bluuebunny/crossref_metadata_2025_split huggingface dataset:
507 LONG-TERM SUBCLINICAL HEART INVOLVEMENT IN PATIENTS WITH IDIOPATHIC INFLAMMATORY MYOPATHIES
Authors: Paolini Federico , Stronati Giulia , Brugiatelli Leonardo , Bastianoni Gianmarco , Alfieri Michele , Moroncini Gianluca , Danieli Maria Giovanna , Guerra Federico 
Published: December 2022

Abstract

Introduction
Global longitudinal strain (GLS) is a validated and reproducible technique for the measurement of LV longitudinal deformation. There is strong evidence of the prognostic value of GLS in patients with HF and is associated with cardiac mortality independent of clinical and cardiac status. In the present study, we aimed to assess whether GLS can reliably predict prognosis in patients diagnosed with idiopathic inflammatory myopathies.

Methods
We conducted a prospective observational study enrolling all consecutive patients referred to our clinic between June 2016 and January 2022 with a confirmed diagnosis of IIM and no overt cardiac disease. For each patient, echocardiogram and GLS calculations were done at baseline and at each follow-up. All patients enrolled have been revalued from two to four times during the median 2.5-year follow-up period.

Results
Forty patients (67% female, mean age 56±12 years) were consecutively enrolled. We detected a significant deterioration of the GLS of the LV (from -19.3±3.6%to -16.8±4.6%; p = 0.006) during the follow-up, while the GLS of the RV was unchanged. We also noticed an increase in the filling pressures as estimated through E/E’, which resulted to be statistically significant (6.5±3.9 to 9.0 ±3.5; p = 0.002), underlying a subclinical diastolic dysfunction of the LV. Traditional markers of systolic dysfunction, such as EF and TAPSE, did not show a significant worsening during the follow-up period. Patients developing a reduction in LV GLS &gt;5% during follow-up experienced an increased incidence of cardiovascular-related death (19% vs 6%; p=0.03).

Discussion
Cardiovascular manifestations constitute a major cause of death in myositis. Although these complications rarely occur clinically, subclinical manifestations such as conduction disturbances, ventricular dysfunction, myocardial infarction and arrhythmias are often observed. This risk appears to increase especially in the first years after diagnosis. There is therefore a problem of the early recognition of patients who may develop heart disease. Several studies propose to use magnetic resonance imaging as a gold standard to select patients at risk, but its high cost and limited availability make it difficult to use as a screening test. The GLS, consistently with the estimated filling pressures, has demonstrated a high predictive power of cardiovascular-related death in our study, without a modification of the traditional systolic function values.

Conclusion
The GLS has a high power predictive, it is low cost and highly repeatable, therefore in our opinion, it could play an important role as a screening test in identifying patients with a diagnosis of IIM at higher risk of developing cardiovascular-related death.
80 GENETIC MYOCARDITIS: ADVANCED WORKUP AND TREATMENT
Authors: Peretto Giovanni , De Luca Giacomo , Villatore Andrea , Sala Simone , Di Resta Chiara , Palmisano Anna , Vignale Davide , Campochiaro Corrado , De Gaspari Monica , Rizzo Stefania , Busnardo Elena , Ferro Paola , Gianolli Luigi , Benedetti Sara , Basso Cristina , Dagna Lorenzo , Esposito Antonio , Della Bella Paolo 
Published: December 2022

Abstract

Background
Preclinical data support the rationale for targeting myocardial inflammation in genetic nonischemic cardiomyopathies. However, no consistent clinical reports have been provided so far.

Methods
We describe a series of patients (n=25) with genetic cardiomyopathy and active myocardial inflammation (AMI) proven by multimodal diagnostic workup. Patient-tailored immunosuppressive therapy was empirically started to target myocardial inflammation whenever feasible. Multiple outcomes were retrospectively assessed by a dedicated multidisciplinary disease unit.

Results
Patients carrying desmosomal (DGV), cytoskeletal (CGV) and membrane gene variants (MGV), were 12 (48%), 10 (40%), and 3 (12%), respectively. DGV carriers uniformly presented with myocarditis-like chest pain and ventricular arrhythmias, whereas heart failure symptoms were found only in CGV carriers. Dilated cardiomyopathy phenotype and ring-like pattern on cardiac magnetic resonance allowed the best discrimination among genotypes. AMI was proven by endomyocardial biopsy in all cases, and by noninvasive imaging in 21 (83%). IST was started in 17 patients (71%) with no safety issues. By the end of follow-up (median 69 months, range 21-182), signs of AMI were documented in 6/18 IST receivers (33%) and 4/7 untreated cases (57%). For most genotypes, paucity of adverse events was noted while on immunosuppression as compared to the off-treatment period.

Conclusion
Our preliminary data provide the clinical rationale for the research and targeting of AMI in a spectrum of genetic nonischemic cardiomyopathies.
505 A NEW NEXN GENE VARIANT LEADING TO FOETAL ONSET MIXED PHENOTYPE CARDIOMYOPATHY WITH UNKNOWN PROGNOSIS
Authors: Ratti Angelo , Irene Picciolli , Rinaldi Berardo , Bedeschi Maria Francesca , Colli Anna Maria , Carugo Stefano 
Published: December 2022

Abstract

Background
Idiopathic dilated cardiomyopathy (DCM) is a common cardiac disease phenotype known to result from many different triggers. Among possible etiologies, genetically determined forms have been more and more acknowledged: recent investigations made possible the identification of risk alleles and disease-causing variants encoding for structural components of the cardiac muscle. The NEXN gene encodes the protein nexilin, an essential protein for Z-disk stability. Homozygous NEXN variants are rare and associated with poor prognosis: fetal and perinatal death or severe DCM in infants.

Case description
we present the case of a male infant, born at term, with prenatal diagnosis of cardiomyopathy. Postnatally, DCM with severely reduced systolic function was confirmed (LVEDd 19 mm, +2 z-scores; LVEF 25%), along with biventricular hypertrophy. The infant required medical treatment for heart failure; atrial tachyarrhythmia responsive to amiodarone was associated. Family history oriented for a genetic and familiar form of DCM (consanguinity and sudden death at a young age on both sides). Whole Exome Sequencing revealed in the proband the homozygous variant c.1156dup (p.Met386fs) in the NEXN gene (NM_144573.4), and both parents were confirmed to be carrier of the same variant at the heterozygous state. This NEXN variant has not been described previously. Complete cardiac evaluation was performed in first-degree relatives, including echocardiography and Cardiac Magnetic Resonance; the mother shows early signs of CMP, such as LBBB with mildly reduced LVEF (53%), while the only remarkable finding for the father is mild left ventricular hypertrophy.

Discussion
genetic DCM can have different clinical courses. It is important to reach the diagnosis: family history is essential in raising suspicion, while infectious causes and inborn error of metabolism must be ruled out. Only a few cases of NEXN-related CMP are described in literature; surprisingly, and unlike what was previously described, we observed in our patient a favorable clinical course during time, suggesting a different severity compared to so far known NEXN variants.

Conclusion
This case helps to broaden present knowledge about NEXN variants and their clinical expression in humans, supporting the inclusion of the NEXN gene in the investigation of patients with DCM, in order to understand phenotypes, guide therapy, and provide proper genetic advice.
560 A RARE CASE OF PERICARDIAL SARCOMA
Authors: Ruggiero Rossella , Di Cesare Annamaria , Fabrizio Michele , Di Domenico Assunta , Gardi Andrea , Moriconi Veronica , Gaudenzi Eleonora , Spighi Lorenzo , Piovaccari Giancarlo 
Published: December 2022

Abstract
A 47 years old man, with no previous medical history, presented to the emergency department for dyspnea increased over the prior weeks. The echocardiographic evaluation revealed a severe pericardial effusion. Moreover, a round-shaped, indeterminate mass was detected into the pericardium, partially enhanced after injection SonoVue R contrast. After the pericardiocentesis, to exclude a severe dilatation of the coronary sinus, the patient underwent to a coronary angiography that showed a diffuse vascularization of the region of the mass. A CT-scan were then performed, and confirmed the presence of a well-defined mass placed near to the left atrial-ventricular junction into the pericardium. The magnetic resonance revealed a limited area of infiltration of the lesion into the myocardium. Finally the patient underwent surgical asportation, and the biopsy showed an high-grade sarcoma. Primary cardiac tumors are rare diseases and in the majority of the cases are of benign nature. Among malignant tumor of the heart sarcoma is the most common. Despite their rarity, malignant heart cancers are associated with high mortality and the prognosis is mostly influenced by the localization of the tumor, its biological aggressiveness, and the difficulty in obtaining surgical radicality. Cardiac sarcomas are most likely to arise from the right atrium followed by right ventricle and less often from the epicardium. In these cases, the asportation of the mass can be challenging, and the risk of myocardial narrowing can affect the area of resection.
We reported a rare case of primary cardiac sarcoma mainly localized into the pericardium. Due to its position, the surgical asportation of the tumor was considered complete and our patient was discharge from the hospital without further complication and with a program of chemotherapy.
At six months follow-up no recurrence of disease was observed.
674 FOCUSING ON ADHERENCE TO INFECTION PREVENTION AND CONTROL RECOMMENDATIONS: FACILITATORS AND BARRIERS. AN EXPLORATORY STUDY OF THE ROLE OF HEALTHCARE PROVIDERS IN CARDIOLOGY SETTINGS
Authors: Chiaretti Ludovica , Ruggiero Antonio , Chiala´ Oronzo 
Published: December 2022

Abstract

Introduction
Cardiovascular devices and their associated placement, management and maintenance require adequate adherence to infection prevention and control (IPC) programs, which are paramount to patient and healthcare provider safety. The lack of control of adverse events and complications due to non-compliance with guidelines and recommendations is clearly a potential risk that needs to be known and avoided. Therefore, it is crucial to identify and describe the potential enabling factors and existing barriers to the use of and adherence to infection control guidelines and recommendations by healthcare providers in the cardiovascular setting.

Methods
An observational study was conducted involving 53 healthcare providers from cardiovascular settings. An online survey was used to explore sociodemographic information, attitudes towards evidence-based practice and its application (IPC-COQ), factors influencing adherence to standard precautions (FIASP Adapted), and potential barriers and facilitators.

Results
The sample was mostly composed by female providers (66%) with a mean age of 41 years. The Attitude domain score (IPC-COQ) was 3.87 (+-0.44), while the Utilization domain score was 3.20 (+- 0.61). The overall FIASP score was 2.84 (+-0.44). The main barriers to adherence reported were: poor relationship between health providers and patients, heavy workload, limited availability of PPE; of the facilitators of adherence, the most frequently cited were: interest in infection control in my department, personal motivation to adhere to EPB, peer involvement. A significant relationship was found between the domains IPC-COQ, FIASP and facilitators and barriers.

Discussion
The identification of potential barriers and facilitators to adherence to IPC recommendations in cardiology could serve as a starting point for the development and implementation of guideline and recommendation adherence strategies by healthcare providers. This will naturally support decision-making processes from which to infer which of these factors need to be removed and which need to be strengthened in order to improve the efficiency and utility of IPC programs in cardiology settings.
413 CARDIOVASCULAR CHANGES MICROGRAVITY-RELEATED: SYSTEMATIC REVIEW OF THE LITERATURE
Authors: Vecchiet Arisa , Li Vigni Myriam Letizia , Satiro Francesco 
Published: December 2022

Abstract

Introduction
Microgravity-relevant space is characterized by different environmental conditions than terrestrial space, including microgravity, vacuum/hypovaria, acceleration, extreme temperature, ionizing radiation, and confinement/isolation.
The space environment induces biomechanical and cardiovascular stress during both takeoff and landing, exposing astronauts to the development of cardiovascular disease (CVD). This literature review focuses on cardiovascular changes that can be traced to an aerospace mission. (Baran, R., et All. 2021).

Materials and Methods
A systematic literature review was conducted to assess the cardiovascular changes that occur on the human body in microgravity. The search was performed using PUBMED as the database. The inclusion criteria established, for an article to be used, was the relevance of the topic.
The choice of articles that were subsequently included in the study was based on predetermined questions and inclusion and exclusion criteria in accordance with PRISMA (Preferred Reporting Items for Systematic reviews and Meta-Analyses). The quality of the study was assessed by two reviewers and then discussed with a third reviewer using the DIXONWOOD checklist.

Results and Discussion
Baran, R. et all studied cardiovascular changes during spaceflight in vivo and related molecular changes in vitro that, were outside the scope of pre-flight cardiovascular disease. These changes are related to several factors, including the absence of orthostatic pressure, and reduced blood pressure, which results in a depletion of cardiac workload, sometimes leading to cardiac atrophy, i.e., reduced ventricular size in cosmonauts following a mission. In the face of these, the scientific community is investigating through pre- and post-flight simulation tests the adaptation and prevention of the cardiovascular system.
An exposure to microgravity greater than 6 months cascades numerous physiological adaptations. Indeed, there is a shift in body fluids, arrhythmias, and an increase in systolic output (35-46%) and cardiac output (18-41%). Despite these increases, astronauts may enter a hypovolemic state and cardiogenic shock (10-15% decrease in volaemia). (Baran, R., et All. 2021). In addition to this, prolonged stay in Space, exposes, the cosmonaut to syncopal events of cardiogenic origin in the re-entry and post-landing phase. (Meck, J. V., et All. 2001).

Conclusions
Exposure to microgravity conditions results in significant changes in cardiovascular function. Further clinical studies in larger samples are, however, needed to understand and prevent microgravity exposure-related CVD.
For future applications and theoretical-practical investigations, there also emerges the need, in view of increasingly longer and riskier missions̀ health teleconsultations, for the presence of the health care team on board the shuttle or at the bases alreadỳ present in Earth orbit.
376 CARDIAC CONTRACTILITY MODULATION: A NEW THERAPY FOR HEART FAILURE
Authors: Pettillo Vincenzo , Artiola Gaetano 
Published: December 2022

Abstract

Introduction
Cardiac Contractility Modulation is a therapy for chronic Heart Failure patients with EF 25-45% in sinus rhythm with QRS &lt; 130 ms clinically symptomatic (NYHA Class &gt; II) despite optimised medical therapy. It is made possible by the implantation of the OPTIMIZER SMART® device. The CCM works by delivering a non-excitatory high-energy bipolar signal, synchronised with local electrical activity (or cooperating with another pre-existing CIED), in the absolute ventricular refractory period, by means of two actively fixating leads, positioned in the interventricular septum and spaced at least 1-2 cm apart.

Purpose
Assessment and feedback of the CCM therapy process in patients with dilated cardiomyopathy. Educational update for health professionals. Patient education.

Method
A Pubmed search correlated with clinical data and field experience was performed.

Results
Patients with severe Dilated Cardiomyopathy already on optimised medical therapy. The mechanism of action demonstrated a remodelling of the left ventricle with a so-called 'metabolic' therapy. In CCM therapy, electrical stimulation is delivered to the heart muscle during the absolute refractory period. In this phase, electrical signals activate the mobilisation of calcium ions in cardiomyocytes. CCM signals applied during the absolute refractory period cause an increase in cytosolic calcium during systole, resulting in improved cardiac contraction without an increase in oxygen consumption.

Conclusions
CCM therapy has shown to improve contractility and cardiac performance improving the quality of life of patients undergoing this treatment by reducing symptoms of fatigue and dyspnoea; it has reduced the number of hospitalisations for moderate to severe heart failure. Healthcare professionals recognise The typical ECG of a patient implanted with a CCM device shows a 'spike' in the absolute refractory period of the cardiac cycle: the 'R wave' of the QRS complex without being alarmed. The patient has been trained to be autonomous in charging. OPTIMIZER SMART® is recharged externally with a weekly rechargeable battery through a mini induction charger, rechargeable by itself.
320 THE POTENTIAL ROLE OF ECHOCARDIOGRAPHY IN THE PRE-PARTICIPATION SCREENING: RESULTS OF AN OBSERVATIONAL RETROSPECTIVE STUDY
Authors: Donati Federica , Guicciardi Claudio , Lodi Elisa , Modena Maria Grazia , Palermi Stefano , Biffi Alessandro , Fernando Frederick 
Published: December 2022

Abstract

Introduction
Preparticipation screenings (PPs) have been conceived for the potential to prevent SCD in young athletes by early identification of hidden cardiac diseases. Commonly used protocols include history collection, physical examination and baseline electrocardiogram. Nevertheless, transthoracic echocardiography has been hypothesized to have a primary role in the PPs.

Aim of the Study
The aim of the present paper was to evaluate the potential role of echocardiogram in identifying life-threatening cardiovascular abnormalities that might be undetected by commonly used PPS.

Methods
We retrospectively reviewed a corporate wellness program - database and analyzed data recorded from 2017 to 2022 to compare two medical models: a “standard” PPS including medical history, physical examination, electrocardiogram (ECG) and exercise stress test (EST); an “advanced” PPS comprising history, physical examination, ECG, EST and echocardiography. The primary outcome of the study was to assess the efficacy of the advanced PPS in detecting CV abnormalities compared to the “standard” PPS.

Results
We analyzed data from 500 subjects (420 male, 33.69 ± 7.9 mean aged) enrolled for the first time in the corporate wellness program between 2017 and 2022. 339 (67.8%) subjects had no pathological findings at “standard” PPS and, even if would have not required further evaluation including echocardiogram, performed the ultrasound exam anyway (“advanced” PPS): 31 (9.1%) showed some abnormal findings at echocardiography.

Conclusions
Even if with some limitations, performing a screening echocardiogram could help to detect subjects with some form of cardiovascular morpho-functional abnormalities, otherwise difficult to diagnose with “standard” PPS protocol.
887 THE EFFECTS OF CONCURRENT AEROBIC PLUS RESISTANCE EXERCISE ON BLOOD PRESSURE VALUES AND VARIABILITY AND ARE BLUNTED IN HYPERTENSIVE SUBJECTS WITH NONDIPPING NOCTURNAL BLOOD PRESSURE PROFILE
Authors: Caminiti Giuseppe , Marazzi Giuseppe , Perrone Marco Alfonso , Iellamo Ferdinando , Sposato Barbara , Franchini Alessio , Di Carlo Roberto , Volterrani Maurizio 
Published: December 2022

Abstract

Background
The purpose of this study was to investigate the effects of 12-weeks of concurrent aerobic plus resistance exercise training (CT) on short –term blood pressure variability (BPV) and BP values in hypertensive patients with nondippper BP nocturnal pattern and underlying coronary artery disease.

Methods
The study included 64 patients who were divided into two groups according to the nocturnal BP pattern: dipping pattern (29 pts) and nondipping (35 pts). Before starting CT and at 12 weeks patients underwent ergometric test, assessment of 1-repetiton mximun (1RM), and 24/h BP monitoring (24-h ABPM).

Results
after CT Twenty-four/h systolic BPV and daytime systolic BPV decreased significantly in the dipping group (-1.2 mmHg and -1.4 mmHg respectively) while they were unchanged in the nondipping group (-0.2 mmHg and -0.1 mmHg). Twenty-four/h systolic BP and daytime systolic BP decreased significantly in the dipping group (-8.3 mmHg and -8.5 mmHg respectively) while were unchanged in the nondipping group (-1.2 mmHg and -0.7 mmHg). Nighttime systolic BP and BPV was unchanged in both groups.

Conclusion
the effects of CT on BP and BPV are blunted in hypertensive subjects with nondipping BP pattern. Further studies are needed in order to find out more effectives training modalities for these patients