Cardiac amyloidosis without increased left ventricular wall thickness.

Area of Science:

• Cardiology
• Hematology
• Medical Diagnostics

Background:

• Immunoglobulin light chain (AL) cardiac amyloidosis is a condition affecting the heart.
• Left ventricular wall thickness (LVWT) is a key echocardiographic parameter.
• Assessing LVWT's impact on clinical outcomes in AL cardiac amyloidosis is important for patient management.

Purpose of the Study:

• To determine the frequency of normal LVWT in AL cardiac amyloidosis patients.
• To evaluate the influence of LVWT on clinical outcomes in this patient group.
• To identify predictors of survival in AL cardiac amyloidosis.

Main Methods:

• Retrospective analysis of 117 patients with systemic AL amyloidosis.
• Categorization into three groups: cardiac amyloidosis with LVWT >12 mm, LVWT ≤12 mm, and no cardiac amyloidosis.
• Comparison of echocardiographic parameters and survival rates across groups.

Main Results:

• No significant differences in left ventricular ejection fraction, left atrial volume index, e', or E/e' between patients with LVWT >12 mm and LVWT ≤12 mm.
• Patients without cardiac amyloidosis showed significantly better echocardiographic parameters.
• Median survival times varied significantly: 422 days (LVWT >12 mm), 729 days (LVWT ≤12 mm), and 2080 days (no cardiac amyloidosis).
• Age, NT-proBNP level, E/e', and complete hematologic remission predicted survival.

Conclusions:

• Approximately one-third of AL cardiac amyloidosis patients present with normal LVWT (≤12 mm).
• Normal LVWT does not preclude the diagnosis or impact of AL cardiac amyloidosis.
• Early and sensitive diagnostic methods are essential for timely intervention and improved survival, regardless of LVWT.
• Multivariate analysis identified key prognostic factors for survival in AL cardiac amyloidosis.