Background: In this study, we aimed to describe the impact of MBs on atherosclerosis and survival, in patients with coronary artery disease (CAD). Methods: We retrospectively studied 1920 consecutive patients who underwent conventional coronary angiography for suspected CAD. Atherosclerotic load (AL), defined as the sum of degrees of stenosis, and general atherosclerotic load (GAL), representing the sum of AL, were compared between patients with MB and a control group without MB; patients in these groups were similar in age and sex. We assessed survival at 10 years after the last enrolled patient. Results: Prevalence of MB was 3.96%, predominantly in the mid-segment of left anterior descendent artery (LAD). In the presence of MB, GAL was lower (158.1 ± 93.7 vs. 205.3 ± 117.9, p = 0.004) with a lesser AL in the proximal (30.3 ± 39.9 vs. 42.9 ± 41.1, p = 0.038) and mid-segments (8.1 ± 20.0 vs. 25.3 ± 35.9, p < 0.001) of LAD. Based on a Multinominal Logistic Regression, we found that the presence of MB on LAD (regardless of its location on this artery) is a protective factor against atherosclerotic lesions, decreasing the probability of significant stenosis, especially of those ≥70%, on the entire artery (B −1.539, OR 4660; 95% CI = 1.873–11.595, p = 0.001) and on each of its segments as well: proximal LAD (B −1.275, OR 0.280; 95% CI = 0.015–5.073; p = 0.038), mid-LAD (B −1.879, OR 6.545; 95% CI = 1.492–28.712; p = 0.013) and distal LAD (B −0.900, OR 2.459, 95% CI = 2.459–2.459, p = 0.032). However, 10-year survival was similar between groups (76.70% vs. 74.30%, p = 0.740). Conclusion: The presence of MB on LAD proved to be a protective factor against atherosclerosis for the entire artery and for each of its segments, but it does not influence long-term survival in patients with CAD.

Transthyretin amyloid cardiomyopathy (ATTR-CM) is an infiltrative disorder characterized by extracellular myocardial deposits of amyloid fibrils, with poor outcome, leading to heart failure and death, with significant treatment expenditure. In the era of a novel therapeutic arsenal of disease-modifying agents that target a myriad of pathophysiological mechanisms, timely and accurate diagnosis of ATTR-CM is crucial. Recent advances in therapeutic strategies shown to be most beneficial in the early stages of the disease have determined a paradigm shift in the screening, diagnostic algorithm, and risk classification of patients with ATTR-CM. The aim of this review is to explore the utility of novel specific non-invasive imaging parameters and biomarkers from screening to diagnosis, prognosis, risk stratification, and monitoring of the response to therapy. We will summarize the knowledge of the most recent advances in diagnostic, prognostic, and treatment tailoring parameters for early recognition, prediction of outcome, and better selection of therapeutic candidates in ATTR-CM. Moreover, we will provide input from different potential pathways involved in the pathophysiology of ATTR-CM, on top of the amyloid deposition, such as inflammation, endothelial dysfunction, reduced nitric oxide bioavailability, oxidative stress, and myocardial fibrosis, and their diagnostic, prognostic, and therapeutic implications.

Left ventricular non-compaction (LVNC) with preserved ejection fraction (EF) is still a controverted entity. We aimed to characterize structural and functional changes in LVNC with heart failure with preserved EF (HFpEF). Methods: We enrolled 21 patients with LVNC and HFpEF and 21 HFpEF controls. For all patients, we performed CMR, speckle tracking echocardiography (STE), and biomarker assessment for HFpEF (NT-proBNP), for myocardial fibrosis (Galectin-3), and for endothelial dysfunction [ADAMTS13, von Willebrand factor, and their ratio]. By CMR, we assessed native T1 and extracellular volume (ECV) for each LV level (basal, mid, and apical). By STE, we assessed longitudinal strain (LS), globally and at each LV level, base-to-apex gradient, LS layer by layer, from epicardium to endocardium, and transmural deformation gradient. Results: In the LVNC group, mean NC/C ratio was 2.9 ± 0.4 and the percentage of NC myocardium mass was 24.4 ± 8.7%. LVNC patients, by comparison with controls, had higher apical native T1 (1061 ± 72 vs. 1008 ± 40 ms), diffusely increased ECV (27.2 ± 2.9 vs. 24.4 ± 2.5%), with higher values at the apical level (29.6 ± 3.8 vs. 25.2 ± 2.8%) (all p < 0.01); they had a lower LS only at the apical level (−21.4 ± 4.4 vs. −24.3 ± 3.2%), with decreased base-to-apex gradient (3.8 ± 4.7 vs. 6.9 ± 3.4%) and transmural deformation gradient (3.9 ± 0.8 vs. 4.8 ± 1.0%). LVNC patients had higher NT-proBNP [237 (156–489) vs. 156 (139–257) pg/mL] and Galectin-3 [7.3 (6.0–11.5) vs. 5.6 (4.8–8.3) ng/mL], and lower ADAMTS13 (767.3 ± 335.5 vs. 962.3 ± 253.7 ng/mL) and ADAMTS13/vWF ratio (all p < 0.05). Conclusion: LVNC patients with HFpEF have diffuse fibrosis, which is more extensive at the apical level, explaining the decrease in apical deformation and overexpression of Galectin-3. Lower transmural and base-to-apex deformation gradients underpin the sequence of myocardial maturation failure. Endothelial dysfunction, expressed by the lower ADAMTS13 and ADAMTS13/vWF ratio, may play an important role in the mechanism of HFpEF in patients with LVNC.

Carney complex (CNC) is a rare autosomal dominant syndrome. Spotty skin pigmentation is the major clinical manifestation of CNC, followed by cardiac myxomas, benign tumors that usually present with features from the classical triad of obstructive cardiac, embolic and non-specific constitutional symptoms (NCS). NCS are caused by the overproduction of interleukin-6 (IL-6), a pro-inflammatory cytokine which mediates the induction of intercellular adhesion molecule 1 (ICAM-1) and promotes endothelial dysfunction and atherosclerosis. Thus, myxomas may be directly linked to an increased risk of atherosclerotic events. We report here a case of a 74-year-old woman with left atrial myxoma, skin pigmentary abnormalities, thyroid disorder and extensive atherosclerosis, with non-embolic occlusion of infrarenal abdominal aorta.

Aims Numerous diagnostic criteria for excessive trabeculation (ET), or so-called “noncompaction”, score the extent of the trabecular layer, yet whether the trabeculations themselves have a negative or positive impact on pump function is largely unknown. This study aimed to measure the ejection fraction (EF) of the trabecular layer and its impact on pump function assessment. Methods and results We retrospectively analyzed cardiac magnetic resonance (CMR) findings in patients with ET of the left ventricle (LV). The LV was labelled into four regions: compact wall, central cavity (CC), trabeculations, and intertrabecular recesses (IR). For each label we calculated the systolic fractional volume change (SFVC) in short-axis images (n=15) and systolic fractional area change (SFAC) in 4-chamber images (n=30), by dividing end-systolic to end-diastolic values. We measured the EF of IR, CC, and total cavity (TC). Three methods to calculate EF of the TC were compared: trabeculations included (per guidelines), IR excluded (Jacquier criterion), trabeculations contoured and excluded (contour-EF). The SFVC and SFAC of the compact wall were similar with SFVC and SFAC of trabeculations (both P>0.05). In contrast, the IR were more diminished in systole by comparison with the CC, having lower SFVC (39±17% vs. 56±16%, P<0.001) and SFAC (37±22% vs. 72±12%, P<0.001). EF of the IR was also greater than EF of the CC (61±17% vs. 44±16%, P<0.001). Excluding IR from the TC or including trabeculations underestimates the contour-EF (44±16% and 40±12%, respectively, vs. 51±16%; both P<0.001). Conclusions The trabecular layer has a high EF. Values of key prognostic indicators are better when this is accounted for. Graphical AbstractEF, ejection fraction; LVEF, left ventricular ejection fraction; SAX, short-axis; 4c, 4 chamber. *P<0001