Left atrial dysfunction in light-chain cardiac amyloidosis and hypertrophic cardiomyopathy - a comparative three-dimensional speckle-tracking echocardiographic analysis from the MAGYAR-Path Study

INTRODUCTION: While cardiac amyloidosis (CA) is a rare systemic disease characterized by extracellular deposition of protein-derived fibrils, hypertrophic cardiomyopathy (HCM) is histopathologically characterized by myocyte hypertrophy and disarray, interstitial fibrosis, and small intramural coronary arteriole dysplasia. The aim of the present study was to compare left atrial (LA) volumetric and functional characteristics between light-chain (AL) CA and HCM by three-dimensional (3D) speckle-tracking echocardiography (STE). METHODS: The AL-CA group initially consisted of 17 patients with AL-CA, but one patient was excluded due to inadequate image quality, and so the study population consisted of 16 patients (mean age: 64.0+/-9.6 years, five men). Their results were compared with data on 20 age-matched HCM patients (mean age: 59.8+/-5.2 years, 10 men) and on 16 age-matched healthy controls (mean age: 58.2+/-7.2 years, six men). Complete two-dimensional Doppler echocardiography and 3D-STE were performed in all cases. RESULTS: Significantly increased LA volumes were observed in both AL-CA and HCM compared with the control group. Only active atrial emptying fraction was found to be significantly reduced in AL-CA patients compared to controls. Peak global and mean segmental circumferential, longitudinal and area strains showed significant reductions in AL-CA patients compared with controls, but only peak mean segmental longitudinal strain differed significantly between HCM patients and controls. While no differences were demonstrated in global and mean segmental strain at atrial contraction between HCM patients and controls, AL-CA patients showed reductions in certain strain parameters compared to controls and HCM patients. CONCLUSIONS: Different patterns of LA functional characteristics were demonstrated in AL-CA and HCM patients by 3D-STE