Dilated Cardiomyopathy. From Genetics to Clinical Management

The current definition of dilated cardiomyopathy (DCM) is relatively simple: it is a heart muscle disease characterized by left ventricular (LV) or biventricular dilation and systolic dysfunction in the absence of either pressure or volume overload or coronary artery disease sufficient enough to explain the dysfunction. In the last 30 years, prognosis of patients with DCM has dramatically been improved with few similarities in the history of cardiology and medicine. Typically, in the 1980s, the average survival rate was approximately 50% in a 5-year follow-up. Nowadays, at 10 years of follow-up, the survival/free from heart transplant rate is far beyond 85%, and the projection of this improvement is significantly better for those who have had DCM diagnosed in the late 2010s. This improvement in outcomes is fundamentally due to a better characterization of etiological factors, medical management for heart failure, and device treatment, like the implantable cardioverter defibrillator (ICD), for sudden cardiac death prevention. However, other milestones should be recognized for the improvement in the survival rate, namely, the early diagnosis due to familial and sport-related screening, which allow detection of DCM at a less severe stage, and the uninterrupted, active, and individualized long-term follow-up with continuous reevaluation of the disease and re-stratification of the risk

Oxygen Uptake Efficiency Slope at a Glance: A Fascinating Index Carrying Unsolved Questions

Since 1996, when it was first introduced by Baba et al, oxygen uptake (VO2) efficiency slope (OUES) has represented a controversial index of cardiopulmonary efficiency

Pragmatic electrocardiogram tracings in non-ischaemic dilated cardiomyopathy: diagnostic and prognostic role

Dilated cardiomyopathy (DCM) is a primitive heart muscle disease characterized by a great heterogeneous aetiology and prognostic outcome. Dilated cardiomyopathy is an umbrella term encompassing different aetiologies that might require specific treatments. It principally affects young and male adults, with high-risk arrhythmic competitive risk. Unfortunately, the prevention of major ventricular arrhythmic events remains a clinical challenge. In the era of advanced multimodality imaging and widely available genetic testing, electrocardiogram (ECG) continues to represent a reliable diagnostic tool, for specific work up of every single patient. However, approaching DCM patients, only a cardiomyopathy-oriented reading makes the role of ECG central in the management of DCM, both for diagnosis, prognosis, and therapeutic management. In this paper, we present four ECGs of four different DCM patients, in order to guide a cardiomyopathy-oriented ECG reading, emphasizing its impact in an early, cost-effective, and personalized diagnostic and prognostic work up in this specific setting

SGLT2-inhibitors: Should they be considered anti-remodeling drugs?

Heart failure (HF) is a complex syndrome characterized by multiple aetiologies and a progressive clinical course with a strong impact in terms of morbidity, mortality and public health costs. According to the neurohormonal hypothesis, HF with reduced ejection fraction (HFrEF) is considered a neurohormonal disease and HF patients benefit from the use of medications that interfere with and modulate the negative effects of neurohormonal systems (i.e. permanent renin–angiotensin–aldosterone system activation). The foundation of HF treatment includes the combination of well-known neurohormonal antagonists such as angiotensin receptor neprilysin inhibitors, beta-blockers, and mineralocorticoid receptor antagonists. However, recently, Sodium-Glucose Cotransporter-2 Inhibitors (SGLT2-i) have emerged as a new additional cornerstone of HF treatment – they are now regarded as one of the four keystone drugs to be introduced as first line therapy in HFrEF (class I recommended drug). Moreover, SGLT2-i have been shown to decrease combined endpoints of cardiovascular mortality and worsening HF regardless of ejection fraction (EF), and also to prevent the onset of HF in patients who are at high cardiovascular risk. The pathophysiologic mechanisms that may explain the benefit in clinical outcomes of the SGLT2-i in patients with HF are still incompletely understood. Therefore, it is of great interest to analyze the biological changes, which may occur in patients taking SGLT2-i because this may be helpful to elucidate how SGLT2-i may lead to improved cardiovascular outcomes. In this context, the metanalysis published in the European Journal of Internal Medicine by Fan et al. is timely and relevant because it evaluates the potential structural and functional impact of SGLT2-i in human heart giving possible translational understandings of the biologic consequences caused by SGLT2-i

Heart failure with reduced ejection fraction and monogenic dilated cardiomyopathy: distinct diseases? Insights from randomized controlled trials

The genetic component of heart failure with reduced ejection fraction (HFrEF) is traditionally considered as part of the non-ischaemic aetiology. Dilated cardiomyopathy (DCM) has been recognized as the most heterogeneous amongst the classical cardiomyopathy phenotypes, with >250 genes which have been causally related with the disease

Editorial: Proceedings and predictions in cardiac amyloidosis: unsolved mysteries and challenges for the future

Perceptions of amyloidosis have changed dramatically over the recent years following major advances in diagnosis and therapeutic strategies, especially in the field of cardiac amyloidosis

Notch1 signaling stimulates proliferation of immature cardiomyocytes

The identification of the molecular mechanisms controlling cardiomyocyte proliferation during the embryonic, fetal, and early neonatal life appears of paramount interest in regard to exploiting this information to promote cardiac regeneration. Here, we show that the proliferative potential of neonatal rat cardiomyocytes is powerfully stimulated by the sustained activation of the Notch pathway. We found that Notch1 is expressed in proliferating ventricular immature cardiac myocytes (ICMs) both in vitro and in vivo, and that the number of Notch1-positive cells in the heart declines with age. Notch1 expression in ICMs paralleled the expression of its Jagged1 ligand on non-myocyte supporting cells. The inhibition of Notch signaling in ICMs blocked their proliferation and induced apoptosis; in contrast, its activation by Jagged1 or by the constitutive expression of its activated form using an adeno-associated virus markedly stimulated proliferative signaling and promoted ICM expansion. Maintenance or reactivation of Notch signaling in cardiac myocytes might represent an interesting target for innovative regenerative therapy

Standardizing the role of endomyocardial biopsy in current clinical practice worldwide

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Pianeta Cuore 3.0 istruzioni per conoscerlo e mantenerlo sano

Il testo è caratterizzato dall’essenzialità, dalla schematicità e dalla chiarezza e nasce dalle domande più frequenti che i Pazienti e i familiari pongono sulle varie cardiopatie, prefazione all'edizione del 2010, e sugli aspetti diagnostico-terapeutici in Cardiologia. Indugia maggiormente sulla prevenzione cardiovascolare e le cardiopatie coronariche perché, attraverso la prevenzione è possibile ridurre i nuovi casi di malattia e le recidive di malattia, massimizzando i risultati delle procedure e tecnologie terapeutiche avanzate delle quali oggi disponiamo. Include un utilissimo glossario che illustra il significato delle parole più ricorrenti nel gergo cardiologico la cui comprensione non sempre è immediata