Publications by authors named "G Sinigiani"

Importance: Patients with transthyretin (ATTR) cardiac amyloid infiltration are increasingly diagnosed at earlier disease stages with no heart failure (HF) symptoms and a wide range of cardiac amyloid infiltration.

Objective: To characterize the clinical phenotype and natural history of asymptomatic patients with ATTR cardiac amyloid infiltration.

Design, Setting, And Participants: This cohort study analyzed data of all patients at 12 international centers for amyloidosis from January 1, 2008, through December 31, 2023.

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Article Synopsis
  • Cardiac amyloid infiltration significantly impacts survival in systemic light-chain (AL) amyloidosis, with guidelines suggesting early therapy changes for patients not responding well, regardless of cardiac amyloid severity.
  • The study aimed to compare serum biomarkers, echocardiography, and cardiovascular magnetic resonance (CMR) with extracellular volume (ECV) mapping in understanding cardiac amyloid and its prognostic value.
  • Findings revealed that ECV is a strong independent predictor of mortality, showing different relationships between the depth of hematological response and survival based on specific ECV thresholds over 1- and 6-month periods.
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Article Synopsis
  • This study analyzed the effectiveness and tolerability of SGLT2 inhibitors in patients with transthyretin cardiomyopathy (ATTR-CM), a condition previously excluded from related clinical trials.
  • Out of 2,356 ATTR-CM patients, 260 received SGLT2 inhibitors, and after matching for certain variables, it was found that SGLT2i treatment led to less deterioration in heart failure symptoms and better kidney function over time.
  • The findings suggest that SGLT2i treatment not only had a low discontinuation rate but also significantly reduced the risks of all-cause mortality, cardiovascular mortality, and heart failure hospitalizations in these patients.
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Background: Atrial fibrillation (AF) is common in patients with cardiac amyloidosis (CA) and is a significant risk factor for heart failure hospitalization and thromboembolic events.

Objective: This study was designed to investigate the atrial electrofunctional predictors of incident AF in CA.

Methods: A multicenter, observational study was conducted in 4 CA referral centers including sinus rhythm patients with light-chain (AL) and transthyretin (ATTR) CA undergoing electrocardiography and cardiac magnetic resonance imaging.

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