bluuebunny/crossref_metadata_2025_split huggingface dataset:
C67 ATRIOVENTRICULAR BLOCK DUE TO CARDIAC CONDUCTION TISSUE INFILTRATION: A CLINICAL PRESENTATION OF TRANSTHYRETIN CARDIAC AMYLOIDOSIS
Authors: Manguso G , Alfarano M , Ballatore F , Ajmone F , Verardo R , Magnocavallo M , Bagnato G , Ciaramella P , Maggio E , Vernile A , Costantino J , Mariani M , Frustaci A , Lavalle C , Vizza C , Chimenti C 
Published: May 2023

Abstract

Background
In cardiac amyloidosis, the deposition of insoluble amyloid fibrils can affect the cardiac conduction tissue, causing bradyarrhythmic clinical manifestations, often in the advanced stages of the disease. In this clinical case, atrioventricular block represented the clinical onset of transthyretin cardiac amyloidosis.

Clinical case
A 68–year–old man was admitted to our ward for recurrent syncopal episodes. He had in his medical history bilateral carpal tunnel syndrome. The electrocardiogram documented sinus rhythm with preserved QRS voltages, right bundle branch block, left anterior hemiblock, and first and second degree atrioventricular block with mean ventricular rate of 38 beats/min. A color–Doppler echocardiogram revealed increased left and right ventricular thickness (maximum thickness 16 and 10 mm, respectively), diastolic dysfunction, and preserved ejection fraction (60%). The patient underwent a coronary angiography, which was normal, and an endomyocardial biopsy of the left ventricle, which revealed the presence of hypotrophic and degenerated cardiomyocytes surrounded by large areas of amorphous substance that exhibited apple–green birefringence in polarized light after staining with Congo red, suggesting the diagnosis of cardiac amyloidosis. Sections of conduction tissue were included in the specimens, which appeared on histology as small myocytes positive by immunohistochemistry for HCN4, suggestive of conduction tissue cardiomyocytes. The conducting tissue was almost completely replaced by amyloid deposits positive by immunohistochemistry for transthyretin. The affected patient underwent a genetic test which revealed the mutation of the TTR gene (p.Thr79Lys). The patient subsequently underwent pacemaker implantation and started treatment with Tafamidis due to the absence of neurological involvement.

Conclusion
In patients presenting clinically with syncope and conduction abnormalities, the diagnosis of cardiac amyloidosis should always be considered, because they may represent the clinical onset of the disease.
C73 COVID–19 DIAGNOSIS AND MORTALITY IN PATIENTS WITH ACUTE MYOCARDIAL INFARCTION ADMITTED IN ITALY DURING THE NATIONAL OUTBREAK
Authors: D’Errigo P , De Luca L , Rosato S , Giordani B , Cuomo M , Duranti G , Mureddu G , Tavilla A , Badoni G , Seccareccia F , Baglio G 
Published: May 2023

Abstract

Background
and objective: There are limited data on the clinical impact of Covid–19 in patients with acute myocardial infarction (AMI). Aim of this work is to assess the impact of Covid–19 infection on mortality in AMI patients admitted during the national outbreak in Italy.

Methods
Retrospective nationwide cohort study enrolling consecutive AMI patients admitted between March,11st and May 3rd, 2020 (national outbreak) and the equivalent periods of the previous 5 years in Italy. The main outcomes were 30–day and 6–month all–cause mortality.

Results
The actual number of AMI admission during the 2020 outbreak in Italy was significantly reduced as compared to that expected based on the trend of the previous 5 years (STEMI: 4048 vs 5523, p&lt;0.0001; NSTEMI: 4981 vs 8633, p&lt;0.0001). A Covid–19 diagnosis was reported in 4.2% of STEMI and 3.5% of NSTEMI patients. In STEMI patients the 2020 expected rate of 30–day and 6–month mortality was 9.2% and 12.6%, compared to observed rates of 10.8% (p=0.016) and 14.4% (p=0.017), respectively. In NSTEMI patients the 30–day and 6–month expected mortality rates in 2020 were 6.5% and 12.2%, compared to observed rates of 8.3% (p=0.001) and 13.6% (p=0.041), respectively. Excluding patients diagnosed with Covid–19, the mortality rates become consistent with the trend of the previous 5–year for STEMI, but remain higher for NSTEMI. After multivariate adjustment, diagnosis of Covid–19 resulted an independent predictor of both 30–day mortality (OR=4.7, p&lt;0.0001 for STEMI; OR=4.5, p&lt;0.0001 for NSTEMI) and 6–month mortality (OR=3.6, p&lt;0.0001 for STEMI; OR=3.8, p&lt;0.0001 for NSTEMI).

Conclusion
During the 2020 national outbreak in Italy, a concomitant diagnosis of Covid–19 was associated with a significantly higher rate of mortality in both STEMI and NSTEMI patients. Excluding patients diagnosed with Covid–19, the mortality rates become consistent with the previous 5–year trend for STEMI, while they remain higher for NSTEMI.
P5 DISCOVERY OF A NEW HATTR GENETIC VARIANT: A CASE REPORT OF A PATIENT WITH CARDIAC UPTAKE AT 99–MTC–DIPHOSPHONATES SCINTIGRAPHY AND ABNORMAL MONOCLONAL PROTEINS TESTS
Authors: Fargione L , Pandolfi A , Carugo S , Spaziani C , Vicenzi M 
Published: May 2023

Abstract

Introduction
Cardiac amyloidosis is an infiltrative disease that remains an under–diagnosticated cause of heart failure. Diagnostic algorithms focus on identifying the two most frequent forms of the disease, light chains amyloidosis (AL) and transthyretin amyloidosis (ATTR), by the initial use of 99mTc–DPD scintigraphy coupled to assessment of monoclonal proteins. Etiological distinction is and of pivotal importance since the availability of specific disease modifying treatments.

Case Description
We report the case of a 61–year–old man hospitalized for new onset of heart failure. Past medical history highlights bilateral carpal tunnel syndrome and monoclonal gammopathy of undetermined significance (MGUS). Cardiovascular examination reveals clinical signs of pulmonary and peripheric congestion. ECG shows conduction disturbances and low QRS voltage. Transthoracic echocardiography reveals left ventricular hypertrophy, normal systolic function (LVEF 58%), 3rd degree LV diastolic disfunction and mild pericardial effusion. Laboratory tests shows abnormal levels of TnT and pro–BNP. A diagnosis of cardiac amyloidosis is considered, and further exams are executed: cardiac magnetic resonance, haematological laboratory tests and 99mTc–DPD scintigraphy (tab. 1). Since both bone scintigraphy and monoclonal proteins tests are positive, diagnosis of cardiac amyloidosis cannot be made without non–invasive modalities (fig.1) and the patient is referred to an Amyloidosis Centre. Haematological evaluation and abdominal fat pad biopsy show stability of haematological disease and confirms the deposition of amyloidogenic TTR both in wild–type and mutated Ile88Arg variants. The results of genetic analysis confirms the presence of a heterozygous mutation of TTR gene (p.Ile88Arg – c.263T&gt;G) (fig. 2). This mutation is not reported in database ClinVar nor in gnomAD, but according to the ACMG criteria it can be considered likely pathogenic. Considering the final diagnosis of cardiac hATTR the patient is considered eligible for treatment with Tafamidis.

Discussion and Conclusion
Despite clinical history with cardiac conduction system abnormalities and bilateral carpal tunnel syndrome, some gene TTR variants can bring false negative results at 99mTc–DPD scintigraphy, so ATTR form should not be excluded basing on non–invasive modalities. Performing a compete diagnostic workup led us to discover a new TTR pathogenic variant and confirm ATTR form without performing endomyocardial biopsy.
P7 INJECTABLE LOOP RECORDER IMPLANTATION IN CHILD WITH DANON SYNDROME AND VENTRICULAR EXTRASYSTOLES
Authors: Catucci S , Lombardi M , Maiorano A , Meliota G , Pirolo T , Massari E , Vairo U 
Published: May 2023

Abstract

Introduction
Danon syndrome is a rare X–linked storage disorder caused by mutation of the LAMP2 gene, which is expressed in the heart with hypertrophic cardiomyopathy, heart failure and arrhythmias.

Clinical case
Little C. comes to our observation at the age of 3 years for ventricular extrasystoles. The maternal grandmother died suddenly at the age of 36, with the autopsy of hypertrophic cardiomyopathy being found. The mother, who died at the age of 28 from heart failure, was suffering from Danon syndrome with non–obstructive hypertrophic cardiomyopathy with dilatative evolution, episodes of atrial fibrillation and sustained ventricular tachycardia, WPW syndrome, already subjected to ICD implantation and on list for cardiac transplantation. The little girl presents a normal echocardiogram at our first evaluation; Holter documents numerous isolated, monomorphic ventricular extrasystoles. Genetic analysis is started to ascertain the presence of the germline mutation in the LAMP2 c.928G&gt;A gene (related in the literature to Danon disease). During the follow–up the patient, always asymptomatic for syncope and / or palpitations, presents the appearance of slight concentric hypertrophy of the left ventricle; to Holter persistence of monomorphic, non–repetitive ventricular extrasystoles. At the age of 5, injectable loop recorder implantation is performed. The procedure is well tolerated and with no complications. The sensing of the R wave (1.03 mV) was great. Beta–blocker therapy is also undertaken. At remote monitoring, some false episodes of tachyarrhythmias due to double counting due to the presence of high voltage T waves are observed in the first month after the implant; after reprogramming the gain, false alarms do not occur. Over the course of about 3 years, monitoring with loop recorder allowed to document the absence of sustained brady and tachyarrhythmias. At the clinical follow–up, a reduction in the arrhythmic burden was observed, with almost total disappearance of ventricular extrasystoles.

Conclusions
The loop recorder implant is a safe and reliable procedure in pediatric age for the monitoring of patients at arrhythmic risk who do not have indication for defibrillator implantation. Danon syndrome is expressed phenotypically with hypertrophic cardiomyopathy in the second to third decade of life; it is, therefore, necessary to identify paediatric subjects who may be most at risk of sudden arrhythmic events.
P18 PROGNOSTIC IMPACT OF MITRAL REGURGITATION BEFORE AND AFTER TRANSCATHETER AORTIC VALVE REPLACEMENT IN PATIENTS WITH SEVERE LOW FLOW, LOW GRADIENT AORTIC STENOSIS
Authors: Ferruzzi G , Silverio A , Cimmino M , Corcione N , Bellino M , Attisano T , Baldi C , Morello A , Biondi–Zoccai G , Citro R , Vecchione C , Galasso G 
Published: May 2023

Abstract

Background
There is little evidence about the prognostic role of mitral regurgitation (MR) in patients with low flow, low gradient aortic stenosis (LFLG–AS) undergoing transcatheter aortic valve replacement (TAVR). Aims: The aim of this study was to assess the prevalence and outcome implication of moderately severe to severe MR in patients with LFLG–AS before and after TAVR.

Methods
This study included consecutive patients with LFLG–AS undergoing TAVR at two Italian high–volume centres. The study population was categorized according to the baseline MR severity and to the presence of MR improvement at discharge. The primary outcome was the composite of all–cause death and hospitalization for worsening heart failure (HF) up to one year. The secondary outcomes were the single components of the primary outcome.

Results
The study included 268 patients; 57 (21%) patients showed MR&gt;2+. Patients with MR&gt;2+ showed a lower one–year survival free from the primary outcome (Log–Rank &lt;0.001; Figure1), all–cause death (Log–Rank &lt;0.001), and HF hospitalization (Log–Rank &lt;0.001) compared to patients with MR≤2+. At multivariable analysis, baseline MR&gt;2+ was an independent predictor of the primary outcome (p&lt;0.001). Among patients with baseline MR&gt;2+, MR improvement was reported in 24 (44%) cases after TAVR. The one–year survival free from the primary outcome was significantly higher in patients with MR improvement than in those without (Log–Rank = 0.009), and both groups had a significantly lower survival free from the primary outcome when compared with patients with baseline MR &lt;2+ (Figure 2). One–year survival free from the all–cause death (Log–Rank = 0.036) and HF hospitalization (Log–Rank = 0.005) was consistently higher in patients with MR improvement than in those without.

Conclusions
In this study, the presence of moderately severe to severe MR in LFLG–AS patients undergoing TAVR portend a worse clinical outcome at one year. TAVR may improve MR severity in nearly half of the patients, resulting in a potential outcome benefit after discharge.
P19 INCIDENTAL DIAGNOSIS OF MASSIVE MOBILE LEFT VENTRICLE THROMBI FOLLOWING COVID–19 INFECTION IN A HEART FAILURE PATIENT
Authors: Mapelli M , Celeste F , Conte E , Palermo P , Mancini E , Maiolo G , Ghulam Ali S , Agostoni P 
Published: May 2023

Abstract

Background
Intracardiac thrombus can be a complication of ischemic heart disease and cardiomyopathies. The advancements in imaging modalities have increased the capacity to diagnose this condition helping to reduce the associated complications (i.e. embolization). Previous reports suggest a possible link between intracardiac thrombosis and COVID–19 infection, especially in patients with severe “endotheliitis”, pneumonia, or myocarditis. COVID–19 effects on the cardiovascular system are partly evaluated. Venous and arterial thrombosis is commonly associated with COVID–19 but detection of intracardiac thrombus has not been frequently reported. Case Description: A 71–year–old woman, known to have a non–ischemic dilated cardiomyopathy with reduced ejection fraction (EF) and a previous transcatheter edge–to–edge mitral valve repair (MitraClip), was admitted after a routine echocardiogram showing new onset multiple, highly mobile, left ventricle (LV) masses. The patient, fully vaccinated against Sars–Cov–2, experienced a paucisymptomatic COVID–19 infection 1 month before, followed by a full recovery. A transthoracic echocardiogram performed 3 months showed no LV masses. On admission she was completely asymptomatic with no clinical signs of heart failure or systemic embolization. A multimodality imaging evaluation (contrast echocardiography, cardiac computed tomography, cardiac magnetic resonance) confirmed a severe dilation of the LV with severe EF reduction, and 3 mobile LV masses. The largest mass was adhered to the middle portion of the anterolateral wall (maximum diameter 49x15 mm). A diagnostic endomyocardial biopsy and cardiac surgery were excluded due to prohibitive embolic/procedural risk and an anticoagulant treatment with warfarin was started with a progressive reduction of the masses’ dimension at transthoracic echocardiography. Thus, a diagnosis of exclusion of LV thrombosis was made. After 2–week a complete resolution of the masses was documented with no clinical or embolic events.

Conclusion
The pro–thrombotic nature of COVID–19 infection is well known. This case demonstrates how vulnerable patients, i.e. those with heart failure, may experience thrombotic complications following non–severe COVID–19 infection and despite having completed the vaccine course. Although currently unconfirmed by dedicated clinical trials, more assiduous echocardiographic monitoring and/or the use of anticoagulant therapies could yield a benefit in selected patients.
P21 INFECTIVE ENDOCARDITIS COMPLICATED BY BLOCKED ATRIAL FIBRILLATION AND AORTIC VALVE RUPTURE: A CASE REPORT
Authors: De Maio J , Caputi A , Martelli A , Bagni E 
Published: May 2023

Abstract
A 63–year–old male patient with a mechanical mitral prosthesis and permanent atrial fibrillation in TAO was hospitalized for heart failure during a SARS CoV–2 infection in April 2022; the transthoracic echocardiogram performed during the hospitalization showed an ejection fraction of 38% The patient was rehospitalized in September 2022 due to worsening general conditions during suspected pneumonia. The hospitalization was complicated by brady AF with heart rate &lt; 30 b.p.m. symptomatic for asthenia and dyspnoea despite the suspension of beta–blocker and digitalis therapy. Therefore, we decided on the implantation of a temporary pacemaker in urgency and subsequently a definitive pacemaker (Essentio MRI – Boston Scientific). During the hospitalization, the clinical conditions continued to be precarious; a transthoracic echocardiogram revealed suspicious formation of endocardic nature at the aortic valve with consensual severe regurgitation. This datum was confirmed with the TEE echo. At this point, in the suspicion of infective endocarditis, antibiotic therapy was set up and the patient was taken to our reference cardiac surgery where he underwent aortic valve replacement with a mechanical prosthesis, reconstruction of the coronary sinus and of the outflow tract of the left ventricle with heterologous pericardial patch. Currently, the patient performs regular clinical follow up at our Center and is in good general condition.
P26 TRANSIENT GLOBAL AMNESIA AS A POSSIBLE CONSEQUENCE OF ICA DISSECTION: A CASE REPORT
Authors: Natale F , Loffredo F , Molinari R , Alfieri R , Covino S , De Luca L , Limatola M , Mollo N , Pezzullo E , Cimmino G 
Published: May 2023

Abstract

Case Report
A 42–year–old woman was carried to emergency room lamenting sudden memory loss. Her husband witnessed the onset of the episode. No recent head trauma was reported, blood pressure was under control. Anterograde and retrograde memory loss was present and confirmed by a neurologist. No other neurological signs were elicitable. Blood exams and toxicological analysis were negative. ECG, chest radiography, and cerebral TC showed no abnormalities. At physical examination a carotid bruit could be appreciated. The patient was asymptomatic. Subsequently an eco–colour–Doppler of carotid artery was performed showing a left common and internal carotid artery dissection. The diagnosis of ICA dissection was confirmed with a contrast CT furthermore excluding the coexistence of an aortic dissection. The anterograde amnesia resolved 15 h after the admission. A neurologist confirmed that the patient experimented an episode of transient global amnesia. After a vascular surgery consultation, a conservative treatment was deemed appropriate. After a 6 month follow up the patient didn’t complain of any other neurological symptom or sign. Transient global amnesia (TGA) is characterized by reversible anterograde and retrograde amnesia of sudden onset that resolves spontaneously within 24 h. The pathophysiological mechanisms underlying this puzzling neurological syndrome are yet not completely clarified. Some studies support an ischemic aetiology for TGA and cases of TGA following ICA–stenting or aortic dissection with involvement of the supra–aortic branches are reported in literature, thus supporting the hypothesis that a cause–effect relationship between ischemic pathologies of supra–aortic trunks and TGA might be present. In the case here presented, a Doppler ultrasound of carotid arteries performed in a patient presenting with TGA, asymptomatic for neck pain, led to a diagnosis of ICA dissection. In order to not miss a spontaneous ICA dissection as a rare ethology of TGA, we suggesto perform an eco–colour–Doppler of the epi–aortic vessels in patients affected by such amnesic syndrome.
P36 A CHALLENGING CASE OF MINOCA
Authors: Zagarese G , Candelora A , Vinco G , Giovannelli C , Angheben C , Moggio P , Musuraca G , Cemin C , Del Greco M 
Published: May 2023

Abstract
Myocardial infarction in the absence of obstructive coronary artery disease (MINOCA) accounts for 5–6% of all myocardial infarctions. The causes that can lead to this diagnosis are many and not always clearly identifiable; in fact, despite optimal work–up, the cause of MINOCA remains undetermined in 8–25% of patients and diagnosis represents a real challenge for the cardiologist clinician. Because patients with MINOCA have clinical outcomes comparable to those with myocardial infarction with obstructive coronary artery disease, they should undergo a careful diagnostic work–up in order to set up a therapy related to the specific diagnosis.

Introduction
A 51–years old woman presented to the emergency room after a sudden onset of an oppressive, prolonged and non–radiating chest pain, with gradual resolution after intravenous nitroderivates therapy. Smoking was the only cardiovascular risk factor. A recent uncomplicated COVID 19 infection (2 months before hospital admission) was the only event reported in clinical history. Physical examination was unremarkable with the exception of an apical mid–end systolic murmur. The electrocardiogram was normal and the Troponin T peak was 391 pg/ml. An echocardiogram showed a normal biventricular function without alterations of segmental kinetics and mitral valve prolapse with moderate degree regurgitation. The patient was admitted to the Intensive Care Unit department and underwent a coronary angiography study with evidence of main epicardial coronaries free from stenosing atherosclerotic lesions. In addition, a cardiac magnetic resonance (CMR) was performed before discharge, with evidence of normal biventricular systolic function in the presence of limited area of ​​late enhancement with ischemic pattern at the level of the middle segment of the inferolateral wall of the left ventricle without edema. In the light of the clinical instrumental picture, a diagnosis of MINOCA was made.

Discussion
This clinical case is an example of how sometimes the cause relating to a MINOCA cannot always be well identified. However, the multi–imaging approach and in particular the resonance allowed to identify a localized area of ​​late enhancement with an ischemic type pattern. The origin of the ischemia could be dated from a picture of distal embolization, from a microvascular disease or from a myocardial stretching due to prolapse.
P37 MULTIMODALITY DIAGNOSTIC APPROACH TO A JUVENILE CASE OF SUSTAINED VENTRICULAR TACHYCARDIA
Authors: Castello L , Di Fusco S , Colivicchi F 
Published: May 2023

Abstract

Clinical case
31–year–old male subject, former competitive athlete, with family history of coronary artery disease and no further cardiovascular risk factors or previous cardiac events. Previous SARS–CoV2–related paucisymptomatic infection (January 2022) and subsequent episode of fever (August 2022) with negative SARS–CoV2 nasal swab. In September 2022 he reported sudden onset of palpitations after hearty meal. Because of such symptoms, the patient underwent Holter ECG showing 90 episodes of ventricular tachycardia (VT) and one episode of sustained VT. Based on such evidence, the patient went to the emergency room (ER). During the ER stay, blood exams (blood count, high–sensitivity troponin, BNP, CRP, D–dimer) and ECG were normal and SARS–CoV2 test was negative. The patient was then hospitalized in cardiac ICU. The echocardiogram showed left ventricle with normal dimension, thickness and wall motion (EF: 65%, mean GLS: –20%), trivial mitral and aortic regurgitation, no pericardial effusion. Subsequently, a cardiac coronary CT scan was performed showing absence of atherosclerotic lesions and myocardial bridge of the middle segment of LAD artery. The diagnostic pathway went on with cardiac magnetic resonance (CMR) demonstrating focal hypokinesia and wall thickness reduction of the anteroseptal–anterior junctional wall at middle–distal level (minimum thickness: 3 mm) where a millimetric area of late gadolinium enhancement with transmural distribution was described; absence of myocardial oedema. In addition, an EPS was performed with electroanatomical high–density/definition mapping of the left ventricle showing normal volumes, signals and activation and absence of LAVA/LV. Taking into account such findings, a subcutaneous defibrillator was implanted for primary prevention and a control CMR was scheduled in the follow–up period.

Conclusions
This case underlines the central role of CMR in identifying areas of myocardial fibrosis that can represent the electroanatomical substrate for triggering ventricular arrhythmias. In this case, the arrhythmic events are associated with a focal area of transmural fibrosis and wall thinning, usually indicating ischaemic damage, nonetheless in the presence of normal coronary arteries.