bluuebunny/crossref_metadata_2025 huggingface dataset:
P271 CASEOUS CALCIFICATION OF THE MITRAL ANNULUS: ROLE OF 3D ECHOCARDIOGRAPHY
Authors: Fede G , Abate G , Belluardo P , Guccione N , Scollo V , Sulsenti G , Ficili S
Published: May 2023

The caseous calcification of the mitral annulus (CCMA) is a rare degenerative condition of the mitral valve (0.067% prevalence in the general population) that generally affects older women with hypertension, chronic kidney disease and dyslipidemia. Usually CCMA is asymptomatic and it is associated with mild to moderate mitral regurgitation and it can cause severe mitral valve dysfunction that requires surgical treatment. CASE 1: A 84–year–old woman, with arterial hypertension, was evaluated in our clinic to perform a transthoracic echocardiogram (TTE) for a previous finding of heart murmur. TTE reveled at the level of the posterior mitral annulus a hyperechoic roundish formation with a central eco–shiny area with a maximum diameter of 20x20 mm. A tridimensional trans–esophageal echocardiography (3D TEE) allowed a better characterization of the mass showing well–defined contours, adhered to the posterior mitral annulus, systolic dislocation of the anterior mitral leaflet, with slight mitral regurgitation. Considering the morphological characteristics of the mass we concluded for a presumptive diagnosis of CMAC. We recommended regular follow–up to the patient. After 6 and 12 months the mass did not change in size and echostructure. CASE 2: An 88–year–old woman with chronic kidney disease, high blood pressure and chronic ischemic heart disease was admitted to our ward for acute heart failure. TTE showed a large an echodense mass (43 × 19 mm) involving the posterior mitral annulus, with a central echo–lucent area. A 3D TEE was performed with better mass characterization: involvement of the entire posterior mitral ring, reduced movement of the posterior mitral flap, anterior mitral flap with normal morphology and normal mobility. In consideration of the echocardiographic characteristics we concluded for the presence of CMAC. We discharged the patient with indication for a regular clinical and echocardiographic follow–up.

Conclusions

The 3D TTE provides adequate morphostructural information that allows in most cases to characterize the CCAM. When the transthoracic approach is difficult due to poor acoustic windows, 3D TEE can be used. Only in rare cases it is necessary to use second–level diagnostic tests such as CT and Cardiac Magnetic Resonance Imaging.
P283 PULMONARY, CARDIAC AND NEUROLOGIC FOLLOW–UP AFTER SEVERE COVID–19 PNEUMONIA
Authors: Franco E , Oberto L , Gagliardi L , Barra M , Ruffini S , Cavallone E , Moretti C
Published: May 2023

Background

Since December 2019 COVID–19 has been burdening all health systems worldwide. However, pulmonary and extrapulmonary sequelae of COVID–19 after recovery from the acute disease are still under study. Purpose: The aim of this study was to investigate pulmonary impairments, neurologic disorders and the prevalence of right ventricle dysfunctions in patients with severe COVID–19 pneumonia, almost six months after discharge from hospital. Material and methods: Hospitalized COVID–19 patients with severe pneumonia, requiring mechanical ventilation were included in the study and evaluated at about 6 months after hospital discharge. Pulmonary function tests, echocardiography, EMG were performed to all patients, and a sitting psychotherapy was proposed.

Results

A total of 56 patients (median age 64 years) with severe COVID–19 pneumonia were included in the study. The disease was complicated by pulmonary embolism in 5 patients, by cerebral stroke in 1 patient, by bacterial infections in 4 patients, by pericarditis in 1 patient, by Guillain Barrè syndrome in 2 patients. At almost 6 months after hospital discharge, 17 patients (30%) had dyspnea NYHA II, 1 was in NYHA III (1.8%), and 4 (7%) suffered from symptoms of fatigue. Pulmonary function tests did not reveal any significant limitation (FVC: 96.9% {78–101}; FEV1: 99% {72–103}; FEV1/FVC 81.3% {76–85}; except for slightly reduced DLCO (61% {69–95}). Only 3 (5%) patients developed a severe DLCO alteration, among them only one patient had acute pulmonary embolism. There were no echocardiographic impairments, in particular right chambers were normal in size and function (median TAPSE=23 mm, median S1 = 12 cm/sec) and there were no cases of pericardial effusion. From the neurological point of view, in addition to the 2 patients who have developed a Guillain Barré syndrome, the patients recovered from COVID–19 who have left the Resuscitation of our Hospital have manifested after–effects of Critical Illness Myopathy and Neuropathy (CRIMYNE), a form of neuromuscular pathology considered in all respects an "organ failure" linked to the stay in intensive care caused by inflammatory mediators.

Conclusions

Hospitalized patients with severe COVID–19 pneumonia, requiring mechanical ventilation, are unlikely to develop pulmonary long–term impairments or cardiac dysfunction after discharge, but frequently suffer from symptoms of fatigue and persisting dyspnea on effort, due to CRIMYNE syndrome.
P287 RELATIONSHIP OF HYPOVITAMINOSIS D WITH HEART FAILURE DEVELOPMENT, HEART TRANSPLANTATION NECESSITY AND MORTALITY AMONG INDIVIDUALS WITH DILATED CARDIOMYOPATHY
Authors: Aleksova A , Fluca A , D‘Errico S , Munaretto L , Zhou X , Sinagra G , Janjusevic M
Published: May 2023

Background

Non–ischemic dilated cardiomyopathy (DCM) is a progressive heart disease often affecting young individuals leading to heart failure (HF) development, heart transplantation (HT) and adverse outcome. We aimed to evaluate the prevalence of hypovitaminosis D among Italian patients with DCM and its prognostic value during follow–up.

Methods

Plasma vitamin D levels were measured in 204 patients with DCM enlisted from The Trieste Registry of Cardiomyopathies. The endpoints during follow–up were defined as the incidence of major ventricular arrhythmias (MVAs), HT, hospitalization for HF and all–cause mortality.

Results

The median concentration of vitamin D in our cohort was 20.7 (15.53–29.3) ng/ml) and the majority of enrolled individuals were vitamin D deficient (54.4%). Vitamin D levels positively correlated with left ventricular ejection fraction (LVEF) (R=0.216, p=0.002) and patients with LVEF > 35 have significantly higher plasma vitamin D concentration compared to patients with LVEF <= 35 (respectively, 22.7 (17.35 – 30.5) ng/ml vs 19.2 (13.0 – 26.35) ng/ml, p=0.009). During a median follow–up of 58.47 (38.05 – 74.97) months, 7.8% of patients died, 3.9% had HT, 12.3% of individuals were hospitalized for HF, and 12.7% suffered from MVAs. Deceased individuals, patients requiring HT and hospitalized for HF during follow–up were observed to have significantly lower plasma vitamin D values compared to living and event–free individuals at the end of follow–up (respectively, 18.65 (9.49 – 20.7) ng/ml vs 21.3 (15.75 – 29.58) ng/ml, p=0.046, 14.35 (7.03 – 17.85 ng/ml vs 20.9 (15.9 – 29.48) ng/ml, p=0.021, 15.5 (9.56 – 21.35) ng/ml vs 21.7 (16.2 – 29.5) ng/ml, p= 0.012). In addition, patients suffering from MVAs had lower vitamin D values compared to individuals without events, although not significant (18.8 (14.48 – 24.48) ng/ml 21.56 (15.6 – 29.53) ng/ml, p=0.219). Unfortunately, Kaplan–Meier analysis and multivariate Cox regression analysis did not show an association and prognostic feature of vitamin D levels with events as expected probably due to the limited number of samples.

Conclusions

Hypovitaminosis D is common in patients with DCM. Given that lower levels of vitamin D are noted among patients with MVA, HT, HF hospitalization and poor outcome, future studies with a larger cohort are needed to confirm its predictive value. Vitamin D supplementation should be highly recommended as part of preventive therapies in patients with DCM.
P294 BIOPROSTHETIC AORTIC VALVE REPLACEMENT IN PATIENTS AT INCREASED RISK OF EARLY STRUCTURAL VALVE DEGENERATION
Authors: Paduvakis I , Cerillo A , Petrini F , Manca A
Published: May 2023

Background

Biological prostheses are increasingly been used in young patients to avoid anticoagulation. The Edwards Inspiris aortic bioprosthesis is extremely promising in this population, since it has shown reduced calcification of the prosthetic leaflets in preclinical and early clinical studies. Little data are available, however, in patients younger than 65. This study investigates the hemodynamic performance and clinical outcome of the Inspiris bioprosthesis in patients aged 56 ± 10.7 years.

Methods and Results

Between may 2018 and December 2022, 342 patients aged 56 ± 10.7 years underwent AVR with the Edwards Inspiris bioprosthesis at our institution. 37 of them (10.8%) were younger than 40, and 98 (28.6%) had a BMI >30 kgm2. A minimally invasive approach was used in all isolated AVR patients. Associated procedures were performed in 157 (45.9%) patients. There were 56 redo patients (16.4%). 7 patients died (2%). Major complications included bleeding (32), respiratory failure (20) and BAV requiring PMK (4). The mean follow up was 23 ± 15.2 months (range 2–55). Late valve–related events included endocarditis (5) requiring reoperation. There were no thromboembolic events, valve thrombosis or SVD cases. At follow up, all except four patients reported a significant improvement of their symptomatic status (98.8% were in NYHA II) and quality of life.

Conclusion

Our study demonstrates excellent early haemodynamics and promising results at up to four years in a cohort of very young patients. Further studies with longer follow–up are needed.
P301 TAVI IN PATIENT SUFFERING FROM NIEMANN – PICK DISEASE (ACID SPHINGOMYELINASE DEFICIENCY) WITH CONCOMITANT SITUS INVERSUS AND DEXTROCARDIA
Authors: De Feo D , D’Anzi A , Caragnano V , Pestrichella V , Scialpi A , Laronga G , Lafranceschina C , Tiecco F , Scicchitano P , Ciccone M , Iliceto S
Published: May 2023

Abstract Acid sphingomyelinase deficiency (ASMD) – also known as Niemann–Pick (NP) disease – is a rare, autosomal recessive disorder which is characterized by deficiency of the lysosomal enzyme acid sphingomyelinase (ASM), thus resulting in excessive storage of lipids in organs (i.e. spleen, liver, lung, bone marrow, lymph nodes, and vascular system). Literature provided few cases of moderate–to–severe valvular heart disease due to ASMD, mostly in adulthood. We reported the case of a patient with B–subtype NP disease which was diagnosed during his adulthood. NP disease was associated to situs inversus. Specifically, a severe, symptomatic aortic stenosis was also identified and the need for surgical or percutaneous intervention was deemed. The heart team chose Trans Aortic Valvular Implantation (TAVI) which was successfully performed with no further complications at follow–up.
P303 ECMO–PROTECTED HIGH RISK PCI IN UNUSUAL CLINICAL AND ANATOMICAL SCENARIO
Authors: Rossi S , Perfetti M , Mantini C , Cicchitti V , Zimarino M
Published: May 2023

Background

Non–emergent complex high–risk indicated procedure (CHIP) identify complex PCI according with clinical, anatomical and procedural features and prevent intraprocedural hemodynamic compromise represents a major issue. The aortic valve bypass (AVB) has been an uncommon surgical option performed by creating a heterotopic second left ventricular output bypassing native stenotic aortic valve with flow redistribution between the native left ventricular outflow tract and the apical–aortic bypass. AVB was phased out in “TAVI–era”.

Case Report

A 78 years–old obese and hypertensive male, with pulmonary fibrosis and previous AVB for severe aortic stenosis judged at unacceptable high surgical risk 10 years before, was admitted to the emergency department for chest pain and palpitation. Patient was in Killip class I. ECG showed rapid atrial fibrillation without ST–segment elevation. Atrial tachyarrhythmia, chest pain and palpitation spontaneously simultaneously resolved. A type 2 NSTEMI–ACS was suspected according to clinical features and pathological Hs–troponin. Multimodality imaging documented normal systolic function, normally operating AVB, paradoxical low flow severe native aortic valve stenosis, a chronic type B aortic dissection from distal anastomosis of AVB retrogradely extended to left subclavian artery origin and a wide aneurysm of the abdominal aorta (Fig. 1). Coronary angiography documented severely calcific multivessel disease. Prompt and complete revascularization was considered mandatory and a surgical redo was discarded. The use of an intraprocedural hemodynamic support was advisable, but anatomical context represented substantial contraindication to IABP and Impella. Heart Team decided to perform VA–ECMO–protected–PCI. After lesion preparation double kissing crush was performed on LMCA and single stents were implanted in proximal LAD and proximal RCA (Fig. 2). Despite signs of transient hemodynamic instability the patient tolerated the procedure thanks to the ECMO support. Complete myocardial revascularization was obtained without complications and the subsequent clinical course was uneventful.

Discussion

In this CHIP patient, hemodynamic support was vital to allow a safe complete myocardial revascularization. ECMO is a demanding system, not routinely used for “protected PCI”; however, the case here reported underlines that ECMO can be a feasible option, prerequisite in uncommon cases where other standard tMCS are contraindicated.
P308 REAL–WORD MONOCENTRIC EXPERIENCE OF DRUG ELUTING BALLOON PERFORMANCE
Authors: Venturi G , Bacchion F , Mugnolo A , Zamboni A
Published: May 2023

Introduction

Recently, drug eluting balloon (DEB) angioplasty has gained increased interested for both in stent restenosis lesions and de novo coronary lesions.

Objectives

This study aimed to evaluate performance of DEB in a single center real world experience and to assess predictors of outcome after DEB angioplasty.

Methods

We retrospectively evaluated patients undergoing angioplasty with drug coated balloon in the Cath Lab of Mater Salutis Hospital (Legnago). Clinical and angiographic characteristics of the groups were analyzed. The primary endpoint was a composite of all–cause mortality, any coronary revascularization and acute coronary syndromes assessed as per patients analysis at last available follow–up. Secondary endpoint was evaluation of target lesion failure (TLR) in a per lesion analysis.

Results

Between January 2018 and September 2022, 99 patients received angioplasty with DEB implantation at the Mater Salutis CathLab, Legnago, accounting for a total of 103 procedures and 112 lesions. Most patients were male (83,8%) and mean age was 66,73±9,5 years. Among 112 lesions treated, 63,4% were in stent restenosis lesions while 36,6% were de novo lesions. DEB used for de novo lesions were significantly smaller than ones used for in stent restenosis (2,23±0,30 mm vs 3,01±0,52 mm; p value<0.001). 19 patients were lost at follow up soon after discharge. Among the 81 patients remaining, with a median of 24 months (IQ1–3 6–37), composite endpoint occurred in 15 patients (18,5%) leading to an overall events–free survival of 81,5%. TLF occurred in 4 out of 99 lesions avalaible at clinical follow–up (4.3%), with a TLF free–survival of 95.7%. In all 4 failure cases, DEB were used for in stent restenosis. Accordingly, TLF free–survival for de novo lesions was 100%.

Conclusion

In our real–world monocentric experience, patients undergoing DEB angioplasty had good cardiovascular events free survival. Of note, DEB performance proved excellent outcome in de novo coronary artery lesions.
P315 GENETIC RESISTENCE TO CLOPIDOGREL AND COMPLEX PCI: A CASE REPORT
Authors: Ossola P , Ciampi C , Grieco N , Di Matteo I , Emanuela P , Giannattasio C , Oliva F
Published: May 2023

A 35–year–old man presents to the attention of the cardiology department for anterior STEMI. On history he reports hypertension, atrial fibrillation with CHA2DS2–VASc 2 on Rivaroxaban therapy, a previous PCI in the anterior interventricular artery, and a known genetic resistance to Clopidogrel (CYP2C19*2 mutation in heterozygosity) that emerged during diagnostic investigations for marked hyperhomocysteinemia (MTHFR A1298C variant in heterozygosity). Coronary angiography showed endoluminal thrombosis at the ostium of the anterior descending artery and an intrastent thrombosis. IVUS analysis, moreover, shows underexpansion and a concomitant protrusion of the stent at the ostium of the coronary. Thus, angioplasty with positioning of a drug eluting stent was performed. Antithrombotic therapy in these patients is usually triple therapy with ASA, clopidogrel and an anticoagulant. In this case the management of therapy was guided by the patient‘s genetic. Therefore, considering the low hemorrhagic risk and the high thrombotic risk, it was decided to undertake combination therapy with Dabigatran 150mg and Ticagrelor 90 BID in accordance with the Re–Dual PCI treatment trial. Thromboembolic events affect high mortality and morbidity, especially in the 30 days following the angioplastiy procedure. There is a part of the population that may have a poor response to the drugs used and most frequently to Clopidogrel. Genetic polymorphisms present in the coding regions for cytochrome CYP2C19 are involved in the altered metabolism and activation of the drug. The "genetic guided DAPT" used in the POPular Genetics study, was not inferior to Ticagrelor/Prasugrel therapy with regard to thrombotic events but showed a significant reduction in minor bleeding. Since long–term mortality post PCI is predominantly related to the occurrence of hemorrhagic rather than thrombotic events, the reduction of bleeding is very valuable in clinical practice. This case emphasizes the importance for the possible interindividual genetic variability and the most personalized approach possible in treatment choices. The feasibility in terms of cost and events saved of introducing "genetic–guided" DAPT into everyday clinical practice remains to be defined.
P323 WELLENS PATTERN, MYOCARDIAL BRIDGING, AND BIVENTRICULAR ARRHYTHMOGENIC CARDIOMYOPATHY: A SINGLE "TRIPLE" DIAGNOSIS
Authors: Ossola P , Bassi I , Pirola R , Gallone G , Occhi L , Milani M , Spanò F , Regazzetti M , Pedrotti P , Quattrocchi G , Soriano F , Giannattasio C
Published: May 2023

Wellens‘ syndrome describes an electrocardiographic pattern characterized by T–wave changes in the antero–septal leads that make this finding highly suggestive of a lesion of the proximal segment of the anterior interventricular artery. Case report. A 58–year–old patient from China with a silent medical history presented to the emergency department with angor, dizziness, asthenia, and blurred vision. The electrocardiogram shows a Wellens type A pattern, and blood chemistry tests collected in the emergency department show liver, kidney function and blood count within normal limits. High–sensitivity troponin T values stand at 34 ng/mL. The echocardiogram shows preserved biventricular systolic function in the absence of major regional wall motion abnormality. Given the symptoms, troponin values, and electrocardiographic pattern, the patient underwent coronary angiopgraphy, which showed lesion–free epicardial coronary arteries but with a muscular bridge in the mid–proximal section of the anterior interventricular artery, with no obvious significant systolic–diastolic caliber changes at subsequent coronary CT analysis. To better assess possible myocardial ischemic damage, the patient underwent MRI (magnetic resonance), which showed an entirely unexpected result. In fact, T1 sequences revealed biventricular adipose infiltration at the anterolateral and inferolateral walls of the left ventricle, with homosedial enhancement to nonischemic pattern on late post–contrast images: this picture was considered suggestive of biventricular arrhythmogenic cardiomyopathy. In the absence of history of syncope and documented arrhythmias on prolonged rhythm monitoring during hospitalization, no indication was made for further diagnostic and therapeutic investigations from the arrhythmological point of view. Thus, beta blocker and ace inhibitor therapy was set, and the patient was started on an outpatient cardiology follow–up course and genetic counseling for him and first–degree family members was scheduled. Selecting the correct imaging method becomes crucial for a correct diagnosis, especially in cases in which symptomatology is inconclusive.
P325 CARDIAC SARCOIDOSIS PRESENTING WITH ATRIOVENTRICULAR BLOCK AND SUBSEQUENT CARDIAC ARREST: A CASE REPORT
Authors: Caldarella Y , Foroni M , Spagnolo P , Brunello G , Migliore F , Sarais C , Perazzolo Marra M , Iliceto S , Rigato I
Published: May 2023

A 53–year–old male patient with no significant past medical history and no familiar history for cardiac diseases presented to the emergency department for asymptomatic bradycardia. The ECG showed complete atrioventricular block in the absence of pathological findings on the echocardiogram and no coronary lesions were found on coronary angiography. The patient underwent pacemaker (PM) implantation. A few months later the patient suffered from cardiac arrest in sustained ventricular tachycardia, effectively treated with DC shock. The echocardiogram showed mild right ventricular dilation and dysfunction with regional wall motion abnormalities, especially in the subtricuspid site. Cardiac magnetic resonance imaging (MRI) showed focal areas of delayed enhancement with a non–ischaemic pattern and distribution with a hook sign, highly suggestive of cardiac sarcoidosis. FDG PET showed cardiac, hepatic, splenic, and lymph nodal uptake. In the suspicion of systemic sarcoidosis, an hepatic biopsy was performed, being the liver the most accessible site. The histological examination showed the presence of non–caseating granulomas. Therefore, a diagnosis of Systemic Sarcoidosis (SS) with Cardiac involvement was made and the PM was upgraded to CRT–D in secondary prevention. The patient was discharged with cortisone therapy. Three months after discharge, a new total body PET–CT scan was performed and showed, in comparison to the previous one, the absence of areas of significant hypermetabolism in the cardiac, hepatic and bone fields. Cardiac sarcoidosis (CS) is a rare inflammatory granulomatous myocardial disease of unknown etiology that should be suspected in young patients with atrioventricular conduction disturbances and complex ventricular arrhythmias. The diagnostic approach is challenging: imaging techniques (MRI, PET) can be suggestive of CS but biopsy with histological demonstration of the non–caseous granuloma is mandatory. Endomyocardial biopsy (which has suboptimal sensitivity due to focal lesion) could be avoided if there is a more accessible and less invasive extracardiac site for biopsy. Early diagnosis relies on multidisciplinary collaboration and is essential for staging and long–term management of the disease and prevention of sudden cardiac death. Cortisone therapy remains the mainstay of treatment.
P333 A RARE CAUSE OF SHOCK: AORTO–PULMONARY FISTULA AS AORTIC ANEURYSM COMPLICATION
Authors: Ferraro I , Baralis I , Coletta G , Rossini R
Published: May 2023

Clinical case

A 69–year–old man with right shoulder discomfort, malaise for one hour and hemodynamic instability was admitted to our Emergency Department (ED). The patient had a history of arterial hypertension, diabetes, renal failure on peritoneal dialysis and ischemic cardiomyopathy with preserved ejection fraction treated by angioplasty and stent implantation on right coronary. At first medical contact out of hospital the patient appeared confused with severe hypotension (BP 70/40 mmHg). ECG transmitted to our Cardiology Unit showed sinus tachycardia with repolarization abnormalities consistent with diffuse ischemia. The patient was centralized with clinical suspicion of acute coronary syndrome complicated by cardiogenic shock. In ED, BP was 65/35 mmHg. On physical examination extremities were cold and mottled, crackles in the lower lung zones and turgid jugulars were present. Pulses were weak but present. ECG was repeated and no evolutive signs were detected. Echocardiography finding were: not dilated left ventricle with global systolic function at the lower limits, moderate aortic insufficiency, right ventricle with dimensions at the upper limits and hypocontractile, moderate tricuspid regurgitation, inferior vena cava dilated with preserved inspiratory collapse. Aortic root was non dilatated but aortic arch was 55 mm with no images of dissection flap. At blood test lactates were 4,2 with metabolic acidosis, troponin was just above the upper limit, inflammation indices were negative and electrolytes were in range. Vasopressor support with norepinephrine was started. With the high suspicion of acute aortic disease, CT angiography was performed showing a voluminous aneurysm of the aortic arch of 70x56 mm rupturing into the left pulmonary artery (Figure 1,2). Concomitant bilateral iliac artery dissection was detected. Cardiac and Vascular Surgeons were promptly alerted to plan tan emergent surgical intervention whose complexity was increased by the difficult vascular cannulation for iliac dissection. However, the patient‘s hemodynamic rapidly deteriorated requiring intubation and, after subsequent episodes of cardiac arrest in pulseless electrical activity (PEA), his death was assessed.
P340 REMOTE MONITORING IN HEART FAILURE: EFFECTIVE TOOL TO REDUCE HOSPITALIZATIONS AND MORTALITY
Authors: Iulianella R , Perna S , Schirripa V , Sciahbasi A , Chialastri C , Rizzo V , Scicchitano M , Loricchio M , Granatelli A
Published: May 2023

Introduction

The prevalence of heart failure in the general population in the idustrialized countries is estimated to be around 2%, a prevalence that increases with age until it reaches 10% at 70 aa of age. The disease is burdened by a high mortality rate and high expense related mainly to repeated hospitalizations. Patients with low ejection fraction heart failure are often implanted with devices, which have the ability to be monitored remotely. The Ministry of Health‘s 2017 annual report on hospitalization activity shows dramatic data: 60% of patients with heart failure are re–hospitalized within one year of discharge and in 14% of cases even within one month of discharge with an annual mortality rate of 18%. The aim of our study is to investigate whether remote monitoring allows improving the outcome of patients with heart failure in terms of reduction of hospitalizations and reduction of all–cause mortality.

Materials and Methods

In the year 2021, 241 patients with heart failure and reduced ejection fraction, implanted with devices of mean age 73 yr (+/– 11 yr) were remotely monitored in our center; Other population data: see Table 1. On the occasion of transmissions presenting with alarm events, our operational flow chart provides: first telephone contact and possible subsequent outpatient follow–up within 24 h working hours.

Results

During the year 2021, all–cause mortality in our population was 10.7% (26 patients), while re–hospitalizations were 70 (28.8%). As a result of alarms reported to remote monitoring, 83 patients were contacted whose therapy was changed or device reprogrammed. The causes of alarms were in order: reduced thoracic impedance, arrhythmic events, and reduced biventricular pacing rate.

Conclusions

The use of remote monitoring in heart failure patients and device wearers allows early and timely diagnosis of clinical changes that anticipate heart failure exacerbation and active intervention by modifying the clinical course, often avoiding hospitalization, with a significant social and economic impact. Thanks to the operational organization of our center, the management of remote monitoring has allowed a low rate of hospitalization and low mortality year compared with literature data.
P353 A CASE OF FABRY DISEASE WITH ISOLATED CARDIAC INVOLVEMENT
Authors: Magnano R , Pezzi L , Verrengia E , D‘Alleva A , Fulgenzi F , Corazzini A , Vitulli P , Genovesi E , Forlani D , Di Marco M , Paloscia L
Published: May 2023

A 64–year–old patient,hypertensive,ex–smoker,no family history of CAD or MCI,with electrocardiographic abnormalities during checkups for sports activity at age 40,came to our observation for reported brief episodes of heart palpitation.On ecg sinus rhythm,PR 200 msec,elevated QRS voltages,negative T waves from V3–V6 and infero–laterally,isolated BEV. Echocardiogram showed asymmetric left ventricular hypertrophy with involvement of septum and mid–apical segments,absence of obstruction,grade II diastolic dysfunction,enlarged left atrium,ectasia of aortic bulb,ascending aorta and arch with mild valvular insufficiency. Cardiac MRI showed asymmetric hypertrophy with prevalent involvement of the SIV and apex,normal systolic function.Right ventricle within limits.LAM elongated,biatrial dilatation.Areas of enhancement with intramural extension to the inferior SIV and extensive subendocardial area to the infero–lateral wall,picture suggestive of Fabry disease. Holter ecg was performed:RS at mean fc 61 bpm,numerous isolated BEVs and pairs,1 run of TVNS of 11 beats,for which dual–chamber ICD implantation was performed in primary prevention.Genetic examination showed absence of alpha galactosidase enzyme activity and mutation in exon 5 of GLA gene(p.N215S). The patient had no other systemic symptoms typical of Fabry‘s disease such as CRI,acroparesthesias,angiokeratomas,cornea verticillata,gastrointestinal disturbances nor central neurological involvement.In addition,he was starting enzyme replacement therapy with alpha galactosidase on a home regimen.Hypertrophic cardiomyopathy is a frequent finding in clinical practice.In MF, the age of cardiac involvement is usually later than in other forms of cardiomyopathy. Cardiac MRI is the most appropriate technique to place a correct differential diagnosis;numerous studies have demonstrated preferential localization of areas of "delayed enhancement" at the infero–lateral and posterolateral basal wall,present even in the absence of frank ventricular hypertrophy. Systemic involvement, may help in the differential diagnosis because of the presence of characteristic symptoms.In the reported clinical case,however,the diagnosis is even more insidious since in late forms or cardiac variants of MF,as with the p.N215S gene variant,cardiomyopathy may be the only significant clinical manifestation.
P356 MARCHE REGIONAL NETWORK FOR CARDIAC AMYLOIDOSIS AND HYPERTOPHIC CARDIOMYOPATHY: INSIGHTS FROM 3–YEAR COLLABORATIVE EXPERIENCE
Authors: Vagnarelli F , Lofiego C , Terzi F , Romandini A , Contadini D , Olivieri R , Falchetti E , Lanari A , Caffarelli F , Scipione P , Viozzi V , D‘Agostino S , Tullio M , Matarrese A , Flori M , Capodaglio I , Patani F , Tofoni P , Lombardi G , Pietrucci F , Giorgetti R , Onofri V , Tortora C , Sgattoni C , Dello Russo A , Tarsi G , Perna G
Published: May 2023

Background and Methods

The management of patients with Cardiac Amyloidosis (CA) and Hypertrophic Cardiomyopathy (HCM) is complex and requires specific skills. Collaboration between centres remains essential because not all centres can perform complex diagnostic techniques (such as endomyocardial biopsy and mass spectrometry) or prescribe disease–modifying therapies, Therefore, in 2019 we created a collaborative network for diagnosis and treatment of CA (TTR and AL) and HCM (sarcomeric and phenocopies) with the aim of enhancing disease awareness among physicians and favoring appropriate access to innovative diagnostic tools/therapies. The network includes all 14 Cardiology Departments and outpatient clinics from 5 Local Health Units. At Referral Centre of the Regional University Hospital, all diagnostic and therapeutic tools necessary for a comprehensive management of CM patients including genetic testing, endomyocardial biopsy, ablation of complex ventricular arrhythmias, and septal myectomy for HOCM, are currently available and at disposal for the entire network.

Results

Thanks to this network, from December 2019 to December 2022, 283 patients were referred to our Center (fig. 1). Of these, 136 (48%) had CA, 122 (43%) were diagnosed with HCM (68 with obstructive form and 54 with non–obstructive form), 12 (4%) with Anderson Fabry Disease (AFD) and 13 (5%) with other forms of cardiomyopathy (miscellanea: arrhythmogenic, LVNC, etc). As for temporal trends in diagnosis of CM patients, in the first 2 years the diagnosis of HCM was more frequent than that of CA, while the number of CA patients has steadily grown, overtaking that of HCM patients in the last year (fig.2). The number of AFD diagnoses was consistently low over the 3 years, despite the spread use of genetic testing in all HCM patients. All patients with CA (136) were managed according to current algorythm with need of EMB in uncertain cases (8). Of the 136 patients with AC, 122 had TTR while 14 had AL (Fig.3). All patients with ATTR (122) underwent genetic testing: in 14 cases the presence of mutations in the TTR gene was documented (8 Ile68Leu; 4 Val30Met; 2 Val122Ile). 51 ATTR patients were treated with tafamidis, while 3 patients with variant–TTR and neuropathy received patisiran iv.

Conclusions

Implementing specific clinical network provided excellent results, allowing a precise phenotype/genotype characterization and favoring appropriate access to specific disease–modifying drugs.
P364 LOW–DENSITY LIPOPROTEIN CHOLESTEROL REDUCTION IN PATIENTS AT VERY HIGH CARDIOVASCULAR RISK
Authors: Cherubini A , Tollardo M , Palcic S , Scagnetto A , Russo G , Capelletto C , Bollini M , Di Lenarda A
Published: May 2023

Background

Guidelines of treatment of hypercholesterolemia in patients at very high cardiovascular (CV) risk have progressively lowered the target LDL–cholesterol (LDL–C). In real life LDL–C targets are difficult to reach. Aim of this study was to evaluate the achievement of LDL–C targets and the prescription of lipid–lowering therapy (LLT) in a large cohort of outpatients at very high CV risk.

Methods

we considered 11350 patients at very high CV risk, mean age 70 years evaluated in Our Center from 2017 to 2020. The study population was divided in 3 groups according to the presence of one or more very high risk CV score components. For each group lipid–lowering therapy, LDL–C goal and prognosis were assessed.

Results

The very high CV risk population was composed maily by patients with coronary artery disease (CAD). We found an increse of LLT after the index cardiologic visit (from 50,5 to 75,8%) and high–efficacy (HE) LLT from 22% to 27,9%. Consequently we found an increase in the achievement of LDL–C target <55 mg/dl (1.4 mmol/l): from 3,2% to 8,3% 6 months after the visit. In the group with more CV risk components the LLT and HE LLT were more prescribed, the LDL–C target was more reached and the mortality rate were higher (22,3% vs 8,4% in patients with only one CV risk component).

Conclusions

in the very high CV risk population the target of LDL–C is rarely reached. We identificated patients with more CV risk components with worse prognosis and need of HE LLT.
P365 MANAGEMENT OF DYSLIPIDAEMIA AND CARDIOVASCULAR RISK REDUCTION IN HIV PATIENTS
Authors: Gatto M , Cicalini S , Bellagamba R , Biava G , Antinori A
Published: May 2023

Background

At our dedicated cardiology outpatient clinic, patients with HIV infection are seen daily. This patient population undergoes routine haematochemical analyses including lipid assessment every six months.

Methods

Patients with HIV infection referred to the dedicated cardiology outpatient clinic in the period between March and September 2022 were retrospectively enrolled. For each of these patients, the medical history was collected and the global cardiovascular risk, lipid assessment (total cholesterol, LDL–C, HDL–C, triglycerides), hypolipidemic therapy (if any) and, in accordance with the most recent guidelines of the European Society of Cardiology, the achieved LDL target were assessed.

Results

200 patients with mean age 59±15 years with HIV infection on antiretroviral treatment since 20±11 years, 92% male, were enrolled. 58% of patients had hypertension, 46% vasculopathy, 44% smoking, 14% diabetes, 10% chronic ischaemic heart disease and 4% previous stroke/TIA. Among these patients, 30% had a very high cardiovascular risk, 42% a high cardiovascular risk, 22% a moderate cardiovascular risk and 6% a low cardiovascular risk. Only 60% was on hypolipidemic therapy (statin in 50 per cent of cases and statin+ezetimibe in 10 per cent). Patients with a very high cardiovascular risk had an average LDL–cholesterol of 119.2±45.5 mg/dl, patients with a high cardiovascular risk 122.4±43.6 and only 12% of them was found to meet the guideline LDL targets.

Conclusions

Assessment and management of cardiovascular risk is crucial in order to reduce mortality and morbidity. In some particular clinical settings such as for patients with chronic infectious diseases (e.g. HIV) more attention must be paid to the LDL–cholesterol target in relation to the individual cardiovascular risk, which is often high or very high. Correct therapeutic management of dyslipidaemia through the use of the fixed statin/ezetimibe combination and the most innovative hypolipidemic therapies such as bempedoic acid, PCSK9 inhibitors, and inclisiran is the keystone in order to achieve the target.
P366 FULMINANT MYOCARDITIS WITH SPONTANEOUS RESOLUTION AFTER COVID 19 AND ALLERGIC EPISODE: MIS–A OR ALLERGIC MYOCARDITIS?
Authors: D’ Elia S , Catapano D , Cimmino G , Pezzullo E , Alfieri R , Golino P , Loffredo F
Published: May 2023

Clinical case

23–year–old male. No medical history Not vaccinated for Sars–Cov2; Recent Sars–Cov2 infection (negative antigenic swab on 03/01/22); Pharyngodynia treated with ibuprofen + ciprofloxacin and amoxicillin ac clavulanic acid(suspended for purpuric lesions on hands) After 5 days from onset of symptoms, access to PS for loss of consciousness. Laboratory: ph 7.4; Lat 3.4 EKG: sinus tachycardia HR 126 bpm ECHOCARDIOGRAM: Non–dilated left ventricle, marked reduction in FE (15%) spontaneous echocontrast in ventricular cavity chest TC: hypodense tissue in anterior mediastinum; adenopathy in peribronchial site, distended gallbladder transfer to Vanvitelli Cardiac ICU Amine support with augmented DBT for hypotension and tachycardia LAB: Tni 2193; Nt proBNP 27796; PCR 26; PCT 4; crea 1,4, Lat 1 CATH DX + EMB after 24 hours EBM: …Mixed cellularity endomyocarditis, predominantly lymphocytic, presence of eosinophils… enrollment in the MYTHS study: placebo group (saline solution) after 10 day MRI:Normal biventricular systolic function. Tissue characterization images consistent with the presence of limited area of active myocardial damage and with nonischemic pattern involving the basal segment of the infero–lateral wall of the left ventricle.

Discussion

The case has been a source of considerable discussion to establish the etiology of myocarditis. Although with numerous doubts, after careful evaluation of the histologic picture, the case falls under possible MIS–A (MULTISYSTEM INFLAMMATORY SYNDROME in adult) Cardiac manifestations of MIS–A include myocarditis, pericarditis, and arrhythmias, which can rapidly lead to cardiogenic shock.
P372 PULMONARY EMBOLISM IN CARDIAC INTENSIVE CARE UNIT–REAL–WORLD EXPERIENCE OF A TERTIARY CENTER
Authors: Morosato M , Mager R , Morciano D , Ricchetti G , Eustachio A , Margonato A , Slavich M
Published: May 2023

Introduction

Pulmonary thromboembolism represents a therapeutic challenge for patients admitted to cardiology intensive care units, given the variety of clinical presentations and available treatment opportunities.

Methods

This retrospective, single–center study recruited patients hospitalized in cardiology intensive care units with a diagnosis of pulmonary thromboembolism between April 2010 and May 2022. For each hospitalized patient, Wells and PESI scores were calculated, the location of the PE was identified, and acute PE resolution at discharge was determined. Patients with cardiac arrest (CCA) and/or cardiogenic shock were identified, and RV longitudinal systolic function (TAPSE) and pulmonary arterial systolic pressure (PASP) were studied. Patients were then stratified based on their clinical risk of in–hospital or 30–day mortality (early mortality); acute phase and discharge treatments and patients who developed major bleeding complications were identified.

Results

A total of 148 patients were recruited, with a median age of 66 years and predominantly male (51.4%). In 6.4% of patients, in–hospital death occurred (from 0% in low–risk patients to 25% in high–risk patients), 12.2% developed cardiogenic shock and/or CCA. 25.0% of patients had an ongoing neoplasm. Systemic thrombolytic therapy and Sonos were the most effective in terms of mortality (p=0.015) and RV function at discharge in high–risk patients, although associated with a high risk of bleeding. Intermediate–low–risk patients treated with reperfusion therapy showed better RV function parameters, although the sample size did not allow for statistical significance.

Conclusions

The results obtained are in line with those of the literature with regard to epidemiology, total hospital stay, correlation between acute PE and neoplasms, in–hospital death and FU death. Furthermore, Sonos represents an effective alternative to full–dose systemic thrombolysis in high–risk patients who cannot undergo the latter. Finally, in intermediate–low risk patients, there may be a role for reperfusion therapy in selected patients with very low bleeding risk.
P374 THE BAD COUSIN OF PULMONARY EMBOLISM: PRIMARY PULMONARY ARTERY SARCOMA
Authors: Tempestini F , Tinti M , Natale E , Giuliani M , Colucci F , Putini R , Cianni R , Gabrielli D
Published: May 2023

A young man of 29 years presented to the Emergency Room reporting worsening dyspnea for about 2 weeks. He had never had previous cardiovascular disease nor had risk factors for them. Hemodynamics were stable, but arterial blood gases showed hypocapnic hypoxia. The ECG revealed a S1Q3T3 pattern, incomplete right bundle branch block and signs of right ventricular overload. D–dimer was 4493 ng/ml. CT pulmonary angiogram (CTPA) showed massive bilateral embolism with thrombotic material in the right ventricle (RV) and almost complete obliteration of pulmonary trunk and main pulmonary arteries; moreover, there were subpleural nodularities in upper pulmonary lobes. Echocardiogram showed a dilated and hypokinetic RV, moderate tricuspid regurgitation with a sPAP of 80 mmHg, thrombotic pulmonary trunk obliteration and floating thrombi adhered to the tricuspid valve. UFH therapy was started. After ten days, there was no improvement in clinical status, echocardiography and CTPA. So, we decided to perform mechanical thrombectomy. In addition to thrombotic material, fibrous tissue was extracted and histologically analyzed. At the end of the procedure, the patient became hemodynamically unstable (BP 80/40 mmHg and HR 130 bpm) with dyspnea and tachypnea. ABG showed mixed acidosis. Right ventricular function worsened due to high afterload for the persistence of thrombotic material in the pulmonary trunk, defining a picture of obstructive shock. We started fibrinolysis (with rtPA), enoximone and norepinephrine, but asystole occurred and the patient eventually died. The histological examination revealed that the mass was a pleomorphic rhabdomyosarcoma of the pulmonary artery. Primary pulmonary artery sarcoma is an extremely rare and histologically heterogeneous disease. It develops mainly from great pulmonary arteries and then it grows up in the lumen of vessels. It may involve pulmonary valve and RV and invade pulmonary parenchyma and loco–regional lymph nodes. Diagnosis is challenging, because it simulates pulmonary embolism in clinical, echocardiographic and radiological presentation. Suspicion may arise from lack of benefit from anticoagulant therapy. Nodular pulmonary infiltrates are typical. There are two surgical approaches: pneumectomy or endarterectomy (as palliation). Prognosis is poor: mean survival of 1,5 month without therapy and 10 months with pneumectomy. In addition to the progression of the disease, death can occur for obstructive shock, as in our patient.
P378 HEART FAILURE DURING ACUTE CORONARY SYNDROME AND THE LONG–TERM RISK OF INCIDENT CANCER. THE ABC–9* STUDY ON HEART DISEASE
Authors: Mahmoud H , Berton G , Cordiano R , Cavuto F , Merotto D , Menegon F , Bagato F , Dal Bo A , Mahmoud M , Sitta N
Published: May 2023

Introduction

Recent reports have suggested a higher risk of incident cancer among patients with pre–existing Heart failure (HF) in community–based cohorts.

Methods

To investigate the association between heart failure (HF) during hospitalization with acute coronary syndrome (ACS) and the very long–term cancer risk, we enrolled 572 patients admitted with ACS to 3 Italian hospitals and discharged alive and free from neoplasia. Patients were prospectively followed for 24 years or until death.

Results

All except for three patients completed the follow–up, representing 6440 person–years. Patients‘ mean age was 66 ± 12 years and 70% were males. Baseline clinical HF was diagnosed in 192 (34%) patients. During follow–up, 129 patients (23%) developed cancer; of them, 103 with no HF [27% of patients without HF] and 26 had baseline HF [14% of patients with HF]. The incidence rates for cancer were 18 and 21 per 1000 person–years for patients with and without baseline HF, respectively (p = 0.62). The risks for cancer associated with HF were (HR: 0.96; 95% CI: 0.62–1.47; p=0.85). Similar associations were observed among both genders and in patients younger than 75 years of age. Yet, in patients older than 75 years, the unadjusted risk was (HR: 0.30; 95% CI: 0.09–0.92; p=0.04). However, the fully adjusted risk was (HR: 0.39; 95% CI: 0.12–1.30; p=0.13). The unadjusted HRs for incident cancer were (HR: 0.88; 95% CI: 0.45–1.74; p=0.73) and (HR: 0.91; 95% CI: 0.48–1.71; p=0.77) for patients with HFrEF and HFpEF respectively. We observed a positive interaction between age and LVEF for the risk of cancer onset (HR: 1.002; 95% CI: 1.003 –1.004; p=0.02) in the unadjusted model. Results were the same in the fully adjusted model.

Conclusions

A lack of association between clinical HF at admission for ACS and the long–term cancer risk has been observed in this prospective study of unselected ACS patients. A positive independent interaction between age and LVEF has been also observed.