Abnormal conduction-induced cardiomyopathy: a poorly explored entity

A dyssynchronous biventricular activation, which can be determined by left bundle branch block, chronic right ventricular pacing, frequent premature ventricular complexes, or pre-excitation, can cause a global abnormal contractility, thus leading to systolic dysfunction and left ventricular remodelling in a unique nosological entities: abnormal conduction-induced cardiomyopathies. In this clinical scenario, the mainstay therapy is eliminating or improving LV dyssynchrony, removing the trigger. This usually ensures the improvement and even recovery of cardiac geometry and left ventricular function, especially in the absence of genetic background. A multidisciplinary approach, integrating advanced multimodal imaging, is essential for the systematic aetiological definition and the subsequent evaluation and aetiology-guided therapies of patients and their families. This review aims to describe mechanisms, prevalence, risk factors, and diagnostic and therapeutic approach to the various abnormal conduction-induced cardiomyopathies, starting from reasonable certainties and then analysing the grey areas requiring further studies

Teaching Modern Languages on Ancient Roots. Anche le pietre parlano

Possono le pietre raccontarci qualcosa? Si possono imparare le lingue osservando i resti archeologici di Verona, Tarragona e Kalamata? Perché le lingue hanno una storia? Perché abbiamo bisogno di rappresentare le parole? In questo volume poniamo anche altri interrogativi e cerchiamo alcune risposte

Left ventricular wall thickness and severity of cardiac disease in women and men with transthyretin amyloidosis

Aims: Cardiac amyloidosis (CA) is due to a deposition of amyloid fibrils in the heart causing an increase in wall thickness. A left ventricular (LV) wall thickness ≥12 mm plus at least one red flag should raise the suspicion of CA. As normal values of LV wall thickness are lower in women, the adoption or the same cut-off values for men and women could lead to underdiagnosis or delayed diagnosis in women. We investigated the relationship between LV wall thickness and the severity of cardiac involvement in women and men with transthyretin (ATTR) CA. Methods and results: We evaluated 330 consecutive patients diagnosed with ATTR-CA at three centres (Pisa, n = 232; Brescia, n = 69; Trieste, n = 29). Interventricular septum (IVS) and posterior wall (PW) thickness values were lower in women (n = 53, 16%) than men, but most differences were abolished when indexing by body surface area (BSA), height, or height, suggesting similar disease severity when accounting for the smaller body size of women. PW thickness indexed for height was even higher in women. We also searched for correlations between IVS and PW thickness and other indicators of the severity of cardiac disease. IVS values indexed by height displayed tighter associations with N-terminal pro-B-type natriuretic peptide values than non-indexed IVS values. Similarly, indexed values displayed closer relationships with relative wall thickness, E/e' ratio, and tricuspid annular plane systolic excursion. Conclusions: Indexed LV wall thickness values, particularly by height, reflect more accurately the severity of cardiac involvement than non-indexed values

P2X7 receptor involvement in the pathogenesis of Hidradenitis Suppurativa

The P2X7 receptor (P2X7R) is an ATP-gated plasma membrane ion channel widely distributed in human tissues, the highest expression being in cells of the immune and inflammatory systems, especially of the myeloid lineage [1]. The P2X7R plays different functions depending on the cell type and agonist concentration. One of the most relevant P2X7R activities consists in the regulation of inflammatory responses mainly through NLRP3 inflammasome activation and IL-1β processing and release [2]. Hidradenitis suppurativa (HS) is a chronic skin disease characterized by recurrent, painful nodules and abscesses of apocrine glands. Follicular occlusion and subsequent rupture are central events in HS. HS pathogenesis is not yet understood and many researches have recently focused on possible involvement of components of the immune system, mainly cytokines. Indeed, dysregulated cytokine expression, e.g. IL-1β, TNF-α, IL-6, was found in skin, plasma and immune cells of HS patients [3-6]. The aim of this study was to investigate a possible role of P2X7R in the pathogenesis of HS, also in view of a possible use of P2X7R antagonists in the treatment of this pathology with few options for effective therapy. For this 30 HS patients compared to 30 matched healthy control subjects, have been studied as regard skin biopsies, plasma and peripheral blood mononuclear cells (PBMC). Results show increased P2X7R expression in skin biopsies of HS patients respect to healthy controls (Fig. 1). Higher P2X7R immunostaining was shown by cheratinocytes as well as by some inflammatory cells in the derma, mainly macrophages and plasma cells. Plasma IL-1β levels were increased in HS patients respect to healthy controls suggesting a role for this cytokine in disease development. In apparent contrast with the last finding, PBMC from HS patients appeared defective in IL-1β release upon P2X7R stimulation. This is in agreement with deregulated and compartmentalized cytokine responses found by other authors [3,6]. Further research is necessary to identify the main source of circulating IL-1β as well as the expression of inflammasome components in HS lesional and possibly perilesional skin. Open image in new window Figure 1. Increased P2X7 immunostaining of skin section from HS patient (A) compared to control subject (B). 1. Bours MJ, Dagnelie PC, Giuliani AL, Wesselius A, Di Virgilio F (2011) P2 receptors and extracellular ATP: a novel homeostatic pathway in inflammation. Front Biosci (Schol Ed) 3:1443-1456. 2. Giuliani AL, Sarti AC, Falzoni S, Di Virgilio F (2017) The P2X7 receptor-interleukin-1 liaison. Front Pharmacol 8:123. 3. Kelly G, Hughes R, Mc Garry T, van den Born M, Adamzik K, Fitzgerald R, Lawlor C, Tobin AM, Sweeney CM, Kirby B (2015) Dysregulated cytokine expression in lesional and nonlesional skin in hidradenitis suppurativa. Br J Dermatol 173:1431-1439. 4. Xu H, Xiao X, He Y, Zhang X, Li C, Mao Q, Wu X, Wang B (2017) Increased serum interleuikin-6 levels in patients with hidradenitis suppurativa. Postepy Dermatol Alergol 34:82-84. 5. Van der Zee HH, de Ruiter L, van der Broecke DG, Dik WA, Laman JD, Pren EP (2011) Elevated levels of tumor necrosis factor (TNF)-alpha, interleukin (IL)-1beta and IL-10 in hidradenitis suppurativa skin: a rationale for targeting TNF-alpha and IL-1beta. Br J Dermatol 164:1292-1298. 6. Kanni T, Tzanetakou V, Savva A, Kersten B, Pistiki A, van der Veerdonk FL, Netea MG, van der Meer J, Giamarellos-Bourboulis EJ (2015) Compartimentalized cytokine responses in hidradenitis suppurativa. PLoS One 10:e0130522

Conoscere e Curare il Cuore 2024 - Le cardiomiopatie da disturbo di conduzione

Capitolo dedicato alle cardiomiopatie da disturbo di conduzion