Evaluation of Left and Right Ventricular Function Using 2D Strain Imaging in Patients with Cardiac Amyloidosis

Introduction: Cardiac involvement is a leading cause of morbidity and mortality in primary light chain (AL) amyloidosis. Diastolic dysfunction is the earliest echocardiographic abnormality and may occur before cardiac symptoms develop. We used 2D strain analysis for evaluating biventricular systolic and diastolic function in patients with cardiac AL amyloidosis, and assessed the prognostic value of these measurements.

Methods: Twenty four consecutive patients with cardiac amyloidosis were studied. Patients’ charts and laboratory data were reviewed to obtain survival data, clinical severity of heart failure, treatment protocol and biochemical/light chain response to therapy. Strain and strain rate (SR) measurements were obtained from 16 segments of the left ventricle (LV), and from 3 free wall segments of the right ventricle (RV). The relationships between clinical, laboratory and echocardiographic parameters were evaluated.

Results: LV global longitudinal strain and SR were lower in non-survivors than survivors. LV segmental analysis showed better myocardial shortening of all segments in survivors compared to non-survivors. Apical-lateral and mid-septal data reached statistical significance but a clear trend was noted in all other segments as well. Global peak early diastolic SR (LSR_dias) was significantly lower in non-survivors. Global and segmental longitudinal systolic strain of the RV free wall was lower in non-survivors than in survivors. Statistical significance for this parameter was reached for the apical RV free wall segment. Global LSR_dias of the RV was significantly lower in non-survivors. After a median follow up of 37 months, 8 patients had died (33.3%). Clinical variables and conventional echocardiographic parameters were entered into a multivariate regression model predicting mortality. Adding global LV and RV free wall longitudinal strain further improved the prognostic utility of the model.

Conclusions: The results of the present study support that 2D speckle tracking-derived biventricular systolic and diastolic deformation improves the risk assessment of patient with this disease.