA. In our clinical case, our patient's will to not undergo surgery was considered, and therefore, a conservative management with strict follow-up and medications was chosen.

Tricuspid regurgitation (TR) poses a significant healthcare burden and is a major concern for patients who experience debilitating symptoms and face a poorer prognosis. Cardiologists are showing renewed interest in TR, as the previous belief that it was merely a bystander of left-sided heart disease has evolved. As a result, more transcatheter techniques addressing TR are emerging. Although a clear impact on mortality from these transcatheter tricuspid valve interventions (TTVI) has not yet been demonstrated, the improvement in symptoms and quality of life for patients is substantial, leading to increased use of these procedures in clinical practice. In this review, we focus on multimodality imaging as an essential tool for quantifying TR severity, assessing right ventricular (RV) function, understanding the underlying mechanisms, selecting the appropriate intervention, and ensuring thorough and accurate preprocedural planning to minimize complications.

Papillary fibroelastomas (PFs) are small and pedunculated left side valves associated mass, that frequently causing cerebral embolization. We present the case of a 69-year-old male with a history of multiple ischemic strokes and a small pedunculated mass in the left ventricle outflow tract, highly suggestive of a rare case of PF in an atypical localization. Due to the clinical history and the echocardiographic aspect of the mass, he underwent surgical excision and Bentall intervention for concomitant aortic root and ascending aorta aneurysm. The pathological analysis of the surgical specimen confirmed the diagnosis of PF.

Propofol infusion syndrome (PRIS) is a rare but potentially lethal side effect of propofol. In most cases it shows various combinations of signs such as unexplained metabolic acidosis, rhabdomyolysis, hepatomegaly, renal failure, hypertriglyceridemia, malignant arrhythmia and rapidly progressive cardiac failure. The development of coved ST elevation in the right precordial leads of the electrocardiogram (ECG), similar to that seen in the type I Brugada syndrome may be the first sign of cardiac instability. There is no specific treatment for PRIS. Successful management consists of an early recognition of its signs followed by a prompt propofol infusion termination. We present the case of a 35-year-old male affected by mild hypertension. He was found by his wife during a transitory loss of consciousness episode. He had resulted positive to Sars Cov 2 infection a day before and was symptomatic for fever and myalgia. An ambulance was immediately called and the patient was transferred to the emergency department for a suspected out-of-hospital-cardiac arrest. The initial one-lead ECG performed by the emergency physician was unremarkable. On arrival he was in a coma state but with stable hemodynamics. ECG showed only an asymmetric T wave inversion in V4-V6 leads. The cardiac echocardiogram did not show any major alterations. In the meantime, due to worsening of respiratory function, orotracheal intubation was performed and the patient was sedated with propofol, midazolam and fentanyl. Subsequently, an episode of atrial fibrillation was documented. Amiodarone infusion was started and the patient reverted to sinus rhythm after a few hours. The following day two episodes of Torsade de Pointes during prolonged QTc (660 ms) occurred. These arrhythmias were treated successfully with magnesium sulfate infusion. Blood analysis showed severe hypokalemia that was immediately corrected. After the hemodynamic stabilization the ECG showed a pattern highly resembling the Brugada pattern type 1 in the right precordial leads. Moreover CPK, myoglobin, high sensitivity troponin I levels started to rise, along with creatinine, triglycerides and markers of hepatic injury. Propofol had been administered continuously for eight days, so PRIS was suspected as the primum movens of this clinical scenario. Propofol infusion was immediately interrupted. Thereafter, the patient gradually improved and was extubated. As soon as the patient's hemodynamic conditions allowed it, a coronary CT and a cardiac MRI were performed, but were unremarkable. To further evaluate the case, a flecainide challenge test was performed, but no significant ECG change was induced. Nonetheless, given both the history of ventricular arrhythmia, the young age of the patient and the unexplained transitory loss of consciousness a subcutaneous defibrillator was implanted as a form of secondary prevention.

Platypnea orthodeoxia syndrome (POS) is characterized by dispnoea and a fall in oxygen saturation levels when in the upright position that resolves lying down in the supine position. It is secondary to a right to left shunt (R-L shunt), typically trough a patent foramen ovale (PFO). Normally, in the presence of a PFO, R-L shunt does not occur unless there is a clinical condition that raises right side pressures like venous thromboembolism, hydrothorax, pneumothorax or chronic pulmunary hypertension. Although its physiopathological mechanisms are not entirely understood, in the POS, R-L shunt mostly occurs due to anatomical alteration of the interatrial septum in the upright position. Venous flow therefore can pass from the inferior vena cava through the PFO or an atrial septal defect (ASD) to the left side of the heart. We present a case of a 77 y.o. woman with no relevant past medical hystory, that was admitted to hospital care because of a mild pulmonary embolism and deep vein trombosis of the right popliteal vein. She was promptly treated for PE with direct oral anticoagulation achieving the resolution of the embolism. Despite the CT scan showed the complete resolution of the embolism her dysponoea did not improve. Her blood gas analysis showed normocapnic hypoxiemic respiratory insufficiency with a suspicious lowering of oxygen saturation levels when in the upright position which however improved in the supine position. She also had a transient episode of dysarthria and hypostenia to the right upper limb, the negative head CT scan was suggestive of a transient ischaemic attack. A pulmonary scintigraphy showed arterious renal perfusion as in a right to left shunt. She then underwent a transoesophageal echocardiography which showed an hypermobile interatrial septum with evidence of a patent foramen ovale with left to right shunt. The exame was in fact executed in the supine position. Given these findings, we concluded for a diagnosis of POS. Because of this records, the severity of the shunt and the suggestive clinical hystory of ortodeoxya she underwent PFO closure positioning an Amplatzer PFO Occluder 25 mm. The procedure was carried out without complications and led to complete resolution of the symptoms and the signs of platipnoea-ortodeoxya.

Abstract Introduction ST-segment elevation myocardial infarction (STEMI) is one of the most lethal manifestations of ischemic cardiomyopathy. Diagnosis is made through clinical signs, symptoms and, most importantly, the surface ECG. Several conditions mimicking the STEMI repolarization pattern have been hitherto recognised and their identification is of utmost importance in order to provide the correct treatment. Case report A 81-years old man affected by multiple sclerosis and dysphagia was admitted to our hospital for continuous vomiting. His anamnesis was relevant for an episode of bowel obstruction; ECG upon admission exhibited ST-elevation in D1, aVL, V5 and V6 with specular ST-depression in the inferior leads. The patient was unconscious, therefore making it impossible to report any kind of symptoms, in addition, bedside echocardiography showed a thoracic fluid collection completely obscuring cardiac chambers. Physical examination displayed tachypnoea and, most importantly, a tender abdomen with no bowel movements; this specific finding added to the anamnesis and the echocardiography called into doubt the possibility of a real myocardial infarction. Preliminary laboratory findings pointed out high levels of C-reactive protein, procalcitonin and both indirect and direct bilirubin. Being hesitant about the next step, we took a second look at the only clue we had so far, the ECG. At a deeper analysis, the aspects of repolarization did not exactly resemble the convex ST-elevation seen in STEMI presentations, rather reminding (in D1 and aVL) of a pattern called “spiked helmet sign”, described as an index of poor prognosis and increased intrathoracic pressure in critically ill patients. Considered a high suspect of bowel obstruction, a nasogastric tube was placed and, soon after, 3 litres of dark fluid were drained from his stomach. In the following minutes, we witnessed the progressive normalization of the ST alterations initially observed, thus excluding the necessity of a cath lab activation. A CT scan was then performed showing a faecaloma extending from the rectum to the descending colon creating a complete obstruction associated with intestinal pneumatosis and gastric distension. As soon as troponin levels came out normal, the patient underwent surgery, but, unfortunately, he died a few days later due to intervention-related complications. Discussion Although current literature reports several cases of STEMI phenocopies, with a minority referring to bowel obstruction, this is one of the rarely reported circumstances with no instrumental findings or patient symptoms available to support the diagnosis. As most physicians know, diagnoses are rarely straightforward and instrumental findings, especially in the context of an emergency, may drive to incorrect deductions if not properly analysed or incoherent with the clinical context. The “old-fashioned” anamnesis and clinical evaluation may be the keys to unlocking the true diagnosis in difficult contexts where pitfalls can mislead the clinician to the most obvious and quickest conclusion.

Abstract Background left ventricular non-compaction (LVNC) cardiomyopathy is an often underdiagnosed and under-classified disease deriving from the incomplete development of ventricular myocardium. Clinical presentations may be variable and uncommon, ranging from an apparent lack of functional anomalies to heart failure, ventricular arrhythmias and, in some cases, even ischemic stroke. Despite great improvements in diagnostic performance there is still a widespread lack of evidence regarding the prognosis and management of affected patients. Methods all consecutive patients admitted to our Cardiology Institution from October 2009 to August 2022 fulfilling LVNC criteria by echocardiography or cardiovascular magnetic resonance (CMR) or both, were consecutively enrolled. CMR has been performed wherever possible. All patients underwent a complete cardiological visit, a 12-lead ECG and echocardiography at baseline, whereas at follow-up, if a complete visit was not possible, information regarding patients’ endpoints was acquired through telephonic contact. Additional diagnostic exams or implantation of a cardiac device were also performed if indicated. The primary endpoint was a composite of at least one between: sustained ventricular arrhythmias, an appropriate ICD intervention and sudden cardiac death. Secondary endpoints included supraventricular arrhythmias, unplanned cardiac hospitalizations, acute decompensated, chronic heart failure and ischemic stroke. Risk predictor analyses were not performed as the overall event rates were low and the risk for type II error was high. Results forty patients (26 males; age 45±17) were prospectively enrolled and followed up for a median of five years. CMR and echocardiography were overall agreeing on the majority of the diagnoses, with 62.5% of patients meeting the echo criteria and 70% of patients meeting the CMR criteria for LVNC. The incidence of the primary endpoint was 1.8% per years. Male gender and late gadolinium enhancement (LGE) were correlated with an increased incidence of the primary endpoint, while LVEF, NC/C or functional status were not associated with a significantly increased risk of the composite endpoint. HF diagnosis was the most common endpoint (6.1% annual incidence). The annual incidence of supraventricular arrhythmias was 3.0% and the annual incidence of stroke was 0.7%. Twenty-four patients (60%) experienced at least one hospitalization during follow-up. Unplanned hospitalizations represented 20% of all hospitalizations and were mainly HF-related. Planned hospitalizations were performed for elective procedures such as atrial fibrillation cardioversion, ablation, coronary angiography or diagnostic check-ups. Discussion in patients with LVNC, there is an increased incidence of cardiac-related outcomes than in the general population; furthermore, male gender and myocardial fibrosis are associated with increased risk of events. This trend highlights the importance of a prompt diagnosis and, obviously, of a correct knowledge of such disease.

Abstract Introduction Takotsubo syndrome is a clinical syndrome characterized by typical anamnestic features together with typical ECG and echocardiographic findings. Comparing with the available literature not so many cases of takotsubo syndrome after pacemaker implantation can be found. Furthermore, there are only few articles talking about ECG features in these patient. The case we described allows to observe dynamic ECG alterations in a patient with electro-induced ventriculograms Case Description A 90-year-old male was admitted to the emergency room for important fatigue associated with severe bradycardia (25/min). His cardiovascular history was silent, and his past medical history was characterized by high blood pressure, chronic pulmonary obstructive disease and anemia due to iron deficiency. The ECG showed second degree AVB type 2, with phases of 2:1 AVB and paroxysmal third degree AVB on continuous monitoring. The routine blood tests showed normal T troponin and BNP was 420 pg/mL. The echocardiogram revealed normal biventricular dimensions and systolic function with moderate aortic valve stenosis. The patient underwent urgent permanent DDD pacemaker implantation without previous isoproterenol administration. During the procedure he referred important pain on the site of the wound, and he became confused and agitated. The procedure was complicated by massive pneumothorax that needed quick decompression. On the 2nd day after pacemaker implantation the ECG revealed electro-induced atrium-guided ventriculograms and began to modify with only mild ST-segment elevation in V2 and initial T-wave inversion from V3 to V6 and in I - II - aVF. On the next days, T-waves became deeper and QTc prolonged to 540 ms. These abnormalities were then gradually resolved on the 11th day. Mild transient attenuation of the amplitude of the QRS complexes in V2 – V3 leads on day 1 could be reported. Another echocardiogram was then performed, which showed new apical akinesis with “apical ballooning” aspect and EF of 40%. TnT and BNP values increased. Coronary angiogram was not performed due to patient rejection, so that coronaropathy could not be excluded with certainty. Nevertheless, the patient had only high blood pressure as cardiovascular risk factor and that the probability of the diagnosis of takotsubo cardiomyopathy was assessed of 76,9% by InterTAK diagnostic score, so that we considered Takotsubo syndrome the most likely diagnosis. Therefore, the patient's therapy was then optimized with an increase in the dosage of ACE inhibitors. At one month follow-up the ECG remained stable, and the echocardiogram showed a preserved ejection fraction (EF = 55%), without alterations of the segmental contractility. BNP and TnT values were normal. Therapy was left unmodified. Conclusions Takotsubo syndrome should be consider a rare but possible complication of pacemaker implantation. This is true especially for patients affected by frailty and cognitive impairment. There are no specific ECG criteria for takotsubo syndrome in patients with electro-induced ventriculograms, but anomalies of the repolarization are similar to those in patients with spontaneous ventricular activity. Transient attenuation of the QRS complexes voltages could be seen even just in the precordial leads and it is generally present in the very acute phase.

Abstract Introduction The mechanisms of cardiotoxicity during CAR-T cell therapy remain unclear to this day. We present the case of a 63-year-old woman with diffuse large B-cell lymphoma, who underwent CAR-T cell therapy. Five days after the infusion, she developed takotsubo cardiomyopathy. This is one of the rare cases of CAR-T cell-induced takotsubo cardiomyopathy. Case Description a 63-year-old woman with a diffuse large B-cell lymphoma and without significant cardiovascular history was treated with CAR-T cell therapy. The patient had arterial hypertension, dyslipidemia and history of CAD in the family. Prior to CAR-T cell therapy, she underwent baseline cardiac evaluation with an echocardiogram which showed a normal biventricular function. Within 24 hours of the infusion of CAR-T cells, she developed grade I cytokine release syndrome (CRS) with high-grade fever and sinus tachycardia. On day four, she developed grade three immune effector cell associated neurological syndrome which was treated with tocilizumab and dexamethasone. On day five laboratory testing showed a disproportionate elevation of BNP compared to hs-TnI. An ECG reported diffuse new-onset T wave inversion in all the precordial leads and a prolonged QT interval, and an echocardiogram showed severely reduced left ventricular ejection fraction (EF = 30%) with evidence of apical ballooning and right ventricular systolic dysfunction. Coronary angiography showed significant stenosis of the middle segment of the circumflex artery, which poorly could explain the clinical presentation of the patient. Ventriculography was also performed, which confirmed the ultrasound findings. During the next 48 hours, ECG and left ventricular function improved along with a gradual reduction of BNP and hs-TnI. We concluded that takotsubo syndrome was the most likely diagnosis. The InterTAK score was 89, which corresponded to a probability of takotsubo of 99.4%. The patient's therapy was then optimized with an increase in the dosage of angiotensin receptor antagonists and beta blockers. One month after CAR-T cell infusion, echocardiography showed complete recovery of biventricular function and ECG completely normalized, together with the values of BNP and TnI-hs. Medical treatment was left unmodified. Conclusions the pathophysiology of left ventricular systolic dysfunction during CAR-T cell therapy is unclear, but the main hypotheses are IL-6 mediated myocardial depression during CRS, stress-induced or takotsubo cardiomyopathy, and direct toxicity from CAR-T cells. From the currently available data from retrospective studies, cardiovascular events strongly overlap with CRS and, particularly, with high-grade CRS. Therefore, there is a strong rationale for early treatment with tocilizumab as it has been postulated from retrospective data a lower risk of cardiovascular events with earlier administration of tocilizumab during CRS. Surveillance for cardiotoxicity in patients receiving CAR-T cell therapy is mandatory for prompt recognition and treatment of cardiovascular complications. Our understanding of CAR-T cell-induced cardiomyopathy is still limited, and data regarding predictive factors for persistent cardiac dysfunction are lacking. It is important to differentiate cardiovascular events related to CAR-T cell therapy from epiphenomenon of CRS and capillary leak, to allow for a broader assessment of cardiac events among future CAR T-cell trials.

Blood culture negative endocarditis (BCNE) accounts for 5-10% of all cases of endocarditis. The most common cause of this outcome is initiation of antibiotics prior to cultures. The second most common etiology of negative blood culture is infection secondary to intracellular organisms, which require specific serological tests to be detected. Less frequently, the cause BCNE is noninfectious endocarditis. In this case, it could be secondary to systemic inflammatory diseases or malignancies. We present the case of a 64-year-old male who presented to his physician complaining of fever, malaise and generalized weakness. He was treated with oral antibiotics and with evidence of initial improvement, but after a few days the fever recurred. His past medical history was significant for cardiac surgery: he had an aortic bioprosthesis implanted for severe regurgitation secondary to left coronary cusp prolapse. Furthermore, he underwent mitral and tricuspid valve annuloplasty for severe regurgitation secondary to diffuse prolapse of these valves. After recurrence of the fever, he presented to his local hospital. A CT scan was executed and showed left basal lobe hypoattenuation. Given the context, this finding led to a diagnosis of community-acquired pneumonia. He was then treated accordingly and improved. A few days later, the fever returned, and he went back to his local hospital. This time he was visited by a cardiologist, and a transthoracic echocardiogram showed the presence of a vegetation adherent to the aortic bioprosthetic valve. Revising the initial CT scan, the lobar consolidation initially seen was interpreted retrospectively as an atelectasis. On that same day, due to the presence of a painful red eye, the patient was visited by an ophthalmologist. Retinal examination revealed the presence of lesions strongly suggestive of fungal endophthalmitis. Considering these results, fungal endocarditis was suspected. In view of the patient's complexity and the need for a cardiac surgery, the patient was transferred to our hospital and, after performing blood cultures to identify the pathogen, immediately started on an empirical broad-spectrum antibiotic and antifungal therapy. On admission a cerebral CT scan showed the presence of asymptomatic cerebral septic emboli. Due to the presence of cerebral lesions deemed at high hemorrhagic risk, no immediate surgical indication was posed, despite the suspicion of fungal endocarditis. Fortunately, the patient responded to medical treatment alone. However, blood cultures never revealed the presence of a defined pathogen. Therefore, the final diagnosis was that of blood culture negative endocarditis.

Abstract Hypertrophic cardiomyopathy (HCM) is a common disease, mostly inherited, with a prevalence of 1:500. Cornerstone of the clinical management of HCM patients is stratifying the risk of sudden cardiac death (SCD) which is the most frightening complication despite its relatively low incidence (0,9% per year). Although mathematical risk scores, as proposed by ESC, are the most used to assess the probability of arrhythmic complications leading to SCD, they have been associated with low sensitivity, precluding some high-risk patients from prophylactic ICD implantation. Among the tools at clinicians’ disposal for risk stratification in HCM patients, the latest AHA/ACC guidelines propose an “individual risk markers strategy”, based on the presence of ≥1 clinical feature such as a family history of SCD, extreme left ventricular hypertrophy (LVH), unexplained recent syncope, NSVT, late gadolinium enhancement (LGE) at CMR, systolic dysfunction and LV apical aneurysm. We present a case that lets us reflect on arrhythmic risk stratification. P.C. is a 58 y.o. male with a history of septal hypertrophy (19 mm) and T wave inversion but without an established diagnosis of HCM. He did not have any familiar history of syncope or SCD. He came to the emergency department for epistaxis and was admitted to the cardiology unit due to the previously mentioned findings, where he underwent cardiac-CTA and CMR showing myocardial bridge of left anterior descending artery, LVH with apical aneurysm and LGE areas localized on the apex (transmural) and on the interventricular septum (subendocardial). No signs of dynamic LVOT obstruction or atrial enlargement emerged from echocardiography. During the observation, asymptomatic NSVT were recorded on continuous ECG monitoring. According to the ESC risk prediction score (3.7%) ICD was not strictly indicated but considering the CMR high-risk profile (according to the suggestions of AHA/ACC) we proposed an ICD implantation anyways. The patient refused any invasive procedure and was implanted with a loop recorder. At 12 months remote monitoring showed a single episode of self-terminated sustained polymorphic ventricular tachycardia (02:23 mm: ss) symptomatic of pre-syncope. The patient was then immediately reached and admitted for ICD implantation in secondary prevention. According to the patient's will, and the no need for bradycardia therapy, an S-ICD was implanted. Figure 1LV apical aneurysm at CMRFigure 2Remote Monitoring

Cardiac amyloidosis (CA) is characterized by extracellular protein fibril deposition in the myocardium leading to restrictive heart failure. Both atrial and ventricular arrhythmias are common in CA. Study have shown up to one half of patients with CA die suddenly. However, the most common cause of sudden death has been historically through to be secondary electromechanical dissociation rather than a lethal ventricular arrhythmia. We present the case of a 84 years old man, with a history of hypertension, dyslipidaemia, and prior smoking. In June 2020 the patient was admitted to the emergency room of our hospital due to an episode of hemodynamically unstable sustained ventricular tachycardia (SVT). The patient underwent electrical cardioversion with restoration of sinus rhythm. An echocardiogram that showed a slightly reduced ejection fraction (FE = 45%), severe concentric hypertrophy, grade 3 diastolic dysfunction with high pressures in the left ventricular (LV) cavity, and a reduced GLS (-13.8%) with a typical apical-sparing aspect. A cardiac magnetic resonance (Fig.1). was performed showing a diffuse area of LGE with a subepicardial pattern involving left ventricular and atrial segments, compatible with myocardial storage disease. In order to complete the diagnostic workout, we performed a bone scintigraphy (Fig.2). (Positive for CA, with Perugini score grade 2), a genetic test (negative for hATTR-CA mutations) and free light chain in serum (negative for AL-CA). The patient was discharged at home after ICD implantation in secondary prevention and prescribed appropriate heart failure therapy. In May 2022 the patient was readmitted in emergency room for dyspnea during an arrhythmic storm characterized by several SVTs and ICD interventions, and atrial fibrillation (FA). After stabilization of clinical parameters, the patient was hospitalized in our ward. We optimized medical therapy with metaprololo 100 mg 1 cp BID, cordarone 600 mg ev and mexiletina 200 mg cp BID. During the hospitalization we succeeded in reducing SVT burden. However, ventricular PVCs and slow SVT remained. For this reason, we decided to perform an electrophysiological study (EPS) followed by catheter ablation. The EPS found two low voltage areas (Fig.3). The first were found under the aortic valve; the second was an area of dense scar and fragmented potentials along the basal-posterior wall. After ablation of the first area we succeeded in removing clinical PVCs. During the ablation of the second area the procedure was complicated by ventricular fibrillation that required advanced life support and many electrical shocks. The patient was discharged at home in absence of further episodes of SVTs with optimized therapy At a three moths follow up no arrhythmic events were recorded.

Abstract Introduction Retinol binding protein (RBP) is a protein synthesized in the liver which forms, along with transthyretin (TTR) the retinol-transport-complex. This complex ligates retinol and thyroxine and transports them in the human plasma. TTR is a protein longer and heavier than RBP. It ligates RBP preventing its renal filtration. Our hypothesis is that the relationship between RBP and TTR is dual: TTR avoids renal filtration of RBP, but also RBP itself, functioning as an endogenous ligand, has an action on TTR by stabilizing and avoiding TTR misfolding. Misfolded TTR proteins form amyloid fibrils rich in beta-cross-sheets that can infiltrate healthy tissues altering their physiology. The infiltration of these fibrils in the cardiac tissue leads to the well-known condition of TTR cardiac amyloidosis (ATTR CA). Our hypothesis is that the lack of TTR stabilization that can happen with low blood levels of RBP is a risk factor for developing ATTR CA. The main goal of our study is to understand if high or normal values of RBP can help exclude any pathological condition linked to the accumulation of misfolded TTR proteins. Methods All patients referred to our Cardiomyopathy Clinic with a clinical and echographic suspicion of CA were consecutively enrolled. All patients underwent a complete diagnostic workup in order to confirm or exclude CA, comprehending lab tests (including RBP4), SPECT, cardiac MR, genetic testing and cardiac biopsy as recommended by current recommendations. At the end of the diagnostic process, patients with ATTR-wt CA were enrolled as cases, and patients with no CA were considered as controls. Patients with ATTR-mutated CA or AL CA were excluded from the present analysis. Results Fifty-nine consecutive patients (42 males, age 77+/-13 years) were enrolled. Of those, 27 had a definitive diagnosis of ATTR-wt CA, while 32 had another diagnosis. Mean levels of RBP4 were not different between patients with and without CA (5.4+/-1.9 vs. 5.0+/-1.8 mg/dl; p=ns) as were Troponin I, BNP and NT-proBNP levels (all p=ns). Among patients with a definitive diagnosis of ATTR-wt CA and a positive SPECT, RBP4 levels were similar between Perugini 2 and 3 scores (5.4+/-2.1 vs. 5.6+/-2.0 mg/dl; p=ns). Discussion From our findings, it seems that there is no significant correlation between low levels of RBP and the presence of ATTR CA as we found no difference in the distribution of RBP levels between cases and controls. It also did not seem to be a useful marker to stratify the levels of myocardial infiltration as long as there is no difference in RBP between Perugini score 2 and 3.

Abstract Aims Left atrial appendage aneurysm (LAAA) is a rare condition mostly due to congenital malformations or secondary causes. Methods and results Since very few cases are described in the literature, there is uncertainty in treatment and prognosis. Diagnosis is achieved by advanced imaging as transesophageal echocardiography (TEE), which also allows the detection of thrombus, moreover cardiac magnetic resonance (CMR) could be more specific in describing sizes and relationships with surrounding anatomical structures. Surgical aneurysmectomy could be indicated in the majority of cases, especially if compression of other cardiac chambers or mediastinal structures are present. Medical therapy can include tromboprophylaxys and arrhythmias management. Since high quality evidence is scarce, a shared decision making by Heart Team approach should be considered. We present the case of a 47 years old male who came to our attention for palpitations and epigastric pain. The ECG showed high ventricular rate atrial fibrillation (AF) with wide QRS (left bundle branch block morphology). Due to haemodynamic instability the patient underwent urgent electrical cardioversion and coronary angiography showed patent coronary arteries. He had a giant left auricle appendage diagnosed twelve years before and was on antiarrhythmic prophylaxis for previous AF episodes. A TEE was performed and confirmed the diagnosis of LAAA also showing hypokinetic anterior-apical wall due to the interplay with the giant aneurysm. Subsequent CMR showed no LGE and confirmed the absence of thrombus in the LAAA. After Heart Team consultation surgical treatment was proposed to the patient who refused any invasive procedure. Therefore medical treatment was achieved by direct oral anticoagulation and antiarrhythmic therapy with betablockers and flecainide per os. Moreover, a loop recorder for longitudinal monitoring was implanted. At 6 months of follow-up the patient was asymptomatic except for a brief paroxysm of self-limited AF. 510 Figure 1CMR scan showing giant left atrial appendage aneurysm. (A) Transversal view. (B) Frontal view. (C) Sagittal view.510 Figure 2TOE mid oesophageal 57° showing giant left atrial appendage.