Clinical and imaging characteristics of patients with cardiac amyloidosis- a single center observational study

Area of Science:

• Cardiology
• Cardiovascular Imaging
• Amyloidosis Research

Background:

• Amyloidosis involves protein fibril deposition, with cardiac involvement significantly impacting prognosis.
• Distinguishing between transthyretin (ATTR) and amyloid light-chain (AL) amyloidosis is crucial for patient management.
• Multimodality imaging plays a key role in assessing cardiac involvement and outcomes in amyloidosis.

Purpose of the Study:

• To investigate the clinical characteristics, outcomes, and long-term mortality in patients with ATTR and AL amyloidosis.
• To compare the prevalence of cardiac involvement and associated risk factors between ATTR and AL amyloidosis.
• To identify imaging predictors of mortality in cardiac amyloidosis.

Main Methods:

• Retrospective cohort study of 94 patients diagnosed with amyloidosis (2010-2022).
• Utilized multimodality imaging including ECG, echocardiography, and cardiac magnetic resonance (CMR).
• Performed survival analyses to determine mortality predictors.

Main Results:

• ATTR amyloidosis patients were older, with more males, and higher prevalence of cardiac involvement (e.g., atrial fibrillation) compared to AL amyloidosis.
• AL amyloidosis patients had more frequent pleural and pericardial effusions.
• Left atrial dilatation and pleural effusion on echocardiography were strong mortality predictors, while typical late gadolinium enhancement (LGE) on CMR was not.

Conclusions:

• Both AL and ATTR amyloidosis are associated with poor prognoses.
• Cardiac involvement, specifically left atrial dilatation and pleural effusion, are significant predictors of mortality.
• Typical LGE on CMR is not a reliable predictor of mortality in cardiac amyloidosis.