bluuebunny/crossref_metadata_2025_split huggingface dataset:
1116 DOUBLE-CHAMBERED RIGHT VENTRICLE AND VENTRICULAR SEPTAL DEFECT IN AN ELDERLY WOMAN: A RARE CASE.
Authors: Cristiano Mario , Iuliano Giuseppe , Rispoli Antonella , Soriente Lucia , Ciccarelli Michele , Galasso Gennaro , Vecchione Carmine , Citro Rodolfo 
Published: December 2022

Abstract

Introduction
Double-chambered right ventricle (DCRV) is a rare congenital heart defect with right ventricular outflow tract (RVOT) obstruction. The right ventricle (RV) is divided into anatomically proximal high-pressure and distal low-pressure chambers by abnormal muscle bundle. DCRV is frequently associated with others congenital heart defects, particularly ventricular septal defects (VSDs). Although its typically presents during childhood and adolescence, it can also present in adulthood.

Case Presentation
An 84-years-old woman was admitted to our hospital, in emergency department, with a 30-days history of worsening dyspnea and exercise intolerance. She was Ukrainian and did not speak Italian or English. The patient past medical history was unknow except for untreated bilateral glaucoma complicated by blindness. Vital signs were notable for tachycardia, tachypnea (respiratory rate, 28/minute), blood pressure of 118/76 mm Hg, SpO2 of 91%. Physical exam revealed left-sided parasternal systolic murmurs, abolished vesicular murmur at lung bases and jugular vein distension with hepatojugular reflux. The ECG showed atrial fibrillation. Chest X-ray showed moderate cardiomegaly, bilateral pleural effusions, and pulmonary congestion. Transthoracic echocardiography (TTE) was performed and revealed a normal-sized left ventricle with mildly reduced left ventricle ejection fraction (EF 48%), left atrial enlargement, biventricular hypertrophy with asymmetrical interventricular septal hypertrophy. Also, we found massive right atrium and enlarged right ventricle with reduced longitudinal contractility (TAPSE of 13 mm and tricuspid annular tissue Doppler S’ velocity = 7.0 cm/sec). Color flow Doppler in parasternal short-axis view revealed a turbulent systolic flow into the right ventricle. Continuous-wave spectral Doppler analysis showed a peak velocity of 5.6 m/ sec corresponding to a peak gradient of 120 mmHg. Real time 3D-TTE confirmed the of mid-ventricular obstruction due to abnormal trabecular tissue. Therapy including diuretics, beta-blockers and anticoagulants was started. Subsequently, a transesophageal echocardiography (TOE) confirmed the presence of an anomalous mid-ventricular muscle bundle and revealed an associated small sub-aortic ventricular septal defect (VSD) leading to the diagnosis of acute RV failure due to double-chambered RV with VSD and atrial fibrillation. Due to the high risk of complications, patient was considered not amenable for surgery. She was discharged on medical therapy.

Conclusions
We report a rare case of DCRV and VSD diagnosed in an elderly patient. Due to its rarity, DCRV continues to be misdiagnosed, especially in adulthood. Three-dimensional echocardiography and TOE were most useful tool to define diagnosis and pathophysiology in such an elderly and non-compliant patient.
49 GLOBAL LONGITUDINAL STRAIN TO EVALUATE THE SUCCESSFUL OF MEDICAL TREATMENT IN SECONDARY CARDIAC LYMPHOMA: A CASE REPORT
Authors: Tonia D´amico Anthea , Giubertoni Ailia , Degiovanni Anna , Patti Giuseppe 
Published: December 2022

Abstract

Background
Cardiac lymphoma is often lately diagnosed and associated with a poor outcome because of delayed treatment. We report the case of a patient with diffuse large B-cell lymphoma (DLBCL) massively infiltrating the heart, who showed significant improvement in echocardiographic features after high-dose chemotherapy.

Case summary
A 39-year-old female was admitted to our hospital after a vasovagal syncope secondary to excisional biopsy of an inguinal lymph node. Symptoms and signs were consistent with heart failure. Brain natriuretic peptide was markedly elevated (472 pg/ml). Transthoracic echocardiography (TTE) revealed moderate hypertrophy of both ventricles, with preserved left ventricular ejection fraction, reduced function of the right ventricle and a monophasic diastolic pattern. Left Ventricular Global longitudinal strain (LV GLS) was significantly reduced (up to -5%). These findings led us to the strong suspicion of biventricular infiltrative heart disease. Positron emission tomography-computed tomography (PET-CT) scan showed metastases to the kidneys, mesenteric and skeletal systems. The results of histological analysis and immunohistochemical staining confirmed the diagnosis of stage IV-B DLBCL. Prompt and aggressive treatment was initiated, performing a multiparametric cardiac monitoring based on biomarkers and TTE prior to and after each cycle of chemotherapy. We early observed a significant reduction of myocardial infiltration with an impressive recovery of LV GLS up to -10% after the 1st cycle of chemotherapy. The echocardiographic abnormalities of the right chambers resulted totally restored. Complete regression of the cardiac lymphoma was obtained, with a recovery of LV GLS up to -11.2% at the end of protocol and -16.8% at the 1st month follow-up. The PET-CT scan confirmed the recovery of the heart.

Discussion
In this case, functional heart improvement due to total regression of cardiac lymphoma has been early detected by strain imaging. GLS could be an early indicator of therapy responsiveness and may be implemented in routinely evaluation of these patients with infiltrative cardiac diseases.
311 CORONARY CT ANGIOGRAPHY A NEW PROMISING TOOL IN HEART TRANSPLANTED PATIENTS: FROM CLINICAL AND ECONOMICAL BENEFITS TO CORONARY INFLAMMATION DETECTION
Authors: Dellino Carlo Maria , Pergola Valeria , Scarpa Francesco , Storer Vittorio , Galzerano Domenico , Tessari Chiara , Fraiese Angela , Motta Raffaella , Gerosa Gino , Iliceto Sabino , Mele Donato 
Published: December 2022

Abstract

Background
Heart transplanted patients are mainly monitored with invasive diagnostic techniques for detecting cardiac allograft vasculopathy (CAV). However coronary CT angiography (CCTA) is a new promising tool that brings clinical and economical benefits.

Objectives
primary aim: demonstrate the non-inferiority of CCTA in comparison to invasive coronary angiography (ICA), in terms of radiation and contrast dose, costs, in-hospital stay and complications. Other aims: analyse in the subgroup of patients undergoing CCTA the role of immunological and non-immunological risk factors and the role of pericoronary-fat-attenuation-index (pFAI) in predicting CAV.

Methods
Between March 2021 and May 2022, 179 consecutive heart transplanted patients underwent either CCTA (78 patients) or ICA (101 patients) based on medical preference to study CAV. The 78 patients who underwent CCTA were also divided in patients with no previous CAV (npCAV, previous ISHLTV=0) and patients with previous CAV (pCAV, ISHLTV≤1). CAV progression was considered if there was any progression in ISHLTV.

Results
CCTA delivered lower radiation doses in comparison with ICA (3,52 mSV [1.46-7.23] versus 10,8 mSV [8.8-20.3]; p=0.03) and required also less in-hospital stay (0.5 ± 0,2 hours versus 23.7 ± 12.31 hours; p&lt;0.001), lower costs (120 euros versus 2800 euros; p&lt;0.001) and less contrast agent (60.4 ± 8.7 ml versus 95.68 ± 47.6ml; p&lt;0.001). The rate of complications of CCTA and ICA was low in both of them (0 (0%) vs 3 (3%); p=0,258). Among the non-immunological risk factors for CAV, only smoking was related with the progression of CAV (0% with ISHLTV=0 and 15% with ISHLTV 1; p=0.015). Among immunological risk factors, TNF was the only independent predictor in the progression of CAV (HR 8.23; IC 95% 1.47-45.81; p=0.019). PFAI either as a continuous variable or as a categorical variable (&gt;-70.1HU) did not show a statistically significance in the progression of CAV (p=NS).

Conclusions
CCTA is superior to ICA in terms of radiation and contrast dose, costs and in-hospital stay. TNF was the only independent predictor related with the progression of CAV. PFAI, didn't reach statistically significance probably due to the small sample size. Further studies are necessary to understand the role of pFAI in this subset of patients.
585 SPONTANEOUS CORONARY ARTERY DISSECTION: THE ROLE OF CORONARY CT ANGIOGRAPHY IN THE FOLLOW-UP MANAGEMENT
Authors: Dellino Carlo Maria , Pergola Valeria , Continisio Saverio , Gentili Alessandto , Montonati Carolina , De Conti Giorgio , Motta Raffaella , Iliceto Sabino , Tarantini Giuseppe , Mele Donato 
Published: December 2022

Abstract

Background
Spontaneous coronary artery dissection (SCAD) is one of the causes of acute coronary syndrome (ACS) and sudden death (SD). This condition predominantly affects young women with a few or no conventional risk factors for CAD. Diagnosis is traditionally made with invasive coronary angiography (ICA); nevertheless, coronary computed tomography angiography (CCTA) is going to be a new useful tool in the acute diagnosis and at follow-up in these patients. Conventional treatment could involve a conservative approach with medical therapy or an invasive approach with percutaneous coronary intervention (PCI).

Methods
We retrospectively analyzed data of 57 SCAD patients followed up with Coronary CT angiography (CCTA) at our centre. Clinical and angiographic (invasive and non invasive) data were collected at baseline and at the follow-up. The primary outcome was a composite of all cause mortality and hospitalization for cardiovascular cause evaluated at 1690,7±1082,3 days; the secondary outcome was the evaluation of the vessels with CCTA at 777,9 ± 271,6 days.

Results
57 patients were divided in 2 groups: 46 patients underwent a conservative treatment (80,7%) and 11 patients a PCI treatment (19,3%). The first group is composed of 15,2% male and the second of 27,3% male (p=0,387), mean age is 52,8±11,1 years vs 48,0±10,7 years (p=0,201). Patients treated with PCI has a significative higher incidence of smoking habits (45,5% vs 15,2%; p=0,042), peripheral arteriopathy (18,2% vs 0%; p=0,034), higher troponin peak (40425,8 vs 13436; p=0,011) and lower ejection fraction (51,4±11,0 vs 57,1±7,6; p=0,050). Moreover the PCI population has a more common involvement of 2 vessels (72,7% vs 6,5%; p&lt;0,001), of the left main coronary artery (45,4% vs 0%; p&lt;0,001) and of the circumflex coronary artery (45,4% vs 2,2%; p=0,001). At the follow up, there were no statistical differences for the primary and secondary outcomes between the 2 groups (p&gt;0,05). Among patients treated with conservative therapy, there were a more frequent recurrence of SCAD in those treated with DAPT than in those treated with SAPT (33,3% vs 5,9%; p=0,033).

Conclusions
in patients with SCAD, conservative management is comparable to PCI treatment in terms of clinical and angiographic outcomes. Among patients treated with conservative therapy, DAPT at discharge was independently associated with a higher rate of SCAD recurrence at follow-up.
809 A CASE OF CASEOUS MITRAL ANNULUS CALCIFICATION
Authors: Diana Davide , Diana Giovanni , Geraci Salvatore , Martello Natale Marco , Savarino Dario , Caramanno Giuseppe 
Published: December 2022

Abstract
Caseous mitral annular calcification (CMAC) is a rare variant of degenerative mitral annular calcification (MAC) with an echocardiographic prevalence of 0.6% of all MACs and an overall prevalence of up to 0.07% in the general population. It primarily affects older patients with hypertension and chronic kidney disease. Due to the general benign prognosis, conservative management of this lesion is recommended in most cases.
Herein we describe the case of a 78-year-old female patient admitted for sustained ventricular tachycardia treated with Amiodaron infusion followed by electrical cardioversion for hemodynamically instability. Her past medical history was remarkable for hypertension, HFpEF and chronic kidney disease. Trans-thoracic echocardiogram showed the presence of an echo-dense mass with central echo-lucency surrounded by calcifications at the posterior peri-annular and leaflet region of the mitral valve, not associated with valvular disfunction.
To define the etiology of the arrythmia, a coronary angiography was performed and excluded significant coronary artery disease. Nevertheless, a round-shape hyperdense mass was observed in the left atrium near the atrio-ventricular groove (figure 1): during injection through coronary vessels the contrast was not visible in the lesion. In order to better define the nature of the mass, a Trans-esophageal Echocardiogram was performed and showed a “toothpaste-like” echo-dense mass with central echo-lucency surrounded by a calcified envelope at the posterior peri-annular region of the mitral valve suggestive for CMAC (figure 2); 3D zoom imaging clarified its relationship with P2 scallop of posterior mitral valve leaflet (figure 3). For the best tissue characterization and to definitely exclude other mass-like lesions, such as myxoma, papillary fibroelastoma, myocardial abscess, vegetations, lipomatous hypertrophy, Cardiac Magnetic Resonance was performed and showed low-signal intensity in both T1- and T2-weighted sequences, reflecting its calcium content. First-pass perfusion sequence reveals no contrast enhancement, whereas late gadolinium enhancement depicts a peripheral rim of enhancement confirming our suspect.
In Conclusion, CMAC is a rare disorder requiring a multimodal imaging approach to differentiate it with other intra-cardiac masses. Its course is generally benign and whether not associated with valvular or hemodynamic significance, it could be considered as a bystander.
Figure 1
Figure 2
Figure 3
503 SPECKLE-TRACKING ECHOCARDIOGRAPHY FOR ARRHYTHMIC RISK ASSESSMENT IN HYPERTROPHIC AND FABRY CARDIOMYOPATHY
Authors: Faro Denise Cristiana , Losi Valentina , Rodolico Margherita Stefania , Licciardi Salvatore , Paola Monte Ines 
Published: December 2022

Abstract

Background
Hypertrophic Cardiomyopathy (HCM) is burdened by sudden cardiac death (SCD) risk of 0.9%/year, and is the most common cause of SCD in young adults. It is an autosomal dominant inherited disease caused by mutations in cardiac sarcomere genes, but the hypertrophic phenotype can also be an expression of cardiac involvement in multiorgan metabolic storage diseases, such as Anderson-Fabry disease (AFD). Mechanical Dispersion (MD) by Speckle-Tracking Echocardiography (STE) has recently emerged as an additional arrhythmic risk marker.

Purpose
Aim of the study was to evaluate LV systolic and diastolic function, global longitudinal strain (GLS) and MD by STE and analyze their association with ventricular arrhythmias in patients with HCM and AFD.

Methods
We included in our analysis 36 patients with HCM, 54 with AFD, of which 10 with left ventricular hypertrophy (AFD-LVH) and 44 without (AFD-N), and 27 healthy subjects. We performed a comprehensive basic echocardiographic study and analyzed GLS and MD (post-processing through EchoPAC 2.02). We also evaluated ventricular arrhythmias (V-AR), including ventricular fibrillation and sustained and non-sustained ventricular tachycardia, by Holter ECG, and the data obtained by cardiac magnetic resonance (CMR) in hypertrophic patients. Data were analyzed by unpaired Student t-test or chi-square/Fisher's exact test as appropriate, and binary logistic regression (SPSS Statistics ver.26).

Results
Diastolic function was impaired in HCM and AFD-LVH patients compared to control and AFD without LVH. GLS was significantly lower in the V-AR group compared to patients without V-AR (9.7±2.9 vs 14.1±4, P=0.007), MD was significantly higher in the V-AR group (111±47 vs 68.1±16, P=0.03). We found a significant association between ventricular arrhythmias and GLS (P=0.005) and between ventricular arrhythmias and MD (P&lt;0.001). We found also a significant association of late gadolinium enhancement at CMR with GLS (P=0.005) and MD (P=0.03).

Conclusions
GLS and MD are useful additional indices in the evaluation of patients with HCM or AFD, also in presence of preserved LV ejection fraction, and promising prognostic predictors to identify patients at high risk for ventricular arrhythmias
Figures: MD in an HCM patient (on the left) and in an AFD-LVH patient (on the right).
911 THE HYPERTROPHIC SEPTAL PERFORATING BRANCH MISINTERPRETED AS INTERVENTRICULAR DEFECT
Authors: Ferraro Daniel , Aquila Iolanda , Volpe Lorenzo , Purri Ilaria , Pasceri Eugenia , Chiarello Claudia , Mascaro Giuseppina , Petullà Maria , Mongiardo Annalisa , Indolfi Ciro 
Published: December 2022

Abstract

Introduction
the septal perforating arteries of the heart usually branch off from the anterior and inferior interventricular arteries and supply the interventricular septum and the conduction system therein.

Aim
we present the case of hypertrophic septal perforating branch in a 71-year-old man who experienced dyspnoea in which was initially misinterpreted as interventricular defect.

Materials and methods
transthoracic echocardiography without and with contrast, coronary angiography and angioTC with 3D reconstructions were performed.

Results
echocardiography showed dilative cardiopathy with EF 30%, akinesia of IVS, apical and inferior wall. Hypokinesia of the remaining walls. At color-Doppler: severe mitral regurgitation and moderate tricuspidal regurgitation. Coronary angiography showed severe multivessel atherosclerotic coronary artery disease. At the echocardiographic control was reported the presence of turbulent flows at the level of medio-basal segment of posterior IVS. Echo-power-Doppler showed no shunts. For persisting suspect of interventricular defect was performed contrast echocardiography that showed hypoperfusion of IVS after contrast infusion but no clear signs of interventricular shunts. To complete the diagnostic iter was performed finally CT-Scan of coronary arteries that reported a presence of hypertrophic septal perforating branch with long intramyocardial course in the interventricular septum, but was no detected interventricular defect.

Conclusions
Septal perforating arteries have a large variability in their anatomy. Particular clincal conditions such as chronic coronary syndromes could lead to a reactive hypertrophy. Is important recognise it due to its similarity with interventricular defect or myocardial bridging effect. A better understanding of these arteries will help physicians to enhance cardiac care for their patients.
1048 MYOCARDIAL WORK AND OUTCOME IN PATIENTS PARADOXICAL WITH LOW FLOW, LOW GRADIENT AORTIC STENOSIS
Authors: Iuliano Giuseppe , Junior Ferruzzi Germano , Migliarino Serena , Cristiano Mario , Bellino Michele , Silverio Angelo , Pamela Peluso Angela , Vecchione Carmine , Ciccarelli Michele , Galasso Gennaro , Citro Rodolfo 
Published: December 2022

Abstract

Aim
The aim of this study was to investigate the potential impact of non-invasive derived myocardial work (MW) indexes on outcome of patients with severe paradoxical low flow, low gradient (PLFLG AS) undergoing transcatheter aortic valve implantation (TAVI).

Methods
Complete demographic, clinical characteristics, laboratory analyses and echocardiographic parameters were collected. Severe PLFLG AS was defined as indexed aortic valve area (iAVA) ≤0.6 cm2/m2, mean transaortic gradient &lt; 40 mmHg and stroke volume index &lt;36 ml/m2 and preserved LVEF &gt;50%. MW was obtained from the non-invasive strain-pressure loop obtained pressure by combining GLS and the left ventricular systolic pressure, which was derived by adding the mean aortic valve gradient to systolic brachial pressure. Constructive MW (CMW), MW index (MWI), MW efficiency (MWE), and wasted MW (WMW) were measured. The normal values ​​considered for the MW parameters were: MWI ≥ 1300 mmHg%; CMW ≥ 1500 mmHg%; WMW &lt; 240 mmHg%; MWE ≥ 90%. Odds ratio, sensitivity and specificity were used to quantify the ability of MW parameters (abnormal vs normal values) in predicting the primary outcome defined as all-causes mortality.

Results
study population included 30 patients with severe PLFLG AS undergoing TAVI. The most frequent comorbidities were hypertension (93%; n=28), dyslipidaemia (66%; n=20), diabetes (23%; n=7). Atrial fibrillation/flutter and chronic kidney disease were identified in 12 (40%) and 18 (60%) patients, respectively. Concomitant coronary artery disease and history of stroke were reported in 23% (n=7) and 10% (n=3), respectively. Society of Thoracic Surgeons score in overall population was mean 11,34±4,34. During median of 209 days (IQR: 104–213 days) all-causes mortality occurred in 13 patients (43%) (just 1 for non-cardiac death). Abnormal values of MWI, CMW and MWE identified significant statistical correlation with primary outcome [(odds ratio for primary outcome: 7.5 (95% confidence interval: 1.4 to 39.8); 7.5 (1.4 to 39.5) and 5.2 (1.1 to 25.3) respectively, Table 1)]. The MWI, CMW and MWE have the same sensitivity (62%) but higher specificity (82% for MWI and CMW, 88% for WMW and 76% for MWE).

Conclusion
In a population of patients with PLFLG-AS characterized by normal ejection fraction the abnormal MW parameters seem to be significantly associated with all-causes mortality during mid-term follow up and might provide additional information on outcome of this peculiar subgroup of patients with AS.
Table 1
1076 AN UNUSUAL PAPILLARY MUSCLE PRESENTATION AT CARDIAC-MRI.
Authors: Figliozzi Stefano , Francone Marco , Bragato Renato Maria , Stefanini Giulio Giuseppe , Condorelli Gianluigi , Monti Lorenzo 
Published: December 2022

Abstract

Background
Cardiac-MRI is the gold-standard non-invasive imaging modality to evaluate cardiac masses.

Case Summary
A 58-year-old man underwent surgical excision of a sacral chordoma. At the time of surgery, there was neoplastic infiltration of bones and soft tissues (immunophenotype: CKMNF116+, EMA+, S100 +/-) and radiotherapy was performed. The patient underwent superior-lobectomy of the left lung six years later because of pulmonary metastasis of the chordoma. Nine years later, the patient was asymptomatic and referred to us for Cardiac-MRI because a suspected cardiac mass at echocardiography. At Cardiac-MRI, we found normal bi-ventricular volumes, wall thickness, wall motion and ejection fraction. Atria, heart valves and pericardium were normal too. In ssFP-cine images, an oval, hypointense mass with sharp margins (26×18 mm) was evident in the context of the posteromedial papillary muscle (Panel, A-B). Compared to the myocardium, the lesion was hyperintense at T2-weighted (Panel, C), native T1-(Panel, D) and T2-mapping sequences (Panel, E). After contrast injection, there was no increased mass signal at first-pass perfusion (Panel, F), whereas the late sequences revealed a soft, heterogeneous enhancement of the central core of the lesion, and a bright peripheral rim (Panel, G-H). These findings were interpreted as a cardiac metastasis of the chordoma and systematic treatment with imatinib was started.

Discussion
Six cases of cardiac metastasis secondary to chordoma were previously described. The present case is the first to provide advanced, multiparametric Cardiac-MRI assessment and represents the first description of a location within the context of a papillary muscle. Cardiac-MRI was essential to identify cardiac metastasis, leading patient's management and targeting appropriate treatment.