Comprehensive Doppler assessment of right ventricular diastolic function in cardiac amyloidosis

To assess right ventricular diastolic function in cardiac amyloidosis, pulsed wave Doppler ultrasound measurements of right ventricular inflow velocities and superior vena cava and hepatic vein flow velocities with respiratory monitoring were performed in 41 patients with primary systemic amyloidosis and two-dimensional echocardiographic features of cardiac involvement. Right ventricular diastolic function was abnormal in 31 (76%) of these patients, the major abnormality being a short deceleration time (less than 150 ms) in 21 (68%), suggesting restriction. In contrast, 7 (23%) of the 31 patients had a decreased ratio of early (E) and late (A) diastolic peak flow velocities and a prolonged deceleration time (greater than 240 ms), suggesting abnormal relaxation. The patients were classified into two groups on the basis of right ventricular free wall thickness: group 1, less than 7 mm and group 2, greater than or equal to 7 mm. Compared with normal values, group 1 showed an increased peak late flow velocity (44 +/- 19 versus 39 +/- 6 cm/s; p less than 0.01) and a decreased E/A velocity ratio (1.1 +/- 0.4 versus 1.5 +/- 0.3; p less than 0.01). Group 2 showed a markedly shortened deceleration time (151 +/- 37 versus 225 +/- 28 ms; p less than 0.01), characteristic of restriction. In the overall group, superior vena cava peak flow velocity was decreased in systole and increased in diastole and flow reversals during inspiration were increased compared with normal values. Hepatic venous flow velocities were similar to those in the superior vena cava except for larger flow reversals in the hepatic vein. Thus, in cardiac amyloidosis, right ventricular diastolic function is abnormal. There is a spectrum of right ventricular filling abnormalities and the restrictive filling pattern is seen only in the advanced stages of the disease.