bluuebunny/crossref_metadata_2025_split huggingface dataset:
990 IMMUNE-MEDIATED NECROTIZING MYOPATHY IN A PATIENT WITH MULTI-VESSEL CORONARY ARTERY DISEASE
Authors: Benvenuto Ciro Pollio , Liuzzo Giovanna 
Published: December 2022

Abstract
Inflammatory muscle diseases (often referred to as “inflammatory myopathies” or “myositis”) are a group of muscle diseases characterized by chronic muscle inflammation, inflammation of tissues associated with the muscles, muscle weakness and occasionally muscle pain (depending on the specific case).
Ischemic heart disease is the leading cause of cardiovascular mortality in Western countries; the underlying cause is coronary atherosclerosis, a multifactorial disease determined by the concomitant presence of classic cardiovascular risk factors and inflammation of the vessel wall.
We present the case of a 60 y/o man who previously underwent CABG (LIMA for ADA e GSV for OM1) for multi-vessel CAD and then developed immune-mediated necrotizing myopathy (probably due to statin treatment) with muscle weakness and pain and with elevation of myolysis markers: in this context, he also presented a recurrence of chest pain with troponin elevation, so that it was needed the integration of functional imaging from myocardial scintigraphy and from cardiac magnetic resonance to understand that troponin elevation (and the chest pain) were due to ischemia and not to muscle damage. This case report underlines how a critical approach to imaging and a deep knowledge of pathophysiology is mandatory in cardiovascular medicine.
403 ECG EXERCISE STRESS TEST PREDICTORS OF LEFT MAIN CORONARY ARTERY DISEASE
Authors: Tremamunno Saverio , Yuksek Ayda Naz , Telesca Alessandro , Cambise Nello , Tinti Lorenzo , Di Renzo Antonio , Belmusto Antonietta , De Vita Antonio , Crea Filippo , Lanza Gaetano Antonio 
Published: December 2022

Abstract

Background
Left main coronary artery disease (LMCAD) heavily affects prognosis of patients with suspected CAD. Thus, its identification/exclusion is an important step in the assessment of these patients. Although being the gold standard to identify LMCAD, invasive coronary angiography (ICA) is burdened by some risks, while coronary computed tomography angiography (CCTA) has still limited availability, quite high costs and associated radiologic risks.
In the past decades, several studies demonstrated the utility of ECG exercise stress test (EST) for the identification of LM disease in patients with suspected CAD. However, the pre-test probability of CAD of subjects undergoing EST has significantly changed in the last decades.
Accordingly, in this study we aimed to assess the predictive value of EST for the presence/absence of LMCAD in a contemporary population of patients with suspected CAD.

Methods
We retrospectively enrolled 495 consecutive patients, referred to our Center between years 2018 and 2021 because of suspected CAD, who underwent both an EST (standard treadmill Bruce protocol) and ICA (within 12 months of the EST). Patients with a history of coronary artery bypass surgery were excluded.

Results
Overall, 24 patients (4.8%) were found to have LMCAD at ICA. Among clinical variables, only male gender (p=0.025) and smoking (p=0.003) were associated with LMCAD. A number of ECG leads with EST-induced ST-segment depression (STD) ≥5 and a maximal STD ≥2 mm were more frequently found in patients with, compared to those without LMCAD (29.2 vs 9.8%, p=0.003; and 58.3 vs 24.8%, p&lt;0.001, respectively). No other EST variable was associated with LMCAD, including ST-segment elevation in lead aVR (4.2 vs. 2.6% in the 2 groups, respectively, p=0.63). On the other hand, among the 206 patients (41.6% of the population) who achieved 85% of maximal heart rate predicted for age and had ST-segment depression &lt; 2mm, LMCAD was present in 1.9% only.

Conclusions
Our data indicate that EST continues to be a valuable tool for predicting the presence/absence of LMCAD in contemporary populations of patients with suspected coronary artery disease.
758 CASE REPORT OF A CATHETER-RELATED THROMBOSIS IN THE RIGHT HEART
Authors: Pezzini Sara , Daus Francesca , Lobiati Elisabetta , Giannattasio Cristina , Savonitto Stefano 
Published: December 2022

Abstract

Introduction
Central venous catheters (CVCs) are commonly used in clinical practice. Their use may be associated to complications like catheter-related thrombosis, that may not only result in vascular and catheter occlusion but also infection, pulmonary embolism, and formation of right heart thromboemboli. Patients who develop catheter-related thrombosis have an increased risk of mortality due to the potential risk of embolization to the pulmonary vasculature.

Case presentation
We describe the case of a 76-year-old man with multiple cardiovascular risk factors, admitted to the Nephrology Department for a suspected UTI. The patient develops a central venous catheter-related thrombosis on the CVC placed in the right jugular vein. The finding of the thrombus was accidental and happened during a transthoracic echocardiography performed to exclude endocarditis. The thrombus was successfully treated with LMWH infusion, preventing complications like pulmonary embolism.

Conclusion
Although generally considered safe, central venous catheters can be associated with complications such as catheter-related venous thrombosis. Intensivists who use these devices should be aware of this possible complication and may use strategies to prevent them in order to improve patient outcome. The present case highlights the importance of maintaining a high index of suspicion for thromboembolic complications in patients with CVCs.
20 LEAD-RELATED SUPERIOR VENA CAVA SYNDROME IN A PACEMAKER-DEPENDENT PATIENT: A HYBRID PROCEDURE
Authors: Pittorru Raimondo , Migliore Federico , Arturi Federico , Tona Francesco , Tarzia Vincenzo , Iliceto Sabino , Gerosa Gino 
Published: December 2022

Abstract
We report a case of a 77-years-old man admitted to our department because of facial plethora, prominent venous collateral over the anterior chest wall, marked swelling of the head and upper extremities. His medical history was notable diabetes, non-Hodgkin lymphoma (NHL), eradicated HCV, AV block, for which he underwent placement of a dual-chamber pacemaker 4 years earlier. A superior vena cava (SVC) syndrome lead-related was documented on angio-CT scan and phlebography. After a multidisciplinary team discussion, a treatment strategy was designed to consist of transvenous lead extraction, SVC reconstruction and epicardial pacing placement. At 5 months follow-up, he was completely relieved of symptoms.

Conclusion
Lead-associated SVC syndrome is a rare condition and there is no standard management protocol. Data are lacking and we need at least a registry to make the treatment homogeneous. In this specific patient, a hybrid procedure based on lead extraction and reconstruction of superior vena cava was safe and effective for the relief of symptoms and maintenance of SVC patency. Heparin alone will not lead to the resolution of the disease if the disease is at an advanced stage.

Learning outcomes
Patients with lead-induced superior vena cava syndrome often required a multidisciplinary approach. A hybrid procedure based on transvenous lead extraction and reconstruction of superior vena cava is safe and effective to achieve relief of symptoms, maintenance of SVC patency, and adequate pacing.
557 RAREFACTION OF MYOCARDIAL LYMPHATIC VESSELS IN PATIENTS WITH CARDIAC AMYLOIDOSIS
Authors: Aimo Alberto , Martinelli Giulia , Bonino Lucrezia , Musetti Veronica , Emdin Michele , Pucci Angela 
Published: December 2022

Abstract

Introduction
In cardiac amyloidosis (CA), amyloid fibers accumulate in the extracellular spaces of the heart. It is reasonable to speculate that this accumulation could lead to a compression and obliteration of lymphatic vessels, and possibly to their eventual disappearance. As lympathic drainage removes proteins from the interstitial spaces, including amyloid precursors, an impairment of lympathic vessels could exacerbate amyloid accumulation, thus triggering a vicious circle.

Methods
We evaluated consecutive endomyocardial biopsies (EMB) performed as part of the standard diagnostic workup for CA or for unexplained left ventricular (LV) hypertrophy, with no specific suspicion of CA. All EMBs were performed in the septal region of the LV. Three samples were analyzed through Congo red staining and immunohistochemistry, with final confirmation of the amyloid subtype through a proteomic approach. Lymphatic vessels were visualized through antibodies targeting the specific marker podoplanin. Echocardiographic variables measured at the time of the EMB were collected.

Results
We examined 15 patients with CA (ATTR-CA, n=10; AL-CA, n=5; 50% men, median age 75 years [interquartile range 71-78]), and 13 patients with CA excluded (55% men, median age 68 years [59-71]). The median density of lymphatic vessels in patients with CA was 0.52 vessels/mm2 (0-3.32), and 10.90 vessels/mm2 (3.10-26.25) in patients without CA (p=0.001). No significant differences were found in LV mass index (p=0.170), interventricular septal thickness (p=0.262), relative wall thickness (p=0.078), or LV ejection fraction (p=0.262), while posterior wall thickness was higher in patients with CA (p=0.042). Three patients with ATTR-CA and 2 patients with AL-CA had no lymphatic vessels on their tissue samples. Patients with ATTR- or AL-CA did not display significant differences in the density of lymphatic vessels, or any metric of increased LV wall thickness or systolic function.

Conclusions
We report for the first time that patients with CA have significantly less myocardial lymphatic vessels than patients without CA, despite no significant differences in the degree of LV wall thickening or LV ejection fraction. These results support the hypothesis that the lymphatic drainage is deranged in patients with CA, possibly promoting further amyloid deposition.
957 EMERGING FROM THE DARKNESS. SUDDEN CARDIAC DEATH IN CARDIAC AMYLOIDOSIS
Authors: Antonelli Giorgio , Cammalleri Valeria , De Luca Valeria Maria , Annibali Ombretta , Nusca Annunziata , Mega Simona , Carpenito Myriam , Ricciardi Danilo , Guerrieri Fiorella , Avvisati Giuseppe , Ussia Gian Paolo , Grigioni Francesco 
Published: December 2022

Abstract
Cardiac amyloidosis (CA) manifests as infiltrative cardiomyopathy with a hypertrophic pattern, usually presenting with heart failure with preserved ejection fraction (HFpEF). In addition, degenerative valvular heart disease, particularly severe aortic stenosis, is commonly seen in patients with CA. However, amyloid fibril deposition may also infiltrate the conduction system and promote the development of electrical disorders, including ventricular tachyarrhythmias (VT), atrio-ventricular block, or acute electromechanical dissociation (EMD). These manifestations can increase the risk of sudden cardiac death (SCD). This review summarizes the pathophysiological mechanisms and risk factors for sudden cardiac death in cardiac amyloidosis and focuses on the major current concerns regarding medical and device management in this challenging scenario.
Heart involvement is the major determinant of survival in patient with amyloidosis and a “sudden death” occurs in approximately two-thirds of patients with cardiac amyloidosis. Moreover, the AL amyloid fibrils are shown to be highly cytotoxic to the ventricular myocardial, explaining why ventricular arrhythmias appear more frequently in AL over ATTR. Proposed mechanisms driving electrophysiological manifestations of CA involve intramural coronaries, microvascular ischemia, or patchy myocardium infiltration of amyloid fibrils, causing the development of anatomical re-entrant circuits responsible for ventricular arrhythmia. The prevalence of non-sustained ventricular tachycardia (NSVT) in AL amyloidosis ranges from 5 to 27% with routine monitoring and 100% during the stem cell transplant period. The guidelines don't provide specific indications regarding insertion of implantabile cardioverter defibrillator (ICD) and they don't clarify whether some patients subtypes can benefit from them.
Amyloid infiltration into the conduction system enhances the genesis of rhythm disturbances, including fatal ventricular arrhythmias and SCD. Current pharmacological anti-arrhythmic therapies are poorly tolerated in CA, and there are no robust recommendations on the management of ventricular arrhythmias in this subset of patients. Furthermore, the benefit of ICD implantation is highly variable according to the different clinical stages of the disease. Therefore, further studies are needed to create a standardized diagnostic algorithm and appropriate treatment strategy for this special population.
620 A RARE CASE OF HYPERTROPHIC CARDIOMYOPATHY
Authors: Bandini Marta , Carrassa Gianmarco , Di Mario Carlo 
Published: December 2022

Abstract

Background
In patients presenting with hypertrophic cardiomyopathy (HCM), the differential diagnosis of “non-sarcomeric” versus “sarcomeric” HCM forms is crucial because of their differences in prognosis and treatment strategies.

Case presentation
We present the case of a 31 years-old male with unexplained syncope, occurring abruptly. His father died of sudden cardiac death (SCD) and his paternal aunt was known for HCM. The electrocardiogram showed left ventricular (LV) hypertrophy and signs of ventricular pre-excitation; the echocardiogram showed marked and asymmetric hypertrophy with normal systolic function. Cardiac magnetic resonance finally confirmed the suspicion of HCM with marked septal fibrosis. In the setting of autosomal dominant HCM coexisting with Wolf-Parkinson-White syndrome, one of the most significant genetic syndrome is PRKAG2-syndrome (PS).

Conclusion
PS represents a rare phenocopy of the classic form of HCM distinguished by ventricular pre-excitation and often accompanied by advanced heart blocks, due to the marked septal fibrosis involving the conduction system. In the presented case, the diagnosis has been then confirmed by genetic testing. What would be the best therapeutic approach in the prevention of SCD? Can subcutaneous defibrillator implantation (S-ICD) be sufficient?
1064 LONG QTC IN HCM: A CONSEQUENCE OF MYOCARDIAL HYPERTROPHY OR A DISTINCT GENETIC DISEASE?
Authors: Cava Francesco , Micolonghi Caterina , Savio Camilla , Musumeci Maria Beatrice , Melato Giacomo Tini , Petrucci Simona , Alesi L , Germani Aldo , Piane Maria , Autore Camillo , Rubattu Speranza Donatella 
Published: December 2022

Abstract
Hypertrophic cardiomyopathy (HCM) is an autosomal dominant condition, characterized by the presence of unexplained left ventricular hypertrophy. This condition is usually associated with electrocardiographic abnormalities including QTc prolongation reported in the 13% of HCM patients. The main explanation for QTc prolongation in HCM is myocardial hypertrophy and the related structural damage. However, mechanisms other than myocardial hypertrophy, including long QT syndrome (LQTS) gene mutations, may also be involved. The addictive effect of 2 disease-causing mutations has been already reported in other pathological conditions. In the present study we investigated the possible co-inheritance of pathogenic variants associated with both LQTS and HCM, exploring the hypothesis of a distinct genetic basis underlying QTc prolongation in HCM. For this purpose, we considered a cohort of 150 HCM patients carrying pathogenic variants in sarcomeric genes. Out of them we selected 25 patients carrying a QTc prolongation not explained by any other cause (drugs, etc). The QTc was considered prolonged if greater than 450 ms in males and greater than 470 ms in females. NGS analysis was performed with Illumina TrueSight Cardio panel genes on Illumina MiniSeq platform to evaluate the presence of pathogenetic variants in LQTS related genes. We identified pathogenic/likely pathogenic variants in LQTS related gene KCNQ1 (c.1781G&gt;A, p. Arg594Gln; c.532G&gt;A, p. Ala178Thr) in two patients (8%) and uncertain significant variants in SCN5A, KCNJ5, AKAP9 and ANK2 in four patients (16%). Although the results of our study are limited by the small number of patients included in the analysis, they highlight a minor contribution of LQTS genes in HCM patients with a QTc prolongation. The screening for ion channel gene mutations should be considered in HCM patients with QTc prolongation, not explained by any other cause. This strategy may be important for risk stratification and treatment planning.
128 AN UNEXPECTED ENDING OF PREGNANCY
Authors: Coccalotto Marco , Cumitini Luca , Giubertoni Ailia , Rossi Lidia , Rosso Roberta , Patti Giuseppe 
Published: December 2022

Abstract

Introduction
Dilated cardiomyopathy (DCM) is a condition whose etiologies may be various. DCM with ventricular disfunction is a frequent complication of chemotherapy. Peripartum cardiomyopathy is characterized by a reduced ejection fraction (EF), presenting toward the end of pregnancy or in the first months after delivery. Lipodystrophy may be associated to dilated and hypertrophic cardiomyopathy.

Material and Methods
A 28-years-old pregnant woman in her late 33rd week was admitted with acute heart failure complicated by pulmonary edema. The patient had history of acute lymphoblastic leukemia during childhood treated with chemo and radiotherapy, followed by hematopoietic stem cell transplantation (HSCT). Afterwards she developed partial acquired lipodystrophy, causing diabetes mellitus, insulin resistance, dyslipidemia, leptin deficiency and steatohepatitis. A mild left ventricular disfunction had been reported before HSCT, with complete recovery at follow up.
After admission, the scenary rapidly deteriorated towards cardiogenic shock, so a caesarean section was performed. Transthoracic echocardiography revealed diffuse hypokinesia conditioning severe left ventricular (LV) dysfunction (EF 24%), moderate mitral and tricuspidal valve regurgitation and left to right shunt in possible atrial septal defect. A cycle of levosimendan was administrated, with subsequent improvement in LV function (EF 32%). Cardiac magnetic resonance (CMRI) showed hypokinetic DCM with no pathological findings in T1, T2, STIR sequences nor LGE. In parallel, empty sella in possible Sheehan Syndrome was hypothesized. A diagnosis of peripartum cardiomyopathy on the basis of a chemotherapy-induced cardiomyopathy in a good compensation status was made, with no evidence of lipodystrophy-related cardiac involvement. After recovery the patient was discharged with optimal cardiological therapy and a wearable cardioverter defibrillator. During follow up, the patient improved ventricular function (EF 40%) without any registered arrhythmia. She will be evaluated for heart transplantation.

Conclusions
Pregnancy in patients with cardiac disease can be life-threatening for mother and fetus, a multidisciplinary approach and a correct timing are crucial to achieve the best possible result. Left ventricular disfunction can be related to many etiologies, and instrumental tests such as echocardiogram and CMRI are crucial to evaluate the cause of the disfunction. Close follow up in patients who underwent chemo/radiotherapy or are affected by metabolic diseases may avoid long-term cardiac complications.
1072 A RARE CASE OF CONSTRICTIVE PERICARDITIS AFTER A STAB WOUND TO THE CHEST
Authors: Del Giudice Carmen , Cosimo Renato , Pezzullo Enrica , D´aquino Malvezzi Marco Caracciolo , Bussani Rossana , Sinagra Gianfranco , Golino Paolo , Loffredo Francesco 
Published: December 2022

Abstract
A 39 year old man presented to the emergency department with dyspnoea on exertion, chest pain and fatigue. Few years before, the patient was stabbed in the left chest thus developing Pneumothorax and mild pericardial effusion successfully treated with chest tube drainage and medical therapy. Physical examination revelead giugular vein distension and lower extremity edema. The echocardiographic evaluation showed typical signs of constrictive pericarditis which was confirmed by cardiac catheterization. Furthermore, because of clinical worsening and the absence of ongoing inflammation on PET-TC, the patient was referred to surgery. Pericardiectomy was successfully performed with rapid symptoms relief and clinical benefits. Histologic examination of pericardium showed the presence of thick fibrotic tissue with clusters of squamous epithelial cells with no cellular atypias or malignancy. Therefore, we hypothesize that following the chest stabbing wound, squamous and epitheloid cells have invaded the pericardial space producing a fibrotic reaction responsable of the development of the constriction. Furthermore multimodality imaging has been essential for diagnosis and management.
28 CONSTRICTIVE PERICARDITIS: A ROOM FOR MULTI-MODALITY IMAGING
Authors: Del Monaco Guido , Tumminello Fabio , Figliozzi Stefano , Pagliaro Beniamino , Fumero Andrea , Barbone Alessandro , Cuko Enea , Mantovani Riccardo , Fazzari Fabio , Cannata Francesco , Curzi Mirko , Catapano Federica , Monti Lorenzo , Francone Marco , Pini Daniela , Stefanini Giulio Giuseppe , Torracca Lucia , Condorelli Gianluigi , Bragato Renato Maria 
Published: December 2022

Abstract

Introduction
The diagnosis of constrictive pericarditis can be challenging through conventional imaging. Novel imaging techniques have been proposed to improve it.

Clinical Case
A 74-year-old man with a previous diagnosis of heart failure with preserved ejection fraction and permanent atrial fibrillation (AF) was admitted because of worsening dyspnea, peripheral edema, and ascites refractory to diuretics therapy. ECG revealed low QRS voltages and AF with normal heart rate and laboratory exams detected increased values of BNP and hepatic stasis indices; blood count and CRP were normal. Echocardiography revealed mildly reduced bi-ventricular pump function (left ventricular ejection fraction = 52%, right ventricular fractional area change = 25%) and slightly increased wall thickness. Atria were moderately dilated (left and right atrium 46 and 44 ml/mq, respectively) and mitral and tricuspid annuli were dilated too. There was inferior vena cava plethora, moderate atriogenic tricuspid regurgitation and mild-to-moderate atriogenic mitral regurgitation. A paradoxical interventricular septal motion and significant respiratory changes on trans-mitral (&gt;25%) and trans-tricuspid (&gt;30%) pulsed Doppler were noted; however, there was no "annulus reversus" (lateral and septal e’ 13 and 11 cm/sec, respectively) at TDI analysis. In contrast, 2D-speckle-tracking global longitudinal strain showed impaired deformation ​​of the lateral segments with relative sparing of the septal segments. The latter pattern has been recently described as "strain reversus" or "hot septum sign". The findings were suspected of constrictive pericarditis, but a chest Computed Tomography excluded pericardial calcifications. Cardiac magnetic resonance (CMR) was then performed revealing a "septal shift" at cine-real time sequences analysis. The pericardium was slightly thickened (4-5 mm) with widespread late gadolinium enhancement but without signs of acute inflammation at T2-weighted imaging. Tricuspid regurgitation was confirmed to be "moderate" after phase-contrast imaging. Cardiac catheterization finally revealed normal coronary arteries and confirmed the diagnosis of constrictive pericarditis (square root sign, equalization of bi-ventricular end-diastolic pressures). The patient underwent pericardiectomy and tricuspid and mitral valve repair and was asymptomatic at 6-months follow-up.

Conclusions
Constrictive pericarditis represents a potentially reversible cause of heart failure but can be easily missed through conventional imaging. In the present case, advanced echocardiography and CMR were essential to come up with an accurate diagnosis, guiding the patient's clinical management with excellent outcome.