bluuebunny/crossref_metadata_2025_split huggingface dataset:
297 DEVELOPMENT OF CORONARY ARTERY DISEASE IN A YOUNG ADULT AFFECTED BY TAKAYASU'S ARTERITIS
Authors: Benvenuto Ciro Pollio , Simonini Chiara , Vaccarella Marcello , Ierardi Carolina , Fedele Annalaura , Leone Antonio Maria 
Published: December 2022

Abstract
Large vessel vasculitis, such as Takayasu's Arteritis (TA), are rare inflammatory diseases affecting multiple vascular districts including the coronary arteries, producing either stenosis and/or aneurysms: these lesions can be found in the same patient and also in the same vessel, with potentially devastating effects. Moreover, TA often affects young people, in the midst of their work and social activity.
Ischemic heart disease is the leading cause of cardiovascular mortality in Western countries and is mainly due to coronary atherosclerosis, whose etiopathogenesis is multifactorial and is closely related to the concomitant presence of classic cardiovascular risk factors and inflammation of the vessel wall.
We present the case of a young adult, sportsman, with multivessel coronary artery disease developed in the context of a TA bursted seven years before and currently in clinical remission. This complex case required a careful literature review and a multidisciplinary approach, since the best treatment option for coronary lesions induced by TA has not been established: a “watchful waiting” strategy was eventually adopted, considering the poor outcome of both percutaneous and surgical revascularization in this group of patients.
656 DIFFERENTIAL DIAGNOSIS BETWEEN TAKOTSUBO SYNDROME AND MYOCARDITIS
Authors: Biddau Mattia , Garau Enrica , Marchetti Maria Francesca , Utzeri Elena , Licciardi Marco , Mandas Martina , Maiani Silvia , Cau Riccardo , Saba Luca , Montisci Roberta 
Published: December 2022

Abstract

Background
In the evaluation of specific non MINOCA, the differential diagnosis between Takotsubo syndrome and myocarditis can be challenging.

Purpose
The aim of our study was to evaluate the parameters that can be more useful to distinguish the pathologies.

Methods
All the patients with specific non MINOCA admitted to our service were enrolled. Informations about clinical characteristic, in-hospital outcome and complications were collected. All the patients have made several blood samples for hS-troponin, CRP, NT-proBNP, ECG with calculation of QTc, cardiac echocardiogram at the admission and at the discharge and diagnosis has always been confirmed by CMR.

Results
Between October 2018 and October 2021 51 specific non MINOCA have been hospitalized in our service (26 Takotsubo and 25 myocarditis). Patients affected by Takotsubo syndrome were more frequent older, females (p&lt;0,0001), had a higher global cardiovascular risk (p=0,046) and had more frequently neurological disorders (p=0,041), autoimmune thyroiditis (p=0,02) and have experienced stressful triggers before hospital admission (p=0,003); patients with myocarditis had, instead, more often an infection before hospital admission (p&lt;0,0001). No differences have been found in clinical or electrocardiographic presentation, although observing the evolution of the ECG, a significative prolongation of the QTc in Takotsubo have been noticed (QTc 552 ± 75,1 vs 409,8 ± 27,7 p&lt;0,0001). Peak troponins, NT-proBNP and CRP were significantly different (p=0,002; p=0,008; p=0,003), as were LVEF and WMSI at the admission (p=0,004 and p&lt;0,0001). From the CMR data LGE and T2 mapping were useful to distinguish the two pathologies (p&lt;0,0001; p=0,001). From the analysis of ROC curves a T2 mapping value superior to 62 msec was able to distinguish the two pathologies with a sensibility of 70%, a specificity of 73%, a positive predictive value of 73% and a negative predictive value of 70%.

Conclusion
There are many parameters useful to distinguish the specific non MINOCA: clinical characteristics; blood sample parameters like troponins, CRP and NT-proBNP; ECG, echocardiographic and CRM. T2 mapping can distinguish the two pathologies with a good sensibility and specificity.
668 TWO CATHETERS (DDD) OR ONE (VDD) FOR ATRIO-VENTRICULAR BLOCK? COMPLICATION RATES OVER A MORE THAN 20 YEARS EXPERIENCE
Authors: Agbariah Andrea , Ruzzarin Alessandro , Manfrin Massimiliano , Donazzan Luca 
Published: December 2022

Abstract

Background
in patients with atrio-ventricular block and spared sinus node function dual chamber (DDD) pacing or ventricular (VDD) pacing are considered as an alternative in clinical practice. Literature lacks validated data on the best treatment choice in this situation. Decision should be made balancing risk and benefit ratio of a single or dual lead procedure.

Aims
to compare complication rates of DDD and VDD implants in a single center experience.

Methods
data from consecutive patients who underwent DDD or VDD implantation in our institution between January 2000 and December 2021 were included in the analysis. Complications consisted of catheter dislodgements (i.e. dislodgements needing a repositioning procedure), catheter related problems (i.e. lead problems needing a substitution) and infections (i.e. pocket infections or lead infections needing antibiotic therapy or extraction). The need of an upgrading procedure in VDD recipients was not considered as a complication but as a further procedure. The rate of generator replacement and its longevity was also recorded.

Results
we performed 2499 pacemaker implantations, among them 1435 (57,4%) were DDD and 1064 (42,6%) were VDD. The majority of DDD and VDD recipients were male (836 patients, 58,3% and 609 patients, 57,2% respectively). The VDD population was significantly older (77,7 years old vs 75,5 years old, p&lt;0,01) at the time of implant.
Complications occurred more often in the DDD than in the VDD group, without reaching statistical significance (7,3% vs 5,7% p=0,13). No statistical difference was found in catheter displacement rate between DDD and VDD cohorts (4,9% vs 3,8%, p=0,21). In the first group, dislodgements were more frequent for the atrial catheter (41) than the ventricular (29).
Catheter-related problems occurred in 25 (1,7%) with a DDD and in 5 (0,5%) patients implanted with a VDD (p&lt;0,01). Infections were also more frequent in patients with dual-chamber pacemakers (1,5%, 22 patients) than in those with single-chamber pacemakers (1,3%, 14 patients) (p=0,67).
Twenty (1,9%) VDD recipients needed upgrading to DDD mode during the follow-up due to chronotropic incompetence, whereas a single patient with a DDD underwent a CRT upgrading to relieve the symptoms of heart failure. During the study period, we changed 121 DDD and 140 VDD generators. Mean device duration was similar in both groups: 91,3 months and 93,3 months (p=0,43).

Conclusions
VDD implantation was the more frequent choice for elderly patients. No significant complications difference occurred in in patients implanted with DDD and with VDD pacemakers, although catheter-related problems occur more frequently in the former population. A small proportion of VDD recipients needed to upgrade the device to DDD. Both pacing choices are equally effective in atrio-ventricular block.
276 CIRCULATING FORMS OF PLASMA TRANSTHYRETIN IN PATIENTS WITH WILD-TYPE TRANSTHYRETIN AMYLOIDOSIS AND EFFECTS OF TREATMENT WITH TAFAMIDIS
Authors: Chiara Sanguinetti , Minniti Marianna , Panichella Giorgia , Vergaro Giuseppe , Aimo Alberto , Castiglione Vincenzo , Caponi Laura , Paolicchi Aldo , Emdin Michele , Franzini Maria 
Published: December 2022

Abstract

Introduction
Transthyretin (TTR) is a homotetrameric 55-KDa plasma protein that transports thyroxin and retinol complexed to retinol-binding protein (RBP). TTR misfolding and aggregation can lead to the extracellular deposition of amyloid (ATTR) representing one of the most frequent forms of amyloidosis in elderly. Aim of this study is to develop a native electrophoretic method to characterize circulating TTR in plasma samples of ATTR patients before and during treatment with tafamidis, a TTR stabilizer.

Methods
Plasma from ATTR patients (n=6), collected before (T0) and during tafamidis treatment, and plasma of healthy controls (n=6) were obtained from Fondazione Toscana G. Monasterio (Pisa, Italy). Plasma samples were separated on a native 4–20% Tris-Gly polyacrylamide gel. Western blot analysis was performed with anti-TTR (DAKO) or anti-RBP (Siemens Healthineer) antibodies. Proteins were detected by Clarity ECL substrate (BioRad).

Results
Circulating forms of TTR were qualitatively similar between ATTRwt 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 protein in 1:1 ratio (∼80 kDa,) or 1:2 ratio (∼100 kDa), and high molecular weight (MW) aggregates (&gt;150 kDa). Neither TTR monomers nor the tetramers were visible. RBP protein was detectable in association with TTR tetramers and some of the higher MW fractions (∼150 kDa, &gt;250 kDa). Following tafamidis treatment, all ATTRwt patients displayed a progressive increase of the intensity of the band corresponding to TTR-RBP complexes, in agreement with the drug stabilizing action on TTR tetramers. Interestingly dimers and trimers, detectable at T0, were progressively lost during tafamidis treatment.

Conclusions
The native electrophoretic allowed us to detect several circulating TTR fractions. Data suggest a similar pattern of circulating TTR both in patients and healthy control: TTR tetramer exists only complexed with RBP and in equilibrium with low and high MW forms. Furthermore, the method allowed to appreciate the stabilizing effect of tafamidis on circulating TTR tetramers complexed with RBP. The study of circulating TTR fractions can expand our knowledge on mechanisms triggering its destabilization even when not mutated.
490 NT-PROBNP AND HIGH-SENSITIVITY TROPONIN T HOLD DIAGNOSTIC VALUE IN CARDIAC AMYLOIDOSIS
Authors: Castiglione Vincenzo , Aimo Alberto , Prontera Concetta , Masotti Silvia , Musetti Veronica , Nicol Martin , Cohen-solal Alain , Logeart Damien , Georgiopoulos Georgios , Chubuchny Vladyslav , Giannoni Alberto , Clerico Aldo , Buda Gabriele , Patel Kiara N , Razvi Yousuf , Patel Rishi , Wechalekar Ashutosh , Lachman Helen , Hawkins Philip N , Passino Claudio , Gillmore Julian , Emdin Michele , Fontana Marianna , Vergaro Giuseppe 
Published: December 2022

Abstract

Background
Cardiac amyloidosis (CA) is associated with an elevation of natriuretic peptides and troponins, predicting outcome. Nevertheless, the diagnostic yield of these biomarkers has not been extensively investigated.

Aim
To evaluate the diagnostic performance for CA of N-terminal pro-B-type natriuretic peptide (NT-proBNP) and high-sensitivity troponin T (hs-TnT).

Methods
Patients with suspected CA (n=1,149) underwent a diagnostic work-up in 3 Centers in Italy, France (n=343, derivation cohort), and United Kingdom (n=806, validation cohort). Biomarker values with either 100% sensitivity or ≥95% specificity were selected as rule-out/rule-in cut-offs, respectively.

Results
In the derivation cohort, 227 patients (66%) had CA, and presented with higher NT-proBNP and hs-TnT. NT-proBNP 180 ng/L and hs-TnT 14 ng/L were selected as rule-out cut-offs, and hs-TnT 86 ng/L as rule-in cut-off. NT-proBNP &lt;180 ng/L or hs-TnT &lt;14 ng/L were found in 7% of patients, ruled out without false negatives. In the validation cohort, 20% of patients (2% false negatives) had NT-proBNP &lt;180 ng/L or hs-TnT &lt;14 ng/L, and 10% showed both biomarkers below cut-offs (0.5% false negatives). These cut-offs refined CA prediction when added to echocardiographic scores in patients with a hematologic disease or an increased wall thickness. In the validation cohort, the 86 ng/L hs-TnT cut-off ruled in 20% of patients (2% false positives).

Conclusions
Cardiac biomarkers can refine the diagnostic algorithm in patients with suspected CA. NT-proBNP &lt;180 ng/L and hs-TnT &lt;14 ng/L reliably exclude the diagnosis (Figure), both in the overall population and subgroups referred for either AL-CA or cardiac (pseudo)hypertrophy.
73 WHICH IS THE CULPRIT OF THIS MYOCARDIAL INFILTRATION?
Authors: Cumitini Luca , Rossi Lidia , Giubertoni Ailia , Airoldi Luisa , Casali Giovanni , Boldorini Renzo , Patti Giuseppe 
Published: December 2022

Abstract

Introduction
Myocardial infiltration by primary cardiac neoplasm is a rare condition and the pathological spectrum includes more widespread benign cell types, providing exceptional diagnostic and therapeutic challenges. Refractory heart failure, pericardial effusion and arrhythmias due to infiltrative mass are the most common clinical manifestations. Multimodality imaging, with echocardiography, computed tomography and magnetic resonance imaging, is essential to determine the characteristics of a cardiac tumor.
Clinical case: A 35-year-old man without cardiovascular risk factors presented with two months of shortness of breath and weight loss. A previous history of acute myeloid leukemia undergoing allogenic bone marrow transplant was reported. Transthoracic echocardiography revealed an apical thrombus in left ventricle with inferior and septal hypokinesia conditioning mildly reduced ejection fraction, circumferential pericardial effusion without cardiac tamponade and abnormal right ventricular thickening (Figure 1). The cardiac magnetic resonance imaging confirmed diffuse thickening of the right ventricular free wall (maximum diameter 30 mm), suspected for myocardial infiltration, extending to the right atrium and left ventricle with an ischemic pattern of the inferior wall (Figure 2). No coronary artery disease was found by coronary angiography, but the right coronary artery was incorporated by the pathological tissue in chest computed tomography scan. Cardiac positron emission tomography demonstrated the presence of neoplastic tissue with increased metabolic activity, whose similar characteristics were also evident in the ascending aorta and aortic arch. Being the percutaneous endomyocardial biopsy inconclusive, a pericardiectomy was performed through a median sternotomy incision, showing widespread cardiac neoplastic infiltration. Histopathological analysis, done on samples taken by surgical myocardial biopsy, revealed tissue involvement by a rare and aggressive cardiac anaplastic large T-cell non-Hodgkin lymphoma (ALK -, Ki-67 90%). Few days after surgery, the patient developed a refractory cardiogenic shock and unluckily died before establishing an adequate therapy.

Conclusion
Primary cardiac lymphoma is very infrequent and the non-specificity of the symptoms makes the diagnosis extremely challenging and often limited to autopsy findings. Our case highlights the importance of an appropriate diagnostic algorithm that can allow an early diagnosis and an adequate therapy of this otherwise fatal pathology. In this condition surgical removal of the tumor in not indicated, being chemotherapy the only effective treatment. However, because of rarity, data are lacking to produce definitive regarding guidelines.
358 A POSSIBLE RARE CASE OF VENTRICULAR LIPOMATOSIS: A STIMULUS FOR A PANORAMA OF FATTY CARDIAC LESIONS
Authors: De Gaetano Fabrizio , Losi Valentina , Tamburino Corrado , Monte Ines Paola 
Published: December 2022

Abstract
We exhibit the case of a 75 years-old man who undertook cardio-oncological assessment for Non-Hodgkin Lymphoma: baseline echo revealed typical features of lipomatous hypertrophy of the interatrial septum (LHIS) and an apical hypoechoic left ventricle (LV) lesion. Five years before, the patient performed MRI (Magnetic Resonance Imaging), which confirmed LHIS, but superabundant epicardial fat extended to the right ventricle and the apical junction between the two ventricles, both characterized by normal ejection fraction, and surrounded the origin of the pulmonary trunk. LV lateral and inferior walls presented lipomatous metaplasia foci and no history or ECG sign of previous myocardial injury could be pointed out. A slight band of late gadolinium enhancement at the basal infero-lateral wall of LV was highlighted. The patient referred episodes of fainting: the Holter-ECG showed pronounced bradycardia which couldn't be explained by other secondary causes. So a bicameral pace-maker was implanted. During our ambulatory evaluation, we decided to repeat MRI, which showed no difference in comparison with the previous one, so excluding malignant degeneration of the fatty mass surrounding the heart. Despite older age and the lack of sudden cardiac death family history we searched for pathognomonic arrhythmogenic cardiomyopathy (ACM) mutations. In consideration of the new evidence of the metabolic risk associated with abundant epicardial fat (EF) we asked for updated laboratory dosage of plasma glucose, LDL cholesterol and triglycerides and we recommended stricter control of blood pressure.
From 4% to 52% of cardiac mass is made up of fat but only EF interacts with myocardium and coronary arteries. Anomalous growth of fat in the heart can present itself as LHIS, excessive lipomatous infiltration or cardiac lipomas (real capsulated tumors) but also ACM is histologically characterized by fibrofatty replacement of cardiomyocites. Overabundant and inflammed EF is now considered a surrogate of metabolic risk and is associated with multivessel coronary disease, the finding of chronic total occlusions (CTO) and microvascular angina.
Identifying fatty heart lesions and defining their clinical and prognostic role remains an intriguing challenge. We suggest a general binary way to follow: the “mechanic one” for lipomas or lipomatous infiltration, that is paying attention to compression phenomena or the generation of arrhythmias, and the “metabolic one”, in other words optimizing cardiovascular prevention when EF is too much. A careful research of imaging features of ACM can't be omitted.
361 PATISIRAN EFFICACY ON MYOCARDIAL FUNCTION IN H-ATTR PATIENTS EVALUATED WITH THE USE OF CMR AND SPECKLE TRACKING ECOCARDIOGRAPHY
Authors: Cannizzo Noemi , Di Lisi Daniela , Damerino Giuseppe , Di Caccamo Leandro , Galassi Alfredo Ruggero , Novo Giuseppina 
Published: December 2022

Abstract

Introduction
Hereditary transthyretin amyloidosis (h-ATTR) is a complex, systemic infiltrative disease characterized by extracellular deposition of transthyretin in different organs. Infiltrative cardiomyopathy and polyneuropathy represent two of the most common and severe manifestations of this pathology. Patisiran is a double-stranded interfering RNA drug, used to stop expression of TTR in h-ATTR. It is recommended in the treatment of cardiac h-ATTR with polyneuropathy or polyneuropathy alone. Recent studies showed the efficacy of Patisiran in slowing the progression of the disease, improving myocardial deformation indices, biomarkers and functional capacity.

Aim
We aimed to illustrate the efficacy of Patisiran on myocardial function and to evaluate the role of Speckle Tracking Echocardiography (STE) and Cardiac Magnetic Resonance (CMR) as markers of subclinical cardiac efficacy of Patisiran in two h-ATTR patients (males, with mean age 55 ± 1,4 years old) with infiltrative cardiomyopathy and polyneuropathy who were scheduled to receive this drug.

Materials and Methods
Before receiving Patisiran (T0) the following tests were performed: cardiovascular physical examination, complete echocardiogram including STE. All these exams were repeated after 6 months or 1 year (T1). CMR was performed at the baseline and three years after.

Results
The CMR evaluation showed, after the use of Patisiran, a significant reduction of myocardial mass (average reduction -13,29%) and myocardial walls thickness (average septal reduction -13,65%). After 1 year follow up myocardial performance indices were also improved: GLS values (average gain 2,3 pt), and myocardial work values (average increase GWI +311,5, GCW +188,5, GWW -114,5, GWE +5,5 pt) at echocardiography.

Conclusion
The results of our experience showed, according to evidences suggested by the APOLLO trial, the efficacy of Patisiran, in patients with cardiac h-ATTR and polyneuropathy, in reducing myocardial mass and improving myocardial function.
STE and CMR could represent useful tools to study cardiac function in A-TTR patients undergoing Patisiran.
391 NEUROLOGICAL DISORDERS AND TAKOTSUBO SYNDROME: CLINICAL FEATURES AND OUTCOMES. RESULTS FROM A MULTICENTER PROSPECTIVE EUROPEAN REGISTRY OF 2300 PATIENTS.
Authors: Vitale Enrica , Santoro Francesco , Cetera Rosa , Ragnatela Ilaria , Mallardi Adriana , Leopizzi Alessandra , Matera Annalisa , Correale Michele , Iacoviello Massimo , Brunetti Natale Daniele 
Published: December 2022

Abstract

Background and aims
Takotsubo syndrome (TTS) is an acute heart failure syndrome featured by a reversible left ventricular systolic dysfunction. However, few data have been reported about prevalence of non-cardiovascular comorbidities as chronic neurological disorders in TTS patients.
Aim of this study was to compare clinical features, short- and long-term outcome among TTS patients with history of neurological disorder.

Methods
2301 patients with TTS were enrolled in a multicenter prospective European registry from July 2002 to November 2021. Clinical features, in- and out-of-hospital MACE, laboratory and imaging data were collected and a long-term follow-up was performed.

Results
Prevalence of history of neurological disorder was 17% (n= 400 pts). Most represented neurological conditions were previous cerebrovascular disorders (ischemic stroke and transient ischemic attack, 36.2%), followed by neurodegenerative disorders (30.7%, including cognitive disorders and motion disorders), tensive migraine (10%) and epilepsy (9.5%).
Neurological Patients developed TTS more frequently after a physical trigger (46.2% vs 33.8%, p&lt;0.01). Sepsis (10%), acute neurological trigger (9.5%) and extreme activity/trauma (8.5%) where the type of physical trigger more prevalent.
During hospitalization neurological patients had longer in-hospital stay (10±11 days vs 8±8 days, p&lt;0.01) and experienced more often in-hospital complications (27% vs 16%, p=0.0001) mainly driven by cardiogenic shock and in-hospital death (12% vs 7.6%; 6.5% vs 2.8%, respectively p&lt;0.01).
Long-term mortality was higher in patients with history of neurological disorders (23.5% vs 10%, p&lt;0.01), with a mean follow up of 2.5 ± 5.8 years.

Conclusions
Patients with history of neurological disorders and TTS have different clinical features and worse prognosis with higher short-term and long-term mortality.