Case 2: Amlyloidosis with reduced LVEF

Patient with progressive dyspnea complaints for almost 1 year. Admitted to our ward with severe heart failure (NYHA IV/IV). The findings on ultrasound were performed with the patient on dobutamine infusion. The ECG showed a sinus tachycardia with microvoltages of the QRS-complex.
2D assessment shows concentric LVH and a reduced LV systolic function based on global hypokinesia, LVEF ±30%. Note the biatrial dilatation, moderate accumulation of pericardial fluid, and thickening of the AV valves, all associated with amyloid heart disease. Diastolic function showed restriction (not shown).

Deformation imaging showed a typical appearance of a reduced to absent longitudinal deformation throughout the LV. Note the low peak systolic values throughout the entire LV  with relative sparing of the apical segments (anterolateral apex appears on the color map bulls-eye, the maximal negative value of -10% is nevertheless severely reduced), other segments show no deformation and no stretching/dyskinesia (regression towards the zero line), also a finding we commonly see in this disorder.

Regional and global longitudinal strain values Amyloid

Post systolic strain Amyloid

Cardiac MRI confirmed our findings described on routine echocardiographic examination. Delayed enhancement showed typical appearance of inappropriate suppression during the delayed enhancement sequence. This is a typical finding on CMR for amyloid depositions