Publications by authors named "Alberto Ponziani"
Article Synopsis
- The study investigates the incidence, mechanisms, and predictors of sudden death (SD) in patients with cardiac amyloidosis, a serious heart condition.
- It analyzed data from 784 patients with either ATTR or AL cardiac amyloidosis, finding that SD is more common in AL patients compared to those with ATTR.
- Key risk factors for SD include previous pacemaker implantation for ATTR patients and the use of beta-blockers and advanced heart failure symptoms (NYHA III-IV) for AL patients.
Background: The introduction of a noninvasive diagnostic algorithm in 2016 led to increased awareness and recognition of cardiac amyloidosis (CA).
Objectives: The purpose of this study was to analyze the impact of the introduction of the noninvasive diagnostic algorithm on diagnosis and prognosis in a multicenter Italian CA cohort.
Methods: This was a retrospective analysis of 887 CA patients from 5 Italian Cardiomyopathies Referral Centers: 311 light-chain CA, 87 variant transthyretin (TTR)-related CA, 489 wild-type TTR-related CA.
Aims: Transthyretin cardiac amyloidosis (ATTR-CA) is a rare and progressive cardiomyopathy caused by amyloid fibril deposition in myocardial tissue. Diagnostic challenges have historically hampered timely detection. Recent advances in noninvasive diagnostic techniques have facilitated ATTR-CA diagnosis.
View Article and Find Full Text PDFArticle Synopsis
- * Early detection is crucial, as the combination of these conditions worsens patient prognosis without proper intervention, and diagnosing can be achieved through clinical signs and non-invasive tests.
- * Percutaneous treatment for aortic valve disease shows better survival outcomes compared to medical therapy alone, and while cardiac amyloidosis does not significantly affect survival post-valve replacement, it may lead to higher rehospitalization rates; disease-modifying treatments for amyloidosis are also under consideration for improving patient outcomes.
Article Synopsis
- The study aimed to explore the prevalence of transthyretin amyloidosis variant cardiomyopathy (ATTRv-CM) among relatives at risk, assess the effectiveness of repeated evaluations, and analyze first-line diagnostic methods like ECG and echocardiogram.
- Among 159 relatives evaluated, 25% were diagnosed with ATTRv-CM, and half of those diagnosed exhibited heart failure symptoms, showcasing a high negative predictive value for screening based on specific criteria.
- The findings emphasize the need for ongoing monitoring, as 13% of patients with ATTRv-CM showed no initial cardiac signs, suggesting regular follow-up testing is crucial for early detection and management.
Article Synopsis
- The study focused on hereditary transthyretin amyloidosis (ATTRv), examining a cohort of 325 patients with various TTR gene mutations over nearly four decades at a tertiary center in Bologna, Italy.
- The research identified three main phenotypes (cardiac, neurological, and mixed), with significant findings that asymptomatic mutation carriers generally had a prognosis similar to healthy individuals and highlighted the importance of family screening for early detection.
- Key determinants of survival included age at diagnosis, heart function measures, and disease-modifying therapies, indicating that ongoing treatment plays a crucial role in managing ATTRv and improving outcomes for affected patients.
Objective: We sought to investigate prevalence, incidence and prognostic implications of permanent pacemaker (PPM) implantation in patients with cardiac amyloidosis (CA), thereby identifying the predictors of time to PPM implantation.
Methods: Seven hundred eighty-seven patients with CA (602 men, median age 74 years, 571 transthyretin amyloidosis (ATTR), 216 light-chain amyloidosis (AL)) evaluated at two European referral centres were retrospectively included. Clinical, laboratory and instrumental data were analysed.
Cardiac amyloidosis is a serious and progressive infiltrative disease caused by the deposition of amyloid fibrils in the heart. In the last years, a significant increase in the diagnosis rate has been observed owing to a greater awareness of its broad clinical presentation. Cardiac amyloidosis is frequently associated to specific clinical and instrumental features, so called "red flags", and it appears to occur more commonly in particular clinical settings such as multidistrict orthopedic conditions, aortic valve stenosis, heart failure with preserved or mildly reduced ejection fraction, arrhythmias, plasma cell disorders.
View Article and Find Full Text PDFAims: 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) in chronic kidney disease.
Methods And Results: A multi-national retrospective study of 3354 patients with suspected or histologically proven cardiac amyloidosis (CA) referred to specialist centres from 2015 to 2021; evaluations included radionuclide bone scintigraphy, serum and urine immunofixation, sFLC assay, eGFR measurement and echocardiography. Seventy-nine percent (1636/2080) of patients with Perugini grade 2 or 3 radionuclide scans fulfilled NBDC for ATTR-CM through absence of a serum or urine monoclonal protein on immunofixation together with a sFLC ratio falling within revised cut-offs incorporating eGFR; 403 of these patients had amyloid on biopsy, all of which were ATTR type, and their survival was comparable to non-biopsied ATTR-CM patients (p = 0.
[Ten questions about transthyretin amyloidosis].
G Ital Cardiol (Rome)
September 2022
Systemic amyloidosis is a hereditary or acquired disease characterized by deposition of amyloid insoluble fibrils into body organs and tissues, causing structural abnormalities and organ dysfunction, i.e. heart failure.
View Article and Find Full Text PDFAims: The use of beta-blocker therapy in cardiac amyloidosis (CA) is debated. We aimed at describing patterns of beta-blocker prescription through a nationwide survey.
Methods And Results: From 11 referral centres, we retrospectively collected data of CA patients with a first evaluation after 2016 (n = 642).