bluuebunny/crossref_metadata_2025_split huggingface dataset:
484 PAPILLARY FIBROELASTOMA OF THE AORTIC VALVE ASSOCIATED WITH MYOCARDIAL INFARCTION, COMPLICATED BY ELECTRICAL STORM AND ENDOVENTRICULAR THROMBOSIS
Authors: Nepitella Alessandro Alberto , Cadeddu Christian , Maiani Silvia , Mandas Martina , Marchetti Maria Francesca , Montisci Roberta 
Published: December 2022

Abstract

Introduction
Papillary fibroelastomas (PFE) are reported to be the most common tumors of the heart valves and are a possible source of systemic embolism, including myocardial infarction.

Case Report
We report the case of a 67-year-old female with no background of cardiovascular disease, admitted to our hospital with worsening dyspnea and chest tightness over the prior 10 days.
The ECG showed a sustained hemodynamically tolerated monomorphic ventricular tachycardia, successfully treated with antiarrhythmic therapy, and ECG in sinus rhythm revealed signs of previous anterior myocardial infarction. Transthoracic echocardiogram showed a severely depressed systolic function (LVEF 19%), akinesia and thinning of the anterior wall and of all middle-apical segments, complicated by left ventricular aneurysm and endoventricular thrombosis. In the ventricular side of right aortic coronary cusp a mobile, pedunculated mass of 12×8 mm was visualized. A subsequent TEE corroborated the suspicion of PFE. She underwent cardiac MRI which showed areas of transmural late enhancement in all akinetic segments. A coronary angiography revealed sub-occlusion of the mid-anterior descending artery with no angiographic evidence of coronary atherosclerosis elsewhere. In view of her reduced LVEF and history of VT, an ICD was implanted. She started oral anticoagulant therapy and complete resolution of the endoventricular thrombosis was evidenced. After several days of electrical instability, characterized by multiple episodes of ventricular tachycardia, the clinical course was characterized by progressive clinical and electrical improvement and she was discharged in good clinical conditions after four weeks. Due to the severe impairment of the systolic function, not susceptible to surgical remodeling and the high operative risk, no indication for surgical removal of the mass was made.

Conclusion
Aortic PFE carry a not negligible risk of acute coronary syndrome, electrical storm and sudden death. It should be considered as the cause of myocardial infarction, and not an incidental finding, especially in patients with no angiographic evidence of coronary atherosclerosis.
558 BIVENTRICULAR PERIOPERATIVE TAKOTSUBO CARDIOMIOPATHY
Authors: Padoan Laura , Sforna Stefano , Parise Antonio , Barengo Alberto , Torlone Francesco , Coiro Stefano , Riccini Clara , Del Pinto Maurizio , Cavallini Claudio 
Published: December 2022

Abstract

Introduction
Perioperative Takotsubo cardiomyopathy (pTC) represents a rare and still not well characterized disease. Biventricular involvement is an uncommon manifestation of TC and is associated with a more severe clinical presentation.

Case report
A 72-year-old male, hospitalized for a laparoscopic left hemicolectomy, was transferred to ICU after having developed severe bradycardia, treated with Adrenalin, and cardiogenic shock during induction of general anesthesia. EKG showed complete atrio-ventricular block, so a temporary PMK was placed. Troponin was raised. Echocardiogram showed severe biventricular disfunction (LVEF 25%, TAPSE 12 mm) with akinesia of medio-apical segments, suggesting a biventricular pTC. Also, there was a reduction of 3D LV longitudinal strain (- 5,8%), particularly of the medio-apical portion, and RV free wall longitudinal strain (- 11%). Coronary angiography resulted negative. Patient's hemodynamic was supported with Noradrenalin and a cycle of Levosimendan, allowing to reach stability. Further echocardiogram showed improvement of biventricular function and longitudinal strain values. Due to the persistency of atrio-ventricular block, a definitive PMK was placed. Patient was discharged after therapy optimization.

Discussion
pTC is a little-known disease, as incidence and etiology are not well definite. Literature suggests a relation between pTC and physical or emotional stress due to surgery to promote an increase of catecholamine release. Other potential factors may be inadequate depth of anesthesia or tracheal manipulation during intubation and catecholaminergic drugs administration. On the other hand, the use of anesthetic volatile agents seems to have a cardioprotective effect. PTC is more common during general anesthesia and when occurs intraoperatively has a worse clinical presentation. Biventricular involvement, which is a rare and severe manifestation of TC, has been reported frequently among pTC patients. To date, due to the lack of a systematic review, there is little knowledge about potential risk factors, prevention strategies and management of pTC.

Conclusions
Despite multiple cases of pTC have been reported, several characteristics of this entity are not fully understood. However, it must be considered as a part of differential diagnosis in patients with anaesthesia-related decompensation. Biventricular involvement represents an infrequent presentation and its commonly associated with life-threating hemodynamic impairment.
240 A PEDIATRIC CASE OF BIVENTRICULAR END-STAGE ARRHYTHMOGENIC CARDIOMYOPATHY
Authors: Agbariah Andrea , Ermacora Davide , Ruzzarin Alessandro , Milewski Priscilla , Nannelli Chiara , Comunello Andrea , Cemin Roberto 
Published: December 2022

Abstract

Background
Arrhythmogenic cardiomyopathy (ACM) is a rare heart muscle disease characterized by cardiomyocyte necrosis and fibro-adipose substitution which contribute both to the genesis of malignant ventricular arrhythmias and the morpho-functional alterations of the ventricles. ACM has always been considered a right ventricular disease with secondary involvement of the left ventricle, but recently a left dominant phenotype has been also described.

Case report
A 14-year-old patient was admitted to our hospital after out-of-hospital cardiac arrest due to ventricular fibrillation, which occurred when she was at rest. A family history of sudden death and dilated cardiomyopathy was retrieved (her brother died at the age of 18 with a dilated heart), but other family members had never been screened to date. Cardiac magnetic resonance imaging (cMRI) appearance was consistent with biventricular dilatation along with severe systolic dysfunction (left ventricular ejection fraction 20%; right ventricular ejection fraction 25%). It showed also thinning and bulging of the basal free wall of the right ventricle proximal to the outflow tract (up to 4 mm) and multiple foci of subepicardial, mid-wall and transmural late gadolinium enhancement (LGE) in both ventricles. Signs of acute decompensated heart failure and a high arrhythmic burden were present. After cardiopulmonary stabilization, she underwent the implantation of a cardioverter-defibrillator (ICD).

Conclusion
We herein report an uncommon case of biventricular AC (‘definite diagnosis’ according to both Task force criteria 2010 and Padua criteria) with advanced morpho-functional dysfunction occurring already in pediatric age, without any alerting symptom until the cardiac arrest (the typical onset of the ACM in children). It shows how severe biventricular involvement may affect young patients with a rapid progression of the disease. It also stresses the need for a strict follow-up of the family members of a proband since his first diagnosis, highlighting the pivotal role of cMRI in the diagnosis of pediatric disease. This has not been thoroughly studied and lacks sensitive and specific diagnostic criteria.
449 HYPERTROPHIC CARDIOMYOPATHY: A CASE OF CHALLENGING ARRHYTHMIC RISK STRATIFICATION
Authors: Belleggia Sara , Coraducci Francesca , Torselletti Lorenzo , Coretti Francesca , Paolini Federico , Alfieri Michele , Bastianoni Gianmarco , Brugiatelli Leonardo , Principi Samuele , Ciliberti Guseppe , Barbarossa Alessandro , Stronati Giulia , Russo Antonio Dello , Guerra Federico 
Published: December 2022

Abstract
Hypertrophic cardiomyopathy (HCM) is a common disease, mostly inherited, with a prevalence of 1:500. Cornerstone of the clinical management of HCM patients is stratifying the risk of sudden cardiac death (SCD) which is the most frightening complication despite its relatively low incidence (0,9% per year). Although mathematical risk scores, as proposed by ESC, are the most used to assess the probability of arrhythmic complications leading to SCD, they have been associated with low sensitivity, precluding some high-risk patients from prophylactic ICD implantation.
Among the tools at clinicians’ disposal for risk stratification in HCM patients, the latest AHA/ACC guidelines propose an “individual risk markers strategy”, based on the presence of ≥1 clinical feature such as a family history of SCD, extreme left ventricular hypertrophy (LVH), unexplained recent syncope, NSVT, late gadolinium enhancement (LGE) at CMR, systolic dysfunction and LV apical aneurysm.
We present a case that lets us reflect on arrhythmic risk stratification.
P.C. is a 58 y.o. male with a history of septal hypertrophy (19 mm) and T wave inversion but without an established diagnosis of HCM. He did not have any familiar history of syncope or SCD. He came to the emergency department for epistaxis and was admitted to the cardiology unit due to the previously mentioned findings, where he underwent cardiac-CTA and CMR showing myocardial bridge of left anterior descending artery, LVH with apical aneurysm and LGE areas localized on the apex (transmural) and on the interventricular septum (subendocardial). No signs of dynamic LVOT obstruction or atrial enlargement emerged from echocardiography. During the observation, asymptomatic NSVT were recorded on continuous ECG monitoring. According to the ESC risk prediction score (3.7%) ICD was not strictly indicated but considering the CMR high-risk profile (according to the suggestions of AHA/ACC) we proposed an ICD implantation anyways. The patient refused any invasive procedure and was implanted with a loop recorder. At 12 months remote monitoring showed a single episode of self-terminated sustained polymorphic ventricular tachycardia (02:23 mm: ss) symptomatic of pre-syncope. The patient was then immediately reached and admitted for ICD implantation in secondary prevention. According to the patient's will, and the no need for bradycardia therapy, an S-ICD was implanted. Figure 1LV apical aneurysm at CMRFigure 2Remote Monitoring
802 DIAGNOSTIC YIELD AND PREDICTIVE VALUE ON LEFT VENTRICULAR REVERSE REMODELING OF GENETIC TESTING IN DILATED CARDIOMYOPATHY
Authors: Bertero Edoardo , Fracasso Giulia , Eustachi Virginia , Coviello Domenico , Cecconi Massimiliano , Giovinazzo Stefano , Toma Matteo , Merlo Marco , Sinagra Gianfranco , Porto Italo , Ameri Pietro , Canepa Marco 
Published: December 2022

Abstract

Background
Genetic testing is one essential element in the diagnostic workup of patients with idiopathic dilated cardiomyopathy (DCM), and genotype-phenotype correlations are still poorly understood. We assessed the diagnostic yield of genetic testing in a selected DCM cohort including both familial and nonfamilial cases and evaluated phenotypic differences and the relationship of left ventricular reverse remodeling (LVRR) with the presence of pathogenic (P) or likely pathogenic (LP) variants of disease-associated DCM genes.

Methods
From 680 outpatients with heart with reduced ejection fraction followed at our Heart Failure Outpatient Clinic we selected subjects with a diagnosis of DCM before 65 years of age and without secondary causes of DCM including ischemic, valvular, hypertensive, or drug-induced heart disease or myocarditis. All patients underwent extensive clinical, laboratory, and imaging evaluation at the time of diagnosis and during regular follow-up visits and were offered genetic investigations with Next Generation Sequencing (NGS) of 42 disease-associated DCM genes.

Results
Of 680 DCM outpatients, 70 fulfilled the definition of idiopathic DCM and 66 gave consent to undergo genetic investigation (median age at diagnosis 57 years, 68% males). A total of 18 pathogenic/likely pathogenic (P/LP) variants were identified in 16 patients (diagnostic yield 24%), the most common (7, 39% of all variants) involving the TTN gene, followed by lamin A/C (n=3), cytoskeleton Z-disk (n=3), motor sarcomeric (n=2), desmosomal (n=1), and ion channel (n=1) gene variants. At the time of DCM diagnosis, patients with P/LP had a higher left ventricular (LV) ejection fraction (LVEF) compared with subjects (31±11% vs 28±8%, p=0.17). After a median follow-up of 53 (IQR 20-111) months, P/LP-positive patients had lower systolic and diastolic blood pressure and higher plasma natriuretic peptide levels than patients without P/LP variants. Patients without P/LP variants exhibited a larger extent of LVRR, as reflected by the increase in LVEF (+14% vs. +1%, p=0.0008) and decrease in LVEDDi (-3 vs. -1 mm/m2, p=0.03) compared with subjects carrying P/LP variants (Figure).

Conclusions
Our results confirm the high diagnostic yield of genetic testing in selected DCM patients and suggest that identification of P/LP variants in DCM portends poorer left ventricular reverse remodeling in response to guideline-directed medical therapy.
102 ICD IMPLANTATION IN PATIENTS WITH HYPERTROPHIC CARDIOMYOPATHY (HCM): A LONG-TERM SINGLE CENTER EXPERIENCE
Authors: Buongiorno Antonia Luisa , Blandino Alessandro , Bianchi Francesca , Masi Andrea Sibona , Mabritto Barbara , Luceri Stefania , Bongioanni Sergio , De Rosa Catia , Tommasello Antonino , Parrini Iris , Grossi Stefano , Musumeci Giuseppe 
Published: December 2022

Abstract

Background
Subcutaneous ICD (S-ICD) is a safe and effective tool in preventing sudden cardiac death (SCD) and it has the potential to overcome the limitations of transvenous ICDs. Because of increased left ventricular mass and unpredictable electrical substrate, concerns regarding the safety and efficacy of S-ICD in hypertrophic cardiomyopathy (HCM) patients have been raised.

Material and Methods
We retrospectively collected clinical data from consecutive HCM patients who underwent ICD implantation and were followed at our Cardiology Department from January 2000 to January 2022.

Results
Among 702 HCM patients, 52 (7.4%) received an ICD (female, 30.7%; mean age at implantation, 49 ± 20; primary prevention, 86.5%; S-ICD, 13%; single-chamber ICD, 50%). During a mean follow up of 8 ± 5 years, 27 shocks occurred in 20 (38%) patients of which 11 appropriate shocks (AS) in 9 (17%) patients and 16 inappropriate shocks (IS) in 11 (21%) patients, respectively after 4.3 ± 3 and 5 ± 4 years. AS were due to ventricular tachycardia (7) and ventricular fibrillation (4) and occurred in 9 patients with transvenous ICD vs. 0 in S-ICD patients (100% vs. 0%, p=0.32). IS were due to AF (8), PSVT (3), sinus tachycardia (2), and oversensing (3), with a non-significant trend towards a higher rate in patients with transvenous ICD compared to those with S-ICD (33% vs 14%, p=0.41). Device-related complications (DC) were recorded in 14 (27%) patients and were secondary to lead fracture (6), pocket hematoma (3), pneumothorax (1), device malfunctioning (1), endocarditis (2), and venous thrombosis (1). Compared to S-ICD, transvenous ICD was associated with a non-significant higher risk of DC (31% vs. 0%, p=0.17).

Conclusions
In HCM patients at high risk of SCD, when compared to transvenous ICD, S-ICD is related to a non-significant lower rate of IS and DC, with similar rate of AS. Further randomized studies as well as large prospective registries are needed to confirm these findings.
525 PROGNOSTIC RELEVANCE OF DEMOGRAPHIC FACTORS IN CARDIAC MAGNETIC RESONANCE-PROVEN ACUTE MYOCARDITIS. A COHORT STUDY
Authors: Cannata Antonio , Roy Roman , Rios Antonio Jordan , Ferone Emma , Cassimon Barbara , Sadler Matthew , Rind Irfan , Rosmini Stefania , Piper Susan , Giacca Mauro , Mcdonagh Theresa , Scott Paul , Bromage Daniel 
Published: December 2022

Abstract

Aims
Acute myocarditis (AM) is a heterogeneous condition with variable estimates of survival. Contemporary criteria for the diagnosis of clinically suspected AM enable non-invasive assessment, resulting in greater sensitivity and more representative cohorts. We aimed to describe the demographic characteristics and long-term outcomes of patients with AM diagnosed using non-invasive criteria.

Methods and Results
A total of 199 patients with cardiac magnetic resonance (CMR)-confirmed AM were included. The majority (n=130, 65%) were male and the average age was 39±16 years. Half of patients were White (n=99, 52%) with the remainder from Black and Minority Ethnic (BAME) groups. The most common clinical presentation was with chest pain (n=156, 78%), with smaller numbers presenting with breathlessness (n=25, 13%) and arrhythmias (n=18, 9%). Patients admitted with breathlessness were sicker and more often required inotropes, steroids, and renal replacement therapy (p&lt;0.001, p&lt;0.001, and p=0.01, respectively). Over a median follow-up of 53 (IQR 34–76) months, 11 patients (6%) experienced an adverse outcome, defined as a composite of all-cause mortality, resuscitated cardiac arrest, and appropriate implantable cardioverter defibrillator (ICD) therapy. Patients in the arrhythmia group had a worse prognosis, with a nearly 7-fold risk of adverse events (hazard ratio [HR] 6.97; 95% Confidence Interval [CI] 1.87–26.00, p=0.004). Sex and ethnicity were not significantly associated with the outcome.

Conclusions
AM is highly heterogeneous with an overall favourable prognosis. Three quarters of patients with AM present with chest pain and this is associated with a benign prognosis. AM presenting with life-threatening arrhythmias is associated with higher risk of adverse events.
583 DEFORMATION MAP OF LEFT VENTRICULAR STRAIN: COMPARISON BETWEEN PATIENTS WITH HYPERTROPHIC CARDIOMYOPATHY AND CONTROLS WITHOUT SIGNIFICANT CORONARY ARTERY STENOSIS
Authors: Del Franco AnnaMaria , Wierzbowska-drabik Karina , Zampieri Mattia , Palinkas Eszter Dalma , Chiti Chiara , Duraj Iwona , Olivotto Iacopo 
Published: December 2022

Abstract

Background
Hypertrophic cardiomyopathy (HCM), being characterized by enhanced contractility, positively responses to treatment with myosin inhibitors which could promote reverse remodelling. Additionally, speckle tracking analysis highlights left ventricular (LV) contractile alterations in HCM, although the pathophysiological meaning and the effect of the target-therapy are still unclear.

Aim
To analyse global and regional longitudinal peak systolic strain in patients with HCM in comparison to age-matched control group without HCM and with known coronary artery anatomy.

Methods
We examined 37 HCM patients (33 with asymmetric septal and 4 with apical hypertrophy) and 67 age-matched controls without HCM or significant coronary stenoses, with transthoracic echocardiography with analysis of segmental and global left ventricular strain (GLS) by AFI method in 16-segment LV model.

Results
Patients with HCM showed higher values of interventricular septal thickness, left atrial diameter and LV ejection fraction, whereas controls presented higher BMI and resting heart rate. In deformation analysis, GLS values were lower in HCM (15.9 ± 4.2 vs 18.4 ± 3.5%, p = 0.002) as well as segmental strain was significantly impaired in marker regions for studied form of HCM, in particular septal, inferior and inferolateral basal and mid segments as well as apical inferior segment for HCM with asymmetric septal hypertrophy; 4 patients with apical form of HCM showed severely impaired strain in all apical segments.

Conclusions
Deformation analysis shows a significant impairment of global and segmental strain values in patients with HCM, in particular for hypertrophic segments. This finding does not definitely imply a certain contractile dysfunction, and further investigation on the effect of myosin inhibitors on strain analysis is strongly recommended.
302 CHALLENGING DECISION IN INCIDENTAL APICAL HYPERTROPHIC CARDIOMYOPATHY DIAGNOSIS
Authors: Fazzini Luca , Angius Simone , Caggiari Ludovica , Cossu Stefano , Contini Francesca Valeria , Martis Alessandro , Pasqualucci Daniele , Valentino Luigi , Corda Marco , Dessalvi Christian Cadeddu 
Published: December 2022

Abstract

Introduction
Apical hypertrophic cardiomyopathy (ApHCM) compared with normal HCM is less frequent, it's more associated with atrial fibrillation (AF) and it has different sudden cardiac death (SCD) risk factors. Authoritative recommendations and guidelines on diagnosis, family screening and especially patient risk stratification don't exist. Taking decisions on ICD implantation can sometimes be challenging.

Patient presentation
A 66-year-old male with a history of arterial hypertension was admitted to our cardiology department because during the last night, while he was driving his car, he would have lost consciousness having a car accident. He rapidly felt well even though he doesn't remember what happened a few minutes shortly before and immediately after the event. No witnesses. Due to traumatic chest pain he went to emergency department where an electrocardiogram was performed with evidence of negative T waves and ST-segment depression on V3-V6 leads.

Diagnosis and management
Physical examination didn't show anything relevant. On heart ultrasound he had normal left ventricle ejection fraction with apical hypertrophy (maximum thickness 18 mm). He underwent coronary angiography which showed normal coronaries. During hospitalization a cardiac magnetic resonance was performed; it described apical hypertrophy with some small areas of intra-myocardium delayed enhancement in apex wall. During hospitalizations no arrhythmic alarms were detected.

Discussion
Pharmacological and electrical treatment options are based on classic HCM aiming to reduce symptoms, incidence of atrial fibrillation, ventricular arrhythmias, and sudden death. Concerning ApHCM, left ventricular outflow tract obstruction is usually absent and therefore pharmacological therapeutic benefits may be lower than in classic HCM. Regarding sudden death prevention, we evaluate ICD implantation according to ESC 5-y HCM SCD risk score which doesn't include potential risk markers for SCD in ApHCM such as apical aneurysm, midcavity gradient and midventricular obstruction with SCD risk underestimation. By the way it's still the most used score being aware that could lead to ICD underutilization. In our case, according to HCM risk score 1.74%, negative family screening, no arrhythmic events for 15 days hospitalization, no previous transient loss of consciousness (TLOC) and dynamics of the car accident, we decided to follow-up the patients in our cardiomyopathies outpatient clinic explaining the TLOC with a sleep disorder.