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.

to study the prevalence and the impact on long-term survival LVT in AL patients.

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<0.0001). Moreover, patients with advanced Mayo Clinic stage more frequently had LVT: 58%, 45%, and 5% in stage III, II, and I respectively (p<0.0001). Patients with severe symptoms more frequently exhibited LVT (63% in NYHA III-IV vs. 33% in NYHA I-II, p=0.0008). During a mean follow-up of 2.9±3 years, 79 deaths occurred (53%). The presence of LVT was significantly associated with reduced 1 and 5-year survival: 51±4% vs. 70±4% and 32±7% vs. 64±6% respectively (p=0.0002). In multivariate analysis, after adjustment for age, gender, NYHA class≥III, clearance of creatininemia, and LV ejection fraction, LVT remained an independant significant marker of mortality (Hazard ratio= 1.9, 95%CI: 1.10-3.34, p=0.02).

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.