Doppler characterization of left ventricular diastolic function in cardiac amyloidosis

Sixty-four patients with primary systemic amyloidosis-53 with two-dimensional echocardiographic features of cardiac involvement (Group I) and 11 without cardiac involvement (Group II)-underwent Doppler echocardiographic assessment of left ventricular diastolic function. Pulsed wave Doppler recordings of left ventricular inflow velocities and pulmonary vein flow velocities with respiratory monitoring in these patients were compared with findings in a normal group. Patients in Group I showed striking abnormalities of left ventricular diastolic filling when classified into subgroups by mean left ventricular wall thickness: early >12 but <15 mm; advanced ≥15 mm. In early amyloidosis, relaxation was abnormal, with decreased peak early velocity (75 ± 20 versus 86 ± 16 cm/s; p < 0.01), increased late velocity (71 ± 22 versus 56 ± 13 cm/s; p < 0.01), decreased early to late velocity ratio (1.2 ± 0.6 versus 1.6 ± 0.5; p < 0.01) and prolonged isovolumic relaxation time (87 ± 15 versus 73 ± 13 ms; p < 0.01) compared with normal values. In advanced amyloidosis, there was a restrictive filling pattern with a markedly shortened deceleration time (148 ± 50 versus 199 ± 32 ms; p < 0.001), decreased pulmonary vein peak systolic flow velocity (34 ± 16 versus 54 ± 2 cm/s; p < 0.01) and increased diastolic flow velocity (55 ± 20 versus 44 ± 12 cm/s; p < 0.01) compared with normal values. Group II and the subgroup with early amyloidosis had similar flow velocity patterns. Thus, this study documents that in cardiac amyloidosis, a spectrum of diastolic filling abnormalities exists; the restrictive filling pattern is seen only in the advanced stages.