Aldostefano Porcari
A5073996612- Amyloidosis: Diagnosis, Treatment, Outcomes
- Parathyroid Disorders and Treatments
- Cardiomyopathy and Myosin Studies
- Viral Infections and Immunology Research
- Eosinophilic Disorders and Syndromes
- Peptidase Inhibition and Analysis
- Medical Imaging and Pathology Studies
- Cardiovascular Function and Risk Factors
- Sarcoidosis and Beryllium Toxicity Research
- Cardiovascular Effects of Exercise
- Neuroendocrine Tumor Research Advances
- Cardiac Imaging and Diagnostics
- Atrial Fibrillation Management and Outcomes
- Cardiac electrophysiology and arrhythmias
- Trace Elements in Health
- Advanced MRI Techniques and Applications
- SARS-CoV-2 and COVID-19 Research
- Cardiac Structural Anomalies and Repair
- Pericarditis and Cardiac Tamponade
- IgG4-Related and Inflammatory Diseases
- Pancreatitis Pathology and Treatment
- Cardiac Valve Diseases and Treatments
- Lanthanide and Transition Metal Complexes
- Cardiac and Coronary Surgery Techniques
- Liver Disease Diagnosis and Treatment
Azienda Sanitaria Universitaria Integrata di Trieste
2018-2025
University of Trieste
2018-2025
The Royal Free Hospital
2022-2025
University College London
2022-2025
European Hospital
2024-2025
ERN GUARD-Heart
2022-2024
Children's Cancer and Leukaemia Group
2022-2024
Amyloidosis Foundation
2022-2024
Brigham and Women's Hospital
2024
Royal Free London NHS Foundation Trust
2022-2024
Diagnostic and therapeutic advances have led to much greater awareness of transthyretin cardiac amyloidosis (ATTR-CA). We aimed characterize changes in the clinical phenotype patients diagnosed with ATTR-CA over past 20 years. This is a retrospective observational cohort study all referred National Amyloidosis Centre (2002-2021) whom was differential diagnosis. identified 2995 suspected ATTR-CA, 1967 had diagnosis confirmed. Analysis by 5-year periods revealed an incremental increase...
Restrictive cardiomyopathy (RCM) is a heterogeneous group of diseases characterized by restrictive left ventricular pathophysiology, i.e. rapid rise in pressure with only small increases filling volume due to increased myocardial stiffness. More precisely, the defining feature RCM coexistence persistent diastolic dysfunction, non-dilated ventricles, and atrial dilatation, regardless wall thickness systolic function. Beyond this shared haemodynamic hallmark, phenotypic spectrum wide. The...
Abstract Transthyretin cardiac amyloidosis (ATTR-CA) is an increasingly recognized cause of heart failure (HF) and mortality worldwide. Advances in non-invasive diagnosis, coupled with the development effective treatments, have shifted ATTR-CA from a rare untreatable disease to relatively prevalent condition that clinicians should consider on daily basis. Amyloid fibril formation results age-related homoeostatic mechanisms wild-type ATTR (ATTRwt) (non-hereditary form) or destabilizing...
Abstract Aims The aims of this study were to assess prescription patterns, dosages, discontinuation rates, and association with prognosis conventional heart failure medications in patients transthyretin cardiac amyloidosis (ATTR-CA). Methods results A retrospective analysis all consecutive diagnosed ATTR-CA at the National Amyloidosis Centre between 2000 2022 identified 2371 ATTR-CA. Prescription was greater among a more severe phenotype, comprising beta-blockers 55.4%,...
Transthyretin amyloid cardiomyopathy (ATTR-CM) is often assumed to be associated with wild-type TTR genotype (ATTRwt) in elderly patients (aged ≥70), some of whom are not offered genetic testing. We sought estimate the prevalence, clinical characteristics and prognostic implications transthyretin (TTR) variants among diagnosed ATTR-CM.Data from consecutive over 70 years age ATTR-CM at UK National Amyloidosis Centre between January 2010 August 2022 were retrospectively evaluated. All...
Abstract Aims To perform evaluation of widely embraced bone scintigraphy-based non-biopsy diagnostic criteria (NBDC) for ATTR amyloid cardiomyopathy (ATTR-CM) in clinical practice, and to refine serum free light chain (sFLC) ratio cut-offs that reliably exclude monoclonal gammopathy (MG) chronic kidney disease. Methods results A multi-national retrospective study 3354 patients with suspected or histologically proven cardiac amyloidosis (CA) referred specialist centres from 2015 2021;...
Transthyretin cardiac amyloidosis (ATTR-CA) is a progressive cardiomyopathy. The clinical course varies among individuals and there are no established measures to assess disease progression. goal of this study was the prognostic importance an increase in N-terminal pro–B-type natriuretic peptide (NT-proBNP) outpatient diuretic intensification (ODI) as markers progression large cohort patients with ATTR-CA. We evaluated landmark survival analysis based on worsening NT-proBNP requirement for...
BACKGROUND: The extent of myocardial bone tracer uptake with technetium pyrophosphate, hydroxymethylene diphosphonate, and 3,3-diphosphono-1,2-propanodicarboxylate in transthyretin amyloid cardiomyopathy (ATTR-CM) might reflect cardiac burden be associated outcome. METHODS: Consecutive patients ATTR-CM who underwent diagnostic scintigraphy acquisition whole-body planar single-photon emission computed tomography (SPECT) images from the National Amyloidosis Centre 4 Italian centers were...
Lay summary•In this manuscript we describe the phenotype and prognosis of patients with cardiac amyloidosis (CA) hypertension diabetes.•A history is common in a diagnosis should not be overlooked these patients.•Diabetes may influence amyloidosis.AbstractBackgroundHypertension diabetes increase risk adverse events general population cardiomyopathies, however, their role still unclarified.Aimsto evaluate effect on clinical outcomes CA.MethodsData from 5 Italian Amyloidosis Referral Centres...
Low QRS voltages (LQRSVs) are a common electrocardiographic feature in patients with light chain amyloidosis (AL) and transthyretin (ATTR) cardiac (CA).The aim of this study was to identify clinical echocardiographic correlates LQRSV investigate their prognostic significance CA.This multicenter, retrospective performed 6 CA referral centers including consecutive AL ATTR CA. LQRSVs were defined as amplitude ≤5 mm (0.5 mV) all peripheral leads. The outcome cardiovascular (CV)...
Abstract Aims Cardiac amyloidosis (CA) is due to a deposition of amyloid fibrils in the heart causing an increase wall thickness. A left ventricular (LV) thickness ≥12 mm plus at least one red flag should raise suspicion CA. As normal values LV are lower women, adoption or same cut‐off for men and women could lead underdiagnosis delayed diagnosis women. We investigated relationship between severity cardiac involvement with transthyretin (ATTR) Methods results evaluated 330 consecutive...
The 6-minute walk test (6MWT) represents a comprehensive functional assessment that is commonly used in patients with heart failure; however, data are lacking transthyretin cardiac amyloidosis (ATTR-CA).
Transthyretin amyloid cardiomyopathy (ATTR-CM) has been traditionally considered a rare and inexorably fatal condition. ATTR-CM now is an increasingly recognized cause of heart failure (HF) mortality worldwide with effective pharmacological treatments. Advances in non-invasive diagnosis, coupled the development treatments, have transformed diagnosis ATTR-CM, which possible without recourse to endomyocardial biopsy ≈70 % cases. Many patients are diagnosed at earlier stage. Echocardiography...
Cardiac involvement is the main driver of clinical outcomes in systemic amyloidosis and preliminary studies support hypothesis that myocardial ischaemia contributes to cellular damage. The aims this study were assess presence mechanisms using cardiovascular magnetic resonance (CMR) with multiparametric mapping histopathological assessment.
Transthyretin cardiac amyloidosis (ATTR-CA) is a progressive and ultimately fatal cardiomyopathy. Biomarkers reflecting multiorgan dysfunction are of increasing importance in patients with heart failure; however, their significance ATTR-CA remains largely unknown. The aims this study were to characterize the multifaceted nature using blood biomarkers assess association between prognosis. This retrospective cohort 2566 consecutive diagnosed 2007 2023. Anemia (39%), high urea (52%),...
Cardiopulmonary exercise testing (CPET) has an established role in the assessment of patients with heart failure. However, data are lacking transthyretin (ATTR) amyloidosis.
Importance Patients with transthyretin (ATTR) cardiac amyloid infiltration are increasingly diagnosed at earlier disease stages no heart failure (HF) symptoms and a wide range of infiltration. Objective To characterize the clinical phenotype natural history asymptomatic patients ATTR Design, Setting, Participants This cohort study analyzed data all 12 international centers for amyloidosis from January 1, 2008, through December 31, 2023. Inclusion criteria were infiltration, defined as an...