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This article discusses the use of cardiac magnetic resonance (CMR) imaging with the late gadolinium enhancement (LGE) technique to assess the presence of a myocardial scar in patients with an anomalous right coronary artery originating from the left sinus of Valsalva (R-ACAOS). The study included 48 patients with R-ACAOS, and 21% of them showed an ischaemic pattern of LGE. These patients were older and had high-risk anatomical features. Although most patients were asymptomatic and had negative provocative tests, the presence of an ischaemic scar suggests a potential risk for sudden cardiac death. CMR may be a useful tool for risk stratification in R-ACAOS patients. [Extracted from the article]

Aims We assessed the feasibility of cardiac magnetic resonance (CMR) and the role of myocardial strain in the diagnostic work-up of patients with acute myocardial infarction (AMI) and a clinical suspicion of cardiac rupture (CR). Methods and results Consecutive patients with AMI complicated by CR who underwent CMR were enrolled. Traditional and strain CMR findings were evaluated; new parameters indicating the relative wall stress between AMI and adjacent segments, named wall stress index (WSI) and WSI ratio, were analysed. A group of patients admitted for AMI without CR served as control. 19 patients (63% male, median age 73 years) met the inclusion criteria. Microvascular obstruction (MVO, P = 0.001) and pericardial enhancement (P < 0.001) were strongly associated with CR. Patients with clinical CR confirmed by CMR exhibited more frequently an intramyocardial haemorrhage than controls (P = 0.003). Patients with CR had lower 2D and 3D global radial strain (GRS) and global circumferential strain (in 2D mode P < 0.001; in 3D mode P = 0.001), as well as 3D global longitudinal strain (P < 0.001), than controls. The 2D circumferential WSI (P = 0.010), as well as the 2D and 3D circumferential (respectively, P < 0.001 and P = 0.042) and radial WSI ratio (respectively, P < 0.001 and P : 0.007), were higher in CR patients than controls. Conclusion CMR is a safe and useful imaging tool to achieve the definite diagnosis of CR and an accurate visualization of tissue abnormalities associated with CR. Strain analysis parameters can give insights into the pathophysiology of CR and may help to identify those patients with sub-acute CR. [ABSTRACT FROM AUTHOR]

Aims Myocardial longitudinal strain (LS) by two-dimensional (2D) speckle-tracking echocardiography has a diagnostic and prognostic role in cardiac amyloidosis (CA). Typically, the apical segments of the left ventricle (LV) are less affected by LS abnormalities, a finding called relative apical sparing (RELAPS). Whether a variable burden of CA might explain the RELAPS remains unknown. We aimed to evaluate the extent, distribution, and deposition pattern of amyloid in autopsy hearts of CA patients and to correlate the histopathology findings with 2D echocardiography. Methods and results This is a retrospective study of whole heart specimens of CA patients who died and underwent autopsy and 2D echocardiography. Amyloid burden quantification was assessed by histomorphometry in each segment at different LV levels. The LS analysis results were compared with the amyloid burden and the base-to-apex distribution. Histopathology investigation of 27 hearts with CA [immunoglobulin light chains (AL) 17 cases and transthyretin (ATTR) 10 cases] demonstrated an amyloid base-to-apex gradient. In 11 CA patients with 2D echocardiography, analysis of LS and histological amyloid burden allowed to identify different patterns: RELAPS (8 cases, 73%), with (2) or without (6) amyloid gradient, normal or mildly reduced LS with diffuse low amyloid (2, 18%), and severely reduced LS with diffuse high amyloid (1, 9%). Conclusion The typical RELAPS pattern at echocardiography is not always explained by a base-to-apex gradient of amyloid burden at histopathology, suggesting that RELAPS might be an epiphenomenon of complex interactions among amyloid infiltration, myocardial structure, and adaptation. [ABSTRACT FROM AUTHOR]

Aims: The aim of this study is to evaluate the clinical features of patients affected by arrhythmogenic cardiomyopathy (AC), presenting with chest pain and myocardial enzyme release in the setting of normal coronary arteries ('hot phase').Methods and Results: We collected detailed anamnestic, clinical, instrumental, genetic, and histopathological findings as well as follow-up data in a series of AC patients who experienced a hot phase. A total of 23 subjects (12 males, mean age at the first episode 27 ± 16 years) were identified among 560 AC probands and family members (5%). At first episode, 10 patients (43%) already fulfilled AC diagnostic criteria. Twelve-lead electrocardiogram recorded during symptoms showed ST-segment elevation in 11 patients (48%). Endomyocardial biopsy was performed in 11 patients, 8 of them during the acute phase showing histologic evidence of virus-negative myocarditis in 88%. Cardiac magnetic resonance was performed in 21 patients, 12 of them during the acute phase; oedema and/or hyperaemia were detected in 7 (58%) and late gadolinium enhancement in 11 (92%). At the end of follow-up (mean 17 years, range 1-32), 12 additional patients achieved an AC diagnosis. Genetic testing was positive in 77% of cases and pathogenic mutations in desmoplakin gene were the most frequent. No patient complained of sustained ventricular arrhythmias or died suddenly during the 'hot phase'.Conclusion: 'Hot phase' represents an uncommon clinical presentation of AC, which often occurs in paediatric patients and carriers of desmoplakin gene mutations. Tissue characterization, family history, and genetic test represent fundamental diagnostic tools for differential diagnosis. [ABSTRACT FROM AUTHOR]

The article presents the discussion on the history of arrhythmogenic cardiomyopathy highlighting the early discoverers and recent genetic work. Topics include discussing the history of arrhythmogenic cardiomyopathy (AC) focusing on the discoveries from the beginning of modern medicine; and autopsy revealing an extensive fibro-fatty replacement of the Right Ventricle free wall.

Aims: Whether a desmosomal (DS)-gene defect may in itself induce life-threatening ventricular arrhythmias regardless of phenotypic expression of arrhythmogenic right ventricular cardiomyopathy (ARVC) is still debated. This prospective study evaluated the long-term outcome of DS-gene mutation carriers in relation to the ARVC phenotypic expression.Methods and Results: The study population included 116 DS-gene mutation carriers [49% males; median age 33 years (16-48 years)] without prior sustained ventricular tachycardia (VT) or ventricular fibrillation (VF). The incidence of the arrhythmic endpoint, including sudden cardiac death (SCD), aborted SCD, sustained VT, and appropriate implantable cardioverter-defibrillator (ICD) intervention was evaluated prospectively and stratified by the presence of ARVC phenotype and risk factors (syncope, ventricular dysfunction, and non-sustained VT). At enrolment, 40 of 116 (34%) subjects fulfilled the criteria for definite ARVC while the remaining were either borderline or phenotype negatives. During a median follow-up of 8.5 (5-12) years, 10 patients (9%) had arrhythmic events (0.9%/year). The event rate was 2.3%/year among patients with definite ARVC and 0.2%/year among borderline or phenotype negative patients (P = 0.002). In patients with definite ARVC, the incidence of arrhythmias was higher in those with ≥1 risk factors (4.1%/year) than in those with no risk factors (0.4%/year, P = 0.02). Mortality was 0.2%/year (1 heart failure death and 1 SCD).Conclusions: The ARVC phenotypic expression is a prerequisite for the occurrence of life-threatening arrhythmias in DS-gene mutation carriers. The vast majority of malignant arrhythmic events occurred in patients with an overt disease phenotype and major risk factors suggesting that this subgroup most benefits from ICD therapy. [ABSTRACT FROM AUTHOR]

Although right ventricular (RV) failure is the main cause of death in patients with pulmonary arterial hypertension (PAH), there is insufficient data about the effects of PAH treatment on RV geometry and function mainly because the RV assessment has been hampered by its complex crescentic shape, large infundibulum, and its trabecular nature. Echocardiography is a widely available imaging technique particularly suitable for follow-up studies, because of its non-invasive nature, low cost, and lack of ionizing radiation or radioactive agent. Real-time three-dimensional echocardiography (RT3DE) has been shown to be accurate in assessing RV and left ventricular (LV) volumes, stroke volumes, and ejection fractions in comparison with cardiac magnetic resonance imaging. In this review, we describe RV structural and functional changes which occur in patients with PAH and strengths and weaknesses of current non-invasive imaging techniques to assess them. Finally, we describe an ongoing multicentre, prospective observational study involving seven centres expert in treating patients with PAH from four different countries. Investigators will use conventional and advanced echo parameters from RT3DE and speckle-tracking echocardiography to assess the extent of LV and RV remodelling before symptom onset and during pharmacological treatment in patients with PAH. Seventy patients who will survive for at least 1 year will be recruited. All the participating institutions will perform comprehensive standard 2D and Doppler as well as RT3DE examinations with a pre-defined imaging protocol. Measurements will be performed at the core echocardiography laboratory by experienced observers who will be unaware of each patient's treatment assignment and whether the examination was a baseline or a follow-up study. Enrolment duration is expected to be 1 year. [ABSTRACT FROM PUBLISHER]

The article presents a case study of a 52-year-old man with metastatic carcinoid disease due to breath shortness and peripheral oedema. His physical examination showed apulsatile and enlarged liver and holosystolic murmur above the left lower sternal border. It notes that a thickened tricuspid leaflet fixed in a semi-open position was found through three-dimensional echocardiography (3DE), a unique imaging technique for the assessment of the heart.