bluuebunny/crossref_metadata_2025_split huggingface dataset:
P317 FATAL EVOLUTION IN A YOUNG WOMAN DUE TO AMYLOIDOSIS HEART FAILURE IN A RARE MULTIPLE MYELOMA DISEASE
Authors: Magistro A , Cicco S , Marozzi M , Narcisi V , Carrieri A , Longo S , Giliberti T , Melaccio A , Solimando A , Lauletta G , Vacca A , Ria R 
Published: May 2022

Abstract

Introduction
Immunoglobulin (Ig) D (IgD) monoclonal gammopathy is a rare subtype of multiple myeloma (MM) associated with a worse prognosis. Ig light chains amyloidosis is a disorder characterized by extracellular deposition of Ig light chains in various tissues, leading to organ dysfunction.

Case
A 29–year–old woman was admitted for dyspnea and tachycardia for moderate efforts. Lab revealed hypogammaglobulinemia with a monoclonal IgD K paraprotein. There was an increase in K free chains, with a dramatic increase in K/λ free chains Ratio. Proteinuria was 1360 mg/24h with a free K chains Bence Jones Protein. An increase in values of Troponin–I (137,5 pg/mL) and NT–proBNP (12527 pg/mL) pointed towards heart involvement. Furthermore, the ECG showed low voltages in the peripheral leads. Echocardiogram showed a moderate concentric left ventricular hypertrophy with diffused myocardial speckled pattern, an ejection fraction (EF) of 53% and PAPs of 71 mmHg and pericardial effusion. Chest CT scan showed bilateral pleural effusion. Spine MRI and whole body PET/CT indicated areas of osteolytic lesions. Plasma cells infiltration (85% CD138, MUM1 and K chains positivity) was present at bone marrow biopsy. Amyloid deposition was detected in abdominal fat tissue sample. Total bone scintigraphy excluded a transthyretin heart deposition. Thus, amyloidosis associate to IgD MM was diagnosed. In few days heart failure worsened (hs–cTnI 156,6 pg/mL; NT–proBNP 26583 pg/mL, EF 48%,) and the patient began complaining non–productive cough, dyspnea, and columnar edema of the lower limbs. She was not eligible for bone marrow transplantation, so daratumumab, bortezomib, melphalan and prednisone were administered. Despite serological improvement after a short five days course of therapy, she worsened with a further reduction of EF (35%). Blood gas analysis showed hypoxemia and lactate increase quickly turned into a cardiogenic shock. She died by cardiac arrest, just three weeks after admission.

Discussion
The present case is remarkable for age and gender of the patient as well as the rapid onset and fast worsening of symptoms, which were related to secondary heart amyloid deposition. Symptoms associated to cardiac amyloidosis are expressions of right heart involvement. The early good results of the therapy did not turn the evolution of disease. The fatal and overwhelming progression of the myocardial involvement led to the patient’s death in less than one month.
P319 SOMETHING MORE OF SIMPLE CARDIAC CONDUCTION DISORDERS
Authors: Barbati G , Scodro M , Rossillo A , Caprioglio F 
Published: May 2022

Abstract

Background
Cardiac amyloidosis (CA) is an infiltrative disease caused by extracellular deposition of amyloid fibrils. Possible cardiac manifestations include heart failure with preserved ejection fraction (EF), aortic valve stenosis, microvascular coronary artery disease, atrial fibrillation (AF), ventricular arrhythmias, and cardiac conduction disorders (CCD). We report a case of advanced CCD in a patient first diagnosed with wild–type transthyretin amyloidosis (ATTRwt).

Case Report
An 88–year–old man went to Emergency Room for chest pain with minimal elevation of troponin I and increase in NTproBNP. The patient reported a recent syncope with ribs fractures. The medical history showed hypertension, paroxysmal AF, carotid atherosclerosis, vascular encephalopathy and prostate cancer. Troponin I monitoring showed plateau values and there were no clinical signs of heart failure. ECG showed regular sinus rhythm with marked first degree atrioventricular block, right bundle branch block and left anterior fascicular block (Fig. 1A). Cardiac telemetry recorded brief phases of atrioventricular dissociation (AVD). Transthoracic echocardiography showed severe left ventricular concentric hypertrophy (LVCH) with granular–sparkling appearance and preserved EF, reduced global longitudinal strain with apical sparing (Fig. 1B), mitral–aortic valve degeneration and dilation of the ascending aorta. Given the advanced CCD with AVD and the recent syncope, the patient underwent a dual–chamber pacemaker implantation. In consideration of the echocardiographic suspicion of CA, following Gillmore’s diagnostic algorithm (GDA), serum electrophoresis and assay of serum free light chains were performed and resulted normal, while 99mTc–DPD bone scintigraphy was positive for significant radiotracer cardiac uptake (Perugini grade 3) (Fig. 1C). Based on this result, we carried out the search for transthyretin gene mutations which resulted negative, and therefore the diagnosis of ATTRwt was made.

Discussion
Advanced CCD in patients with transthyretin CA are present in about 9.5% of cases at the time of diagnosis, especially in older patients. It is therefore essential, in case of concomitant LVCH, to suspect the presence of CA as the cause of CCD by searching for the disease red flags and following the GDA to non–invasively confirm or exclude the diagnosis of CA. A correct and timely diagnosis of CA makes it possible to treat the disease and its complications, improving the prognosis.
P320 LEFT DOMINANT ARRHYTHMOGENIC CARDIOMYOPATHY: HOW TO STRATIFY THE ARRHYTHMIC RISK? EARLY ONSET CLINICAL CASE
Authors: Di Domenico A , De Raffele M , De Carolis B , Vitali F , Guardigli G , Balla C 
Published: May 2022

Abstract

Introduction
Arrhythmogenic cardiomyopathy (ACM) is a genetic disease characterized by the progressive replacement of myocardiocytes by fibroadipose tissue that becomes a susbstrate for ventricular arrhythmias with risk of sudden cardiac death. Arrhythmogenic cardiomyopathy can manifest itself through three different phenotypes: the classic phenotype, the most frequent, involving only the right ventricle, the biventricular variant with possible right or left dominance and the rare variant involving only the left ventricle. We present a case of left ventricular arrhythmogenic cardiomyopathy.

Case Report
16–year–old male patient in complete physical well–being, with no significant medical history and a negative family history of heart disease and/or sudden cardiac death (Fig. 1). He practiced competitive sports for years with periodic medical–sports visits. In 2017, ventricular extrasystole was detected on the electrocardiogram, for which he performed cardiological investigations. On electrocardiogram: sinus rhythm with QRS fragmented into DIII and aVL and negative T waves from V4 to V6 (Fig. 2). On transthoracic echocardiography, evidence of normal cavity dimensions, ejection fraction at lower normal limits (42%) and left ventricular trabeculation, in the absence of significant valvulopathy. He then performed a cardiac MRI that showed: left ventricle moderately dilated and hypokinetic with widespread alterations of myocardial signal secondary to fibroadipose replacement phenomena, configuring a picture of left ventricular dilation and dysfunction referable to arrhythmogenic left ventricular cardiomyopathy (Fig. 3). The patient was also subjected to genetic analysis aimed at searching for mutations of genes involved in arrhythmic heart disease, which was negative. In light of the absence of conclusive scientific evidence on the implantation of a defribrillator in primary prevention in clinical–instrumental cases such as the present, the risk of infection and the risk of inappropriate discharges, it was proposed the implantation of a loop–recorder in order to monitor and record any arrhythmic events, starting beta–blocker therapy. The possibility of continuing competitive physical activity was therefore denied.

Conclusions
Currently, guidelines on ACM and diagnostic criteria focus mainly on the right variant, so there are no clear indications to stratify arrhythmic risk in left ventricular ACM.
P327 PULMONARY EMBOLISM FROM RIGHT–SIDED VALVE ENDOCARDITIS REVEALING RECTAL NEOPLAS. A “LUCKY” CASE
Authors: Patrassi L , Marinaccio L , Bracco A , Marchese D 
Published: May 2022

Abstract
We describe a case of a 64–years–old man, outwardly healthy, presented with dyspnoea for 2–3 months and weight loss, coincident Co–Vid. Lung CT scan showed massive PULMONARY EMBOLISM. Echocardiography showed LONE PULMONARY VALVE INFECTIVE ENDOCARDITYS with large mobyle vegetations swinging between RVOT and pulmonary artery. Streptococcus Gallolyticus was identified on blood culture (often related with colorectal neoplasm). Given targeted antibiotic therapy, a surgical opinion was obtained: advised trial of medical management. Patient underwent a colonscopy, because of the causing germ: a neoplasm of rectal posterior side was found, hystological examination revealed an adenocarcinoma. MNR demonstrated a solid rectal lesion protruding in the lumen, infiltrating muscle and adipose tissue, but not pelvic organs. Team discussion with surgeons and oncologists: indication at neoadjuvant therapy before heart valve surgery. After 12 days of antibiotics a new TOE and CT scan were performed and revealed enlargement of valvular vegetations with severe pulmonary regurgitation and also PE warsened. After new surgical opinion, has been given indication of valve replacement. A Magna Ease bioprostetic valve was implanted; the native valve appeared completely destroyed and histological exam showed white fragments including cusps, granulocytes and bacteria, no growth at the culture. No complications obseved in the post operative period, regular echo follow up. The patient underwent neoadjuvant therapy and then surgery to remove the colorectal mass. Lone pulmonary valve infective endocarditis is a rare disease (1–2%), especially without history of immunodeficiency or drug abuse, we should look for a cause of persistent bacteraemia. Bacteraemia is a frequent condition in presence of colorectal neoplasm or IBD, mostly enterococchi or S.Gallolitycus. Septic pulmonary embolism is a frequent complication. Following guidelines, surgery should be considered only if there is no response to antibiotic therapy, extended tricuspid valve vegetations, recurrent pulmonary embolism. Valve vegetations&gt; 10mm are predictor of poor response to ab therapy and pulmonary embolism.
P328 L‘ INSUFFICIENZA RENALE CRONICA NELLA PROGRESSIONE DELLA STENOSI VALVOLARE AORTICA
Authors: Pagliani L , Rattazzi M , Buso R , Rivaben D , Nicolosi E , Di Naro A , Cernetti C , Antonini–canterin F 
Published: May 2022

Abstract

Background
Calcific aortic valve disease (CAVD) is the western world’s leading degenerative valve disease affecting nearly 2% of the general population. Considered for a long time as the result of a passive process, nowadays CAVD is referred as an active process mediated by different cell types and involving several molecular mediators and cellular pathways.

Aim of the Study
The aim of this study was to investigate the role played by renal function during the progression of CAVD. Materials and Methods – We enrolled 116 patients affected by CAVD from the Cardiology departments of the Treviso Hospital and the High Specialization Rehabilitative Hospital of Motta di Livenza (TV). For each patient at the time of the recruitment a clinical visit was carried out that included the collection of anamnestic, anthropometric and echocardiographic data. Blood samples were collected from each subject at the recruitment to measure the following biochemical parameters: creatinine, lipidic profile, phospho–calcium metabolism. The GFR was estimated by using the CKD–EPI 2009 equation. To investigate the predictive role of renal function in the progression of CAVD, only patients (n = 49) with at least two transthoracic echocardiograms (TTE) were selected for the prospective analysis. These patients were followed for an average of 18.9 ± 11 months. The rate of progression of valvular disease was assessed in terms of difference in indexed valve area per month (ΔAVA/month).

Results
We observed that fast progressors had a significant reduction of eGFR at baseline as compared to slow progressors (60.62 ± 18.83 ml/min/1.73m2 vs. 77.22 ± 11.46 ml/min/1.73m2, p = 0.001). An increased risk of rapid progression of valvulopathy was found in patients with eGFR &lt; 60 ml/min/1.73m2 (RR = 2.64, IC95%=1.50–4.60, p = 0.001) compared to subjects with normal renal function. A close to significance correlation was also found between values of circulating Pi and ΔAVA/month (r = –0.252, p = 0.084).

Conclusions
The present study showed that reduction of renal function, and in particular the presence of CKD (eGFR values &lt;60 ml/min/1.73m2), is significantly associated with an increased risk of rapid progression of calcified aortic valvulopathy; this risk is further increased by the concomitant presence of increased levels of phosphatemia. Overall, these data indicate the potential predictive role of eGFR on the risk of accelerated calcific valvulopathy in non–dialysed patients.
P330 A CASE OF SEVERE AORTIC STENOSIS IN A YOUNG PATIENT WITH BICUSPID AORTIC VALVE, FAMILIAL HYPERCHOLESTEROLEMIA AND CALCIFICATION AT THE SINOTUBULAR JUNCTION
Authors: Brusamolino M , Muratori M , Apostolo A , Mapelli M , Bonalumi G , Nanci G , Werba J , Pepi M , Mantegazza V , Calligaris G , Formenti A , Agrifoglio M , Agostoni P 
Published: May 2022

Abstract

Background
Familial hypercholesterolemia (FH) is a disorder characterized by elevated LDL–C and premature vascular calcifications. Aortic stenosis (AS) is the most frequent complication of bicuspid aortic valve (BAV), often requiring aortic valve replacement. Cardiac surgery in patients with severely calcified ascending aorta is challenging.

Case Presentation
A 28 year old male from Albania presented to the ED for dyspnea and low–threshold angina. The patient had family history for CAD and a sister with known FH treated with PCSK9–i. He had BAV, known hypercholesterolemia (max cholesterol 660 mg/dL), treated since 2015 with rosuvastatin plus ezetimibe, with reported irregular intake. He was treated with PCI and bioresorbable vascular scaffold on LAD coronary artery. He underwent surgical removal of limb xanthomas. At admission, the patient was asymptomatic at rest. Cardiac auscultatory findings included an ejection murmur in the aortic area. He presented upper and inferior eyelid xanthelasmas, bilateral calcaneal tendon thickening, elbows and knee xanthoma removal scars. Blood tests were unremarkable, except for lipid profile (LDL–C 443 mg/dL, HDL 36 mg/dL, TG 73 mg/dL). The echocardiography showed BAV, severe AS (Vmax 4,2 m/s, MPG 41 mmHg, AVA 0.46 cm2/m2), EF 60%. A coronary angiography excluded significant stenosis in the epicardial coronary vessels. An aortic CT scan showed sinotubular junction with preserved diameters and severe multiple parietal calcifications, ascending aorta with diffuse atheromatous disease. The patient underwent mechanical aortic valve replacement, ascending aorta thromboendarterectomy, non–coronary sinus enlargement patch, double CABG (SVG–OM, SVG–LAD) due to diffuse hypokinesia of the left ventricle after the interruption of extracorporeal circulation. At a 3–month outpatient re–evaluation, due to the unsatisfactory response to the regular intake of rosuvastatin plus ezetimibe (TC 309 mg/dL, TG 52 mg/dL, HDL 34 mg/dL, LDL–C 264 mg/dL), a PCSK9–i was prescribed. Genetic studies for FH are ongoing.

Discussion
This case underlines the importance of aortic evaluation before aortic valve replacement, even in young FH patients, in which severe aortic calcification can influence surgical approach.

Conclusion
We described the multidisciplinary management of a severe symptomatic AS in a young male with FH and sinotubular junction parietal calcifications, which represented a challenging substrate for valve replacement.
P336 ELECTRONIC HEALTH RECORDS
Authors: Soriente L , Fresa V , Ruberto M , Di Muro M 
Published: May 2022

Abstract

Introduction
The crisis connected with Covid–19 has had strong economic and social repercussions. However, during the state of emergency with restricted access to healthcare, healthcare institutions and users have tested the potential of dematerialization of services thanks to digitalization. The PNRR have maintained in the health sector (Mission6 Health) a fixed point on the horizontal strategic axis of digitalization and innovation, focusing on proximity networks, intermediate structures, telemedicine for territorial health care (Community Homes and Community Hospitals) and on the research and digitalization of the NHS (Electronic Health Record and Essential Levels of Assistance).Our proposal identifies an integrated path that, with the active participation of professionals from the Hospital, the Territory, GPs, social services and families, it allows an appropriate and rapid taking in charge of the patient in hospital discharge

Methods
From the SDO office with the Quany we found some indicators of Intensive Cardiology: average weight of ordinary DRG (1.8vs2), average hospital stay (9.05vs9.42), rotation index of beds (51vs39) and pertinence of hospitalizations for acute care / mean hospitalization (9.48 vs 9.38) respectively of 2019–2020 in order to be able to identify critical issues and solutions in the pathways of the frail and improve performance.We used the BPI technique by implementing the Electronic Medical Record and integrating modules that can facilitate a protected discharge by reducing the fragmentation of assistance between the different healthcare structures.We have evaluated the medical–legal questions and identified indicators (average time between arrival of the request and evaluation by the figures involved in the Protected Discharge, time average between evaluation and admission to RSA / Intermediate Care, number of patients discharged with treatment on territory.

Expected Results
The mapping of the revised process by sharing informations (patient‘s condition and social context), determines the most appropriate post–discharge intervention setting while containing costs.Conclusion The process of taking charge of the territory allowing for benchmarking and control of the quality levels of the care provided.
P338 PERCEPTION OF THE BENEFITS AND LIMITATIONS OF TELEVISIT IN A GROUP OF PATIENTS WITH CHRONIC HEART FAILURE
Authors: Zanocco A , Calamelli S , Berton A , Cattarina M , Milan S , Sacca S 
Published: May 2022

Abstract

Telemedicine (T) has now become an indispensable operational tool for the management of patients with chronic conditions, in particular for patients with chronic heart failure (HF).
The current COVID 19 pandemic has accelerated the development and application oh this method in daily clinical practice. Up to now, the evaluation of he advantage and disadvantage of televisit by cardiological patients have been very limited. In order to assess the satisfaction and criticalities of this method, we administered a satisfaction questionnaire to 74 patients (49 males and 25 females with an average of 75) followed in our clinic. Of the 74 questionnaire administered, 70 were received. When asked whether the inclusion of T had improved the quality of service 43 patients (61%) answered a lot, 24 patients (34%) sufficiently and 3 patients (4%) not at all. When asked if the organization of the T had been adequate, 50 patients (71%) responded adequately, 19 patients (27%) sufficiently adequate and 1 patient (1%) not at all adequate. When asked about the modalities of connection to the T for 54 patients (77%) they were found to be sìmple, for 13 patients (18%) a little complicated and for 3 patients (4%) difficult, very complicated. When asked what would be proposed to improve the service 7 patients (10%) answered the possible extension of hours also in the afternoon, 3 patients (4%) the provision by the ASL of electromedical devices, 14 patients (20%) the ability to directly request an appointment in T and 38 patients (54%) all options. In detail, 1 patient (1%) requested both the extension of the afternoon schedule and the provision of electromedicals, 4 patients (6%) requested both the extension of the afternoon schedule and the direct request for an appointment, 2 patients (2%) required both electromedical equipment and direct request for an appointment, 1 patient (1%) did not indicate any option. Finally, as regards the degree of satisfaction, 45 patients (64%) were considered totally satisfied, 22 patients (31%) partially satisfied and 3 patients (4%) dissatisfied.

Conclusions
The televisit in the setting of patients with chronic HF, despite the limitations of a limited series, was generally well accepted, easy to access and with some useful operational proposal for public administration.
P339 MENAGEMENT OF CARDIOGENIC SHOCK IN A LOW–VOLUME CARDIOLOGY CENTER
Authors: Biscottini E , Andreoli C , Tutarini C , Quintavalle E 
Published: May 2022

Abstract
Cardiogenic shock (CS) is a desaese burdened by high mortality rates, but lower in high–volume cardiology centers. Early diagnosis and intensive treatment are essential for prognosis. In our low–volume center, for menagement of CS, is used a protocol based on arterial blood gas analysis serial monitoring, invasive blood pressure, echocardiographic hemodynamic parameters; underlying acute ischemia revascularisation; non–invasive mechanical ventilation; use of inotropic/vasopressors drugs and eventually circulatory support by aortic counterpulsation (IABP). Starting from hemodynamic characteristics of the patient, clinician is so supported in his choices in order to achieve an adequate peripheral perfusion. In case of failure to reach targets for each parameter, meaning the persistent shock status, the protocol helps to select patients with low probability of cardiac function recovery and those with age, comorbidities and life expectancy, deserving extracorporeal membrane oxygenation as a bridge to recovery, transplant or ventricular assist device at a high–volume center. The management of CS in our low–volume center could benefit of Impella, an advanced circulatory support allowing the left ventricular unloading and a more likely patient stabilization until the transfer to high–volume center. In low–volume centers, IABP often is the only option avaliable in CS not responsive to inotropic and vasopressors drugs. However, at present, little evidence in literature support its use. A promptly stratification of patient severity prevents to underestimate pre–shock signs and avoids delays causing further hemodynamic worsening. We think that is necessary to build a CS network, similar to that for percutaneous treatment of ST–elevation myocardial infarction.