Left atrial systolic function in primary and familial amyloidosis: Assessment from left atrial volume change

Abstract

Summary:The severity of left ventricular involvement may differ between primary (PA) and familial amyloidosis (FA). This study examined whether differences in left atrial (LA) systolic function are also present. Twenty-eight patients (18 men, 10 women, aged 59 ± 12 years) with PA, 17 (11 men, 6 women, aged 40 ± 11 years) with FA, and 25 normal controls (18 men, 7 women, aged 56 ± 14 years) underwent transthoracic M-mode, two-dimensional, and Doppler echocardiography. Left atrial volumes were determined at mitral valve (MV) opening (maximal, Vmax), electrocardiographic P wave (onset of atrial systole, Vp), and MV closure (minimal, Vmin) from the apical two-and four-chamber views using the biplane area-length method. Left atrial systolic function was assessed with the LA active emptying volume (ACTEV) = Vp-Vmin and fraction (ACTEF) = ACTEV/Vp. The E/A ratio was increased (1.34 ± 0.93 vs. 0.89 ± 0.3), whereas deceleration time was decreased (168.1 ± 33.7 vs. 196.2 ± 34.2 ms) in PA compared with FA (p<0.05). Vmax and Vp were similar in PA and FA and greater than in the controls (46.6 ± 14 vs. 40 ± 11.4 vs. 27.1 ± 6.3 cm 3 /m 2 , p < 0.01, and 33.4 ± 11.6 vs. 29.7 ± 10.8 vs. 16.8 ± 3.8 cm 3 /m 2 , p < 0.01, respectively). The ACTEV was lower in PA and in the controls than in FA (6.7 ± 2 vs. 6.2 ± 2.2 vs. 8.5 ± 3.3, respectively, p < 0.05). The ACTEF was lower in PA than in FA and both were lower than those in the controls (20 ± 5% vs. 28 ± 7% vs. 36 ± 11%, respectively, p < 0.01). Despite a similar increase in LA volume, LA systolic dysfunction is more pronounced in PA than in FA. This is most likely due to the restrictive left ventricular physiology possibly associated with depressed LA contractility in the former.

Diagnosis and Management of the Cardiac Amyloidoses

“…In advanced disease, there is a restrictive transmitral flow pattern characterized by a short deceleration time of the E wave and a low-velocity A wave, with associated abnormalities in pulmonary venous flow. 26,41,42 The decreased transmitral A wave in AL amyloidosis is related not only to late-stage restrictive pathophysiology but also to atrial amyloid infiltration, which results in intrinsic atrial dysfunction 28,29,[43][44][45][46] ; thus, a normal deceleration time can be seen in association with a diminutive A wave. Further insights into cardiac function in AL amyloidosis can be gained by pulsed tissue Doppler imaging, which can demonstrate the presence of diastolic dysfunction more accurately than transmitral and pulmonary flow and can provide evidence of longitudinal systolic impairment before the ejection fraction becomes abnormal.…”