Echocardiographic features of amyloid cardiomyopathy phenotypes in patients with different types of amyloidosis

Aim. To determine the echocardiographic variants of phenotype presentation of amyloid cardiomyopathy (ACM) in patients with different types of amyloidosis.

Materials and methods. The study included 54 patients with ACM: 27 with light chain amyloidosis (15 [56%] males; the median age was 63.0 [56.5; 67.0]) and 27 patients with transthyretin amyloidosis (20 [74%] males; the median age was 73.0 [65.5; 78.5]). Standard echocardiographic parameters and the left ventricular (LV) global longitudinal strain in both groups were evaluated.

Results. Among patients with ACM, the following phenotypes were reported: hypertrophic phenotype (HP), a combination of hypertrophic and restrictive phenotypes (HP+RP), a combination of hypertrophic, restrictive phenotypes and ejection fraction (EF) less than 50% (HP+RP+EF<50%), and patients with minimal structural changes. In patients with HP+RP+EF<50%, significantly greater thickness of the LV posterior wall (p=0.025) and the relative wall thickness (p=0.010) were found; the LV global longitudinal strain was lower (p=0.001). There were significant differences in the size of the right atrium (p=0.036), the systolic pressure in the pulmonary artery (p<0.001), and the presence of a thickening of the free wall of the right ventricle (p<0.007) in the HP+RP group. Involvement of the heart valves was more common in patients with ATTR ACM.

Conclusion. After reviewing the echocardiographic data of patients, various phenotypic presentations of ACM and several correlations between echocardiographic characteristics depending on the type of amyloidosis were determined. Further study of echocardiographic parameters in patients with various types of amyloidosis may be promising for early diagnosis and proper treatment of ACM.