0224: Prevalence and prognostic significance of left-sided valvular thickening in patients with systemic light-chain amyloidosis

Background Cardiac involvement is frequent in systemic light chain amyloidosis (AL). Left-sided valvular thickening (LVT) have been described in AL reflecting heavy infiltration of the valvular endocardium by amyloid proteins. However, the exact prevalence at diagnosis and the prognostic significance of LVT in AL patients have never been investigated. Aims to study the prevalence and the impact on long-term survival LVT in AL patients. Methods and results Between 1998 and 2013, 150 AL patients were included at diagnosis (mean age was 68±11 ans, 59% were male). A comprehensive transthoracic echocardiography was performed at baseline. The presence of LVT was assessed visually and was found in 42% of patients. Compared to patients without LVT, those with LVT have more frequently advanced NYHA functional class. They also had significantly higher left ventricular (LV) wall thickening (p=0.01), LV mass (p=0.02), and mitral E/e’ (p=0.0009) and larger left atrial size (p Conclusion The presence of LVT is a common finding in patients with AL and is associated with impaired both LV systolic and diastolic function, poorer functional status and advanced stage of the disease. In addition, LVT appeared as a powerful marker of mortality. Download full-size image Abstract 0224 – Figure: 5-year survival of AL-patients according to LVT