305 Usefulness of CHA2DS2-VASc scoring system for predicting risk of embolism in patients with cardiac tumours: a single-centre study

Aims The link between pro-protein convertase subtilisin/kexin 9 (PCSK9) and inflammatory-related disease has been supported in several experimental and human studies, which show how the protein could acts independent its canonical mechanism, by activation of inflammatory, apoptotic and immune pathways. Among these, TLR4/NFKB signalling pathway has been found to be one of the main pathways mediating the PCSK9-induced increase of pro-inflammatory and pro-thrombotic molecules, such as TNF-alpha, IL-6, IL-1, MCP-1, and TF. To investigate the direct involvement of hTLR4 in recognition of exogenous human recombinant (hr) PCSK9. Methods and results Experiments were carried out in three cell line: (i) HEK293-hTLR4-GFP cell lines obtained by stable co-transfection of HEK293 cells with the pUNO1-hTLR4-GFP, a plasmid expressing the hTLR4 gene fused to a GFP gene; (ii) HEK293-hTLR4+ cells obtained by co-transfection of hTLR4 receptor and its accessory proteins MD-2 and CD14, and an inducible secreted embryonic alkaline phosphatase (SEAP) reporter gene; (iii) HEK293-Null2 cells, a cell line lacking TLR4 expression, used as negative control. NFkB activity were measured by the SEAP reporter gene assay using a fluorescence detection method, while localization of hTLR4 and exogenous hrPCSK9 by confocal microscopy. hrPCSK9 (1 µg/mL) activates NFkB in HEK293-hTLR4+ cells [SEAP activity-OD 620 nm: from a baseline of 0.18 ± 0.06 to 0.68 ± 0.05 (N = 8), P < 0.001] to an extent comparable to lipopolysaccharide, the specific agonist of TLR4. Co-localization of hrPCSK9 and TLR4s was clearly documented by quantitative confocal microscopy in HEK293-hTLR4-GFP cell line, that comprises the analysis of more than 80 fields in ∼2.105 cells/well and showing a percentage of co-localization of ∼4% in membrane spots (P < 0.01). Conclusions Our data support the results reported in previous studies that attribute to PCSK9 a pathophysiological role in development of chronic inflammation and related-diseases such as atherothrombosis. A behavior that extends far beyond LDLR degradation, via a mechanism that might be mediated at least in part by recognition of PCSK9 by the TLRs and later activation of NFkB intracellular pathway. Future studies will be important to better investigate the specific binding site engaged in the PCSK9-TLR interaction and to discriminate the intracellular transduction pathways involved in this response, in order to provide a theoretical basis for the development of innovative therapeutic strategies.

413 Challenges in the field of cardiac tumours: the surgical experience of Trieste

Aims Cardiac tumors are rare and heterogeneous entities which still remain a diagnostic and therapeutic challenge. The treatment for most cardiac tumors is prompt surgical resection. We sought to provide an overview of surgical results from a series of consecutive patients treated at our tertiary centre during almost a 20-year experience. Methods and results In this single centre study, 55 consecutive patients with cardiac tumor underwent surgical treatment from January 2002 to April 2021. Of these, 42% of patients were male and the mean age was 62 ± 12 years. Almost 27% of patients were symptomatic at the time of the diagnosis, mostly for dyspnoea and palpitations. The most frequent benign cardiac tumor was myxoma (58% of cases), occurring mainly in the left atrium (97%). Pleomorphic sarcoma was the most frequent primary malignant cardiac tumour (7.2% of cases), mainly located in the ventricles (25% left ventricle; 50% right ventricle). In all cases of benign tumors surgery was successful with no relapses. In 50% of cases of pleomorphic sarcoma relapses were observed during follow-up. After a median follow-up of 44 months, 15 (27%) patients died. While malignant tumors presented a limited survival, benign tumours showed a very good prognosis. Conclusions Cardiac tumours require a multidisciplinary work-up to guarantee a prompt diagnosis and appropriate treatment. In our surgical experience, the prognosis of benign tumours was excellent, while malignant tumours had poor outcomes despite radical surgery.

Is the heart involved or not? The diagnostic value of cardiovascular magnetic resonance imaging in the assessment of non-cardiac malignancies and metastatic cardiac tumours

Cardiovascular magnetic resonance (CMR) is a radiation-free, high-spatial resolution technique which provides dynamic assessment of the heart and pericardial tissue. This is particularly useful for the evaluation and characterisation of non-cardiac tumours close to the pericardium for the exclusion of cardiovascular infiltration, and also for the assessment of the extent of myocardial invasion of cardiac metastases. This information can help make key decisions on further management in oncology multidisciplinary meetings. The CMR evaluation and main types of sequences acquired are detailed in this case series to illustrate the application of CMR in the assessment of non-cardiac malignancies and its importance in guiding management.

Big Mitral Annular Calcification (Big MAC) – A case report of a dynamic liquefaction necrosis as a potential source of embolism

Background Mitral annular calcification (MAC) is a degenerative, mostly asymptomatic abnormality in usually elderly patients. Caseous MAC (cMAC) is a rare form with central liquefaction necrosis, which typically involves the posterior annulus of the mitral valve and can cause serious sequelae. However, optimal management of patients with cMAC is not clearly defined. Case Summary In a 71-year-old female patient, MAC was incidentally detected. Tissue characterization with cardiac magnetic resonance (CMR) revealed a cMAC and a conservative approach was chosen. Six months after cMAC diagnosis the patient developed an acute hemi-occlusion of a retinal artery with cholesterol embolism. At this time, CMR showed a liquefied cavity of the cMAC. Except for atherosclerotic plaques in the aorta and carotid arteries, further stroke work-up was negative. Therefore, the conservative approach was continued. During follow-up, the liquefied cavity regressed completely after another 6 months and the patient was free from further events (total follow-up 3 years since diagnosis of cMAC). Discussion A clear diagnosis and quantitative assessment of dynamic processes such as cMAC are made possible by performing CMR with multi-parametric tissue characterization. Dynamic changes in cMAC may have serious clinical implications such as mitral regurgitation or systemic embolization. Among cardiac tumours, thrombus and abscess, cMAC should be included in the differential diagnosis of an intracardiac mass of the posterior mitral annulus in order to avoid further inappropriate diagnostic interventions.

Cardiac myxomas: clinical presentation, diagnosis and management

Cardiac myxomas (CM) are the most common type of primary cardiac tumours in adults, which have an approximate incidence of up to 0.2% in some autopsy series. The purpose of this review is to summarise the literature on CM, including clinical presentation, differential diagnosis, work-up including imaging modalities and histopathology, management, and prognosis. CM are benign neoplasms developed from multipotent mesenchyme and usually present as an undifferentiated atrial mass. They are typically pedunculated and attached at the fossa ovalis, on the left side of the atrial septum. Potentially life-threatening, the presence of CM calls for prompt diagnosis and surgical resection. Infrequently asymptomatic, patients with CM exhibit various manifestations, ranging from influenza-like symptoms, heart failure and stroke, to sudden death. Although non-specific, a classic triad for CM involves constitutional, embolic, and obstructive or cardiac symptoms. CM may be purposefully characterised or incidentally diagnosed on an echocardiogram, CT scan or cardiac MRI, all of which can help to differentiate CM from other differentials. Echocardiogram is the first-line imaging technique; however, it is fallible, potentially resulting in uncommonly situated CM being overlooked. The diagnosis of CM can often be established based on clinical, imaging and histopathology features. Definitive diagnosis requires macroscopic and histopathological assessment, including positivity for endothelial cell markers such as CD31 and CD34. Their prognosis is excellent when treated with prompt surgical resection, with postsurgical survival rates analogous to overall survival in the age-matched general population.

Asymptomatic cardiac tumour with premature ventricular contraction in an athlete: case report

Cardiac fibromas are the second most common benign primary tumour of the heart in the children; the clinical features include chest pain, arrhythmia, low cardiac output due to outflow tract obstruction, and sudden cardiac death. Sports are associated with an increased risk for sudden death in athletes who are affected by cardiovascular conditions predisposing to life-threatening arrhythmias. We present a case report of 10-year-old asymptomatic boy who was referred to the paediatric cardiology department by his general practitioner for cardiac examination before participation in competitive sports. The electrocardiogram showed premature ventricular contractions originated from inferior of left ventricle. A mass was detected by 2D transthoracic echocardiography, and it was found to be compatible with fibroma on MRI. In some cases, cardiac tumours are asymptomatic as in our patient. Electrocardiogram abnormalities require detailed cardiac imaging with echocardiogram, and if necessary CT/MRI. In this article, we emphasise that detailed cardiac examination of individuals before participating in competitive sports is vital.

Why primary malignancies of the heart and pericardium are so rare

Primary Malignant Cardiac Tumours (PMCTs) of the heart and pericardium are extremely rare. The incidence rate of both malignant and benign primary tumours is 0.02%, however only one quarter of these are malignant. The reason why these specific malignancies are so uncommon is not yet well understood. This review aims to determine why the heart and pericardium are such inhospitable hosts to PMCTs.

When cardiac surgery comes to its limits: a case report of pericardial mesothelioma invading the myocardium

Background Primary pericardial mesothelioma (PPM) is a rare form of highly aggressive cancer. Many patients are diagnosed only at an advanced stage. Therefore, the overall survival rate is poor with a median survival of 3 months. In some rare cases, the PPM infiltrates the myocardium causing lethal myocardial dysfunction. Case summary A 66-year-old patient was transferred to our centre with the provisional diagnose of pericarditis of unknown origin. Using extensive cardiac imaging [echocardiography, computed tomography (CT), positron emission tomography–CT, cardiac magnetic resonance imaging, left and right heart catheterization, coronary angiography], PPM was finally diagnosed. After consultation with the oncologists, the heart team decided to resect the tumour first due to impaired haemodynamics and then initiate adjuvant chemotherapy. Intraoperatively, myocardial infiltration of the tumour became apparent, which was not detected preoperatively despite intensive imaging. Complete resection of the PPM was not possible and effective decompression of the ventricle could not be achieved. The patient died on the first postoperative day. Discussion Surgical therapy is indicated in many forms of cardiac tumours. However, when a tumour invades the myocardium, surgery often comes to its limits. In this case, myocardial invasion of PPM could not be detected despite extensive imaging. We therefore suggest that possible myocardial infiltration by PPM, and thus potential limitations of cardiac surgery, should be considered independently of imaging results when therapeutic options are discussed.

Left Ventricle Intramuscular Haemangioma

Cardiac haemangiomas are rare cardiac tumours that are usually asymptomatic and detected incidentally while imaging the heart with echocardiogram. Cardiovascular magnetic resonance (CMR) is a non-ionising imaging modality that allows the diagnosis of cardiac haemangiomas prior to surgery. We report a 36-year old male patient who was referred to the Adult Cardiology Clinic at the Royal Hospital, Muscat, Oman, in 2006 with a history of a left ventricle mass detected on echocardiogram. Further assessment of the mass by CMR revealed that the mass within the left ventricle apical septum contained features that were consistent with a cardiac haemangioma. Due to the surgical risk of ventricular septal defect and the stability of the mass, the patient was managed conservatively and upon followup the patient’s condition remained stable. Keywords: Heart Neoplasms; Hemangioma; Heart, Neoplasm; Magnetic Resonance Imaging; Cardiovascular; Left Ventricle; Coronary Angiography; Case Report; Oman.

Carney complex: left atrial myxoma in a patient with past pituitary microadenoma and lentiginosis

The Carney complex (CNC) is a rare autosomal dominant genetic complex that is characterised by multiple neoplasms consisting of neuroendocrine and cardiac tumours, with only 750 cases reported worldwide as of 2017. Cardiac tumours, in the context of the CNC, are of unique importance since the leading causes of death in patients with CNC are cardiac. To prevent sudden cardiac death and embolic events, a difficult diagnosis must be made and postdiagnostic screenings must be regular. We present a case of a 52-year-old man, with a medical history of pituitary microadenoma and facial lentiginosis, who presented with dyspnoea 2 months after suffering a cerebrovascular accident.