bluuebunny/crossref_metadata_2025_split huggingface dataset:
259 PERICARDITIS AND PERICARDIAL EFFUSION IN CHRONIC GRAFT-VERSUS-HOST DISEASE AFTER ALLOGENIC HEMATOPOIETIC STEM CELL TRANSPLANTATION
Authors: Collini Valentino , De Biasio Marzia , Favaretto Virginia , Fumarola Francesca , Sinagra Gianfranco , Imazio Massimo 
Published: December 2022

Abstract

Background
Pericarditis and pericardial effusion (PE) are rare but potentially life-threatening complications in chronic graft-vs-host disease (cGVHD). Limited data are currently available in this setting, based on small single-center retrospective studies. The purpose of this study is to characterize the clinical presentation, management, and outcomes of pericarditis and PE complicating cGVHD in allogeneic hematopoietic cell transplant recipients.

Material and Methods
We included all consecutive patients referred to our pericardial diseases referral center from 1st May 2008 to 1st May 2022. All patients had no previous cardiovascular or autoimmune diseases and suffered from severe cGVHD with involvement of at least 2 organs. Pericarditis and PE were diagnosed according to ESC guidelines. Conditioning-related toxicity and infections were carefully excluded.

Results
Pericarditis was diagnosed in 4 patients (2 males, mean age 48.5 ±9.0 years), of whom 2 developed mild PE. PE without pericarditis was diagnosed in 5 patients (80% male, aged 53.2 ±8.9 years), in 2 patients it was reported as severe, requiring pericardial drainage. The average time after allogeneic hematopoietic stem cell transplantation was 22 months (range, 4 to 58 months). All patients required escalation of the corticosteroid therapy they were chronically taking, and in 4 cases the therapy was boosted with non-steroidal anti-inflammatory drugs. Only one patient developed a recurrence and died of multiorgan failure associated with severe cGVHD (involving eyes, mucous membranes, skin, joints, liver, pleuro-pericardial effusion) refractory to immunosuppressive therapy. Overall survival was 89% and no patient developed constrictive pericarditis. Mean follow-up was 69±55 months.

Conclusions
Pericarditis and PE related to cGVHD typically occur with mild clinical signs in the context of severe cGVHD, however in our study 22% of patients required pericardial drainage at diagnosis. The mainstay of treatment is high-dose steroids and usually the majority of patients have a favorable outcome.
941 SARCOIDOSIS: THE IMPORTANCE OF MULTIMODALITY IMAGING
Authors: Pezzato Andrea , De Luca Antonio , Radesich Cinzia , Saro Riccardo , Korcova Renata , Pagnan Lorenzo , Dore Franca , Altinier Alessandro , Massa Laura , Zecchin Massimo , Perkan Andrea , Bussani Rossana , Merlo Marco , Sinagra Gianfranco 
Published: December 2022

Abstract
A 55-years old sporty man, without any cardiovascular risk factors nor previously known cardiological history, presented for a cardiological evaluation due to extra-systolic palpitations and dyspnea. The ECG documented a new onset of right bundle branch block, left anterior fascicular block and first degree of atrio-ventricular block. Echocardiography revealed severe left ventricular (LV) systolic dysfunction (LV ejection fraction -EF- 34%), showing also right ventricular (RV) dilatation and disfunction. A 24-hour Holter monitoring did not show any significant arrhythmias and maximal stress test did not reveal any ECG changes nor arrhythmias. Cardiac magnetic resonance (CMR) confirmed the presence of severe biventricular dysfunction; multiple areas of edema and late gadolinium enhancement (LGE) were observed, with ischemic and nonischemic pattern and extensive involvement of the RV and the interventricular septum. In the suspicion of cardiac sarcoidosis a 18-fluorodeoxyglucose positron emission tomography (PET) was performed, confirming the presence of an inflammatory cardiomyopathy in an active phase. A subsequent endomyocardial biopsy was performed, which demonstrated the presence of non-caseating granulomas, signs of inflammation and fibrosis, consistent with the diagnosis of cardiac sarcoidosis. No signs of extra-cardiac involvement were present.
The patient started anti-inflammatory therapy with Prednisone and Metotrexate, along with anti-neurohormonal therapy. However, due to a significant increase in ventricular arrhythmic burden, an implantable cardioverter defibrillator was placed during early follow-up, and Amiodarone therapy was started.
After 5 months of medical therapy, PET scan showed a marked reduction of cardiac inflammation and echocardiography showed a significant LVEF improvement (from 34% to 43%). After slow tapering of steroid therapy Prednisone was stopped, whereas Metotrexate, Amiodarone and anti-neurohormonal therapy were maintained. Follow-up is still ongoing without clinical events.
Sarcoidosis is a systemic inflammatory disease characterized by the presence of non-caseating granulomas in multiple organs. Cardiac involvement is associated with higher incidence of heart failure, ventricular arrhythmias and all-cause mortality. Isolated cardiac involvement is rare but associated with worse prognosis. Multimodality imaging is of paramount importance for the diagnosis and monitoring therapy.
992 EVALUATION OF MYOCARDIAL WORK INDEX IN PREDICTING IN-HOSPITAL COMPLICATION IN PATIENTS WITH TAKOTSUBO SYNDROME
Authors: Migliarino Serena , Cristiano Mario , Iuliano Giuseppe , Ferruzzi Germano Junior , Loria Francesco , Bellino Michele , Silverio Angelo , Attisano Tiziana , Baldi Cesare , Ciccarelli Michele , Galasso Gennaro , Vecchione Carmine , Citro Rodolfo 
Published: December 2022

Abstract

Backgrounds
Tako-tsubo syndrome (TTS) is a reversible heart failure pathology characterized by transient myocardial left ventricular (LV) dysfunction. Although traditionally considered a benign condition in-hospital complications are frequent in this cohort. Non-invasive myocardial work (MW) parameters are considered as emerging indexes in evaluating global and regional myocardial systolic function.

Objective
to investigate the potential association of MW compared to standard echocardiography parameters, such as LV ejection fraction (EF) with in-hospital complications, in TTS patients.

Methods
thirty-eight patients (mean age, 68 ± 12 years, 35 women) with TTS diagnosed with Takotsubo Italian Network criteria were prospectively enrolled and underwent a transthoracic Doppler echocardiography within 24 hours from hospital admission. MW is derived from the non-invasive strain-pressure loop obtained from the 2D strain data, integrating into its calculation the non-invasive brachial arterial pressure. Constructive MW (CMW), MW index (MWI), MW efficiency (MWE), and wasted myocardial work (WMW) were measured. In hospital complications (HC) were defined as a composite of apical LV thrombosis, hypokinetic arrhythmias (HA), supraventricular tachycardia (SVT), acute heart failure (AHF), ventricular tachycardia/fibrillation (VT/VF), cardiogenic shock (CS), respiratory arrest (RA), stroke and cardiac death (CD).
The normal values ​​for the echocardiographic parameters are reported in the Table. Odds ratio, sensitivity and specificity were used to quantify the ability of EF and MW (abnormal vs normal values) in predicting HC.

Results
HC occurred in 16 TTS patients (26% with AHF; 10% with SVT; 8% with CS; 5% with apical LV thrombosis; 5% with HA; 2,6% with RA; 2,6% with stroke; 2% with VT/VF). The MWI and CMW parameters appear to have the best performance in predicting in-hospital complications (odds ratio for having HC: 10.4 (95% confidence interval: 1.2 to 93.3); 8.6 (0.9 to 77.6) respectively, Table), followed by EF (odds ratio 7.0 (0.8 to 64.1)) and WMW (odds ratio 4.8 (0.9 to 26.7)). MWI has the same sensitivity (94%) as CMW and EF, but higher specificity (41% vs 32%). Of note, the highest specificity was obtained by the WMW parameter (41%).

Conclusion
global and regional myocardial performance is transiently impaired in TTS and significantly associated with HC. Although limited by the low number of patients, these results suggest that myocardial work parameters have a good sensistivity and may have a better performance than the EF value in predicting intra-hospital complications. Larger studies will be necessary to confirm these preliminary results.
1041 ELECTROCARDIOGRAPHIC HETEROGENEITY OF PATIENTS WITH VARIANT TRANSTHYRETIN AMYLOID CARDIOMYOPATHY: GENOTYPE PHENOTYPE CORRELATIONS
Authors: Russo Domitilla , Cappelli Francesco , Di Bella Gianluca , Tini Giacomo , Porcari Aldostefano , Cipriani Alberto , Canepa Marco , Merlo Marco , Licordari Roberto , Vianello Pier Filippo , Zampieri Mattia , De Michieli Laura , Perfetto Federico , Sinagra Gianfranco , Autore Camillo , Rapezzi Claudio , Musumeci Maria Beatrice 
Published: December 2022

Abstract
Transthyretin (TTR) amyloid cardiomyopathy due to mutation on TTR gene (vATTR) has extremely different features according to the type of mutation, ranging from a mild to an extensive cardiac involvement. Data about electrocardiographic findings (ECG) in vATTR are limited and not informative of genotype correlation. Aim of this study is to analyze ECG characteristics and their correlation to clinical and echocardiographic aspects in patients with vATTR cardiomyopathy, focusing on different TTR mutations.
This is a multicentric, retrospective, observational study performed in six Italian referral centres for cardiac amyloidosis. We divided patients in two groups, according to the previously described phenotypic manifestations of the TTR mutation. Of 64 patients with vATTR cardiomyopathy, 23 (36%) had prevalent cardiac (PC) TTR mutations and 41 (64%) patients had a prevalent neurological (PN) TTR mutations. Patients with PC mutations were more frequently males and older, with advanced NAC staging. At baseline ECG, atrial fibrillation was more common in patients with PC, while pacemaker induced rhythm in those with PN mutations. Prevalence of atrioventricular and intraventricular blocks, pseudonecrosis pattern and low voltages were not significantly different in both groups. PQ and QRS durations were longer and voltage to mass ratio was lower in PC mutations. Different TTR mutations tend to have distinctive ECG features.
The standard ECG in vATTR is extremely heterogeneous and the specific mutations are associated with distinct instrumental and clinical features. The differences between PN and PC vATTR are only partially explained by the different degree of cardiac infiltration.
662 A CASE OF MILIARY TBC WITH PERICARDIAL INVOLVEMENT
Authors: Padoan Laura , Parise Antonio , Sforna Stefano , Barengo Alberto , Torlone Francesco , Coiro Stefano , Broccatelli Andrea , Rosignoli Daniele , Cavallini Claudio 
Published: December 2022

Abstract

Introduction
Miliary TBC represents a complex disease, quite rare at our latitudes. We present a case with severe pericardial involvement.

Case report
a 25-year-old Pakistani male, recently come in Italy, presented for fever and orthopnea. Anamnesis was difficult because of linguistic barrier. Echocardiogram revealed a large pericardial effusion with signs of tamponade, so a pericardiocentesis was promptly performed and exudative fluid was drained out and sent to laboratory. Blood tests showed anemia, mild hyponatremia, VES elevation and several vitamin deficiencies. Few days after the procedure the patient developed fever with chills, so blood samples for cultural tests were taken, but resulted negative. To better understand the etiology of pericardial effusion and fever a thoraco-abdominal CT was performed and bared multiple micronodular lesions disseminated to lungs, spleen and lever and pleural and peritoneal effusion. At the same time, the DNA search for Koch's bacillus in the pericardial fluid resulted positive for Mycobacterium TBC complex. So, a diagnosis of miliary TBC with pericardial involvement was done and corticosteroid therapy together with antitubercular drugs were started. Encephalic MRI excluded neurological involvement. Patient was isolated and moved to the infectious disease ward, where therapy was continued until recovery.

Discussion
Miliary TBC is a disseminated form due to the hematogenous spread of tubercle bacilli resulting in the formation of multiple tuberculous foci. This manifestation is more frequent in countries where TBC is still endemic (Pakistan, India, Philippines). The disease can progress slowly with few symptoms or acutely (typical of younger) and the identification may be challenging due to its rarity in developed countries and the lack of uniform criteria. Diagnosis is mainly based on the isolation mycobacterial from a specimen or molecular methods such as PCR. Treatment is based on standard antitubercular drugs regimen. The role of corticosteroids is still controversial.

Conclusions
Miliary TBC is a rare disease in developed countries with not well-defined diagnosis criteria and different clinical presentations. However, because of the increase of migration flows, is important to recognize this manifestation, especially when it develops acutely and with life-treating conditions such as pericardial tamponade.
796 LEFT ATRIAL STRAIN ASSOCIATION WITH FUNCTIONAL CAPACITY IN CARDIAC AMYLOIDOSIS
Authors: Pastore Maria Concetta , Giacomin Elisa , Marrese Federica , Ragazzoni Gian Luca , Mandoli Giulia Elena , Focardi Marta , Gozzetti Alessandro , Cavigli Luna , Lisi Matteo , Valente Serafina , D´ascenzi Flavio , Cameli Matteo 
Published: December 2022

Abstract

Background
cardiac amyloidosis (CA) is characterized by an impairment of cardiac diastolic and, in the severe state of the disease, systolic function, with increasing worsening of functional capacity and quality of life (QoL). Six minute walking test (6MWT) and Kansas City Cardiomyopathy Questionnaire (KCCQ) are two well-known and validated methods to assess the impact of heart failure (HF) symptoms and functional capacity. Left atrial strain by speckle tracking echocardiography has emerged as an index of left ventricular (LV) diastolic function and filing pressure, and is also associated with symptoms in HF. However, its possible association with functional capacity and QoL in CA has not yet been investigated.

Objectives
in this observational pilot study, our aim was to evaluate the relationship of left atrial strain with 6MWT and KCCQ in patients with CA (both AL and ATTR).

Methods
we enrolled consecutive patients with CA during routine follow up visits. Patients underwent clinical and echocardiographic evaluation. On the same day, 6MWT was performed and KCCQ was administered. Speckle tracking analysis was performed offline by an experienced operator blinded to the other data. Correlation analysis was conducted using Pearsons’ coefficient and linear regression analysis

Results
overall, 43 patients with CA (25 ATTR, 18 AL) were enrolled. Mean age was 74 ±11, 16% (n=7) were female. Most patients showed normal left ventricular (LV) ejection fraction (55±9) and reduced LV global longitudinal strain (GLS =-12 ± 7%), 32 of them with apical sparing pattern. Mean global peak atrial longitudinal strain (PALS) was 14 (median[IQR]=6.5;23.5), mean 6MWT score = 382±104 and mean KCCQ score= 67 ± 24.
Global PALS showed a strong direct correlation with 6MWT (Fig 1, P= 0.4, R2=0.2, p-value=0.032) and a trend towards correlation with KCCQ (P=0.3, p-value=0.06), although not reaching statistical significance, probably due to the low sample size. The correlation between PALS and 6MWT was even stronger in patients with ATTR (p=0.7, R2 0.4; p-value&lt;0.0001).

Conclusions
our preliminary results show that, global PALS is associated with functional capacity and the burden of HF symptoms in ATTR and AL, suggesting its role as a more objective marker of disease severity in CA.
Fig. 1
291 BIVENTRICULAR ARRHYTMOGENIC CARDIOMYOPATHY: COULD ELECTROCARDIOGRAPHY SEE BEYOND THE CARDIAC MRI?
Authors: Pittorru Raimondo , Marra Martina Perazzolo , De Lazzari Manuel , Migliore Federico , Basso Cristina , Iliceto Sabino , Corrado Domenico 
Published: December 2022

Abstract
We described the case of a 34 year-old young athlete who was referred to our Department because of palpitations and fatigue, arising after hard effort but also present at rest. Family history was unremarkable for sudden cardiac death or cardiomyopathies. Electrocardiography (ECG) evidenced multifocal premature ventricular contractions (PVCs): left bundle branch block (LBBB) morphology and superior/inferior axis; right bundle branch block (RBBB) morphology and undetermined axis. Cardiac MRI showed extensive mid wall and subepicardial late gadolinium enhancement in the postero-lateral left ventricular wall. We decided to perform electroanatomic mapping (EAM) because we didn't explain the origin of the PVCs with LBBB configuration. During EAM we found an area extending from the right ventricular outflow tract to the subtricuspidal lateral inferior wall which it had not described by cardiac MRI. In the low voltage areas, the electrograms inscribed by the catheter's electrodes was fractionated with different waveforms, a finding that corresponds to a deficiency of healthy myocardial tissue. We performed an endomyocardial biopsy in the interventricular septum close to the ventricular out flow tract. The histological examination of the biopsy samples showed fibro-fatty replacement. Therefore genetic testing revealed a pathogenic desmoplakin mutation. According to “Padua Criteria”, we made a diagnosis of biventricular arrhythmogenic cardiomyopathy. We finally implanted a transvenous cardioverter defibrillator.

Conclusion
Diagnosis of arrhythmogenic cardiomyopathy (ACM) is complex and requires the combination of multiple clinical, histological and genetic parameters. This clinical case demonstrated that ECG represent an essential tool in diagnostic work-up of the disease, and electroanatomic mapping is useful in selected cases for the documentation of the diagnosis of arrhytmogenic cardiomyopathy.
172 CLINICAL AND PROGNOSTIC IMPLICATIONS OF RV UPTAKE WITH RADIONUCLIDE SCINTIGRAPHY IN TRANSTHYRETIN CARDIAC AMYLOIDOSIS
Authors: Porcari Aldostefano , Fontana Marianna , Canepa Marco , Biagini Elena , Cappelli Francesco , Gagliardi Christian , Longhi Simone , Pagura Linda , Tini Giacomo , Dore Franca , Bonfiglioli Rachele , Bauckneht Matteo , Miceli Alberto , Girardi Francesca , Martini Anna Lisa , Barbati Giulia , Costanzo Egidio Natalino , Caponetti Angelo Giuseppe , Paccagnella Andrea , Sguazzotti Maurizio , La Malfa Giovanni , Zampieri Mattia , Sciagrà Roberto , Perfetto Federico , Hutt David , Rapezzi1 Claudio , Merlo Marco , Sinagra Gianfranco , Gillmore Julian D 
Published: December 2022

Abstract

Aims
The prognostic role of bone tracer uptake in transthyretin cardiac amyloidosis (ATTR-CA) is controversial. The study investigated the potential prognostic significance of biventricular (BiV) uptake in ATTR-CA.

Methods
Consecutive ATTR-CA patients who had cardiac scintigraphy with acquisition of planar and single-photon emission computed tomography (SPECT) images from the National Amyloidosis Centre (NAC) and four Italian centres were included. Planar BiV uptake was defined in presence of right ventricle (RV) uptake and graded in combination with SPECT imaging. The primary outcome was all-cause mortality.

Results
Among 1422 patients with ATTR-CA, BiV uptake was found in 85% of cases on planar scintigraphy and in 100% of cases on SPECT images. During a median follow-up of 39 months, BiV uptake at planar scintigraphy was associated with a higher all-cause mortality compared to isolated LV uptake (40.5% vs 10.7%, p&lt;0.001), whereas the Perugini scale was not (p=0.27 in grade 2 vs 3). At multivariable analysis, RV uptake at planar scintigraphy leading to BiV uptake (HR 2.80, p=0.001), together with higher age at diagnosis (HR 1.03, p=0.001), V122I TTR variant (HR 1.60, p=0.001), NAC ATTR Stage (HR 1.29, p=0.003), E/e’ (HR 1.02, p=0.044), right atrium area index (HR 1.04, p=0.018) and GLS (HR 1.05, p=0.003) were independently associated with all-cause death. At time-dependent ROC curve analysis, the addition of planar BiV uptake to the NAC stage resulted in improved accuracy of the model for prediction of all-cause death (from AUC 0.74 to 0.79; p&lt;0.001).

Conclusions
Planar RV uptake leading to BiV uptake identified ATTR-CA patients with worse outcome, potentially serving as a novel prognostic marker.
189 PREVALENCE, DISEASE CHARACTERISTICS AND OUTCOMES OF HEREDITARY TRANSTHYRETIN AMYLOID CARDIOMYOPATHY AMONG ELDERLY INDIVIDUALS
Authors: Porcari Aldostefano , Razvi Yousuf , Masi Ambra , Patel Rishi , Ioannou Adam , Rauf Muhammad Umaid , Hutt David F , Rowczenio Dorota , Gilbertson Janet , Martinez-naharro Ana , Venneri Lucia , Whelan Carol , Lachmann Helen , Wechalekar Ashutosh , Quarta Candida Cristina , Merlo Marco , Sinagra Gianfranco , Hawkins Philip N , Fontana Marianna , Gillmore Julian D 
Published: December 2022

Abstract

Aims
Transthyretin amyloid cardiomyopathy (ATTR-CM) is often assumed to be associated with wild-type TTR genotype (ATTRwt) in elderly patients (aged ≥70), some of whom are not offered genetic testing. We sought to estimate the prevalence, clinical characteristics and prognostic implications of variant ATTR-CM (ATTRv-CM) among elderly patients.

Methods
Data from consecutive patients over 70 years of age diagnosed with ATTR-CM at the UK National Amyloidosis Centre between January 2012 and June 2022 were retrospectively evaluated. All patients underwent clinical evaluation, biochemical tests, echocardiography and TTR genotyping. The study outcome was all-cause mortality.

Results
The study population consisted of 2164 patients with ATTR-CM (median age 79 years at diagnosis, 13.2% females, 80.4% Caucasian). ATTRv-CM was diagnosed in 20% (n=431) of the study population of whom 329 (76.3%) carried V122I, 49 (11.4%) T60A, 18 (4.2%) V30M and 35 (8.1%) other pathogenic TTR variants. During a median (range) follow up of 29 (12-48) months, ATTRv-CM was associated with increased all-cause mortality compared to ATTRwt-CM, with the poorest survival observed in V122I-associated ATTRv-CM (p&lt;0.001). Univariable and multivariable binary logistic regression analyses in those with ATTR-CM showed younger age at diagnosis (odds ratio [OR] 0.86 per year, p&lt;0.001), female sex (OR 3.0, p&lt;0.001), Afro-Caribbean ethnicity (OR 59.1, p&lt;0.001), atrial fibrillation (OR 0.65, p=0.015), ischemic heart disease (OR 0.54, p=0.007), peripheral polyneuropathy (OR 3.72, p&lt;0.001) and orthostatic hypotension (OR 3.75, p=0.001) to be independently associated with ATTRv-CM.

Conclusions
Up to 20% of elderly patients with ATTR-CM have a pathogenic TTR variant. These findings support routine sequencing of the TTR gene in all patients with ATTR-CM regardless of age.