bluuebunny/crossref_metadata_2025 huggingface dataset:
Occlusion of the infarct-related coronary artery presenting as acute coronary syndrome with and without ST-elevation: impact of inflammation and outcomes in a real-world prospective cohort
Authors: Bruno Francesco , Adjibodou Boris , Obeid Slayman , Kraler Simon C , Wenzl Florian A , Akhtar M Majid , Denegri Andrea , Roffi Marco , Muller Olivier , von Eckardstein Arnold , Räber Lorenz , Templin Christian , Lüscher Thomas F
Published: May 2023

Background

Patients with ST-segment elevation typically feature total coronary occlusion (TCO) of the infarct-related artery (IRA) on angiography, which may result in worse outcomes. Yet, relying solely on electrocardiogram (ECG) findings may be misleading and those presenting with non-ST-segment elevation acute coronary syndromes (NSTE-ACSs) may have TCO as well. Herein, we aimed to delineate clinical characteristics and outcomes of patients with ACS stratified by IRA location.

Methods

A total of 4787 ACS patients were prospectively recruited between 2009 and 2017 in SPUM-ACS (ClinicalTrials.gov Identifier: NCT01000701). The primary endpoint was major adverse cardiovascular events (MACEs), a composite of all-cause death, non-fatal myocardial infarction and non-fatal stroke at 1 year. Multivariable-adjusted survival models were fitted using backward selection.

Results

A total of 4412 ACS patients were included in this analysis, 56.0% (n = 2469) ST-elevation myocardial infarction (STEMI) and 44.0% (n = 1943) NSTE-ACS. The IRA was the right coronary artery (RCA) in 33.9% (n = 1494), the left-anterior descending coronary artery (LAD) in 45.6% (n = 2013), and the left circumflex (LCx) in 20.5% (n = 905) patients. In STEMI patients, TCO (defined as TIMI 0 flow at angiography) was observed in 55% of cases with LAD, in 63% with RCA, and in 55% with LCx. In those presenting with NSTE-ACS, TCO was more frequent in those with LCx and RCA as compared to the LAD (27 and 24%, respectively, vs. 9%, P < 0.001). Among patients with NSTE-ACS, occlusion of the LCx was associated with an increased risk of MACE during 1 year after the index ACS (fully adjusted hazard ratio 1.68, 95% confidence interval 1.10–2.59, P = 0.02; reference: RCA and LAD). Features of patients with NSTE-ACS associated with TCO of the IRA included elevated lymphocyte and neutrophil counts, higher levels of high-sensitivity C reactive protein (hs-CRP) and high-sensitivity cardiac troponin T, lower eGFR, and notably a negative history of MI.

Conclusion

In NSTE-ACS, both LCx and RCA involvement was associated with TCO at angiography despite the absence of ST-segment elevation. Involvement of the LCx, but not the LAD or RCA, as the IRA represented an independent predictor of MACE during 1-year follow-up. Hs-CRP, lymphocyte, and neutrophil counts were independent predictors of total IRA occlusion, suggesting a possible role of systemic inflammation in the detection of TCO irrespective of ECG presentation.
ANMCO position paper on the management of hypercholesterolaemia in patients with acute coronary syndrome
Authors: De Luca Leonardo , Riccio Carmine , Navazio Alessandro , Valente Serafina , Cipriani Manlio , Corda Marco , De Nardo Alfredo , Francese Giuseppina Maura , Napoletano Cosimo , Tizzani Emanuele , Roncon Loris , Caldarola Pasquale , Gulizia Michele Massimo , Gabrielli Domenico , Oliva Fabrizio , Colivicchi Furio
Published: May 2023

Patients suffering from acute coronary syndrome (ACS) present a high risk of recurrence and new adverse cardiovascular events after hospital discharge. Elevated plasma LDL-cholesterol (LDL-C) levels have been shown to be a causal factor for the development of coronary heart disease, and robust clinical evidence has documented that LDL-C levels decrease linearly correlates with a reduction in cardiovascular events. Recent studies have also demonstrated the safety and efficacy of an early and significant reduction in LDL-C levels in patients with ACS. In this position paper, Italian Association of Hospital Cardiologists proposes a decision algorithm on early adoption of lipid-lowering strategies at hospital discharge and short-term follow-up of patients with ACS, in the light of the multiple evidence generated in recent years on the treatment of hypercholesterolaemia and the available therapeutic options, considering current reimbursement criteria.
C9 THE EFFECT OF ANTHRACYCLINE CHEMOTHERAPY ON PEAK LEFT ATRIAL LONGITUDINAL STRAIN: A METANALYSIS
Authors: Faggiano A , Giangiacomi F , Gherbesi E , Carugo S , Vicenzi M
Published: May 2023

Background

Currently there is a limited number of studies reporting atrial function, evaluated by peak left atrial longitudinal strain (PALS), among patients undergoing anthracycline chemotherapy.

Objective

We investigated this issue through a meta–analysis of echocardiography studies that reported data on: A) PALS before and after anthracycline chemotherapy and B) PALS in patients who have previously received anthracycline chemotherapy versus controls.

Methods

The PubMed, OVID–MEDLINE and Cochrane library databases were systematically analysed to search articles and abstracts published from inception to 29th December 2022. Studies were identified by using Me–SH terms and crossing the following terms: “left atrial strain”, AND “cardiotoxicity”, “anthracycline”, “anthracycline cardiotoxicity” and “echocardiography”.

Results

The meta–analysis included a total of 967 patients from 10 studies. A) The metanalysis of six studies evaluating PALS before and after anthracycline chemotherapy in a total of 343 patients showed a significant PALS worsening after anthracycline (30,05±2,5 vs 26,8±3,1) with standard mean difference (SMD) being –0,27±0,06, p < 0.0001. Figure 1 panel A. B) Five studies evaluated PALS in 359 patients who have previously received anthracycline chemotherapy versus 356 controls. Compared with controls, PALS was significantly worse in the pooled anthracycline group (34,63±1,8 vs 37,64±2,7) with SMD being –0.35 ± 0,08, p < 0.0001. Figure 1 panel B.

Conclusions

Our metanalysis suggests a detrimental effect of anthracycline chemotherapy on atrial function evaluated by PALS.
C12 DAPAGLIFLOZIN INCREASES PAMPK AND REDUCES MYOCARDIAL AND RENAL NF–KB EXPRESSION IN PRECLINICAL MODELS OF SHORT–TERM DOXORUBICIN CARDIOTOXICITY THROUGH MYD–188 AND NLRP3 PATHWAYS
Authors: Quagliariello V , Paccone A , Iovine M , Palma G , Luciano A , Barbieri M , Bruzzese F , Maurea C , Zito F , Sabetta R , Montella M , Franco R , Maurea N
Published: May 2023

Background

Doxorubicin–mediated adverse cardiovascular events are among the leading causes of morbidity and mortality in breast cancer patients. Sodium–glucose cotransporter 2 (SGLT2) inhibitors have recently been shown to be of therapeutic value in patients with type 2 diabetes, chronic kidney disease, and heart failure with reduced ejection fraction (HFrEF), conditions that commonly coexist and are interrelated pathophysiologically. Purpose: We hypothesized that Dapagliflozin (an SGLT2i), administered during doxorubicin, could improve cardiac function in preclinical models

Methods

Female C57Bl/6 mice were untreated (Sham, n=6) or treated for 10 days with doxorubicin i.p at 2.17 mg/kg (DOXO, n=6), DAPA at 12 mg/kg (DAPA, n=6) or doxorubicin combined to DAPA (DOXO–DAPA, n=6). Ejection fraction, radial and longitudinal strain were analyzed through transthoracic echocardiography (Vevo 2100). Cardiac tissue expression of NLRP3 inflammasome, Myd88, DAMPs (galectine 3 and calgranulinS100), pAMPK, NF–kB, and 13 chemokines (IL–1α, IL–1β, IL–2, IL–4, IL–6, IL–10, IL–12, IL17–α, IL–18, IFN–γ, TNF–α, G–CSF, and GM–CSF) were quantified through ELISA and western blot methods.

Results

DAPA improved significantly the EF and prevented the reduction of radial and longitudinal strain after 10 days of treatment with doxorubicin. A reduced expression of NLRP3, MyD88, DAMPs and NF–kB in cardiac tissues was seen in DOXO–DAPA group compared to DOXO mice (p<0.001). Cardiac expression of IL–1β, IL–6, TNF–α, G–CSF and GM–CSF were significantly reduced after treatment with DAPA indicating anti–inflammatory properties. Expression of pAMPK was strongly enhanced in DAPA–DOXO compared to DOXO group. Levels of Calgranulin S100 and galectine–3 were strongly enhanced in DOXO group; on the other hand their expression were reduced by 47.7 and 52.3% in DAPA–DOXO group vs DOXO (p<0.005). Hystology evaluation indicate reduction NF–kB expression in myocardial and renal tissue in DOXO–DAPA vs DOXO. Radial strain (RS) is 38.8% in DAPA–DOXO vs 14.1% in DOXO groups (P < 0.05); longitudinal strain (LS) is – 23.4 % in DAPA–DOXO vs – 10.5% in DOXO groups (P < 0.001)

Conclusion

In this preclinical study, DAPA is able to improve cardiac function and reduce biomarkers involved in heart failure and fibrosis. The overall picture of the study pushes the use of DAPA in prevention of cardiomyopathies induced by anthracyclines in cancer patients.
C15 PERCUTANEOUS EDGE–TO–EDGE REPAIR USING EDWARDS PASCAL SYSTEM FOR SEVERE TRICUSPID REGURGITATION IN CONGENITALLY CORRECTED TRANSPOSITION OF THE GREAT ARTERIES
Authors: D'Agostino A , Berti S , Santoro G , Ravani M , Trianni G , Mariani M
Published: May 2023

Introduction

Congenitally corrected transposition of the great arteries (ccTGA) is a rare cardiac malformation characterized by ‘double’ discordance of atrioventricular and ventriculo–arterial connections, resulting in a systemic tricuspid atrioventricular valve and a systemic right ventricle.

Clinical case

We describe a case report of 54 years–old male patient with ccTGA, diagnosed when he was 3 years–old. Patient performed regular follow–up. Since December 2019, the patient reported onset of effort dyspnoea (NYHA III), fatigue and exercise intolerance. Despite medication optimization, his symptoms persisted. He was referred at our Centre for specialist evaluation. Transthoracic echocardiography showed mild dilatation and dysfunction of right ventricle, massive systemic tricuspid valve regurgitation (EROA 42 mm2), due to multiple jets and systolic pulmonary artery pressure of 45 mmHg. As reported by most recent guidelines, indication was given for surgical replacement of the systemic tricuspid valve. However, the patient refused the cardiac surgery option. Transesophageal echocardiography (TEE) screening showed feasibility of percutaneous approach to tricuspid regurgitation. Being the jet central, the strategy was to place one device implant between the septal and the anterior leaflet (9–3 oriented), as central as possible. Following simultaneous leaflet capture and further optimization of leaflet, the result was excellent. Post–implant deployment TEE revealed only mild–to–moderate residual tricuspid regurgitation, with no tricuspid stenosis (mean gradient 2 mm Hg). The result was a percutaneous bicuspidization of a tricuspid systemic valve. The patient recovered quickly and was discharged to home 2 days after the procedure. Patient experienced significant improvement in functional status (NYHA I), exercise capacity and quality of life at 6 months follow–up.

Conclusions

In ccTGA patients, treatment of severe tricuspid regurgitation and improvement of the systemic ventricle’s systolic function is critical since progression of dysfunction of the RV and tricuspid regurgitation are considered key determinants for morbidity and mortality in these patients. Based on the results obtained in our case, percutaneous edge–to edge repair seems to be a reasonable alternative to the surgical approach and three–dimensional transesophageal echocardiography is imperative for the proper visualization of the leaflets outcome of the procedure and his durability.
C16 NONCOMPACTION OF VENTRICULAR MYOCARDIUM AND X–LINKED RECESSIVE ICHTYOSIS
Authors: Bevere E , Mastroianno S , Greco A , De Luca G , Potenza D , Brunetti N , Salvatori M , Di Stolfo G
Published: May 2023

Noncompact left ventricle is a rare congenital cardiomyopathy characterized by numerous ventricular trabeculae separated by deep intertrabecular recesses. It can occur in isolation or in association with neuromuscular disorders and congenital heart defects. The cause is not known but it is thought to be due to the arrest of the normal maturation process of the myocardium. Among the responsible mutations are taffazzine, ZASP and distrobrevin. It can manifest as left ventricular dilatation and dysfunction, arrhythmias, thromboembolic events, and sepsis. The echocardiogram is the investigation of first choice. Magnetic resonance allows you to more precisely distinguish compact and non–compact layers, thrombi and myocardial fibrosis.

Clinical case

A 19–year–old patient came for syncope. She presented with midfacial hypoplasia, cupped ears, synophrium, high palate, ichthyosis, small hands, and scarring from Achilles tendon lengthening. She denied a family history of cardiovascular disease and sudden cardiac death. Karyotype analysis showed karyotype 46Y, –X, + der t (X; Y) (p22.2; q11.1), with a chromosome derived from the X;Y translocation (a feature already known in the mother and in several maternal relatives ). Coronary angiography, electroencephalogram and brain MRI were within limits. Echocardiogram and MRI showed non–compact myocardium at the left ventricular apex and mild ventricular dysfunction with areas of anterior and posterior dyssynergia (Fig. 1). The ECG showed high QRS voltages. The Holter ECG showed numerous supraventricular extrasystoles even in pairs and runs, rare PVCs, and nocturnal pauses of up to 3 seconds. The patient underwent genetic screening by NGS for sarcomeric mutations which resulted negative. Subsequent genetic analysis showed deletion in two chromosomal regions that could be interpreted as pathogenic (chromosomal region Xp22.33 containing the SHOX gene and involving the deleted locus in Leri–Weill dyschondrosteosis and in the Xp22.33p22.31 region containing the STS gene involving the locus deleted in X–linked recessive ichthyosis) and a duplication in the chromosomal region Yq11.221q11.23 of uncertain significance.

Conclusions

After review of the scientific literature, the association of the illustrated complex deletion and translocation with non–compact myocardium represents a new finding, worthy of being explored in genetically affected patients.
C17 AN UNUSUAL CASE OF RIGHT HEART FAILURE AND PULMONARY HYPERTENSION: WET BERIBERI. THE IMPORTANCE OF DIFFERENTIAL DIAGNOSIS
Authors: Sambenedetto M , Mapelli M , Biondi M , Vignati C , Palermo P , Nepitella A , Muratori M , Agostoni P
Published: May 2023

Introduction

High–output heart failure is a rare form of heart failure. Possible causes include vitamin B1 (thiamine) deficiency. This vitamin plays an important role in the aminoacids energy metabolism and in the transmission of nerve impulses. Thiamine deficiency can affect the cardiovascular (wet beriberi), nervous, and immune systems. Case report: This case report concerns a 73–year–old man with a history of alcohol abuse affected by arterial hypertension, persistent atrial fibrillation, COPD and alcoholic liver disease. Home therapy included low–dose diuretic, mineralocorticoid antagonists, anticoagulant, hypotensive drugs and oral antidiabetic drugs. Due to the occurrence of dyspnoea and progressive swelling of the lower limbs he was hospitalized. Transthoracic echocardiogram (TTE) showed mild biventricular dilatation with normal function. The Doppler study revealed moderate–severe tricuspid regurgitation (anulus dilatation) and indirect signs of an increase in pulmonary pressures. Blood tests showed a picture of cytopenia, altered hepatic synthesis indexes and high values of natriuretic peptides. He performed invasive hemodynamic evaluation with right heart catheterization which showed mild pulmonary hypertension (mPAP 28 mmHg) with normal pulmonary vascular resistance and an increase in cardiac index (CI 4.2 L/min/m2). The cardiopulmonary testing (CPET) indicated a slight reduction in functional capacity (pVo2 77% of predicted value) and initial pulmonary vascular involvement. In the following months the patient manifested haemodynamic instability with the need for intravenous diuretic therapy. At a subsequent check–up, the dosage of thiamin was performed, which was found to be deficient, so he started supplementation. In the next period the patient reported well–being and maintained euvolemia. At a hospital re–evaluation, he performed a new TTE evaluation which showed a reduction in ventricular volumes and tricuspidal regurgitation as well as a new evaluation of invasive cardiac output which showed a reduction in right sections pressure (mPAP 22 mmHg) with normalization of the CI value (2,20 L/min/m2). CPET showed almost the same results of the previous one.

Conclusion

Beriberi is a possible treatable cause of high–output heart failure. Although it is a rare pathology in Western countries, it should always be suspected in some categories of patients such as alcoholics, subjects with eating disorders and those undergoing bariatric surgery.
C27 RBM20 VARIANTS RELATED CARDIOMYOPATHY: AN ITALIAN CASE SERIES
Authors: Manai R , Dusi V , Pidello S , Angelini F , Bocchino P , Gallone G , De Lio G , Gobello G , Saglietto A , Castagno D , Anselmino M , Giustetto C , Brach Del Prever G , Valeria B , Deaglio S , De Ferrari G , Raineri C
Published: May 2023

Background

RNA–binding motif protein 20 (RBM20) regulates post–transcriptional splicing, particularly in sarcomeric, but also in other cardiac genes crucial for homeostasis. RBM20 variants are a rare cause of cardiomyopathy (CMP), but genotype–phenotype correlation is still elusive, starting from the type of CMP (dilated, non–compacted, hypertrophic, and even restrictive phenotypes have been described), and ranging to sex–dependent and arrhythmic expression. Purpose: To describe cardiac involvement in 8 patients with RBM20 variants.

Methods

189 patients (pts) underwent genetic analysis in our center (2019–2022) for suspected genetic CMP (TruSight Cardio panel, Illumina); 72% had a positive genetic report, including pathogenic/likely pathogenic (P/LP) or variants of undetermined significance (VUS). Among them, 9 (5%, 6 males and 3 females, from 6 families) carried a heterozygous RBM20 variant: 5 a sole P variant, 3 an RBM20–VUS associated to a second VUS in another CMP related gene, one an RBM20–VUS associated to a LP variant in BAG3; the last patient was excluded (figure with 8 pts).

Results

Most pts (75%) had a positive family history (FH) for sudden death. Mean age at diagnosis was significantly lower for P variant carriers compared to VUS carriers (25±15 vs 46±2 years, p=0.04); no other significant differences were found between the 2 groups. At diagnosis, 4/8 pts (50%) had NYHA class>2, mean LVEF was 34±18% and mean LV end diastolic volume 83±14 ml/m2; most pts (6/8, 75%) presented with a dilated phenotype, one with a non–compacted and one with a hypertrophic phenotype. Cardiac magnetic resonance, available in 6 pts, showed non–ischemic fibrosis in 3 (50%). At presentation, two pts had non–sustained VT (NSVT), one atrial flutter, one first degree AV block. The median follow–up was 2 years (IQR 2–10). Four pts (50%) underwent ICD implantation at 40 ± 13 years old. One female patient underwent heart transplant at the age of 13 and no one died from end stage HF; 3 pts had NSVT, one patient had atrial fibrillation (successfully treated with ablation), and none developed advanced AV blocks.

Conclusions

This is the first Italian case series of RMB20 variants related CMP. In our case series, RBM20 variants are associated with an early onset cardiac expression both in male and female pts, with LV dilated CMP and heart failure being the most common presentation. None of the pts presented with or developed advanced AV blocks, most suffered from NSVT.
C28 LEFT VENTRICULAR OUTFLOW TRACT VELOCITY–TIME INTEGRAL (LVOT–VTI) IMPROVES RISK STRATIFICATION IN HEART FAILURE PATIENTS WITH SECONDARY MITRAL REGURGITATION
Authors: Gentile F , Buoncristiani F , Sciarrone P , Bazan L , Panichella G , Gasparini S , Chubuchny V , Fabiani I , Taddei C , Poggianti E , Passino C , Emdin M , Giannoni A
Published: May 2023

Background

Reduced left ventricular ejection fraction (LVEF) has been used as a key criterion for the management of mitral regurgitation (MR) in patients with heart failure (HF), including the decision about mitral valve repair. However, LVEF, not taking into account mitral regurgitant volume, may be an imprecise predictor of outcome in HF patients with MR. Conversely, the estimation of the forward volume through the LV outflow tract (LVOT) may be a better metric in this setting. In this regard, LVOT velocity time integral (LVOT–VTI), not relying on geometrical assumptions, has been shown to be more reproducible than the calculated stroke volume (i.e., LVOT–VTI * LVOT cross sectional area).

Objective

To assess the prognostic significance of LVOT–VTI in a contemporary cohort of patients with chronic HF and significant MR.

Methods

Consecutive patients with chronic HF with reduced (≤40%) or mildly reduced (41–49%) LVEF and moderate–to–severe or severe MR, according to the latest European Society of Cardiology criteria, were selected and followed–up for the endpoint of cardiac death.

Results

287 patients were enrolled in the study (74±11 years, 70% men, 46% ischemic etiology, HFrEF 89%, LVEF 30±9%, mean LVOT–VTI 20±5 cm). Most patients showed a NYHA class II (40%) or III (31%) and received beta–blockers (93%), ACE–inhibitors/ARBs or ARNI (77%), and mineralocorticoid receptor antagonists (76%). One hundred and ten patients (39%) had permanent atrial fibrillation and 82 (29%) a cardiac resynchronization therapy device. Over a median follow–up of 33 (16–47) months, 114 patients died, 71 of whom for cardiac cause, and 28 underwent mitral valve repair/replacement. The risk of cardiac death increased proportionally to the decrease of LVOT–VTI (Figure 1, panel A), with an optimal prognostic cut–off of 17 cm (Figure 1, panel B). Reduced LVOT–VTI was associated with higher NYHA class, NT–proBNP concentration, and worse right ventricle systolic function (all p<0.005). At multivariable competing risks regression analysis, a unit decrease of LVOT–VTI (HR 0.87 [95%CI 0.80–0.93], p<0.001), but not of LVEF (p=0.254), was independently associated with a higher risk of cardiac death (Figure 1, panel C).

Conclusion

Left ventricular forward volume, noninvasively estimated through LVOT–VTI, but not LVEF, predicts the risk of cardiac death in patients with chronic HF and significant MR.
C32 CONSTRICTIVE PERICARDITIS WITH PLATYPNEA ORTHODEOXIA
Authors: Gaiero L , Capriolo M , Gallo C , Annone U , Biasco L , Senatore G
Published: May 2023

A 56–year–old woman is admitted to our centre for biventricular heart failure. In anamnesis she reports a submandibular phlegmon complicated by pleuropericarditis at the age of 13 years requiring repeated pericardiocentesis. In the last three years she has been hospitalised twice for ascites and discharged with a diagnosis of idiopathic liver cirrhosis. On admission she presented with peripheral oedema, ascites and hippocratic fingers; oxygen saturation is 80% in room air. On blood tests creatinine, transaminases, albumin, coagulation and NT–proBNP are normal, bilirubin is elevated. An echocardiography is performed showing a calcific spicola impinging the free wall of the right ventricle producing endocavitary obstruction; diffusely thickened pericardium, septal bounce, annulus reversus and expiratory reflux in the suprahepatic veins. Biventricular contractile function is normal and no significant valvulopathy is present. CT scan confirms the presence of diffuse pericardial calcifications, small liver and irregular profile with large ascitic effusion. Decongestive therapy is started with weight loss of 22 kg and regression of oedema but severe hypoxia persists with evidence of platypnea–orthodeoxia syndrome. An echocardiography is repeated showing passage of microbubbles in the left atrium from the sixth beat onwards, compatible with intrapulmonary shunt. A large arteriovenous malformation (AVM) of the right lower pulmonary branch is found at CT angiography, which is also revealed by targeted invasive angiography. Cardiac catheterisation and cardiac MRI confirmed the diagnosis of constrictive pericarditis. Percutaneous closure of the AVM with complete resolution of the hypoxia and subsequent pericardiectomy is performed. The patient is discharged on day XXVII with normal saturation on room air, in good haemodynamic compensation and with normal liver stasis parameters. In conclusion, constrictive pericarditis is a rare cause of heart failure and to our knowledge this is the second case described reporting a pulmonary AVM secondary to constrictive pericarditis.
C33 AN UNEXPECTED CARDIOGENIC SHOCK
Authors: Leiballi E , Lestuzzi C , Viel E , Riccio A , Valente M , Turazza F , Pecoraro R , Pavan D
Published: May 2023

Background

Chemotherapy regime based on 5–fluorouracil (5–FU) are frequently administered in the treatment of gastro–intestinal malignancies, especially colorectal carcinomas. A potential severe side effect of 5–FU is cardiotoxicity with often presents with chest pain related to coronary vasospasm. More serious cardiotoxicity, including dilated cardiomyopathy, ventricular arrhythmia and sudden cardiac death has also reported in the literature. Case report. We present a 40–years–old man with stage IV colon adenocarcinoma with metastases. The patient had no history, or risk factors, of cardiac disease. The patient was started on palliative chemotherapy with FOLFOXIRI (Leucovorin/Irinotecan/Fluorouracil). Approximately 24 h into receiving the first infusional dose of 5–FU the patient developed progressive chest pain. Electrocardiography (ECG) revealed sinus tachycardia without repolarization changes. Cardiac biomarkers indicated a moderate elevated troponin (430–580 ng/L). Echocardiogram revealed severely reduced left ventricular function with an ejection fraction (EF) of 23%. Subsequently, the patient developed a hemodynamic instability treated with noradrenaline and dobutamine, but the patient remained in cardiogenic shock. The patient was transferred to cardiac surgery for ECMO placement. Despite ECMO placement the patient was hemodinamically unstable, it was necessary to position also IMPELLA and IABP. The improvement was very slow and the ventricular function returned to normal after 5 days. A subsequent cardiac MRI showed delayed enhancement with diffuse subepicardial distribution (non–ischaemic pattern).

Conclusion

Our case emphasizes the importance of early recognition of this rare complication and prompt cessation of 5–FU, as cardiac dysfunction in this context is potentially reversible. The precise mechanism remains unclear, but several mechanisms have been proposed including coronary vasospasm, direct toxicity to the myocardium, endothelial dysfunction. It should be noted that these mechanisms were elucidated though animal modeling, case reports, and small clinical studies.
C34 AN UNUSUAL CASE OF CARDIAC ARREST DUE TO TRAUMA–INDUCED QT PROLONGATION
Authors: Astuti M , Somaschini A , Olivotti L , Cornara S , Ghione M , Buscemi M , Buscaglia E , Botta M , Cordone S , Pentimalli F , Bellone P
Published: May 2023

Background

QT prolongation is a well–known cause of sudden cardiac death due to polymorphic ventricular tachycardia triggered by early afterdepolarizations. Acquired long QT is a common finding in several clinical scenarios and invariably represents a high risk feature.

Case Report

An 84 years–old woman presented to our emergency department (ED) after experiencing a blunt chest trauma due to a car accident. She reported mild dyspnea and chest pain exacerbated by breathing and movement while denied palpitations and loss of consciousness. Baseline ECG showed atrial fibrillation with normal ventricular rate and a prolonged QT interval (QTc 640, Fig 1A) not present at previous ECGs. She did not assume any QT–prolonging drug and laboratory tests at admittance revealed troponin I values slightly elevated (29,6 ng/ml; n.v.: <11,6 ng/l) and no other significant abnormalities. A CT scan demonstrated a displaced fracture of the sternal body. Due to the QT prolongation, the patient was admitted for ECG monitoring.. During the stay in our ED the patient suffered a cardiac arrest. A torsade des pointes triggered by a late–coupled premature ventricular complex (PVC) was documented (Fig 1B). The arrhythmia was interrupted with external DC shock and magnesium solfate infusion was started. The coronary angiography demonstrated normal coronary arteries. The patient underwent cardiac magnetic resonance (CMR) four days later which showed transmural necrosis of the middle third of the lateral wall of the left ventricle at the insertion of the anterolateral papillary muscle (Fig 2). No further ventricular arrhythmias were observed and the patient was discharged after QTc normalization. Cardiac arrhythmias are a rare complication of cardiac contusion ranging from isolated PVCs to atrial or ventricular fibrillation. However, the role of the ECG in this peculiar subset is unclear. The most common ECG abnormalities are non specific ST–T wave changes, whereas in our case the marked QTc prolongation resulted in a closer monitoring of the patient allowing a prompt recognition of cardiac arrest. On the other hand CMR proved to be an effective method to identify a cardiac injury showing the typical pattern of myocardial damage associated with cardiac contusion (stress–induced lesions at the insertions of papillary muscles due to sudden increase of left ventricular pressure) and, thus, could be adopted as a useful tool to identify patients at higher risk for arrhythmic complications.
C35 A RARE CASE OF NON–BACTERIAL THROMBOTIC ENDOCARDITIS IN YOUNG WOMAN AFFECTED BY ADVANCED UTERINE CANCER
Authors: Molisana M , Belleggia S , Gizzi G , Ianni U , D’Agostino S , Di Eusanio M , Dello Russo A , Parato V
Published: May 2023

A 37–year–old woman affected by uterine HPV–related adenocarcinoma complicated by paraneoplastic pulmonary thromboembolism, came to the emergency department for recurrent episodes of dysarthria and blurred vision with spontaneous resolution. Home medications: Enoxaparin 5000 UI b.i.d, levofloxacin 500 mg/die, deltacortene 25 mg/die and omeprazole 20 mg/die. Blood analysis: normocytic anemia (Hb 9 gr/dl),elevated D–dimer (22.5 mcg/ml) and elevated C–reactive protein (18.3 mg/dl). Procalcitonin and blood cultures resulted negative. Brain CT scan: ischemic brain lesions of embolic nature in the right precentral gyrus and in the left post central gyrus. EKG: sinus rhythm, T–wave inversion in inferior leads. In the suspect of cardioembolic stroke a transthoracic (TTE) and subsequent transesophageal (TOE) echocardiography was performed: TTE: isoechoic oval–shaped thickening of the aortic valve cusp tips, producing aortic regurgitation with double jet regurgitation. TOE: evidence of multiple isoechoic masses involving all the aortic cusps, without perivalvular aortic extension, producing significant aortic regurgitation due to coaptation– gap (Figure 1). The diagnosis of non–bacterial thrombotic endocarditis (NBTE) was made up. Although the standard medical therapy (anticoagulation and chemotherapy) was promptly started, it wasn’t effective with progression of the disease leading to surgical indications of valve intervention. The patient underwent aortic valve replacement with bioprosthesis avoiding mechanical prosthesis, despite the young age, in anticipation of major surgery for the uterine cancer. Surgical specimen turned negative for microorganism growth confirming the diagnosis of marantic endocarditis. No further episodes of ischemic stroke appeared after the replacement of the valve and the patient had no neurological sequelae. NBTE is a rare condition of non–infective endocarditis characterized by the formation of aggregates of platelets and fibrin that affects usually undamaged heart valves in the absence of bacteremia and mostly occurs in patients with a predisposing state like hypercoagulability. Surgical indications of valve intervention are not well encoded but in most cases they follow the raccomandations for infective endocarditis. Our experience highlights the need of a prompt intervention in order to reduce the damage of systemic embolization and preserve patient’s hemodynamics with a strict checking of disease progression.
C41 CAROTID OCCLUSION AT THE CAROTID SIPHON LEVEL IN A CHILD WITH PROGERIA
Authors: Greco A , Mastroianno S , Bevere E , Massaro R , Coli C , Potenza D , Salvatori M , Brunetti N , Di Stolfo G
Published: May 2023

Introduction

Hutchinson–Gilford Progeria syndrome (HGPS) is a rare disease affecting 1 in 4–8 million children, caused by a sporadic mutation of the LMNA gene and expression of a mutant product called progerin. Patients develop a progressive and severe premature aging disease characterized by failure to thrive, alopecia, bone and joint abnormalities, loss of subcutaneous fat, and severe atherosclerosis. Patients live an average of 14.6 years, and most die in their early teens from myocardial infarction or stroke due to rapidly progressive atherosclerosis.

Clinical case

The 8–year–old patient, affected by progeria, arrived for relapsing strokes. The clinical history began with a picture of severe acro–mandibular hypoplasia with obstruction of the upper airways, treated with early tracheostomy and subsequent percutaneous gastrostomy. Further progeroid phenotypic characters developed were: poor body growth, sui generis facies with prominent eyes and beaked nose, alopecia, micrognathia, discolored, thin, hypoelastic skin; poorly represented panniculus adiposus; scoliosis. Genetic tests have highlighted two pathogenic heterozygous mutations, of the LMNA gene (C.168 C>A) probably deriving from the mother (mosaicism), and the other of the ZMPSTF24 gene, (c.846 C>T) paternally inherited. The major complications are secondary to severe atherosclerosis of the cerebral district, resulting in repeated strokes. Brain MRA (Fig.1 and 2) documented the absence of signal at the level of the right carotid siphon and of the whole intracranial tract of the right carotid artery and poor signal in correspondence with the intracranial tract of the left carotid artery distal to the carotid siphon in a picture of diffuse atherosclerosis. The marked predisposition and the recurrence of ischemic episodes required the patient to have thrombophilic screening, positive for a heterozygous mutation for the prothrombin gene (G20210A), known for thrombophilic predisposition.

Conclusions

Progeria is a rare syndrome that requires an adequate multidisciplinary approach and clinical care management aimed at improving the quality of life of our patients.
C43 RELATIONSHIP BETWEEN ECHOCARDIOGRAPHIC DATA AND CARDIOPULMONARY EXERCISE TEST PARAMETERS IN ADULT CONGENITAL HEART DISEASE PATIENTS: A RETROSPECTIVE CLINICAL STUDY
Authors: Olimpieri A , Ferraro F , Gambardella R , Di Molfetta A , Grandinetti M , Lillo R , Graziani F , Delogu A , Bruno P , Massetti M
Published: May 2023

Introduction

The interpretation of cardiopulmonary exercise test (CPET) in adult congenital heart disease (ACHD) is challenging because of the high variability in cardiac lesions and because the poor exercise tolerance is often associated with abnormal heart function. The aim of this work is to study the correlation between echocardiographic evaluation and CPET parameters in ACHD population.

Methods

This is a retrospective clinical study enrolling all ACHD patients afferent to our institution from March 2016 to February 2020 collecting clinical, CPET and echocardiographic data. Population was divided into three groups and compared: patients with prevalent left heart lesions (L group), patients with prevalent right heart lesions (R group) and patients with single ventricle physiology (SV group). Finally, correlation analysis were performed to assess any relationship between echocardiographic and CPET data.

Results

60 patients were included in the study, 20 were male, median age was 36 [30–46.75] years old, 23 had a prevalent left side pathology, 31 a prevalent right side pathology and 6 had a single ventricle physiology. L group had a significantly lower predicted peak VO2 in comparison to SV group (p=0.03). Statistical analysis showed a correlation between the right ventricular function and the ventilatory reserve in the R group (R2 0.517). In SV group we found correlation between ventricular–arterial coupling and Cardiac mechanical efficiency with the ventilatory reserve (R2 0.6 and 0.65, respectively) and the oxygen pulse (R2 0.97 and 0.98, respectively), and a correlation between the ejection fraction and the Oxygen pulse (R2 0.52). Finally, a correlation between the TAPSE/PAPs index and the ventilation (R2=0.79), the ratio between the oxygen uptake and the workload (R2=0.61) and the oxygen consumption (R2=0.72) was found. Coclusion:In our preliminary experience, echocardiographic parameters of cardiocirculatory efficiency correlate with the oxygen consumption per heart beat evaluated at the CPET in single ventricle patients, and the breath rate is influenced not only by ventricular function but also by the coupling between the ventricle and the arterial system. In patients affected by right–side disease, the breath rate correlate with the right ventricular function. Finally, in the left heart lesion population, a correlation between the TAPSE/PAPs index with the ventilation, the oxygen consumption per beat and the oxygen consumption was found.
C46 ELECTROPHYSIOLOGICAL ASSESSMENT OF ATRIAL TACHYCARDIA SUBSTRATES IN PATIENTS WITH REPAIRED TETRALOGY OF FALLOT
Authors: Mirizzi G , Garibaldi S , Startari U , Rossi A , Panchetti L , Festa P , Ait–ali L , Santoro G , Assanta N , Piacenti M
Published: May 2023

Introduction

Adults with repaired tetralogy of Fallot (rToF) experience episodes of atrial tachycardia (AT) and radiofrequency catheter ablation (RFCA) is often required but systematic evaluation of the mechanisms and recurrences is lacking.

Methods

Between January 2013 and October 2021, 20 rToF patients with AT referred for catheter ablation were enrolled. Electrophysiologic study with 3D electroanatomic mapping with multi–electrode mapping catheters was done, with right atrial bipolar voltage and activation mapping of the AT. Three mechanisms were searched: intra–atrial re–entrant tachycardias (IART), focal (FAT), other. Critical isthmus (CI) for IART was identified with activation mapping. FATs were localized according to the earliest uni/bipolar signal. All induced AT were treated. RFCA was aimed at the earliest activation point for FAT and at the critical isthmus for IART, anchoring lesion to fixed obstacles (valve annuli or scar). Written informed consent was provided.

Results

Among 20 adult (age 46±14 years) and mainly females (n=11, 55%) enrolled pts, 36 AT were documented: 25 (70%) IART, 10 (27%) FAT and only 1 (3%) had a typical atrioventricular nodal reentrant tachycardia. Mean tachycardia cycle length was 307±95 ms. Two ATs were induced in 11 pts, 3 in 4 pts and 4 in 2 during index EPS. Among IART, cavo–tricuspid isthmus (CTI) was the prevalent CI (n=14, 60%), while incisional IART (IARTinc, atriotomy and superior and inferior vena cava orifices, SVC and IVC, respectively) was the second mechanism (n=9, 39%). In two pts, due to non–inducibility, a pre–emptive lesion set comprising in one case CTI and in the second case CTI+SVC–atriotomy–IVC line was performed. Among FAT, in 3 cases the AT was mapped in the coronary sinus, in two at the tricuspid annulus, other at the crista terminalis, right appendage base, posterolateral scar or between atriotomy and SVC. During a median follow–up of 23 months (interquartile range, 6–37) recurrence occurred in 8 pts (25% of pts, 22% of tachycardias). Pts with recurrences were younger (43±7 vs. 48±17 y, p=0.04); no differences were found according to critical isthmus location (4 CTI and 4 IARTinc, 50%, p=ns).

Conclusions

Among rToF pts with AT, IART is the prevalent mechanism and CTI is the prevalent CI; atriotomy scar, however, is involved in a substantial portion of the critical isthmuses of the IART; FAT location is much more variable. Long–term freedom from AT in this clinical setting is encouraging.
C51 INTRAVASCULAR LITHOTRIPSY FOR THE TREATMENT OF SEVERELY CALCIFIED CORONARY STENOSIS IN ACUTE MYOCARDIAL INFARCTION
Authors: Pavani M , Franzè A , Cinconze S , Barbero U , Meliga E , Rognoni A , Nicolino A , Biasco L , Conrotto F , Ugo F , Scacciatella P , Tomassini F , Leoncini M , Menozzi A , Patti G , Rolandi A , Porto I , Musumeci G , Cerrato E , Varbella F
Published: May 2023

Background

Little is known about intraprocedural and mid–term results of intravascular lithotripsy (IVL) use in patients with acute MI.

Methods

In Our retrospective analysis, data from 21 Centers in Italy and Spain between January 2020 and November 2021 were collected. Patients were allocated to the AMI–group when IVL was performed during an acute MI. Primary safety endpoint was defined as freedom from major adverse cardiac events (MACE: cardiac death, myocardial infarction (MI), target vessel revascularization (TLR) or stent thrombosis (ST)) within 30 days of the index procedure, and primary effectiveness endpoint was procedural success (defined as stent delivery with a residual stenosis <20% and without intraprocedural MACE). The mid–term endpoint was 6–months MACE.

Results

A total of 468 patients were retrospectively enrolled. The primary safety endpoint was achieved in 98.8% of patients in the AMI–group and in 98.5% in the non–AMI group (p=NS). We showed a lower incidence of the primary effectiveness endpoint in the AMI–group (89.8%) vs Non AMI–group (94.8%) (p=0.048). The secondary endpoint of 6–months MACE was similar between groups (6.7% vs 4.9% respectively for AMI group and Non AMI–group), but death for cardiac causes occurred more frequently in the AMI–group (4.3% vs 0%, p=0.01).

Conclusions

Intravascular lithotripsy demonstrated to be a safe tool for the treatment of calcified coronary lesions during Acute Myocardial infarction, even if we showed some concern for lower procedural success.
C62 CHALLENGING DIAGNOSIS OF A TRIPLE CARDIAC PATHOLOGY: TRANSTHYRETIN AMYLOIDOSIS WITH ASPECTS OF HYPERTROPHIC OBSTRUCTIVE CARDIOMYOPATHY AND ANOMALOUS CORONARY ARTERY
Authors: Patani F , Tofoni P , Lofiego C , Vagnarelli F , Capodaglio I , Lombardi G , Pietrucci F , Schicchi N , Esposto Pirani P , Perna G
Published: May 2023

A 70 years–old man with a history of systemic hypertension and surgically treated bilateral carpal tunnel was referred for dyspnea on exertion for few months. At physical examination a systolic heart murmur exacerbated by Valsalva maneuver was appreciated; no signs of heart failure. EKG with low–voltage QRS is shown in Fig.1A. Blood tests showed elevated NT–proBNP (1434 pg/ml). Transthoracic echo (ETT) revealed hyperdynamic left ventricle (LV) with severe asymmetric hypertrophy (interventricular septum 23 mm, posterior wall 17 mm), ‘granular sparkling’ appearance of myocardium, grade 2 diastolic dysfunction and ‘apical sparing’ pattern at global longitudinal strain (GLS, Fig.1B). Moreover, elongated mitral leaflets, anomalies of mitral sub–valvular apparatus with apically displaced papillary muscles and systolic anterior motion of the mitral valve (SAM) with dynamic outflow tract obstruction (20 mmHg at rest and 90–100 mmHg with Valsalva maneuver) were seen (Fig. 2A–B–C). For ETT suspicion of a coronary artery anomaly (Fig.3A), the patient underwent coronary CT angiography that confirmed a separate origin of left anterior descending artery (LAD) from right coronary sinus with a long mid–proximal intramyocardial bridge (Fig.3B–C). As a part of evaluation of the hypertrophic phenotype, a 99mTC–HDP bone scintigraphy was performed with high–grade of cardiac uptake (Perugini Score 2, Fig.1C). Screening for monoclonal gammopathy and genetic testing for hereditary amyloidosis resulted negative. Cardiac MRI revealed late gadolinium enhancement (LGE) in midwall septum, diffuse endocardial LGE in the LV basal inferior and posterolateral walls (Fig.1D), elevated T1 mapping and ECV (40–42%) and confirmed the asymmetric hypertrophy with apically displaced papillary muscles (Fig.2D) and SAM. These clinical, EKG and imaging features led to a non–invasive diagnosis of wild–type transthyretin cardiac amyloidosis (ATTRwt) with aspects of hypertrophic obstructive cardiomyopathy (HOCM). The patient started a disease–modifying therapy with Tafamidis and, cautiously, Metoprolol tartrate, with good tolerance and relevant reduction of LVOT obstruction at follow–up. Genetic testing for HCM was also performed. In conclusion, in rare cases hypertrophic phenotype may be challenging, showing features overlap between ATTR and HOCM. Multimodality evaluation is crucial for a correct diagnosis, thus identifying the most effective target–therapy for the underlying hypertrophic phenotype.
C67 ATRIOVENTRICULAR BLOCK DUE TO CARDIAC CONDUCTION TISSUE INFILTRATION: A CLINICAL PRESENTATION OF TRANSTHYRETIN CARDIAC AMYLOIDOSIS
Authors: Manguso G , Alfarano M , Ballatore F , Ajmone F , Verardo R , Magnocavallo M , Bagnato G , Ciaramella P , Maggio E , Vernile A , Costantino J , Mariani M , Frustaci A , Lavalle C , Vizza C , Chimenti C
Published: May 2023

Background

In cardiac amyloidosis, the deposition of insoluble amyloid fibrils can affect the cardiac conduction tissue, causing bradyarrhythmic clinical manifestations, often in the advanced stages of the disease. In this clinical case, atrioventricular block represented the clinical onset of transthyretin cardiac amyloidosis.

Clinical case

A 68–year–old man was admitted to our ward for recurrent syncopal episodes. He had in his medical history bilateral carpal tunnel syndrome. The electrocardiogram documented sinus rhythm with preserved QRS voltages, right bundle branch block, left anterior hemiblock, and first and second degree atrioventricular block with mean ventricular rate of 38 beats/min. A color–Doppler echocardiogram revealed increased left and right ventricular thickness (maximum thickness 16 and 10 mm, respectively), diastolic dysfunction, and preserved ejection fraction (60%). The patient underwent a coronary angiography, which was normal, and an endomyocardial biopsy of the left ventricle, which revealed the presence of hypotrophic and degenerated cardiomyocytes surrounded by large areas of amorphous substance that exhibited apple–green birefringence in polarized light after staining with Congo red, suggesting the diagnosis of cardiac amyloidosis. Sections of conduction tissue were included in the specimens, which appeared on histology as small myocytes positive by immunohistochemistry for HCN4, suggestive of conduction tissue cardiomyocytes. The conducting tissue was almost completely replaced by amyloid deposits positive by immunohistochemistry for transthyretin. The affected patient underwent a genetic test which revealed the mutation of the TTR gene (p.Thr79Lys). The patient subsequently underwent pacemaker implantation and started treatment with Tafamidis due to the absence of neurological involvement.

Conclusion

In patients presenting clinically with syncope and conduction abnormalities, the diagnosis of cardiac amyloidosis should always be considered, because they may represent the clinical onset of the disease.