bluuebunny/crossref_metadata_2025 huggingface dataset:
186 RARE CASE OF A GIANT THYMIC CYST: A CHALLENGING DIFFERENTIAL DIAGNOSIS OF A PARACARDIAC CYSTIC LESION
Authors: Schiavo Maria Alessandra , Foroni Marco , Coccolo Fabio , Graziosi Maddalena , Attinà Domenico , Dolci Giampiero , Galiè Nazzareno
Published: December 2022

Background

Benign thymic cysts are a rare clinical entity, representing approximately 3% of all mediastinal masses. Thymic cysts can be congenital or acquired. We describe a case of a 58-year-old female with a giant benign thymic cyst which entered in differential diagnosis with a pericardial cyst and pericardial effusion.

Case presentation

A 58-year-old female was brought to our hospital due to new onset dyspnoea and fatigue. She had a history of a liquid mediastinal mass accidentally discovered 9 years before which was interpreted at that time as a loculated pericardial effusion (Fig. 1). An echocardiogram was performed which showed increased size of the known lesion without any signs of hemodynamic compromise (Fig. 2). A magnetic resonance showed a voluminous liquid lesion that originated from the anterior mediastinum, extending caudally to the diaphragm with maximum dimensions on the right hemithorax of 16×12×9 cm (antero-posteriorly, transversely and cranio-caudally respectively) and of 10×5×13 cm on the left hemithorax (Fig. 3). It was initially interpreted as a pericardial cyst. All the findings were then collegially reviewed and for the first time it was suspected of being a giant thymic cyst, especially due to its spatial distibution and growth over time; in particular the cranio-caudal growth in both the hemithoraxes suggested that it might have originated from the thymus gland and then, by force of gravity, extended downword bilaterally. The cyst was surgically removed using a unilateral thoracoscopic approach. Histological examination confirmed the thymic origin of the cyst and excluded malignancy. At the three month follow up, the patient presented no symptoms, and the echocardiogram was normal.

Discussion

The echocardiographic features of mediastinal cysts are not lesion-specific, so the origin and location of the cyst can be used for differential diagnosis. Computed tomography has the best spatial resolution, while the magnetic resonance has better capacity to differentiate the content of the cyst, especially when it is not a homogeneous liquid lesion. In our case, spatial distribution and, in particular, growth over nine years were crucial hints to suspect the thymic origin of the lesion. Indeed, the sole evaluation of the last imaging exam would have made the diagnosis more problematic because of its huge dimensions, which made the exact site of origin very challenging to identify.

Conclusion

Our case is a rare example of a giant thymic cyst which was extremely difficult to differentiate from other paracardiac cystic lesions and required integrated imaging and discussion by a team of experts of different specialties to make the correct diagnosis.
876 VENTRICULAR SEPTAL DEFECT AND RIGHT-SIDED INFECTIVE ENDOCARDITIS
Authors: Sforna Stefano , Del Papa Marco , Grikstaite Egle , Sardone Mariagrazia , Savino Ketty
Published: December 2022

Introduction

Right-sided infective endocarditis (IE) accounts for 5-10% of IE cases; it is most frequently associated with intravenous drug abuse but can also be observed in patients with cardiac implantable electronic devices, central venous catheters or congenital heart diseases.

Case report

A 34-year-old female, with unrepaired perimembranous ventricular septal defect (VSD) and right ventricle and tricuspid valve (TV) IE with lung septic embolism treated conservatively with antibiotic therapy 16 years ago, was admitted to our hospital for persistent fever and cough unsuccessfully treated with empiric antibiotic therapy. She denied intravenous drug abuse and recent surgical or dental procedures. Blood cultures resulted positive for multi sensitive Staphylococcus lugdunensis, so antibiotic therapy with Oxacillin and Gentamicin was started. Suspecting IE, transesophageal echo (TEE) was performed and showed, inside VSD, a small fluctuating vegetation and, along the right side of the interventricular septum, next to the VSD, a voluminous hypomobile vegetation; septal and anterolateral TV leaflets appeared thickened and infiltrated by vegetations floating into the right atrium; at color-Doppler moderate TV regurgitation. A whole-body computed tomography showed right lung abscess and right kidney septic infarct. Endocarditis Team, considering hemodynamic stability and inflammatory biomarkers and surveillance blood cultures negativization, decided for medical therapy with echocardiographic follow-up. TEE performed at the end of antibiotic therapy demonstrated complete disappearance of the above described vegetations and surgical VSD closure was scheduled.

Discussion

Patients with unoperated small VSDs and without volume overload signs usually remain asymptomatic and do not require surgery; nevertheless, a small percentage of them develops problems later in life and IE represents one of the main concerns. Even if unrepaired VSD is associated with increased risk of IE compared with the general population, antibiotic prophylaxis is still recommended only for high-risk patients undergoing at-risk dental procedures, while non-specific oral and cutaneous hygiene measures should always be applied. Latest ESC Guidelines for the management of adult congenital heart disease state that in patients with no significant left to right shunt, but a history of repeated episodes of IE, VSD closure should be considered (IIa, C); it's still matter of debate whether closure should be performed after the first episode of IE.

Conclusion

IE should always be suspected in patients with VSD and persistent fever. Optimal oral and skin hygiene are effective prevention measures, while prophylactic antibiotic therapy is indicated only in selected cases. Surgery should be considered after repeated episodes of IE.
114 SURVIVAL OF PATIENTS WITH PULMONARY ARTERIAL HYPERTENSION AND CHRONIC THROMBOEMBOLIC PULMONARY HYPERTENSION LISTED FOR LUNG TRANSPLANTATION
Authors: Magnani Ilenia , Manes Alessandra , Dolci Giampiero , Salvaterra Elena , Daddi Niccolò , Paganelli Gian Maria , Antonacci Filippo , Guarino Daniele , Dardi Fabio , Palazzini Massimiliano , Galiè Nazzareno , Manes Alessandra
Published: December 2022

Background

despite the approval of pulmonary arterial hypertension (PAH) specific therapies, bilateral lung transplantation (LT) remains the therapeutic option of choice for patients that fail to reach an adequate clinical and hemodynamic response on maximal medical therapy.

Methods

we describe the survival of transplant recipients (TR) patients in comparison to not-transplanted (NT) patients in a single tertiary PAH centre. We included patients with Group 1 PAH and non-operable CTEPH who were listed for lung transplantation at our Centre from January 1999 to February 2022. The survival of NT, TR and of all listed patients was evaluated starting from the date of listing (patients were censored as alive at the time of LT in the intention to treat analysis). The survival of TR was also evaluated starting from the date of the LT. Kaplan Meier curves and log rank test were used for survival analysis. Data are expressed as median (interquartile range).

Results

141 patients were included (80% had Group 1 PAH). Fifty-eight (41%) patients were transplanted, after a mean waiting time of 17 (5-36) months. Of the 83 NT patients 53 (38%) died while on the list and 30 (21%) were alive on the list up to February 2022. The survival of all patients since listing (intention to treat analysis) at 1, 3 and 5 years was 83%, 54%, 45% respectively. The survival of TR at 1, 3, 5 and 10 years since transplantation was 67%, 65%, 62% and 57% respectively. When comparing, NT and TR patients’ survival since listing, TR patients had a better survival than NT patients [survival at 1, 3 and 5 years of 88%, 72% and 66% vs 73%, 42% e 34% for TR and NT patients, respectively (p= 0.0002)].

Conclusions

our experience demonstrated that TR's survival at our center is in line with principal European national registries and confirmed that the risk of death in the first-year post-transplant is higher for patients with pulmonary hypertension than it is for other diseases which might require LT. Finally, our study showed that the prognosis of patients listed is extremely poor and that, despite biases in the comparison of non-randomized groups, survival of TR is better than the one of NT patients.
346 IMPACT OF EXERCISE-INDUCED PULMONARY HYPERTENSION ON THE RIGHT VENTRICLE AND WORSENING OF CARDIOVASCULAR RISK IN HIV PATIENTS
Authors: Ridolfi Lorenzo , Biondi Filippo , Morganti Riccardo , Fabiani Silvia , Forniti Arianna , Iapoce Riccardo , De Caterina Raffaele , Madonna Rosalinda
Published: December 2022

Background and Aim

Exercise-induced pulmonary hypertension (ExPH), due to impaired pulmonary vascular and right ventricular contractile reserve on effort,predicts clinical outcomes, such as all-cause mortality or cardiovascular (CV) hospitalizations in patients with dyspnea on effort. We investigated its prognostic significance in HIV patients at risk for Pulmonary Arterial Hypertension (PAH).

Methods

In 52 consecutive HIV patients with either low (n=47) or intermediate probability (n=5) of PH at rest, we evaluated at time 0 and after 2 years the prognostic determinants of CV risk: onset or progression of heart failure/syncope; worsening of functional class; functional performance at the 6-Minute Walking Test and at cardiopulmonary exercise test (CPET); right atrial area; and pericardial effusion. We assigned a severity score 1-3 to each prognostic determinant, derived an overall CV risk score, and its 0-2 years change. Patients were classified as either at low, intermediate or high probability of ExPH at Stress Echocardiography (ESE), while ExPH at CPET was defined as absence of PH at rest, reduced peak VO2, VE/VCO2 >30 at anaerobic threshold, reduced O2 pulse, and ΔVO2/ΔW <9 mL/min/W. We then correlated ExPH at time 0 with clinical worsening (risk score increase >20% after 2 years).

Results

Right ventricle (RV) systolic function was significantly reduced in patients with ExPH compared to those without ExPH at CPET, as well as in patients with intermediate/high compared to those with low probability of ExPH at ESE, who exhibited worse values of TAPSE and FAC (p<0.001 and p=0.01, respectively). A significant higher proportion of patients with ExPH (CPET) or with intermediate/high probability of ExPH (ESE) had higher sPAP (p<0.001), mPAP (p=0.004), higher TRV (p=0.006) as well as higher right atrial area (p<0.001) and indexed right atrial volume (p=0.004). Total pulmonary vascular resistance (expressed by the ratio between TRV and velocity-time integral at the level of right ventricular outflow tract) was higher in patients with ExPH as well as in patients with intermediate/high probability of ExPH (p<0.001). Patients with intermediate/high probability of ExPH at ESE showed a trend towards clinical worsening compared to those with low probability of ExPH, albeit not statistically significant (p=0.137). In patients with low probability of ExPH, none had >20% increased CV risk score after 2 years. We found an association between higher NT-proBNP and the presence or intermediate/high probability of ExPH after 2 years (p=0.048 at CPET, p=0.033 at ESE).

Conclusions

At short follow-up, ExPH is associated with a worsening trend in the CV risk score which, if confirmed after longer follow-up, could contribute to better risk stratification in HIV patients.
729 BIOMARKERS FOR EARLY DIAGNOSIS OF CARDIAC INVOLVEMENT IN SYSTEMIC SCLEROSIS
Authors: Tifi Priscilla , Pala Barbara , Tempestini Federica , Cristiano Ernesto , Bocci Alessandro , Rosato Edoardo , Salerno Gerardo , Romaniello Antonella
Published: December 2022

Introduction

Systemic Sclerosis (SSc) is an autoimmune disease characterized by microvascular damage, immune system dysregulation and fibrosis of skin and internal organs. It is reported that asymptomatic involvement of the heart was found in 70% of SSc patients. The role of biomarkers in the early detection of fibrosis and vascular damage in SSc is not yet completely defined. Serum levels of sST2, Galectin 3, ANP, IL6 are a potential marker of cardiac involvement. In patients with chronic heart failure elevated levels of galectin-3 (>17.8 ng/ml) are a marker of worse prognosis. Moreover, a previous study reported a possible role of galectin-3 as an independent predictor of all-cause and cardiovascular mortality in SSc.

Material and Methods

We enrolled 62 SSc patients (53 females, median age 52 years) who fulfilled inclusion criteria.

35 patients had limited cutaneous SSc and 27 diffuse cutaneous SSc.

30 Healthy Controls (HC) were also enrolled [20 females; median age 50 years].

All SSc patients had normal echocardiographic and cardiac MRI findings.

Results

In SSc patients, the median Galectin value was 16.7 ng/ml, significantly higher than HC median (10,7 ng/Ml).

sST2 and IL-6 median value was 30 ng/mL and 12,3 pg/mL respectively, which resulted significantly higher than the median value of HC patient (21,55 ng/mL and 4,5 pg/mL respectively).

Therefore, there was a significant difference in biomarkers levels in the SSc group compared with controls.

Conclusion

These data suggest that these biomarkers can help identify a subgroup of SSc patients, with normal echocardiographic and RMN findings, who are at risk of developing heart involvement. In fact, all patients are scheduled for yearly echocardiographic re-evaluation as ESC guidelines recommend for earlier detection of pulmonary arterial hypertension (PAH), a leading cause of death in systemic sclerosis (SSc).
330 INTRAVENOUS CANGRELOR INFUSION IN HIGH BLEEDING RISK PATIENTS UNDERGOING PERCUTANEOUS CORONARY INTERVENTION: PRELIMINARY RESULTS OF THE ICARUS REGISTRY
Authors: Benenati Stefano , Gragnano Felice , Scalamera Riccardo , Bertero Edoardo , Capolongo Antonio , Sio Vincenzo De , Musumeci Giuseppe , Annibali Gianmarco , Campagnuolo Salvatore , Galasso Gennaro , Silverio Angelo , Bellino Michele , Centore Mario , Menozzi Alberto , Caretta Giorgio , Rezzaghi Marco , Luca Leonardo De , Veneziano Francesco Antonio , Cirillo Plinio , Rosa Gennaro De , Calabrò Paolo , Porto Italo
Published: December 2022

Aims

To investigate the prevalence of high bleeding risk (HBR) criteria and compare clinical outcomes between HBR and non-HBR patients receiving cangrelor in the peri-percutaneous coronary intervention (PCI) phase.

Methods

Consecutive patients treated with cangrelor in 7 Italian institutions were retrospectively enrolled in the ICARUS (“Intravenous CAngrelor in high-bleeding Risk patients Undergoing percutaneouS coronary intervention”, NCT05505591) registry. HBR status was assessed according to the Academic Research Consortium (ARC-HBR) definition. The primary endpoint was net adverse clinical events (NACE), defined as a composite of cardiovascular death, myocardial infarction, stroke, definite or probable stent thrombosis and Bleeding Academic Research Consortium (BARC) 2, 3 or 5 bleeding, at 48 hours. Secondary endpoints were assessed at 48 hours and throughout the hospital stay.

Results

We enrolled 551 patients undergoing PCI with cangrelor between January 2019 and August 2022. HBR definition was met in 33% of cases. HBR patients were older (76±10 vs. 63±10 years, p<0.001), more frequently affected by peripheral arterial disease (16% vs. 8%, p=0.010), atrial fibrillation (24% vs. 2%, p<0.001), chronic kidney disease (35% vs. 3%, p<0.001), active cancer (7% vs. 0%, p<0.001) and heart failure (17% vs. 7%, p<0.001). At 48 hours, HBR patients had a significantly higher rate of NACE (13% vs. 6%, p=0.004), and BARC 2, 3 or 5 bleeding (12% vs. 4%, p=0.001). In the HBR group, the frequency of the primary endpoint increased proportionally to the HBR score. There was no significant difference with respect to other 48-hour and in-hospital endpoints.

Conclusions

Among consecutive patients treated with cangrelor in the peri-PCI phase, about 30% fulfilled the ARC-HBR definition. In the HBR subpopulation, the incidence of 48-hour adverse events was higher than in the non-HBR subgroup, primarily driven by a higher rate of clinically relevant and major bleeding.
744 LEFT ATRIAL DISSECTION AFTER MITRACLIP IMPLANTATION: ECHOCARDIOGRAPHIC FEATURES
Authors: Carbone Andrea , Aquila Iolanda , Ielapi Jessica , Pasceri Eugenia , Chiarello Claudia , Mascaro Giuseppina , Mongiardo Annalisa , Indolfi Ciro
Published: December 2022

Introduction

the MitraClip system (MC) is the leading transcatheter technique to treat mitral regurgitation (MR), presenting very low adverse event rates. Left atrial dissection (LAD) is defined as a gap from left atrial wall.

Aim

we report a case of LAD occurring after MC implantation, treated conservatively, focusing on suggestive echocardiographic features.

Materials and methods

transthoracic and transesophageal echocardiography with 3D reconstructions were performed.

Results

the patient was a 73-years-old woman with symptomatic severe MR due to flail leaflet, treated with MC. Seven days later, the echocardiography showed a large mass in the left atrium not visible previously, initially thought to be a thrombus. Further investigation using transoesophageal echocardiography showed a large formation, with uneven echogenicity, at the level of the left atrium without signs of obstructing mitral valve inflow. 3D-rendering was also performed, revealing an echolucent area between the endocardium and the left atrial free wall, presenting increase echogenicity portions, suggestive of being LAD with partial thrombosis inside. The patient was treated with unfractionated heparin and after six days the echocardiogram showed complete resolution of thrombotic elements, with residual double-track linear hyperechoic formation within the atrial posterior wall, identifying an anechoic space. Therefore, the echocardiographic features suggested the diagnosis of left atrial intramural hematoma, initially thrombosed, then dissolved.

Conclusions

LAD is a rare complication either of cardiosurgical and transcatheter procedures. Both operator awareness and imaging features are important for diagnosis. 3D rendering can be useful in the early differential diagnosis, facilitating an optimal management.
939 PROGNOSTIC IMPACT OF THE COAPT INCLUSION AND EXCLUSION CRITERIA IN REAL WORLD PATIENTS WITH SECONDARY MITRAL REGURGITATION UNDERGOING MITRACLIP IMPLANTATION
Authors: Serratore Serena , Polimeni Alberto , Mongiardo Annalisa , Spaccarotella Carmen , Messina Chiara , Vizzari Francesca , Velato Letizia , Di Costanzo Assunta , Ciliberto Federica , Indolfi Ciro
Published: December 2022

Background

The COAPT randomized trial has shown a huge benefit in the survival of patients with heart failure and functional mitral regurgitation treated with Mitraclip. However, patients in COAPT trial were highly selected and the clinical course in real-world patients with and without fulfilment of the trial inclusion criteria is unclear.

Methods and Results

The present study examined the clinical outcome in consecutive patients (n=146) with symptomatic moderate-to-severe or severe MR of dominant functional etiology undergoing Mitraclip therapy by the presence of the inclusion criteria of the COAPT trial (left ventricular ejection fraction >20%, left ventricular end-systolic dimension <70 mm, non-commissural primary jet, estimated pulmonary artery systolic pressure <70 mmHg, mitral valve orifice area >4 cm2, no prior mitral valve leaflet surgery or any currently implanted prosthetic mitral valve or any prior transcatheter mitral valve procedure). The primary endpoint was the composite of all-cause mortality or heart failure hospitalization. Secondary endpoints were the single components of the primary endpoint (heart failure hospitalization, all-cause mortality) and cardiovascular mortality. Among 146 patients who underwent Mitraclip implantation 35.6% fulfilled the inclusion criteria of COAPT. The composite endpoint was significantly less frequent in patients fulfilling the COAPT selection criteria than in those not fulfilling the criteria [HR 0.3851 (0.2266 to 0.6642); p=0.0017] (Figure).

Conclusion

In this single center study the outcome of patients with functional mitral regurgitation undergoing Mitraclip therapy was significantly worse in patients not fulfilling COAPT inclusion criteria, indicating that these criteria might help identify futility.
841 ASSOCIATION BETWEEN CARDIORESPIRATORY SYMPTOMS AND LUNG FUNCTION IN PATIENTS WITH LONG-COVID SIGNS
Authors: Salvioni Elisabetta , Mapelli Massimo , Mattavelli Irene , Banfi Cristina , De Martino Fabiana , Vignati Carlo , Mancini Elisabetta , Harari Sergio , Rovai Sara , Tamborini Gloria , Agostoni Piergiuseppe
Published: December 2022

Background

Many patients recovered from COVID-19 infection present a variety of symptoms which limits overall quality of life, as reduced exercise performance, dysfunctional breathing, cough, dyspnea, weakness and anxiety. This condition has been named long COVID. The origin of this symptomatology is still unclear. This study has the aim to analyse the relation between symptoms and respiratory function, focusing on the alveolar capillary membrane.

Methods

Consecutive patients with long COVID 19 symptoms after 6 months were included. Patients underwent full clinical evaluation, laboratory tests, echocardiography, thoracic CT scan, spirometry including alveolar capillary membrane diffusion by means of combined carbon dioxide and nitric oxide lung diffusion (DLCO/DLNO) and cardiopulmonary exercise test. We measured surfactant derive protein B (immature form) as blood marker of alveolar capillary function. A questionnaire allowed to evaluate symptoms.

Results

We evaluated 204 post COVID-19 patients (age 56.5±14.5 y, 89 females (44%), BMI 25.7±4.0, 6% active smokers) referring to our hospital 171±85 days after the end of acute COVID-19 infection (Fig. 1). None of spirometry data was associated with long COVID 19 referred symptoms. SPB was not associated to differences in any of the referred symptoms. Subjects with lower capillary volume (VCap) have more frequently dyspnea, tiredness, fatigability and hair loss (Fig.2). CT scan lung damage correlated with SPB and membrane diffusion but not with VCap, exercise performance or VE/VCO2 slope.

The strongest correlation of SPB were with lung parenchyma damage and Vcap.

Conclusions

Our data suggest that a relevant reduction of alveolar capillary membrane function plays a central role in the long COVID cardiorespiratory symptoms.
465 LONG-TERM MAYOR ADVERSE CARDIOVASCULAR EVENTS AFTER COVID-19 INFECTION: A CLINICAL SCORE FROM A COHORT OF 2575 PATIENTS ENROLLED IN THE MULTICENTER INTERNATIONAL HOPE 2 REGISTRY
Authors: Santoro Francesco , Di Nunno Nicola , Centola Antonio , Cetera Rosa , Valeria Le Rose , Brunetti Natale Daniele
Published: December 2022

Background

Long-term consequences of COVID-19 infection are still partly known. According to some studies several patients may experience long term symptoms; however, predictors of long-term mayor adverse cardiovascular events among (MACE) patients with previous COVID-19 infection are unknown.

Aim of the study

To derive a simple clinical score for risk prediction of long-term MACE among patients with previous covid-19 infection.

Methods

2575 consecutive patients were enrolled in a multicenter, international registry (HOPE-2) from February 2020 to April 2021, and followed-up at long-term. A risk score was developed using a stepwise multivariable regression analysis.

Results

Out of 2575 patients enrolled in the HOPE-2 registry, 1481 (58%) were male, with mean age of 60±16 years. At long-term follow-up overall rate of MACE was 7.9% (202 of 2545 pts, 3.8% death, 1.3% coronary artery disease, 1.2% heart failure admission, 0.9% cardiac arrhythmias, 0.8% venous thrombosis, 0.7% cerebrovascular accident). After multivariable regression analysis, independent predictors of MACE were used to derive a simple prognostic score.

The HOPE-2 prognostic score may be calculated by giving: ½ point for every 10 years of age, 2 points for previous cardiovascular disease, 1 point for increased troponin serum levels during hospitalization, 2.5 points for heart failure and 3 points for sepsis during hospitalization, -1.5 points for vaccination at follow-up. Score accuracy at receiver operating characteristic curve analysis was 0.81.

Stratification into 3 risk groups (0-2, 3-5, and >5 points) classified into low, intermediate and high risk. The observed MACE rates were 0.5% for low-risk patients, 4% for intermediate-risk patients, and 19.5% for high-risk patients (log-Rank p<0.001).

Conclusions

The HOPE-2 prognostic score may be useful for long-term risk stratification in patients with previous COVID-19 infection. High-risk patients may require a strict cardiological follow-up.
835 CARDIOMYOPATHIES: IS IT USEFUL TO ANALYZE MINOR GENES?
Authors: Mazzaccara Cristina , Barretta Ferdinando , Uomo Fabiana , Caiazza Martina , Monda Emanuele , Lombardi Raffaella , Losi Maria Angela , Limongelli Giuseppe , D´argenio Valeria , Frisso Giulia
Published: December 2022

The diffusion of Next Generation Sequencing (NGS)-based approaches has allowed the identification of cardiomyopathies and channelopathies pathogenic mutations in more than 200 different genes. Since also genes considered uncommon for a clinical phenotype are now included in molecular testing, the detection rate of disease-causing variants is increased. Here, we report the prevalence of genetic variants detected in a cohort of 133 patients, among which 73 showed complex or borderline phenotypes or a positive family history for SCD. The analysis was conducted by analyzing an enlarged panel of 129 genes, including 60 main genes associated to the different genetic cardiomyopathies (HCM, DCM, ACM, LQTS, BrS) and 69 genes without a strong or definitive evidence of disease association according to the NIH-funded Clinical Genome Resource (ClinGen), here named “uncommon genes”.

We identified 82 variants, of which 50 (61%) were identified in uncommon genes. Among these, thirty-five (70%) were reported as variants of unknown significance (VUSs), thirteen (26%) as pathogenic (P) or likely pathogenic (LP) mutations and 2 (4%) as novel (likely) benign (B/LB) variants, according to the American College of Medical Genetics (ACMG) classification. Interestingly, about 85% of P/LP variants identified in uncommon genes were in patients showing complex/unclear/borderline phenotypes or a positive family history for SCD.

These data support the need for an extended genetic testing, including genes that are not usually explored due to a currently poor association with the clinical phenotype, particularly when phenotype is borderline, in order to increase the diagnostic sensitivity of inherited cardiomyopathies and channelopathies genetic testing.
269 EFFECTS OF COLCHICINE TREATMENT AFTER MYOCARDIAL INFARCTION ON TOP OF STANDARD THERAPY: EVIDENCE FROM A PRECLINICAL STUDY
Authors: Aimo Alberto , Burchielli Silvia , Aquaro Giovanni Donato , Barison Andrea , Musetti Veronica , Masotti Silvia , Pucci Angela , Galvez-monton Carolina , Martinez-falguera Daina , Passino Claudio , Emdin Michele , Bayes-genis Antoni
Published: December 2022

Background

An intense inflammatory and fibrotic response after myocardial infarction (MI) may lead to scar expansion and left ventricular (LV) remodeling, increasing the risk for ventricular arrhythmias and heart failure. Colchicine is an anti-inflammatory drug that has been reported to improve the outcome of patients with MI; its effects on LV remodeling have not been specifically investigated. We aimed to characterize the effects of the anti-inflammatory drug colchicine in an animal model of reperfused MI.

Methods

In 4-month-old pigs, MI was induced by occluding the LAD artery through an angioplasty balloon for 90 minutes. Pigs surviving MI induction were randomized in a 1:1 fashion to 2 arms:

The pigs underwent a first CMR scan 72 hours after MI induction, and another CMR at day 30. The pigs were then sacrificed the day after the second CMR. The primary efficacy endpoint was LGE mass (as a percentage of LV mass).

Results

The infarcted area at 72 hours (expressed as absolute mass or as a percentage of the LV mass) and the changes over 1 month did not differ significantly between pigs on colchicine plus standard therapy vs. those on standard therapy alone. When assessing the extent of fibrosis on the explanted hearts (as a percentage of the tissue sample areas) there was a trend toward a lower extent of myocardial fibrosis in pigs on colchicine plus standard therapy (p=0.091). The percent differences in LV volumes, ejection fraction and mass across the two CMR scans did not reach statistical difference.

Conclusions

The results from a pig model of reperfused MI suggest that colchicine has no additional effects to standard MI therapy on MI size and LV remodeling.
1134 IN-HOSPITAL ARRHYTHMIC BURDEN REDUCTION IN DIABETIC PATIENTS WITH ACUTE MYOCARDIAL INFARCTION TREATED WITH SGLT2-INHIBITORS: INSIGHTS FROM THE SGLT2-I AMI PROTECT STUDY
Authors: Cesaro Arturo , Gragnano Felice , Paolisso Pasquale , Bergamaschi Luca , Gallinoro Emanuele , Sardu Celestino , Mileva Niya , Foà Alberto , Armilotta Matteo , Sansonetti Angelo , Amicone Sara , Impellizzeri Andrea , Esposito Giuseppe , Morici Nuccia , Oreglia Andrea Jacopo , Casella Gianni , Mauro Ciro , Vassilev Dobrin , Galiè Nazzareno , Santulli Gaetano , Pizzi Carmine , Barbato Emanuele , Calabrò Paolo , Marfella Raffaele
Published: December 2022

Background

Sodium-glucose co-transporter 2 inhibitors (SGLT2-i) have shown significant cardiovascular benefits in patients with and without type 2 diabetes mellitus (T2DM). They have also gained interest for their potential anti-arrhythmic role and their ability to reduce the occurrence of atrial fibrillation (AF) and ventricular arrhythmias (VAs) in T2DM and heart failure patients.

Objectives

To investigate in-hospital new-onset cardiac arrhythmias in a cohort of T2DM patients presenting with acute myocardial infarction (AMI) treated with SGLT2-i vs. other oral anti-diabetic agents (non-SGLT2-i users).

Methods

Patients from the SGLT2-I AMI PROTECT registry (NCT05261867) were stratified according to the use of SGLT2-i before admission for AMI, divided into SGLT2-i users vs. non SGLT2-i users. In-hospital outcomes included the occurrence of in-hospital new-onset cardiac arrhythmias (NOCAs), defined as a composite of new-onset AF and sustained new-onset ventricular tachycardia (VT) and/or ventricular fibrillation (VF) during hospitalization.

Results

The study population comprised 646 AMI patients categorized into SGLT2-i users (111 patients) and non-SGLT2-i users (535 patients). SGLT2-I users had a lower rate of NOCAs compared with non-SGLT2-i users (6.3 vs.15.7%, p=0.010). Moreover, SGLT2-i was associated with a lower rate of AF and VT/VF considered individually (p = 0.032). In the multivariate logistic regression model, after adjusting for all confounding factors, the use of SGLT2-i was identified as an independent predictor of the lower occurrence of NOCAs (OR=0.35; 95%CI 0.14–0.86; p = 0.022). At multinomial logistic regression, after adjusting for potential confounders, SGLT2-I therapy remained an independent predictor of VT/VF occurrence (OR=0.20; 95%CI 0.04–0.97; p = 0.046) but not of AF occurrence.

Conclusions

In T2DM patients, the use of SGLT2-i was associated with a lower risk of new-onset arrhythmic events during hospitalization for AMI. In particular, the primary effect was expressed in the reduction of VAs. These findings emphasize the cardioprotective effects of SGLT2-i in the setting of AMI beyond glycemic control.

Trial registration: Data are part of the observational international registry: SGLT2-I AMI PROTECT. ClinicalTrials.gov Identifier: NCT05261867.
673 A CRACK AND A CLOT
Authors: Rochira Carla
Published: December 2022

A 74-year-old man with a history of ischaemic heart disease (multiple percutaneous coronary interventions (PCI)), broke his ankle. Therefore, he discontinued dual antiplatelet therapy (DAPT) -acetylsalicylic acid plus clopidogrel- and started enoxaparin sodium 4000 UI/day on medical advice. A few days later, he was admitted to the emergency department for refractory chest pain, vomit and syncope. Twelve-lead electrocardiogram (ECG) showed a sinus rhythm interrupted by frequent premature ventricular contractions in a pattern of trigeminy. The transthoracic echocardiogram revealed a mildly dilated LV with normal wall thickness and marked hypokinesis/ akinesis in apical, infero-lateral and antero-lateral walls, with a 33% ejection fraction. High sensitivity cardiac troponin was increased, and a second ECG showed a diffuse ST segment depression. The patient was loaded with aspirin and emergently taken to the cardiac catheterization lab. Coronary angiography showed a very late stent thrombosis of the left circumflex artery (LCx), with occlusion of the extreme distality of the second obtuse marginal artery and a 50-60% intrastent restenosis of the ostium-proximal tract of the left anterior descending artery (LAD). Given the non-obstructive nature of the thrombus, risk of peripheral embolization, TIMI flow grade 3 and improvement of symptoms, the decision was made to adopt a conservative strategy with loading dose of ticagrelor, heparin and tirofiban infusion. Besides, an optical coherence tomography (OCT) control was scheduled for the next day.

OCT of the LCx revealed a well apposed and well expanded stent with a non-obstructive residual thrombus, nevertheless not visible on angiography. Thus, the tirofiban infusion was prolonged up to 48 hours. OCT of the LAD detected a fibro-calcific disease determining a significant restenosis of the distal left main (LM)-LAD ostium. OCT-guided provisional stenting with two drug eluting stents on LM-LAD-LCx was performed successfully. The patient was discharged home on acetylsalicylic acid (lifelong), ticagrelor (for 12 months) and enoxaparin sodium 4000 UI/day (according to specialist indication).

DAPT is superior to anticoagulant therapy in preventing stent thrombosis in patients undergoing PCI, and a more informed inter-specialist communication is needed. Intracoronary imaging can help in the management of stent failure and in the guidance of complex PCI, allowing a patient-tailored treatment.
415 DOES MYOCARDIAL BRIDGING TRIGGER MYOCARDIAL ISCHEMIA?
Authors: Tremamunno Saverio , Pastena Paola , Cambise Nello , Telesca Alessandro , Tinti Lorenzo , Di Renzo Antonio , De Vita Antonio , Lamendola Priscilla , Lombardo Antonella , Crea Filippo , Lanza Gaetano Antonio
Published: December 2022

Background

Myocardial bridging (MB) is a congenital epicardial coronary abnormality, in which a segment of the artery runs through the myocardium, determining dynamic stenosis during systole. Whether MB can be responsible by itself for myocardial ischemia remains unclear. Thus, we aimed to evaluate whether the dynamic stenosis related to MB can cause signs and symptoms of myocardial ischemia.

Methods

We enrolled 41 consecutive patients who underwent coronary angiography because of chest pain suspected for ischemic heart disease and were found to have MB in the absence of any significant flow-limiting coronary stenosis and any other cardiac disease. A group of 14 patients who also underwent coronary angiography because of chest pain suspected for ischemic heart disease, but found to be free of any coronary or cardiac abnormality (including MB) and also showed negative results at exercise stress test (EST) served as controls.

All enrolled patients underwent a maximal treadmill EST according to standard Bruce protocol. A complete echocardiographic examination was performed at rest and at peak exercise.

Results

Baseline EST parameters were comparable between groups, whereas peak diastolic blood pressure (pDBP) was slightly higher in the MB group (p=0.044).

No clinically significant differences were found in baseline echocardiographic parameters, although the E wave deceleration time (DcT) was longer (p=0.038) and global longitudinal strain (GLS) was higher (p=0.05) in the MB group. During exercise, left ventricular ejection fraction (LVEF) improved significantly in both groups (p<0.001), without any difference between the 2 groups. GLS did not show any significant changes with exercise in both groups.

Moreover, among MB patients, no differences were found in stress echocardiographic results between those with positive vs. those with negative EST.

Conclusions

We failed to demonstrate any significant ischemic impairment of LV systolic and diastolic function at maximal EST in patients with MB. Moreover, we also failed to demonstrate any LV dysfunction in MB patients that developed ECG signs of myocardial ischemia during EST, as compared to those without evidence of myocardial ischemia. Thus, our data question about the ability of MB itself to trigger significant myocardial ischemia in clinical practice.
185 BETA-BLOCKERS OR CALCIUM CHANNEL BLOCKERS IN HYPERTROPHIC CARDIOMYOPATHY
Authors: Pinto Giuseppe , Chiarito Mauro , Bacher Anne , Puscas Tania , Condorelli Gianluigi , Reant Patricia , Donal Erwan , Hagege Albert
Published: December 2022

Background

Guidelines recommend betablockers (BB) as first line therapy in symptomatic patients with hypertrophic cardiomyopathy (HCM). A secondary role in the therapeutic algorithm is assigned to non-dihydropyridine calcium channel blockers (ND-CCB). Since no randomized comparison trials between the two drugs have been made so far, the aim of the present analysis was to evaluate the prognostic impact of BB and ND-CCB in a cohort of patients with HCM, either obstructive or not.

Methods

From a cohort of 1434 patients with a diagnosis of HCM included in the French register REMY, we retrospectively analysed 601 individuals treated with BB or ND-CCB. Patients taking both BB and ND-CCB or none, patients who already had an implantable cardioverter defibrillator or a pacemaker and who had already undergone a procedure of atrial fibrillation (AF) ablation or of septal reduction therapy were excluded.

Results

Out of 601 HCM patients, 545 (91%) were treated with BB and 56 (9%) with ND-CCB. At 8-year follow-up no differences were observed as regard the composite endpoint of cardiovascular death, hospitalization for heart failure and hospitalization for AF (133 [24%] vs. 9 [16%] for patients taking BB or ND-CCB respectively, HR 1.84, 95% CI 0.94–3.63). Patients taking ND-CCB were less symptomatic at long-term follow-up but experienced more drug interruptions or changes.

Conclusions

In a real-world cohort of patients with HCM, ND-CCB therapy was not associated with a higher incidence of adverse events compared to BB therapy.
1059 FAMILIAL RISK OF DILATED AND HYPERTROPHIC CARDIOMYOPATHY: A NATIONAL FAMILY STUDY IN SWEDEN
Authors: Ricci Fabrizio , Banihashemi Behzad , Pirouzifard Mirnabi , Sundquist Jan , Sundquist Kristina , Sutton Richard , Fedorowski Artur , Zoller Bengt
Published: December 2022

Aims

To determine the familial incidence of dilated (DCM) and hypertrophic cardiomyopathy (HCM) in first-, second- and third-degree relatives of affected individuals.

Methods and results

In this population-based multigenerational cohort study, full-siblings, half-siblings, and cousin pairs born to Swedish parents between 1932 and 2015 were included and register-based DCM and HCM diagnoses among relatives were ascertained. Adjusted odds ratios (ORs) for DCM and HCM were calculated for relatives of individuals with DCM and HCM compared with relatives of individuals without DCM and HCM for reference. Total study population included 6,334,979 subjects and consisted of 5,577,449 full-siblings, 1,321,414 half-siblings, and 3,952,137 cousins. Overall, 10,272 (0.16%) unique individuals were diagnosed with DCM and 3,769 (0.06%) with HCM. Of these, 7,716 (75.12%) and 2,375 (63.01%) were males, respectively. Familial risk odds ratios (ORs) for DCM were 5.35 (95% confidence intervals (CI): 4.85-5.90) for full-siblings, 2.68 (95%CI:1.86-3.87) for half-siblings, and 1.72 (95%CI:1.12-2.64) for cousins of affected individuals. The ORs for HCM were 42.44 (95%CI:37.66-47.82) for full-siblings, 32.70 (95%CI:21.32-50.15) for half-siblings, and 36.96 (95%CI:29.50-46.31) for cousins of affected individuals. In sex-stratified analysis, relatives of affected females were found more likely to be affected than were relatives of affected males, with stronger aggregation observed for HCM.

Conclusions

Familial risk of HCM and DCM is high and associated with genetic resemblance, with strongest aggregations observed in relatives of affected females with HCM, whereas this association was distinctly attenuated for DCM. The finding of a Carter effect, more pronounced in HCM, suggests a multifactorial threshold model of inheritance.
933 ASSESSMENT OF NON-INVASIVE MEASUREMENTS OF OXYGEN SATURATION AND HEART RATE WITH AN APPLE SMARTWATCH: COMPARISON WITH A STANDARD PULSE OXIMETER
Authors: Mancuso Cinzia , Spaccarotella Carmen , Polimeni Alberto , Pelaia Girolamo , Esposito Giovanni , Indolfi Ciro
Published: December 2022

Background

The most commonly used method to assess peripheral oxygen saturation (SpO2) in clinical practice is pulse oximetry. Smartwatches are widespread and are increasingly being used for digital health information. The smartwatch Apple Watch 6 was developed with a new sensor and an app that allows taking on-demand readings of blood oxygen and background readings, day and night.

Aims

The primary aim of the study was the head-to-head comparison of the measurements of SpO2 and heart rate (HR) by the smartwatch and the standard pulse oximeter. The secondary aim was the comparison of the measurements of SpO2 and HR between subgroups (lung disease, cardiovascular disease, healthy subjects).

Methods and results

We enrolled study participants with lung or cardiovascular disease and healthy subjects. A total of 265 subjects were screened for enrollment in this study. After screening, 257 subjects were included in the present study. The measurements of SpO2 and HR were obtained with the Apple Watch 6 and the Nellcor system and taken within 1 minute of each other in order to ensure comparability between the two devices. The correlation between the two technologies was assessed using linear regression and estimated with Pearson analysis for normally distributed data and Spearman analysis for nonparametric data. A plot of the differences between techniques was created according to the method described by J.M. Bland and D.G. Altmann. We observed a strong positive correlation between the smartwatch and the standard commercial device in the evaluation of SpO2 measurements (r=0.89, p< 0.0001; Figure 1A) and HR measurements (r=0.98, p< 0.0001; Figure 1B). A very good concordance was found between SpO2 (bias, -0.2289; SD, 1.66; lower limit, -3.49; and upper limit, 3.04; Figure 2A) and HR (bias, -0.1052; SD, 2.93; lower limit, -5.84; and upper limit, 5.63; Figure 2B) measured by the smartwatch in comparison with the standard commercial device using Bland–Altman analysis. We observed similar agreements and concordance even in the different subgroups.

Conclusions

In conclusion, our study demonstrates the feasibility and agreement of the Apple Watch 6 compared with the standard device used to assess SpO2 in patients with cardiovascular or lung diseases and in healthy subjects.
698 MONITORING OF CARDIOVASCULAR EVENTS IN COVID 19 POSITIVE SUBJECTS: THE BIOBEAT SYSTEM
Authors: Pecchioli Valerio , Lomartire Nazzareno , Valente Lucia , Gemmiti Maria Paola , Corsi Maria Pia , Pecchioli Lorenzo , Fedele Francesco
Published: December 2022

Background

Connected health technology can enable healthcare professionals to provide multiple solutions to meet the growing demand of care and control of COVID-19 infected patients, by optimizing economic resources and extending the scope of monitoring beyond the hospital. A connected patient has the ability to monitor his state of health with tools that remotely transmit data to his doctor, help him to perform the right therapy thanks to reminder systems or electronic alerts and identifies cardiovascular alterations predictive of a worsening of the disease.

An innovative mobile device, the BioBeat System® with an APP that can be downloaded directly to the Smart Phone, is characterized by sensors already validated according to the regulations of the Food and Drug Administration for vital signs, the European Society Hypertension for the detection of blood pressure.

Purpose

Wearable medical devices and the BioBeat software platform are appropriate for use in different settings for the management of acute, chronic cardiovascular, respiratory and inflammatory pathology, a tool capable of early identification of the instrumental signs of deterioration even before the exclusively clinical recognition that becomes even more difficult if we consider the patients at home.

The aim of this study is to test the sensitivity and specificity of the biobeat wearable system applied in patients with paucisymptomatic COVID-19 infection (group A) and in those with previous SARS-COV2 disease discharged from the hospital who still needed post-acute monitoring (group B) and compared with clinical control, managing to determine early the clinical signs of worsening.

Methods

The data recorded by individual patients are systolic and diastolic blood pressure, heart and respiratory rate, peripheral oxygen saturation and body temperature. Thanks to these parameters it is possible to calculate the NEWS by being able to predict any adverse events early with the automatic calculation of the score. The criteria of deterioration or worsening of the clinical condition are represented by the need to hospitalize the patient, alteration of one of the criteria grouped in the ABCDE.

Results

Table 1 summarises the demographic data of the cases enrolled. We calculated the sensitivity of the methodology related to the alerts detected and the outcome of the patients.

Sensitivity was 86.3% with a 95% CI of 0.71 to 1.03 and a specificity of 7.7%.

Conclusions

Continuous monitoring with biobeat watch showed a high sensitivity in detecting early any alerts predictive of worsening of the disease.
156 IMPACT OF TRANSCATHETER EDGE-TO-EDGE MITRAL VALVE REPAIR ON GUIDELINE DIRECTED MEDICAL THERAPY OPTIMIZATION IN PATIENT WITH SECONDARY MITRAL REGURGITATION
Authors: Pancaldi Edoardo
Published: December 2022

Background

Guideline-directed medical therapy (GDMT) optimization is mandatory before transcatheter edge-to-edge mitral valve repair (M-TEER) in patients with secondary mitral regurgitation (SMR) and heart failure with reduced ejection fraction (HFrEF). However, the effect of M-TEER on GDMT is unknown.

Objective

To evaluate frequency and prognostic implications and predictors of GDMT optimization after M-TEER in patients with SMR and HFrEF.

Methods

This is a retrospective analysis of prospectively collected data from the EuroSMR Registry. The primary events were all-cause death and the composite of all-cause death or HF hospitalization.

Results

Among the 1641 EuroSMR patients, 810 had full datasets regarding GDMT and were included in this study. GDMT optimization occurred in 307 (38%) patients after M-TEER. Proportion of patients receiving ACEi/ARB/ARNI, betablockers and MRA was 78%, 89% and 62% before M-TEER and 84%, 91% and 66% 6-month after M-TEER (All p<0.001). Patients with GDMT optimization had a lower risk of all-cause death (adjusted hazard ratio [HR] 0.62; 95% confidence interval [CI] 0.41-0.93; p=0.020) and the composite of all-cause death or HF hospitalization (adjusted HR 0.54; 95% CI 0.38-0.76; p<0.001) compared with those without. Degree of MR reduction between baseline and 6-month follow-up was an independent predictor of GDMT optimization after M-TEER (adjusted OR 1.71; 95% CI 1.08-2.71; p=0.022).

Conclusions

GDMT optimization after M-TEER in HFrEF patients with SMR occurred in a considerable proportion of patients and is independently associated with lower rates for mortality and HF hospitalizations. A greater decrease in MR was associated with increased likelihood for GDMT optimization.