Impact of Gliflozins on Right Heart Remodeling in Italian Patients with Type 2 Diabetes and Heart Failure: Results from the GLISCAR Real-World Study.

: The effect of sodium-glucose cotransporter 2 inhibitors (SGLT2is) in addition to optimal medical therapy (OMT) on right ventricular (RV) systolic function in patients with heart failure with reduced ejection fraction (HFrEF) is not well established. This study aimed to assess the impact of SGLT2is on RV function using advanced echocardiographic parameters in patients with HFrEF and type 2 diabetes (T2D). : The real-world prospective, observational GLISCAR study enrolled 31 consecutive patients with T2D and HFrEF.

Prognostic Value of Malnutrition, Frailty, and Physical Performance in Transthyretin Cardiac Amyloidosis: Insights From a Prospective Multicenter Cohort Study.

Background: The prevalence of transthyretin cardiac amyloidosis among older adults (often octogenarians) is increasing. We aimed to determine whether age and geriatric syndromes bear any impact on the management and outcomes in transthyretin cardiac amyloidosis and assess the risk of ageism.

Methods: In a prospective, multicenter cohort study, 256 patients diagnosed with transthyretin cardiac amyloidosis from March 2021 to March 2024 underwent comprehensive geriatric assessment (CGA).

Change in prevalence of ATTR variants in Italy - Results from a National Survey.

Introduction: Hereditary transthyretin amyloidosis (ATTRv) is a rare, heterogenous, inherited disorder caused by over 130 gene mutations. Its prevalence was estimated to 4.33/million in 2020 in Italy.

Exercise Training and Rehabilitation in Cardiac Amyloidosis (ERICA) Study: Rationale and Design.

Notwithstanding the dramatic improvement associated with Tafamidis in Heart Failure due to wild-type transthyretin cardiac amyloidosis (ATTRwt-CA), the effect of exercise training on ATTRwt-CA remains unexplored. We hereby present rationale and design of the Exercise training and Rehabilitation in Cardiac Amyloidosis study. This interventional, controlled study will randomize ATTRwt-CA patients into a control group and a training group.

Advances in the Therapy of Light Chain Cardiac Amyloidosis.

Systemic light chain (AL) amyloidosis stems from abnormal production of amyloidogenic immunoglobulin light chains by plasma cells or B-cell disorders. It can present locally or systemically, with systemic forms posing significant mortality risks, especially if cardiac involvement is present. Delayed diagnosis due to nonspecific symptoms leads to progressive organ dysfunction.

RETRACTED: Monda et al. Left Ventricular Non-Compaction in Children: Aetiology and Diagnostic Criteria. 2024, , 115.

The journal retracts the article, titled "Left Ventricular Non-Compaction in Children: Aetiology and Diagnostic Criteria" [...

Genotype-Phenotype Correlations in ATTR Amyloidosis: A Clinical Update.

Hereditary transthyretin-related amyloidosis (hATTR) is the most common form of familial amyloidosis. It is an autosomal dominant disease caused by a pathogenic variant in the TTR gene. More than 140 TTR gene variants have been associated with hATTR, with the Val30Met variant representing the most common worldwide.

The Role of Echocardiography for the Clinical Diagnosis, Risk Stratification, and Management of Cardiac Amyloidosis.

Amyloidosis is a rare, heterogeneous group of diseases characterized by extracellular infiltration and deposition of misfolded fibrils in different organs and tissues. A timely diagnosis is important as it can improve outcome. Echocardiography has emerged as a powerful tool to prompt suspicion and refer patients to second-level evaluation to reach a definitive diagnosis.

OCT angiography indices and the choroidal vascularity index in wild-type transthyretin (TTR) amyloidosis (ATTRwt).

Purpose: Retinal angiopathy represents a well-known ocular manifestation of hereditary transthyretin amyloidosis (ATTRv). Until recently, there have been no reports on retinal changes in ATTRwt. In this retrospective observational clinical study, we aimed to determine whether vessel density (VD) indices and the choroidal vascularity index (CVI) could offer insights into retinal and choroidal vascular changes among patients affected by ATTRwt.

[Clinical and genetic manifestations of left ventricular non-compaction in children].

Left ventricular non-compaction (LVNC) is a myocardial disease characterized by a two-layered structure typically seen at the apical and lateral left portions of the ventricular myocardium, distal to the papillary muscles. While considered a rare disease, its prevalence in children is increasing due to the increased awareness of this condition and improved resolution of imaging techniques. The etiology is heterogeneous, ranging from inherited conditions to acquired diseases.

RETRACTED: Left Ventricular Non-Compaction in Children: Aetiology and Diagnostic Criteria.

Left ventricular non-compaction (LVNC) is a heterogeneous myocardial disorder characterized by prominent trabeculae protruding into the left ventricular lumen and deep intertrabecular recesses. LVNC can manifest in isolation or alongside other heart muscle diseases. Its occurrence among children is rising due to advancements in imaging techniques.

Cardiovascular Involvement in Fabry's Disease: New Advances in Diagnostic Strategies, Outcome Prediction and Management.

Cardiovascular involvement is common in Fabry's disease and is the leading cause of morbidity and mortality. The research is focused on identifying diagnostic clues suggestive of cardiovascular involvement in the preclinical stage of the disease through clinical and imaging markers. Different pathophysiologically driven therapies are currently or will soon be available for the treatment of Fabry's disease, with the most significant benefit observed in the early stages of the disease.

Targeted Therapies in Pediatric and Adult Patients With Hypertrophic Heart Disease: From Molecular Pathophysiology to Personalized Medicine.

Hypertrophic cardiomyopathy is a myocardial disease defined by an increased left ventricular wall thickness not solely explained by abnormal loading conditions. It is often genetically determined, with sarcomeric gene mutations accounting for around 50% of cases. Several conditions, including syndromic, metabolic, infiltrative, and neuromuscular diseases, may present with left ventricular hypertrophy, mimicking the hypertrophic cardiomyopathy phenotype but showing a different pathophysiology, clinical course, and outcome.

Prevalence and clinical significance of right ventricular pulmonary arterial uncoupling in cardiac amyloidosis.

Background: This study aims to evaluate the prevalence and the clinical significance of the right ventricular pulmonary arterial (RV-PA) uncoupling in patients with cardiac amyloidosis (CA).

Methods: The study population consisted in 92 consecutive patients with CA (age 71.1 ± 12.

Imaging findings of right cardiac amyloidosis: impact on prognosis and clinical course.

Cardiac involvement from amyloidosis is of growing interest in the overall literature. Despite cardiac amyloidosis (CA) has been considered for a long time a rare disease, the diagnostic awareness is increasing mainly thanks to the improvement of diagnostic softwares and of imaging techniques such as cardiac magnetic resonance  (CMR). Some authors have observed an increase of prevalence rate of CA; moreover it's often underestimated because clinical manifestations are aspecific.