bluuebunny/crossref_metadata_2025_split huggingface dataset:
CARDIAC AMYLOIDOSIS IN ELDERLY WITH POSITIVE SCINTIGRAPHY AND MONOCLONALITY: A DIAGNOSTIC DILEMMA
Authors: Alfarano M , Giordano C , Marchionni G , Berni S , Ballatore F , Costantino J , Tocci M , Lavalle C , Vizza C , D‘Amati G , Chimenti C 
Published: May 2024

Abstract

Background and Aims
Clinical identification of elderly patients with both wild–type transthyretin amyloidosis (ATTR) and monoclonal gammopathy of unknown significance (MGUS) is increasing. For this reason, accurate amyloid histologic subtyping is crucial in case of concomitant positivity of bisphosphonate scintigraphy and monoclonal protein testing. We report the diagnostic work–up of cardiac amyloidosis in this specific patient subtype in our tertiary referral center.

Methods
Among 133 patients who received the diagnosis of cardiac amyloidosis from March 2021 to March 2023, 21 (16%) presented with an abnormality of both bisphosphonate scintigraphy and at least one of the serum monoclonal protein tests. Patients underwent abdominal fat biopsy/needle aspiration as the first diagnostic procedure. In case of negative results, an endomyocardial biopsy (EMB) was planned. Amyloid was identified at histology by Congo Red staining. Amyloid typing was obtained by immunohistochemistry using a recently developed amyloid antibody panel AmYkit (Amimed) applicable on fixed paraffin–embedded tissues in an automated platform. The results were compared with those obtained with standard immunohistochemistry.

Results
Sixteen of the 21 patients were older than 75 years (76%, 81±10 ys) with a grade 3 (62%) or 2 (33%) scintigraphy. A grade 1 positive scintigraphy was detected in one patient. Serum immunofixation revealed a previously unknown monoclonal protein peak in 78%, while 22% had a history of MGUS. None of the patients had hematologic disorders. TTR pathogenic mutation was detected in one patient. On pathologic examination, abdominal fat was negative for amyloid in all cases, while Congo Red staining was positive in all examined EMBs. Immunohistochemistry performed with standard antibodies was not able to discriminate between AL and ATTR, due to signal overlapping of variable intensity. Conversely, our preliminary results with AmYkit panel of antibodies demonstrate absence of signal overlapping in over 90% of cases, allowing accurate histologic discrimination between AL and TTR.

Conclusion
Based on our clinical and histologic observations, in elderly patients with concomitant positivity of both bisphosphonate scintigraphy and monoclonal protein testing, TTR is the most frequent type of cardiac amyloid. EMB is a useful diagnostic tool to confirm the clinical suspicion of amyloidosis. Accurate amyloid subtyping is granted by a more sensitive panel of antibodies.
TAFAMIDIS FOR TREATMENT OF TRANSTHYRETIN CARDIAC AMYLOIDOSIS: A REAL WORLD EXPERIENCE
Authors: Vagnarelli F , Brugiatelli L , Lofiego C , Capodaglio I , Patani F , Tofoni P , Maurizi K , Pietrucci F , Romandini A , Contadini D , Lanari A , Bianco M , Terzi F , Paci M , Olivieri R , Flori M , Dello Russo A , Perna G 
Published: May 2024

Abstract

Background
A regional network for cardiac amyloidosis (CA) was created in 2019 with the aim of enhancing disease awareness among physicians and favoring appropriate access to innovative diagnostic tools/therapies. The network includes all 14 Cardiology Departments and outpatient clinics from 5 Local Health Units. Therapies for variant (ATTR–v) and wild–type (ATTR–wt) CA have recently become available in Italy. Selected centres with expertise in cardiomyopathies were identified to prescribe new modifying disease drugs. Tafamidis is the first medication approved for the treatment of ATTR–C. The landmark ATTRACT trial showed a reduction in cardiovascular mortality and cardiovascular–related hospitalization in patients treated with tafamidis compared to placebo. From October 2021 tafamidis can be prescribed in Italy according to AIFA criteria (only NYHA I/II patients eligible).
The aim of our study is to present the experience of our Network in prescribing tafamidis and to compare the clinical baselibe characteristics of our population and the ATTRACT trial population.

Methods
Thanks to our network , 189 patients with ATTR–CA were referred to our Center. 58 ATTR patients were treated with tafamidis according to AIFA criteria, while 5 patients with variant–TTR and neuropathy received patisiran iv. The causes of exclusion from tafamidis therapy are shown in figure 1. We prospectively collected all the patientst treated with tafamidis from January 2022 until November 2023. All the data about clinical characteristics, laboratoy findings ECG, echo, bone scintigraphy and concomitant treatment were collected during routin follow–up visits anc compared to those of ATTRACT trial population.

Results
The main findings of our study are summarized in Table (fig,2) The age of our population was similar to that of the patients enrolled in the ATTR–ACT trial (75,94 ± 11.4 vs. 74,5±7.2, P = 0.21). Of note, our population does not include patients in NYHA class III as the AIFA prescriptive criteria exclude these patients from treatment in Italy. this implies a substantial difference in the distribution of the NYHA class compared to the attract population (p = 0.001). The outcome of our patient was excellent, the drug was well tolerated. Conclusions: Implementing specific clinical network provided excellent results, allowing a precise phenotype/genotype characterization and favoring appropriate access to specific disease–modifying drugs.
EFFICACY OF PERCUTANEOUS STELLATE GANGLION BLOCK ACCORDING TO VENTRICULAR ARRHYTHMIA CYCLE: A SUB–ANALYSIS OF THE STAR STUDY
Authors: Baldi E , Rordorf R , Compagnoni S , Dusi V , Sanzo A , Gentile F , Frea S , Gravinese C , Cauti F , Iannopollo G , De Sensi F , Gandolfi E , Frigerio L , Crea P , Zagari D , Casula M , Sangiorgi G , Persampieri S , Dell‘Era G , Patti G , Colombo C , Mugnai G , Notaristefano F , Barengo A , Falcetti R , Perego G , D’Angelo G , Tanese N , Currao A , Sgromo V , De Ferrari G , Savastano S 
Published: May 2024

Abstract

Introduction
The percutaneous stellate ganglion block (PSGB) is an effective treatment for electrical storm. The STAR study (184 PSGBs performed on 131 patients in 19 Italian centers) demonstrated a significant reduction in delivered electrical therapy (ATP/shock) in patients with electrical storm comparing both the 12 hours before and after PSGB, and 1 hour before and after PSGB. The current aim is to assess whether the efficacy of PSGB is influenced by the arrhythmias’ cycle length prior to the procedure.

Methods
This is a sub–analysis of the STAR study. We divided PSGBs into two groups according to the cycle length of the arrhythmia preceding PSGB: the fastVT–VF group included PSGBs performed following a ventricular tachycardia (VT) with a cycle &lt;400 msec or a ventricular fibrillation (VF), whereas the slowVT group included PSGBs performed following a VT with a cycle ≥400 msec.

Results
The cycle length of the arrhythmia was available for 170 PSGBs. The fastVT–VF group included 127 PSGBs, performed on males in 83% of cases, with a mean age of 65±12 years, ejection fraction (EF) of 28±13% and ischaemic aetiology in 64%, while the slowVT group included 45 PSGBs, performed on males in 84% of cases, with a mean age of 68±13 years, EF of 24±11% and ischaemic aetiology in 60%. Comparing the number of electrical therapies delivered between the hour after and before PSGB in the two groups separately there was a significant reduction for both fastVT–VF group (Hodges–Lehmann median difference –3.5 [95%CI –4 to –2.5], p&lt;0.001) and slowVT group (Hodges–Lehmann median difference –0.5 [95%CI –2 to 0], p=0.03). Comparing the difference in electrical therapies delivered in the first hour after PSGB compared to the previous hour between the two groups, a significantly greater reduction was observed for fastVT–VF group than for slowVT group (–2 [IQR –6 to 0] vs 0 [IQR –2 to 0], p&lt;0.001). Considering the whole population, having a fast VT–VF was significantly associated with a reduction in therapies delivered in the hour following PSGB both at univariable analysis (OR 3.5, 95%CI 1.7–7, p&lt;0.001) and after correction for the number of therapies delivered in the hour before (OR 2.5, 95%CI 1.1–5.6, p=0.03).

Conclusions
PSGB is an effective procedure to reduce the number of electrical therapies delivered in patients with both fast and slow ventricular arrhythmias, but the extent of benefit seems greater in those with fast ventricular arrhythmias or ventricular fibrillation.
MID–TERM OUTCOMES OF ENDOSCOPIC MITRAL VALVE REPAIR VIA RIGHT ANTERIOR MINI–THORACOTOMY
Authors: Squiccimarro E , Margari V , Kounakis G , Visicchio G , Pascarella C , Carbone C , Paparella D 
Published: May 2024

Abstract
The adoption of less–invasive mitral repair (MVr) surgery is increasing, backed by evidence of satisfactory short–term results and lower major morbidity. We assessed mid–term follow–up results of our experience, and compared two techniques: isolated leaflet resection and neochord implantation for posterior leaflet (PML) prolapse. Between 2012–2021, 700+ operations were performed with a video–assisted right mini–thoracotomy (RMT) approach. We report 309 consecutive patients who had endoscopic MVr, including those receiving concomitant tricuspid repair, ablation for atrial fibrillation, closure of patent foramen ovale and/or left atrial appendage. The early and mid–term follow–up results were ascertained. Primary outcome was the incidence of mortality and significant mitral regurgitation (MR) during follow–up which were summarized by Kaplan–Meier estimator and compared between treatment arms via log–rank test. Secondary outcomes were the early–postoperative results including 30–days mortality and major complications. All patients had endoscopic MVr via RMT at the 3rd/4th intercostal space (IS). A soft–tissue retractor is used, accompanied or not by a rib spreader, and two ports in the fourth and sixth IS are used for 3D thoracoscopy and carbon dioxide insufflation. Femoral vessels exposure through a right groin incision and echo–guided cannulation sec. Seldinger is achieved post–heparinization. Thoracic fascia bisection is performed during single–lung ventilation using a double–lumen endotracheal tube, integral to our anesthetic management, including percutaneous right internal jugular vein cannulation. The pericardium is opened and an aortic root cannula for cardioplegia delivery and venting is placed via working incision. After establishing cardiopulmonary bypass, a Chitwood clamp is passed through the second IS and cardioplegia is delivered. Endoaortic balloon clamping is primarily used in redo cases. With ring annuloplasty, 136 (44%) patients received PML resection (122 isolated) and 97 (31%) PML chords implantation (88 isolated). Forty–nine patients had annuloplasty alone. In–hospital mortality was 1.0%. Mean follow–up was 29±22 months (max 8.3 years). Kaplan–Meier 5–year survival was 97.3±1.0%. MR (3/4+) free–survival at 5 years was 94.5±2.3%. Subgroup time–to–event analysis for the indexed outcomes showed no statistical significance between techniques. In conclusion, endoscopic MVr is safe with excellent mid–term outcomes.
ROLE OF CORONARY CT IN THE EMERGENCY ROOM IN THE PATIENTS WITH CHEST PAIN
Authors: Verde M , Cavalieri A , Matera S , Macali L , Buffa V , Madau M , Petrolati S , Taberini L , Terranova A , Polletta B , Stolfi A , Gabrielli D 
Published: May 2024

Abstract

Objective
Assess the use of Coronary CT in patients attending the emergency room (ED) with chest pain and/or doubtful signs/symptoms of acute coronary syndrome (ACS).

Background
Patients coming to the ED for chest pain are an important challenge. According to the guidelines (ESC 2023) it is necessary a fast selection between those who need hospitalization and urgent coronary angiography (rule–in) and those who can be discharged home (rule–out). However, it is difficult to obtain an early diagnosis and therefore patients often need a prolonged observation.

Materials and Methods
We subjected to Coronary CT 104 patients (pts) with symptoms and/or alteration of parameters suspected for ACS (ECG, echocardiography, high sensitivity troponin) arrived in the cardiological holding (cardiological observation) of the San Camillo Forlanini Hospital from the emergency room. Clinical–instrumental data, incidence of positive results on Coronary CT and concordance with coronary angiography, waiting time for the examination and length of hospitalization were retrospectively evaluated.

Results
The average age of the pts was 56 ± 2 years, 65 men (62.5%) 39 women (37.5%). Coronary CT exams were performed on the first day after admission to the ED in 83% of cases. Pts presented with chest pain (61% noncardiac, 13% cardiac), and/or palpitations (10%), and/or dyspnea, presyncope, or syncope (overall 16%). CT was performed in 79% of cases due to the association of symptoms and instrumental findings (ECG 54% of cases, hs troponin 34%, echocardiogram 12%); in 21% only for clinical findings. Coronary CT excluded obstructive coronary artery disease in 95 out of 104 pts (91.3%) and was positive in 6/104 pts (5.8%), with coronary angiographic correspondence in 5 cases. In 3/104 (2.9%) the test resulted doubtful/positive (angiographic confirmation in a single case). Only 8.7% of pts underwent coronary angiography (those with positive or doubtful CT result). In all pts with chest pain only (21%) the Coronary CT was negative. Troponin positivity (34% of pts) was not strictly associated with the presence of coronary artery disease: in 94% of pts with positive hs troponin (average value 500 pg/mL) the CT scan did not show critical lesions. The average stay of pts with negative Coronary CT was 1.5 days.

Conclusions
Coronary CT can significantly reduce the use of coronary angiography and the length of stay in pts coming in ED with chest pain and/or doubtful signs/symptoms of ACS.
IMPACT OF DIFFERENT P2Y12 INHIBITORS IN ACUTE CORONARY SYNDROME PATIENTS WITH SEVERE CHRONIC KIDNEY DISEASE: INSIGHTS FROM THE CORALYS REGISTRY
Authors: Giacobbe F , Giuseppe G , Stefano S , Arianna M , Andrea G , Sagazio E , Marchini F , Wanha W , De Filippo O , Bruno F , D‘Ascenzo F , De Ferrari G 
Published: May 2024

Abstract

Background
The impact of the most potent P2Y12i ticagrelor and prasugrel after acute coronary syndrome in patients with chronic kidney disease (CKD) remains unclear. Indeed, this condition is associated to both higher thrombotic and hemorragic risk, as a consequence newer P2Y12i are seldom used. Most evidence comes from real–world study but is limited to GFR under 60 ml/min whereas no data are available regarding patients with severe CKD (stages IV–V).

Methods
Consecutive patients with acute coronary syndrome (ACS) treated with percutaneous coronary intervention (PCI) and severe CKD defined as GFR under 30 ml/min not on dialysis from the CORALYS (Incidence and Predictors of Heart Failure After Acute Coronary Syndrome) registry were included. Incidence of Net Clinical Adverse Events (NACE) defined as MACE (a composite of cardiovascular death, myocardial infarction and unplanned revascularizations) in addition to major bleedings (classified as BARC 3–5) was our primary endpoint. MACE and their single components, all bleedings, major bleedings and hospitalizations for heart failure were the secondary ones.

Results
Of 14699 patients in the CORALYS registry, 257 presented with severe CKD not on dialysis and had available data on antiplatelet regimen at discharge. Patients were divided into two groups according to the type of P2Y12i prescribed at discharge: 193 (75.1%) were given clopidogrel while 64 (24.9%) with ticagrelor or prasugrel. Our cohort was predominantly male (153, 59.5%) with an average age 75.8 ± 10.9 years old. Average GFR was 21.8 ± 6.3 ml/min. Over a median follow–up of 459 days. Ticagrelor and Prasugrel were associated to lower rates of NACE compared to Clopidogrel, driven by an excess of CV mortality and myocardial infarction (77 (39.9%) vs 16 (25.0%), Adj Hr 0.41 95%CI 0.23–0.74, p=0.03). No difference was reported regarding overall bleeding events (5.7% vs 4.6% with clopidogrel and new P2Y12i respectively, p=0.81) and major bleedings 1.7% vs 4.6% with clopidogrel and new P2Y12i respectively, p=0.15).

Conclusions
In ACS patients treated with PCI with severe CKD ticagrelor and prasugrel were associated to lower risk of NACE, MACE, CV death and myocardial infarction compared to clopidogrel. Regarding bleedings, no significative difference was reported despite a relative increase of major bleedings in the group treated with newer P2Y12i.
DAPAGLIFLOZIN EFFECT ON CARDIOPULMONARY, ECHOCARDIOGRAPHIC FUNCTION, BIOMARKERS AND NYHA CLASS IN HFREF PATIENTS
Authors: Mapelli M , Mattavelli I , Salvioni E , Mantegazza V , Garlaschè A , Vignati C , Gugliandolo P , Capovilla T , Paganin C , Nepitella A , Piotti A , Caputo R , Nava A , Campana N , Agostoni P 
Published: May 2024

Abstract

Background
Sodium–glucose co–transporter–2 inhibitors (SGLT2i) are currently a standard therapy for patients with heart failure with reduced ejection fraction (HFrEF).
This study aims to assess the effects of Dapagliflozin on exercise capacity, cardiac biomarkers, fluid retention, renal and pulmonary function. Methods: We prospectively enrolled stable HFrEF outpatients (left ventricular ejection fraction, LVEF&lt;40%, New York Heart Association, NYHA, class II or III) eligible for SGLT2i and performed serial cardiopulmonary exercise tests (CPET), pulmonary function tests (spirometry and diffusing capacity of the lungs for carbon monoxide, DLCO), laboratory and echocardiographic assessments before and after 6 months of treatment, to evaluate effects on exercise capacity, ventricular remodelling, cardiac biomarkers, fluid retention, renal and pulmonary function.

Results
49 patients (86% males, age 64±12 years) on optimal medical therapy (84% on sacubitril/valsartan; 98% on β–blockers, 84% on MRAs) were evaluated. We observed an increase in LVEF (32.8±7.1 vs. 35.5±8.3%; p&lt;0.001) and a reduction in left ventricular end–diastolic (EDV) and end–systolic (ESV) volumes (EDV: 209±83 vs. 199± 77 mL, p=0.004; ESV: 143±73 vs. 133± 68 mL, p&lt;0.001). There were no significant changes in peak oxygen uptake, while ventilatory efficiency during exercise (VE/VCO2 slope) showed a significant improvement (fig.1). Hemoglobin (Hb) and hematocrit levels improved, while renal function sodium and potassium levels remained stable, as did blood urea nitrogen, while BNP, NT–proBNP, ST2, and hs–TNI did not reach statistical significance in this sub–population. NYHA functional class improved, together with a significant decrease of MECKI (Metabolic Exercise test data combined with Cardiac and Kidney Indexes) score, from 3.9% to 2.7% with a positive impact on 2–year prognosis.

Conclusion
Medium–term treatment with Dapagliflozin demonstrated beneficial effects on LV remodelling, ventilatory efficiency and functional status. An improvement in VE/VCO2 slope and MECKI score paralleled with an enhancement in echocardiographic parameters and NYHA class was observed. Our study did not detect medium–term effects on spirometry values, DLCO, fluid retention and NT–proBNP. These results suggest that some favourable effects could unfold over a longer period of time. Further studies with longer follow up are desirable to assess Dapagliflozin effects even in such a well–treated HF population.
WEARABLE CARDIOVERTER DEFIBRILLATOR IN PATIENT WITH TRANSIENT RISK OF SUDDEN CARDIAC DEATH : SINGLE CENTRE EXPERIENCE
Authors: Bolognesi M , Rossi L , Biagi A , Sticozzi C , Comastri G , Puccio F , Pisati M , Aschieri D 
Published: May 2024

Abstract

Background
Cardiovascular disease is still the first cause of death, with sudden cardiac death ( SCD) as a first presentation in 25% of cases, related to coronary artery disease. Implantable cardioverter defibrillator (ICD) demontrated a reduction of SCD in high risk patients with good functional status. However the risk of SCD should be transient, so the use of a wearable cardioverter defibrillator (WCD) is considered in current guidelines and in clinical practice. The purpose of this study was to investigate the safety and the adherence to WCD in a real world population; moreover we reviewed the rate of ICD implantation after WCD use.

Methods
We considered consecutively 96 patients discharged from Piacenza cardiology department for potentially transient high risk of SCD, weared a WCD from August 2017 to June 2023. The patients were discharged in high risk mode of SCD with WCD protection and followed through remote monitoring or outpatient.A clinical and echocardiographic evalutation was performed at baseline and repeated at the end of the WCD use period.

Results
Out of 95 patients , with average age 66,0 ±11,1 years old and baseline average left ventricular ejection fraction (LVEF) 28,6±7,5, 81,2% were males, 72,9% suffered from arterial hypertension, 26% diabetes mellitus, 13,5% peripheral vascular disease, 14,6% chronic renal failure, 4,1% previous stroke.66% of these patients weared WCD for severe systolic disfunction in ischemic cardiac disease after recent myocardial infarction, after percutaneous coronary intervention or coronary artery bypass graft, 4% after removal of an infected ICD, 6% whilst awaiting completion of diagnostic tests (chanalopathies/right arrhythmogenic ventricular cardiomyopathy), 24% newly diagnosed cardiomyopathy. The average wearing time of WCD was 22,7±3,1 hours daily. We received these events: 1 sustained ventricular tachicardia, 2 non sustained ventricular tachicardia, 5 atrial fibrillation with 3 T wave oversensing. One patient recedived inappropriate shock. One death was detected due to severe heart failure. After 56,0±37,8 days of average wearing time 52% of patients were scheduled for ICD implantation.

Conclusions
In our experience we may consider that WCD use is effective, safe and with a good adherence in all patients. The WCD allows saving resources preventing inapprpriate ICD implantation in patient with transient SCD risk.