bluuebunny/crossref_metadata_2025 huggingface dataset:
706 ETIOLOGICAL ROLE OF STRESS IN ACUTE CORONARY SYNDROME: THE STRESS-ACS-ACTION STUDY
Authors: Magnani Giuia , Denegri Andrea , Bottioni Federico , Gurgoglione Filippo Luca , Tuttolomondo Domenico , Noni Manjola , Barocelli Federico , Vrenozaj Rosi , Catellani Davide , Indrigo Elia , Donelli Davide , Maccari Chiara , Andreoli Roberta , Corradi Massimo , Ossola Paolo , Marchesi Carlo , Ardissino Diego , Niccoli Giampaolo
Published: December 2022

Background

Acute coronary syndrome (ACS) is the main cause of mortality worldwide and despite the adherence to guidelines it is still burdened by an unacceptable risk for cardiovascular (CV) events recurrence, highlighting the need to identify other than traditional cardiovascular risk factors (CVrF) implicated in atherosclerotic plaque instability. In this regard, psychosocial stress appears to be a crucial player in the development of CV disease. Nevertheless, stress is not easy to standardize and the mechanisms by which it promotes coronary artery disease (CAD) are poorly understood.

Materials and Methods

We therefore prospectively enrolled patients with ACS, stable coronary artery disease (CAD) undergoing percutaneous coronary intervention (PCI) and subjects presenting traditional CVrF but without established CV disease. Multimodality cortisol assessment, expression of acute and chronic stress, through blood, urine and hair samples collection was ascertained at baseline. A regression analysis was performed to assess the relationships between significant variables at univariate analysis.

Results

Fifty patients were enrolled in the present study. Cortisol levels in blood and urine were numerically higher in patients with ACS compared to CAD patients and subjects with traditional CVrF only. Hair cortisol levels did not differ between the three groups. The regression analysis showed an inverse correlation (R= -,532, p<0.001 and R=-,615, p<0.001 respectively) between urinary cortisol (UC) and UC/creatinine ratio and left ventricular ejection fraction (LVEF).

Conclusion

The preliminary results of our study showed that patients with ACS did not have significantly higher levels of hair cortisol compared to stable patients. The finding of an inverse relationship between higher UC, UC/C ratio levels and lower LVEF values support a link between a hyperactivity of the hypothalamic-pituitary-adrenal axis and a worse ACS presentation. These preliminary data will be implemented with serial multimodality assessment of cortisol that allow potential implications in diagnosis and outcome.
947 THE IMPACT OF UEFA EURO 2020 FOOTBALL CHAMPIONSHIP ON TAKOTSUBO SYNDROME: RESULTS OF A MULTICENTER NATIONAL REGISTRY
Authors: Ielapi Jessica , Polimeni Alberto , Spaccarotella Carmen , Ccu Investigators Group , Indolfi Ciro
Published: December 2022

Objectives

The UEFA 2020 European Football Championship held in multiple cities across Europe from June 11 to July 11, 2021, was won by Italy, providing an opportunity to examine the relationship between emotional stress and the incidence of cardiovascular events.

Methods and Results

Cardiovascular hospitalizations in the Cardiac Care Units of 49 hospital networks in Italy were assessed by emergency physicians during the UEFA Euro 2020 Football Championship. We compared the events that occurred during matches involving Italy with events that occurred during the remaining days of the championship as the control period. Acute cardiovascular events were assessed in 1235 patients. Cardiovascular events during the UEFA Euro 2020 Football Championship semifinal and final, the most stressful matches ended with penalties and victory of the Italian team, were assessed. A significant increase in the incidence of Takotsubo Syndrome (TTS) by a factor of 11.41 (1.6 - 495.1, P<0.003), as compared with the control period, was demonstrated during the semifinal and final, whereas no differences were found in the incidence of ACS [IRR 0.93(0.74-1.18), P=0.57]. No differences in the incidence of ACS [IRR 0.98 (0.87-1.11; P=0.80)] or TTS [IRR 1.66(0.80-3.4), P=0.14] were found in the entire period including all matches of the UEFA Euro 2020 compared to the control period.

Conclusions

The data of this national registry demonstrated an association between the semifinal and final of UEFA Euro 2020 and TTS suggesting that it can be triggered by also positive emotions as the victory of the European Football Championship finals.
512 ACADEMIC RESEARCH CONSORTIUM HIGH BLEEDING RISK CRITERIA ASSOCIATED WITH ONE-YEAR NACES IN A MULTICENTER COHORT OF OLDEST OLD STEMI PATIENTS
Authors: Ruzzarin Alessandro , Zilio Filippo , Muraglia Simone , Unterhuber Matthias , Baessato Francesca , Bonmassari Roberto , Flaim Massimo , Zanon Sofia , Oberhollenzer Rainer , Donazzan Luca
Published: December 2022

Introduction

The balance between thrombotic and bleeding events after myocardial revascularization is of paramount importance. Patients with higher bleeding risk are at high risk for complications. Age and frailty are also major risk factors for complications. We sought to evaluate the incidence of major bleeding events (BE) and their impact on prognosis in a real life oldest old (i.e. >85 years) ST elevation myocardial infarction (STEMI) patients population.

Methods

We evaluated all consecutive oldest old STEMI patients hospitalized in two hub-centers between January 2010 and June 2021. Patients were stratified according to Academic Research Consortium for High Bleeding Risk (ARC-HBR) in high bleeding risk (HR) and non-HR (nHR) patients. BE were defined according to the Bleeding Academic Research Consortium (BARC) criteria, BARC 3 or 5. Patients were also divided in groups according to anti-thrombotic therapy (AT) regimens at discharge: single antiplatelet therapy (SAPT), dual antiplatelet therapy (DAPT) or triple therapy (DAPT plus oral anticoagulation) (TT). Net adverse clinical event (NACE) was defined as a composite of all-cause mortality, myocardial infarction, stroke, or major bleeding. Follow-up data were determined from local clinical records during a twelve-months period.

Results

340 oldest old (mean age 88.6 ± 2.9 years, 52.6% female) STEMI patients were eligible for the analysis. 161 patients (47.4%) were categorized as nHR and 179 patients (52.6%) as HR.

AT regimens at discharge were: 28 patients with SAPT (8.2%), 276 with DAPT (81.2%) and 36 with TT (10.6%). The overall incidence of BE was 4.6% within 12 months, without difference between nHR and HR patients (3.0% vs 5.9%, p=0.16). No BE were found in the SAPT group while the incidence of BE in the HR group was not statistically different between the DAPT and TT regimens (11.2% vs 9.5%; p=0.8).

The incidence of NACE was statistically higher in HR than nHR patients (40.9% vs 25.5%, p<0.01) at 1-year. In addition, time-to-NACE was statistically inferior in HR group than nHR group (256.2 days vs 294.2 days, p<0.01) (Figure).

Conclusions

Our oldest old STEMI population was characterized by a large proportion of HR patients according to ARC-HBR criteria. This population experienced a higher rate of NACE in a shorter time when compared to nHR patients in the same age group.
299 LEFT VENTRICULAR APICAL THROMBUS
Authors: Tansella Donatella , Rigato Ilaria , Marra Martina Perazzolo , Famoso Giulia
Published: December 2022

LV thrombus is a complication of AMI. It is estimated that 40-60% of patients with large anterior wall MI will develop a LV thrombus. The risk is highest during the first 3 months following AMI. Accurate and timely detection is important as it poses a high risk for thromboembolic events. Advances in technology have improved the detection rate of intracardiac thrombi, but several uncertainties still remain regarding the optimal treatment strategy within daily clinical practice. We report a case of large LV thrombus in a 61-year-old male with extensive anterior wall MI.

A 61-year-old man, hypertension, former smoker, subjected to epatic orthotopic transplant for HCC and treated with immunosuppressive therapy (Everolimus), following development of lymphocytic leukemia in fu. He underwent thoracic ct, wich showed a hypodense imaging in the left ventricular apex referable to thrombotic formation large 2cm, with endocardial rim hypointense like ischemic outcomes. The ECG demonstrated Q waves in anterior leads and biphasic T waves in the same leads. The patient was absolutely asyntomatic. A transthoracic echocardiography, showed the apical mass (2×0,9cm) very mobile and akinesia and thinning of the left ventricol middle-apical wall with apical aneurysmatic evolution. A CMR, confirmed the presence of the thrombus and showed defects at the subendocardial layer of the anterior wall and interventricular septal with also involvement of all the apex; the burden of late gadolinium enhancement (ischemic pattern) extended to the same segment. Coronary angiography showed subocclusion involving the second tract of the left anterior descending (99%) and critical coronary stenosis of the third tract of the right coronary (70%). The thrombus was completely removed surgically through left atriotomy, in prevention of embolization due to the thrombus dimension, the motility and the labile docking station. The histological examination confirmed the thrombotic nature of the mass. International guidelines agree on recommending anticoagulation therapy for patients affected by left ventricular thrombosis. OAC with vitamin K antagonists should be started as soon as LVT is identified, as first-line therapy and parenteral anticoagulation should be discontinued when effective therapeutic range with warfarin has been achieved (INR of 2–3). European and American guidelines stressing the efficacy of parenteral therapy with heparin; there are no randomized trials that have examined the efficacy of VKA anticoagulation compared with parenteral therapy. DOACs are attractive alternatives because of their potential efficacy and safety even though there is no trial that proves the effect of DOACs in LVT. Oral anticoagulation should be discontinued as soon as thrombus resolution has been established. Surgical removal of the LVT is an option for patients with high embolic risk, as in our patient. The high morbidity and mortality of this approach outweigh the benefits of performing surgery solely for the indication of LVT.
983 COMPARISON OF DEMOGRAPHIC, CLINICAL, BIOCHEMICAL AND IMAGING AND IMAGING FINDINGS IN HYPERTROPHIC CARDIOMYOPATHY PROGNOSIS: A NETWORK META-ANALYSES
Authors: Beltrami Matteo , Olivotto Iacopo , Figliozzi Stefano , Pateras Kostantinos , Niccoli Flavia , Bampatsias Dimitrios , Finocchiaro Gherardo , Chiribiri Amedeo , Masci Pier Giorgio , Georgiopoulos Georgios
Published: December 2022

Background

Despite hypertrophic cardiomyopathy (HCM) being the most common inherited heart disease and conferring increased risk for heart failure (HF) and sudden cardiac death (SCD), risk assessment in HCM patients is still largely unresolved.

Objective

To synthesize and compare the prognostic impact of demographic, clinical, biochemical, and imaging findings in patients with HCM.

Methods

We searched PubMed, Embase, and Cochrane Library for studies published from inception to November 2020, and the endpoints were: i) all-cause death; ii) an arrhythmic endpoint including SCD, sustained ventricular tachycardia, ventricular fibrillation, or aborted SCD; iii) a composite endpoint including i) or ii) plus hospitalization for HF or cardiac transplantation. We performed a pairwise meta-analysis obtaining the pooled estimate separately for the association between baseline variables and study endpoints. A random-effects network meta-analysis (NMA) was subsequently used to comparatively assess the prognostic value of outcome predictors.

Results

One-hundred-eleven studies with 56,792 HCM patients were included. Among others, increased BNP/NT-proBNP, late-gadolinium-enhancement (LGE), positive genotype, impaired global longitudinal strain and presence of apical aneurysm conferred increased risk for the composite endpoint. At NMA, LGE showed the highest prognostic value for all endpoints and was superior to all other predictors except NYHA class≥II. A multiparametric imaging-based model was superior in predicting the composite endpoint compared to a pre-specified model based on conventional risk factors.

Conclusions

This network meta-analysis supports the development of multiparametric risk prediction algorithms, including advanced imaging markers additively to conventional risk factors, for refined risk stratification in HCM.
153 CARDIAC METASTASES: WHEN YOU HEAR THE HOOFBEATS YOU SHOULD ALSO THINK ABOUT ZEBRAS
Authors: Boccadoro Alberto , Gibiino Federico , Scala Antonella , Vitali Francesco , Tonet Elisabetta , Campo Gianluca
Published: December 2022

Background

Cardiac metastases (CM) are rare findings and their diagnosis is often challenging due to the large panel of symptoms they can cause. CM are frequently clinically silent, and their diagnosis is frequently made post-mortem. Even in the rare cases in which they clinically manifest themselves, it's harsh to distinguish them from other (more common) cardiovascular conditions presenting with the same panel of symptoms. The diagnosis could be very challenging, in particular in subjects with occult primary neoplasm. Electrocardiogram, although not specific, is frequently abnormal and imaging tools play a key role in the diagnostic process.

Case Report

A 75-year-old man went to the emergency room for chest pain and fever. The pain differs from that experienced in previous myocardial infarction. Laboratory tests showed plateau rise in troponins and the electrocardiogram showed diffuse changes in depolarization and repolarization. Transthoracic echocardiographic assessment showed a non-dilated left ventricle (LV) with a huge thickening of the interventricular septum and of the LV apex. The inferior wall was akinetic and the ejection fraction of the left ventricle was 45%; diffuse pericardial effusion was also present. To characterize the nature of this finding, a chest CT scan an a Cardiac Magnetic Resonance were performed. The diagnosis of a cardiac metastasis and its occult primary lung cancer was then made.

Discussion

In this clinical case, metastases manifest themselves mimicking an acute coronary syndrome, albeit with atypical characteristics; the atypical symptoms and the instrumental findings made us hypothesize that we were not dealing with a myocardial infarction. The peculiarity of this clinical case lies in the fact that the onset of symptoms led to the diagnosis of the cardiac metastasis and, subsequently, of the occult primary lung tumor. Our case report warn us that, sometimes, when you hear the hoofbeats you should also have to think about zebras.
1152 INVESTIGATION ON THE HIGH INCIDENCE OF THE ATTRV-CAUSING TRANSTHYRETIN VARIANT VAL142ILE IN CENTRAL ITALY
Authors: Cappelli Francesco , Argiro´ Alessia , Zampieri Mattia , Giotti Irene , Boschi Beatrice , Frusconi Sabrina , Buxbaum Joel , Gennarelli Massimo , Polimanti Renato , Olivotto Iacopo , Perfetto Federico , Mazzarotto Francesco
Published: December 2022

The p.Val142Ile variant in transthyretin (encoded by the TTR gene) is the most common genetic cause of transthyretin-related amyloidosis. This allele is particularly prevalent in communities of African descent compared with populations of different ancestries, where its frequency is two orders of magnitude lower. For this reason, p.Val142Ile has always been considered an “African” variant, with limited studies performed on individuals of European descent. However, recent reports of higher-than-expected prevalence in European-ancestry populations question the African specificity of this allele. Here we show that the high recurrence of p.Val142Ile in central Italy is due to a founder effect and not to recent admixture from African populations, highlighting how this may be the case in other communities. This suggests a probable underestimate of the global prevalence of p.Val142Ile, and further emphasizes the importance of routine inclusion of TTR in gene panels used for clinical genetic testing in hypertrophic cardiomyopathy (independently of the patient's geographical origin), that transthyretin-related amyloidosis can mimic. Figure.Principal Component Analysis (PCA) of the 16 probands together with the 25044 individuals of the 1000 Genomes Project (Phase 3) having been genotyped both within the 1000 Genomes Project (NA10851_1KG) and in-house (NA10851_internal). The proband clustering within the smear of native Americans is A228.
1107 PLASMA CIRCULATING TRANSTHYRETIN FORMS IN PATIENTS WITH WILD TYPE TRANSTHYRETIN CARDIAC AMYLOIDOSIS
Authors: Castiglione Vincenzo , Sanguinetti Chiara , Panichella Giorgia , Minniti Marianna , Caponi Laura , Aimo Alberto , Paolicchi Aldo , Emdin Michele , Vergaro Giuseppe , Franzini Maria
Published: December 2022

Background and aims

Wild type transthyretin (TTR) cardiac amyloidosis (ATTRwt-CA) is caused by the misfolding, aggregation and tissue deposition of native TTR, leading to cardiac structural remodeling. In the last decade, the validation of non-invasive diagnostic approaches and the development of novel therapeutic options targeting the amyloidogenic cascade, such as the TTR stabilizer tafamidis, have dramatically changed the epidemiology and the prognosis of the disease. Nonetheless, the basic pathophysiological mechanisms underlying TTR misfolding and aggregation are still poorly understood. We aimed to characterize circulating TTR forms in plasma samples of ATTRwt-CA patients before and after treatment with tafamidis through a native electrophoretic method.

Methods

Plasma samples from 6 male patients with ATTRwt amyloidosis (median age 82 years, IQR 80-83), collected before (T0) and during tafamidis treatment, and from 6 healthy controls were collected. Plasma samples were separated on a native 4–20% Tris-Gly polyacrylamide gel. Western blot analysis was performed with anti-TTR or anti-retinol-binding protein (RBP) antibodies. Proteins were detected by Clarity ECL substrate.

Results

Circulating TTR forms were qualitatively similar between ATTRwt-CA patients at T0 and controls. In both groups, the most represented forms were TTR dimers or trimers (∼37-50 kDa), TTR tetramers complexed with RBP in 1:1 ratio (∼80 kDa) or 1:2 ratio (∼100 kDa), and high molecular weight (MW) aggregates (>150 kDa). Neither TTR monomers nor tetramers could be detected. RBP protein was detectable in association with TTR tetramers and some of the higher MW fractions (∼150 kDa, >250 kDa). Following tafamidis treatment, all ATTRwt-CA patients displayed a progressive increase of the intensity of the band corresponding to TTR-RBP complexes, in agreement with the a stabilizing action on TTR tetramers. Interestingly, dimers and trimers, detectable at T0, were progressively lost during tafamidis treatment.

Conclusions

TTR tetramer exists only complexed with RBP and in equilibrium with low and high MW forms, without apparent qualitative differences between ATTR-CA and healthy controls. Tafamidis increases circulating TTR tetramers complexed with RBP. Evaluation of TTR isoform may prove useful as a circulating biomarker for the assessment of response to treatment in patients with ATTRwt-CA.
62 MYOCARDIAL WORK IN CARDIAC AMYLOIDOSIS AND HYPERTENSIVE HEART DISEASE: A PRELIMINARY REPORT
Authors: Trimarchi Giancarlo , Campisi Mariapaola , Luongo Alfredo , De Sarro Rosalba , Poleggi Cristina , Lofrumento Francesca , Licordari Roberto , Mancinelli Anna , Dattilo Giuseppe , Recupero Antonino , Zito Concetta , Carerj Scipione , Di Bella Gianluca , De Gregorio Cesare
Published: December 2022

Objective

Assessment of myocardial work (MW) by pressure-strain loops is a recently introduced ultrasound-based technique allowing a noninvasive estimation of left ventricular (LV) myocardial performance, in a more precise fashion than ejection fraction (LVEF) and global longitudinal strain (GLS). Early studies have shown that MW clusters, such as efficiency (MWE), global wasted work (GWW), global constructive work (GCW) and myocardial work index (MWI) are likely to impair in most patients with hypertrophic heart. These markers can be predictive of myocardial dysfunction from the early stages of the disease and a trend towards fibrosis. To date, there are only scanty information about MW analysis in patients with heart failure (HF) and preserved/mildly reduced left ventricular ejection fraction (LVEF), as in those with cardiac transthyretin amyloidosis (ATTR). This pilot study aimed at evaluating differential MW features in aTTRCA vs hypertensive patients.

Methods

26 male patients, 13 affected by ATTR wild-type (mean aged 74±6 years) and 13 with hypertensive heart disease (HHD, mean aged 63±8 years), were investigated by transthoracic speckle-tracking strain ultrasound. Offline pressure-strain loop analysis was performed by a dedicated software. LV mass index (LVMI), LVEF, GLS, MWE, GWW, GCW and MWI were recognized.

Results

Main findings are reported in Table underneath, and the reference MW values provided by the European multi-centre NORRE study* are also displayed. As expected, ATTR patients showed greater LVMI and lower LVEF than those with HHD. MW indices were almost all impaired in ATTR patients, whereas they were in the lower range of normality in the HHD group. Noteworthy, all patients showed normal GWW values, indicative of a complex pathophysiology conveying different HF settings.

Conclusions

Findings from this hypothesis generating research indicate that MW is significantly impaired in ATTR compared to HHD patients, whose values, however, were in the low range of normality. Global wasted work was the sole marker to be normal in both groups, suggesting further studies still needed to shed lights on the intricate pathophysiology of myocardial function in cardiac amyloidosis.
183 GLOBAL LONGITUDINAL STRAIN BY SPECKLE TRACKING ECHOCARDIOGRAPHY IN SUBCLINICAL ORTHOTOPIC HEART TRANSPLANT REJECTION
Authors: Mancuso Filippo , Fontana Alessandra , Messina Margaret , Cipriani Manlio , De Gregorio Cesare
Published: December 2022

Objective

heart transplant (HT) patients need to be strictly monitored, especially within the first months after surgery by advanced and sensitive techniques, in order to rule out possible acute or subacute cellular rejection (CR) and hinder poor outcomes. This includes frequently repeated endomyocardial biopsy (EMB) and biomarkers. Echocardiography has emerged as an easily available and powered technique able to detect morphofunctional changes in various clinical settings. More recently, speckle tracking ultrasound and left ventricular (LV) strain have been documented as more accurate tools for detecting early myocardial dysfunction in most cardiac diseases. This study aimed at recognizing whether strain echocardiography can improve early diagnosis of subclinical CR.

Methods

We studied 32 patients, mean aged 52±4 years, who had undergone heart transplantation over the last 3 years at IRCCS ISMETT-UPMC (Palermo, Italy). They were scheduled for EMBs on a regular basis, and multisite biopsy specimens served to make diagnosis of CR. Conventional and advanced ultrasound measurements were performed on the same day of EMB. LV ejection fraction (LVEF) was assessed by using the Simpson rule biplane method and global longitudinal strain (GLS) by speckle tracking. Diastolic function was achieved by PW-Doppler/TDI sampling, as the E/E’ ratio. Echocardiographic findings were also related to anti-HLA antibodies (HAb).

Results

Multiple EMBs and ultrasound studies were performed in each patient. Echocardiographic findings from those likely to be healthy (CR- group) or with possible CR (CR+ group) are summarized in Table underneath (numbers are the average values of the various appraisals). Overall, 300 EMBs were negative (score 0-1) and 94 were positive (score 2-3). Global longitudinal strain and E/E’ ratio were significantly impaired in the first group, despite preserved LVEF. Tacrolimus therapeutic levels were similar between the groups, whereas a higher title of HAb was recognized in the CR+ group (13.3% vs 10.8%, p=0.012).

Conclusions

Cardiac ultrasound was confirmed to be a useful technique for monitoring HT patients over time. In those likely to have CR as by EMB specimens and HAb title, GLS and diastolic indices were significantly impaired irrespective of LVEF, suggesting speckle tracking echocardiography as a choice modality for detecting subclinical LV dysfunction in patients at risk of CR.
433 A PRO-ARRHYTHMIC DOUBLE HIT
Authors: Graziano Francesca , Brunetti Giulia , Cipriani Alberto , Cecere Annagrazia , Bauce Barbara , Rigato Ilaria , Basso Cristina , Pilichou Kalliopi , Iliceto Sabino , Corrado Domenico , Marra Martina Perazzolo , Zorzi Alessandro
Published: December 2022

A 42-year-old man went to the emergency room complaining about dyspnea and palpitations. He reported an episode of atrial fibrillation (AF) with symptoms of dizziness 8 years before. He had a family history of ischemic cardiopathy, his father has suffered from AF since a young age.Acute course: The ECG revealed AF at high ventricular frequency. The echocardiography demonstrated mild reduction of the left ventricular function (EF 45%) with diffuse hypokinesis, and mitral valve prolapse (MVP) with mild mitral regurgitation. The blood tests showed a troponin peak of 750 ng/L (n.v. 0–34 ng/L). The suspect was acute coronary syndrome. Thus, he was treated with dual antiplatet therapy and low doses of beta-blockers, and he underwent coronarography, which demonstrated no significant lesions. So, after a transesophageal echocardiography that excluded thrombosis, and a loading dose of amiodarone, a successful electrical cardioversion was performed. The discharge diagnosis was AF with mild left ventricular disfunction. Three months later: He came back to the emergency room because of suffering some days of progressive epigastric and retrosternal pain radiating to the shoulders. The ECG and the echocardiography were unchanged, the troponin-I values were 79 ng/L-74 ng/L. Therefore, he underwent cardiac magnetic resonance (CMR), which showed normal biventricular dimensions and function (LVEF 64%, LVEDV 80 ml/mq), MVP with mitral annular disjunction (MAD) and curling of the infero-lateral basal wall (Figure A). The native T1, T2 and ECV values were increased, and there was a wide left ventricular subepicardial late gadolinium enhancement (LGE). The conclusion was MVP and active myocarditis. Moreover, he underwent endomyocardial biopsy that revealed focal lymphocytic infiltration, PCRs for common cardiotropic viruses or bacteria were negative. Six months later: The basal ECG was characterized by low QRS voltages in limb leads (Figure B). The CMR revealed a wide left ventricular non-ischemic fibrosis with a ring-like pattern (Figure C). The 24-hours Holter ECG showed premature ventricular beats with a right bundle branch block/superior and inferior axis morphology (Figure D), in keeping with MVP. The next step was the genetic testing that resulted positive for a Filamin-C (FLNC) mutation associated with cardiomyopathy. The final diagnosis was “Hot-phase of Left Ventricular Arrhythmogenic Cardiomyopathy (ACM), MVP and paroxysmal AF”. Discussion: The ACM is a rare hereditary heart muscle disease, and the “hot phase” represents its uncommon clinical presentation. Mutations in FLNC gene could be involved in the pathogenesis of ACM. Moreover, there are few cases in literature about the correlation between FLNC mutations and MVP. FLNC mutation may be a cross-sectional substrate for both these pro-arrhythmic conditions.
207 RE-DEFINING ARRHYTHMOGENIC CARDIOMYOPATHY: CHARACTERIZATION AND LONG-TERM PROGNOSTIC IMPLICATIONS
Authors: Setti Martina , Merlo Marco , Gigli Marta , Munaretto Laura , Paldino Alessia , Stolfo Davide , Loco Carola Pio , Medo Kristen , Barbati Giulia , Graw Sharon , Ribichini Flavio Luciano , Ferro Matteo Dal , Taylor Matthew , Sinagra Gianfranco , Mestroni Luisa
Published: December 2022

Background

Arrhythmogenic dilated cardiomyopathy (AR-DCM) combines phenotypical aspects of dilated cardiomyopathy (DCM) and risk of sudden cardiac death (SCD), typical of the arrhythmogenic form (ACM). However, AR-DCM is often ambiguously defined leaving clinicians uncertain on how to identify these high-risk patients.

The aims of the study were to re-define AR-DCM based on outcome related arrhythmic markers and to test the usefulness of the novel AR-DCM definition in identifying arrhythmogenic genotypes (i.e., LMNA, FLNC, RBM20, and desmosomal genes).

Materials and methods

Consecutive DCM patients with genetic evaluation and Holter ECG monitoring or telemetry in two referral institution were analyzed. The arrhythmic markers tested to define AR-DCM were: SCD or major ventricular arrhythmias (MVA), unexplained syncope, rapid nonsustained ventricular tachycardia (nsTV), ≥1000 premature ventricular contractions/24 hours, or ≥50 ventricular couplets/24 hours. Patients were labeled as Early AR-DCM if criteria were met within 12 months from enrolment. The primary endpoint was a composite of SCD/MVA; the secondary endpoint was a composite of all-cause mortality/heart transplant/LVAD implantation (D/HTx/LVAD).

Results

Among the 743 DCM patients included, 290 had disease-related variants (39%), 94 (30%) of these carried arrhythmogenic genotype. Early AR-DCM was identified in 429 (58%) patients. During a median follow-up of 7.0 [2.2-13.8] years, among arrhythmic markers the occurrence of syncope and/or nsVT within 12 months from enrolment were the only arrhythmic markers independently associated with SCD/MVA (Figure), while the occurrence of early MVA and/or nsTV emerged as the strongest long-term predictors of D/ HTx/LVAD. Family history of MVA was also independently associated with primary and secondary endpoints, and together with MVA, nsTV or unexplained syncope increased the agreement between AR-DCM and arrhythmogenic genotypes in 1 out 2 patients.

Conclusions

A combination of early (i.e., within 1 year from diagnosis) MVA or nsVT or unexplained syncope might be proposed as a clinically useful new definition of AR-DCM, especially if associated to family history of MVA. This definition in fact allows clinicians to anticipates worse long-term arrhythmic and global outcomes, and to accurately identify malignant arrhythmogenic genotypes.
259 PERICARDITIS AND PERICARDIAL EFFUSION IN CHRONIC GRAFT-VERSUS-HOST DISEASE AFTER ALLOGENIC HEMATOPOIETIC STEM CELL TRANSPLANTATION
Authors: Collini Valentino , De Biasio Marzia , Favaretto Virginia , Fumarola Francesca , Sinagra Gianfranco , Imazio Massimo
Published: December 2022

Background

Pericarditis and pericardial effusion (PE) are rare but potentially life-threatening complications in chronic graft-vs-host disease (cGVHD). Limited data are currently available in this setting, based on small single-center retrospective studies. The purpose of this study is to characterize the clinical presentation, management, and outcomes of pericarditis and PE complicating cGVHD in allogeneic hematopoietic cell transplant recipients.

Material and Methods

We included all consecutive patients referred to our pericardial diseases referral center from 1st May 2008 to 1st May 2022. All patients had no previous cardiovascular or autoimmune diseases and suffered from severe cGVHD with involvement of at least 2 organs. Pericarditis and PE were diagnosed according to ESC guidelines. Conditioning-related toxicity and infections were carefully excluded.

Results

Pericarditis was diagnosed in 4 patients (2 males, mean age 48.5 ±9.0 years), of whom 2 developed mild PE. PE without pericarditis was diagnosed in 5 patients (80% male, aged 53.2 ±8.9 years), in 2 patients it was reported as severe, requiring pericardial drainage. The average time after allogeneic hematopoietic stem cell transplantation was 22 months (range, 4 to 58 months). All patients required escalation of the corticosteroid therapy they were chronically taking, and in 4 cases the therapy was boosted with non-steroidal anti-inflammatory drugs. Only one patient developed a recurrence and died of multiorgan failure associated with severe cGVHD (involving eyes, mucous membranes, skin, joints, liver, pleuro-pericardial effusion) refractory to immunosuppressive therapy. Overall survival was 89% and no patient developed constrictive pericarditis. Mean follow-up was 69±55 months.

Conclusions

Pericarditis and PE related to cGVHD typically occur with mild clinical signs in the context of severe cGVHD, however in our study 22% of patients required pericardial drainage at diagnosis. The mainstay of treatment is high-dose steroids and usually the majority of patients have a favorable outcome.
941 SARCOIDOSIS: THE IMPORTANCE OF MULTIMODALITY IMAGING
Authors: Pezzato Andrea , De Luca Antonio , Radesich Cinzia , Saro Riccardo , Korcova Renata , Pagnan Lorenzo , Dore Franca , Altinier Alessandro , Massa Laura , Zecchin Massimo , Perkan Andrea , Bussani Rossana , Merlo Marco , Sinagra Gianfranco
Published: December 2022

A 55-years old sporty man, without any cardiovascular risk factors nor previously known cardiological history, presented for a cardiological evaluation due to extra-systolic palpitations and dyspnea. The ECG documented a new onset of right bundle branch block, left anterior fascicular block and first degree of atrio-ventricular block. Echocardiography revealed severe left ventricular (LV) systolic dysfunction (LV ejection fraction -EF- 34%), showing also right ventricular (RV) dilatation and disfunction. A 24-hour Holter monitoring did not show any significant arrhythmias and maximal stress test did not reveal any ECG changes nor arrhythmias. Cardiac magnetic resonance (CMR) confirmed the presence of severe biventricular dysfunction; multiple areas of edema and late gadolinium enhancement (LGE) were observed, with ischemic and nonischemic pattern and extensive involvement of the RV and the interventricular septum. In the suspicion of cardiac sarcoidosis a 18-fluorodeoxyglucose positron emission tomography (PET) was performed, confirming the presence of an inflammatory cardiomyopathy in an active phase. A subsequent endomyocardial biopsy was performed, which demonstrated the presence of non-caseating granulomas, signs of inflammation and fibrosis, consistent with the diagnosis of cardiac sarcoidosis. No signs of extra-cardiac involvement were present.

The patient started anti-inflammatory therapy with Prednisone and Metotrexate, along with anti-neurohormonal therapy. However, due to a significant increase in ventricular arrhythmic burden, an implantable cardioverter defibrillator was placed during early follow-up, and Amiodarone therapy was started.

After 5 months of medical therapy, PET scan showed a marked reduction of cardiac inflammation and echocardiography showed a significant LVEF improvement (from 34% to 43%). After slow tapering of steroid therapy Prednisone was stopped, whereas Metotrexate, Amiodarone and anti-neurohormonal therapy were maintained. Follow-up is still ongoing without clinical events.

Sarcoidosis is a systemic inflammatory disease characterized by the presence of non-caseating granulomas in multiple organs. Cardiac involvement is associated with higher incidence of heart failure, ventricular arrhythmias and all-cause mortality. Isolated cardiac involvement is rare but associated with worse prognosis. Multimodality imaging is of paramount importance for the diagnosis and monitoring therapy.
992 EVALUATION OF MYOCARDIAL WORK INDEX IN PREDICTING IN-HOSPITAL COMPLICATION IN PATIENTS WITH TAKOTSUBO SYNDROME
Authors: Migliarino Serena , Cristiano Mario , Iuliano Giuseppe , Ferruzzi Germano Junior , Loria Francesco , Bellino Michele , Silverio Angelo , Attisano Tiziana , Baldi Cesare , Ciccarelli Michele , Galasso Gennaro , Vecchione Carmine , Citro Rodolfo
Published: December 2022

Backgrounds

Tako-tsubo syndrome (TTS) is a reversible heart failure pathology characterized by transient myocardial left ventricular (LV) dysfunction. Although traditionally considered a benign condition in-hospital complications are frequent in this cohort. Non-invasive myocardial work (MW) parameters are considered as emerging indexes in evaluating global and regional myocardial systolic function.

Objective

to investigate the potential association of MW compared to standard echocardiography parameters, such as LV ejection fraction (EF) with in-hospital complications, in TTS patients.

Methods

thirty-eight patients (mean age, 68 ± 12 years, 35 women) with TTS diagnosed with Takotsubo Italian Network criteria were prospectively enrolled and underwent a transthoracic Doppler echocardiography within 24 hours from hospital admission. MW is derived from the non-invasive strain-pressure loop obtained from the 2D strain data, integrating into its calculation the non-invasive brachial arterial pressure. Constructive MW (CMW), MW index (MWI), MW efficiency (MWE), and wasted myocardial work (WMW) were measured. In hospital complications (HC) were defined as a composite of apical LV thrombosis, hypokinetic arrhythmias (HA), supraventricular tachycardia (SVT), acute heart failure (AHF), ventricular tachycardia/fibrillation (VT/VF), cardiogenic shock (CS), respiratory arrest (RA), stroke and cardiac death (CD).

The normal values ​​for the echocardiographic parameters are reported in the Table. Odds ratio, sensitivity and specificity were used to quantify the ability of EF and MW (abnormal vs normal values) in predicting HC.

Results

HC occurred in 16 TTS patients (26% with AHF; 10% with SVT; 8% with CS; 5% with apical LV thrombosis; 5% with HA; 2,6% with RA; 2,6% with stroke; 2% with VT/VF). The MWI and CMW parameters appear to have the best performance in predicting in-hospital complications (odds ratio for having HC: 10.4 (95% confidence interval: 1.2 to 93.3); 8.6 (0.9 to 77.6) respectively, Table), followed by EF (odds ratio 7.0 (0.8 to 64.1)) and WMW (odds ratio 4.8 (0.9 to 26.7)). MWI has the same sensitivity (94%) as CMW and EF, but higher specificity (41% vs 32%). Of note, the highest specificity was obtained by the WMW parameter (41%).

Conclusion

global and regional myocardial performance is transiently impaired in TTS and significantly associated with HC. Although limited by the low number of patients, these results suggest that myocardial work parameters have a good sensistivity and may have a better performance than the EF value in predicting intra-hospital complications. Larger studies will be necessary to confirm these preliminary results.
1041 ELECTROCARDIOGRAPHIC HETEROGENEITY OF PATIENTS WITH VARIANT TRANSTHYRETIN AMYLOID CARDIOMYOPATHY: GENOTYPE PHENOTYPE CORRELATIONS
Authors: Russo Domitilla , Cappelli Francesco , Di Bella Gianluca , Tini Giacomo , Porcari Aldostefano , Cipriani Alberto , Canepa Marco , Merlo Marco , Licordari Roberto , Vianello Pier Filippo , Zampieri Mattia , De Michieli Laura , Perfetto Federico , Sinagra Gianfranco , Autore Camillo , Rapezzi Claudio , Musumeci Maria Beatrice
Published: December 2022

Transthyretin (TTR) amyloid cardiomyopathy due to mutation on TTR gene (vATTR) has extremely different features according to the type of mutation, ranging from a mild to an extensive cardiac involvement. Data about electrocardiographic findings (ECG) in vATTR are limited and not informative of genotype correlation. Aim of this study is to analyze ECG characteristics and their correlation to clinical and echocardiographic aspects in patients with vATTR cardiomyopathy, focusing on different TTR mutations.

This is a multicentric, retrospective, observational study performed in six Italian referral centres for cardiac amyloidosis. We divided patients in two groups, according to the previously described phenotypic manifestations of the TTR mutation. Of 64 patients with vATTR cardiomyopathy, 23 (36%) had prevalent cardiac (PC) TTR mutations and 41 (64%) patients had a prevalent neurological (PN) TTR mutations. Patients with PC mutations were more frequently males and older, with advanced NAC staging. At baseline ECG, atrial fibrillation was more common in patients with PC, while pacemaker induced rhythm in those with PN mutations. Prevalence of atrioventricular and intraventricular blocks, pseudonecrosis pattern and low voltages were not significantly different in both groups. PQ and QRS durations were longer and voltage to mass ratio was lower in PC mutations. Different TTR mutations tend to have distinctive ECG features.

The standard ECG in vATTR is extremely heterogeneous and the specific mutations are associated with distinct instrumental and clinical features. The differences between PN and PC vATTR are only partially explained by the different degree of cardiac infiltration.
662 A CASE OF MILIARY TBC WITH PERICARDIAL INVOLVEMENT
Authors: Padoan Laura , Parise Antonio , Sforna Stefano , Barengo Alberto , Torlone Francesco , Coiro Stefano , Broccatelli Andrea , Rosignoli Daniele , Cavallini Claudio
Published: December 2022

Introduction

Miliary TBC represents a complex disease, quite rare at our latitudes. We present a case with severe pericardial involvement.

Case report

a 25-year-old Pakistani male, recently come in Italy, presented for fever and orthopnea. Anamnesis was difficult because of linguistic barrier. Echocardiogram revealed a large pericardial effusion with signs of tamponade, so a pericardiocentesis was promptly performed and exudative fluid was drained out and sent to laboratory. Blood tests showed anemia, mild hyponatremia, VES elevation and several vitamin deficiencies. Few days after the procedure the patient developed fever with chills, so blood samples for cultural tests were taken, but resulted negative. To better understand the etiology of pericardial effusion and fever a thoraco-abdominal CT was performed and bared multiple micronodular lesions disseminated to lungs, spleen and lever and pleural and peritoneal effusion. At the same time, the DNA search for Koch's bacillus in the pericardial fluid resulted positive for Mycobacterium TBC complex. So, a diagnosis of miliary TBC with pericardial involvement was done and corticosteroid therapy together with antitubercular drugs were started. Encephalic MRI excluded neurological involvement. Patient was isolated and moved to the infectious disease ward, where therapy was continued until recovery.

Discussion

Miliary TBC is a disseminated form due to the hematogenous spread of tubercle bacilli resulting in the formation of multiple tuberculous foci. This manifestation is more frequent in countries where TBC is still endemic (Pakistan, India, Philippines). The disease can progress slowly with few symptoms or acutely (typical of younger) and the identification may be challenging due to its rarity in developed countries and the lack of uniform criteria. Diagnosis is mainly based on the isolation mycobacterial from a specimen or molecular methods such as PCR. Treatment is based on standard antitubercular drugs regimen. The role of corticosteroids is still controversial.

Conclusions

Miliary TBC is a rare disease in developed countries with not well-defined diagnosis criteria and different clinical presentations. However, because of the increase of migration flows, is important to recognize this manifestation, especially when it develops acutely and with life-treating conditions such as pericardial tamponade.
796 LEFT ATRIAL STRAIN ASSOCIATION WITH FUNCTIONAL CAPACITY IN CARDIAC AMYLOIDOSIS
Authors: Pastore Maria Concetta , Giacomin Elisa , Marrese Federica , Ragazzoni Gian Luca , Mandoli Giulia Elena , Focardi Marta , Gozzetti Alessandro , Cavigli Luna , Lisi Matteo , Valente Serafina , D´ascenzi Flavio , Cameli Matteo
Published: December 2022

Background

cardiac amyloidosis (CA) is characterized by an impairment of cardiac diastolic and, in the severe state of the disease, systolic function, with increasing worsening of functional capacity and quality of life (QoL). Six minute walking test (6MWT) and Kansas City Cardiomyopathy Questionnaire (KCCQ) are two well-known and validated methods to assess the impact of heart failure (HF) symptoms and functional capacity. Left atrial strain by speckle tracking echocardiography has emerged as an index of left ventricular (LV) diastolic function and filing pressure, and is also associated with symptoms in HF. However, its possible association with functional capacity and QoL in CA has not yet been investigated.

Objectives

in this observational pilot study, our aim was to evaluate the relationship of left atrial strain with 6MWT and KCCQ in patients with CA (both AL and ATTR).

Methods

we enrolled consecutive patients with CA during routine follow up visits. Patients underwent clinical and echocardiographic evaluation. On the same day, 6MWT was performed and KCCQ was administered. Speckle tracking analysis was performed offline by an experienced operator blinded to the other data. Correlation analysis was conducted using Pearsons’ coefficient and linear regression analysis

Results

overall, 43 patients with CA (25 ATTR, 18 AL) were enrolled. Mean age was 74 ±11, 16% (n=7) were female. Most patients showed normal left ventricular (LV) ejection fraction (55±9) and reduced LV global longitudinal strain (GLS =-12 ± 7%), 32 of them with apical sparing pattern. Mean global peak atrial longitudinal strain (PALS) was 14 (median[IQR]=6.5;23.5), mean 6MWT score = 382±104 and mean KCCQ score= 67 ± 24.

Global PALS showed a strong direct correlation with 6MWT (Fig 1, P= 0.4, R2=0.2, p-value=0.032) and a trend towards correlation with KCCQ (P=0.3, p-value=0.06), although not reaching statistical significance, probably due to the low sample size. The correlation between PALS and 6MWT was even stronger in patients with ATTR (p=0.7, R2 0.4; p-value<0.0001).

Conclusions

our preliminary results show that, global PALS is associated with functional capacity and the burden of HF symptoms in ATTR and AL, suggesting its role as a more objective marker of disease severity in CA.

Fig. 1
291 BIVENTRICULAR ARRHYTMOGENIC CARDIOMYOPATHY: COULD ELECTROCARDIOGRAPHY SEE BEYOND THE CARDIAC MRI?
Authors: Pittorru Raimondo , Marra Martina Perazzolo , De Lazzari Manuel , Migliore Federico , Basso Cristina , Iliceto Sabino , Corrado Domenico
Published: December 2022

We described the case of a 34 year-old young athlete who was referred to our Department because of palpitations and fatigue, arising after hard effort but also present at rest. Family history was unremarkable for sudden cardiac death or cardiomyopathies. Electrocardiography (ECG) evidenced multifocal premature ventricular contractions (PVCs): left bundle branch block (LBBB) morphology and superior/inferior axis; right bundle branch block (RBBB) morphology and undetermined axis. Cardiac MRI showed extensive mid wall and subepicardial late gadolinium enhancement in the postero-lateral left ventricular wall. We decided to perform electroanatomic mapping (EAM) because we didn't explain the origin of the PVCs with LBBB configuration. During EAM we found an area extending from the right ventricular outflow tract to the subtricuspidal lateral inferior wall which it had not described by cardiac MRI. In the low voltage areas, the electrograms inscribed by the catheter's electrodes was fractionated with different waveforms, a finding that corresponds to a deficiency of healthy myocardial tissue. We performed an endomyocardial biopsy in the interventricular septum close to the ventricular out flow tract. The histological examination of the biopsy samples showed fibro-fatty replacement. Therefore genetic testing revealed a pathogenic desmoplakin mutation. According to “Padua Criteria”, we made a diagnosis of biventricular arrhythmogenic cardiomyopathy. We finally implanted a transvenous cardioverter defibrillator.

Conclusion

Diagnosis of arrhythmogenic cardiomyopathy (ACM) is complex and requires the combination of multiple clinical, histological and genetic parameters. This clinical case demonstrated that ECG represent an essential tool in diagnostic work-up of the disease, and electroanatomic mapping is useful in selected cases for the documentation of the diagnosis of arrhytmogenic cardiomyopathy.