Estimated total amyloid burden from 18F-florbetaben PET predicts all-cause mortality in light-chain cardiac amyloidosis.

Background And Aims: The positron emission tomography (PET) tracer 18F-florbetaben is a promising diagnostic tool for light-chain cardiac amyloidosis (AL-CA). A greater cardiac uptake might signal more amyloid burden and a worse outcome. We aimed to assess the prognostic significance of 18F-florbetaben uptake in AL-CA.

Exercise limitations in amyloid cardiomyopathy assessed by cardiopulmonary exercise testing-A multicentre study.

Aims: Amyloid cardiomyopathy is caused by the deposition of light chain (AL) or transthyretin amyloid (ATTR) fibrils, that leads to a restrictive cardiomyopathy, often resulting in heart failure (HF) with preserved or reduced ejection fraction. This study aimed to determine whether cardiac output reduction or ventilation inefficiency plays a predominant role in limiting exercise in patients with amyloid cardiomyopathy.

Methods: We conducted a multicentre prospective study in patients with AL or ATTR cardiomyopathy who underwent cardiopulmonary exercise testing across four centres.

Clinical and prognostic significance of central and obstructive apneas in patients with transthyretin cardiac amyloidosis.

Aims: Central (CA) and obstructive apneas (OA) are highly prevalent in patients with chronic heart failure (HF) and transthyretin cardiac amyloidosis (ATTR-CA) is an increasingly recognized HF etiology. This study aimed to investigate the prevalence and impact of CA and OA in patients with ATTR-CA.

Methods: Consecutive patients with ATTR-CA underwent a 24-hour ambulatory cardiorespiratory monitoring to evaluate the prevalence and severity of breathing disorders.

Tetrameric Transthyretin as a Protective Factor Against Alzheimer's Disease.

Transthyretin (TTR) is a tetrameric protein traditionally recognized for its role in transporting thyroxine and retinol. Recent research has highlighted the potential neuroprotective functions of TTR in the setting of Alzheimer's disease (AD), which is the most common form of dementia and is caused by the deposition of amyloid beta (Aβ) and the resulting cytotoxic effects. This paper explores the mechanisms of TTR protective action, including its interaction with Aβ to prevent fibril formation and promote Aβ clearance from the brain.

Eligibility for vericiguat in a real-world, contemporary heart failure population.

Aims: Vericiguat is a soluble guanylate cyclase stimulator and improves survival in patients with heart failure (HF) with reduced ejection fraction (HFrEF) and an increased risk of decompensation. As real-world data on how many patients could be eligible for vericiguat therapy derive from outdated registries, we aimed to assess eligibility in a prospective cohort of patients with HF.

Methods And Results: Data from consecutive HF patients undergoing an elective ambulatory visit at five university hospitals from 3 July to 28 July 2023 were collected.

The Role of Scintigraphy with Bone Radiotracers in Cardiac Amyloidosis.

Cardiac amyloidosis (CA) is caused by the myocardial deposition of misfolded proteins, either amyloid transthyretin (ATTR) or immunoglobulin light chains (AL). The paradigm of this condition has transformed, since CA is increasingly recognized as a relatively prevalent cause of heart failure. Cardiac scintigraphy with bone tracers is the unique noninvasive technique able to confirm CA without performing tissue biopsy or advanced imaging tests.

Pathophysiology of Cardiac Amyloidosis.

Amyloidosis refers to a heterogeneous group of disorders sharing common pathophysiological mechanisms characterized by the extracellular accumulation of fibrillar deposits consisting of the aggregation of misfolded proteins. Cardiac amyloidosis (CA), usually caused by deposition of misfolded transthyretin or immunoglobulin light chains, is an increasingly recognized cause of heart failure burdened by a poor prognosis. CA manifests with a restrictive cardiomyopathy which progressively leads to biventricular thickening, diastolic and then systolic dysfunction, arrhythmias, and valvular disease.

Effects of sacubitril-valsartan on remodelling, fibrosis and mitochondria in a murine model of isoproterenol-induced left ventricular dysfunction.

Background: Sacubitril/valsartan has been demonstrated to promote left ventricular (LV) reverse remodelling and improve outcomes in patients with heart failure (HF) with reduced ejection fraction (EF). Its molecular and tissue effects have not been fully elucidated yet, due to the paucity of preclinical studies, mostly based on ischaemic models. We aimed to evaluate the effects of sacubitril/valsartan on LV remodelling, myocardial fibrosis and mitochondrial biology in a murine model of non-ischaemic LV dysfunction.

Case Report: Role of numerical simulations in the management of acute aortic syndromes.

Penetrating aortic ulcer (PAU) represents a subset of acute aortic syndromes characterized by high rupture risk and management challenges, particularly in elderly patients with significant comorbidities. This case report showcases a 75-year-old patient with a history of coronary artery bypass graft (CABG) and with multiple PAUs involving the aortic arch, deemed unfit for conventional open surgery. A branched aortic endograft with a pre-cannulated side component for the left subclavian artery (LSA) was employed to preserve the patency of the previous CABG.

Safety and efficacy of levosimendan in patients with cardiac amyloidosis.

Background: Patients with cardiac amyloidosis (CA) often experience heart failure (HF) episodes. No evidence is available on inotropic therapy. This study aims to fill this gap by examining the safety and efficacy of levosimendan.

Disease features and management of cardiomyopathies in women.

Over the last years, there has been a growing interest in the clinical manifestations and outcomes of cardiomyopathies in women. Peripartum cardiomyopathy is the only women-specific cardiomyopathy. In cardiomyopathies with X-linked transmission, women are not simply healthy carriers of the disorder, but can show a wide spectrum of clinical manifestations ranging from mild to severe manifestations because of heterogeneous patterns of X-chromosome inactivation.

[Obstructive sleep apneas and cardiovascular diseases].

Obstructive sleep apneas (OSA) are a breathing disorder characterized by recurrent apneas and hypopneas associated with complete or partial obstruction of the upper airways during sleep, resulting in disturbed sleep architecture, repeated hypoxemia and awakenings, and daytime sleepiness. OSA syndrome affects up to 34% of men and 17% of women in Western countries. Abnormalities in upper airway anatomy (frequently due to obesity), muscle tone, or neural control of breathing are the main causes.

[Critical reading of cardiovascular trials with neutral or negative findings].

Randomized controlled trials showing a significant benefit are met with enthusiasm because they may change the standard of care for patients who share the clinical and pathophysiologic characteristics of trial participants. Nonetheless, a well-designed and fully executed trial with neutral or negative findings also represents a critically important investigation deserving careful scientific scrutiny. In this paper we propose a 10-step approach to the interpretation of neutral or negative trials to exclude important methodological issues before concluding that the treatment really does not work.

Valvular heart disease in patients with cardiac amyloidosis.

Cardiac amyloidosis (CA) is an underdiagnosed condition caused by the deposition of misfolded proteins, namely immunoglobulin light chains and transthyretin, in the extracellular spaces of the heart. Any cardiovascular structure can be affected by amyloid infiltration, including the valves. Amyloid accumulation within the cardiac valves may lead to their structural and functional impairment, with a profound impact on patients' prognosis and quality of life.

Different Continuous Training Intensities Improve Echocardiographic Parameters, Quality of Life, and Functional Capacity in Heart Failure Patients with Reduced Ejection Fraction.

Background: Multiple comorbidities and physiological changes play a role in a range of heart failure conditions and influence the most effective approach to exercise-based rehabilitation. This research aimed to examine and compare the outcomes of continuous training at three different intensities, focusing on left ventricular (LV) remodeling, functional capacity, and quality of life among patients with heart failure with reduced ejection fraction (HFrEF).

Methods: In this randomized control trial, a total of 60 male patients (average age: 54.

Patient-reported outcome measures for transthyretin cardiac amyloidosis: the ITALY study.

Background: Transthyretin cardiac amyloidosis (ATTR-CA) has a deep impact on the quality of life (QoL), yet no specific patient-reported outcome measures (PROMs) for ATTR-CA exist.

Methods: The ITALY study involved 5 Italian referral centres (Pisa, Pavia, Ferrara, Florence, Messina) enrolling consecutive outpatients with ATTR-CA.

Results: Two 30-item questionnaires were created for wild-type (wt) and variant (v) ATTR-CA.