223 Adverse functional significance of delayed enhancement on cardiac MRI in primary systemic amyloidosis

Introduction

Primary systemic amyloidosis (AL), a plasma cell dyscrasia, is often associated with a fatal outcome if there is advanced cardiac involvement. Myocardial delayed enhancement (DE) has been reported on cardiac MRI but the functional significance of such finding is not established.

Purpose

Our aim is to determine the functional significance of myocardial delayed enhancement (DE) on MRI by comparing systolic and diastolic function of AL patients with delayed enhancement (DE+) versus those without (DE-) using MRI and echocardiography.

Methods

23 patients (11 females; 61 ± 10 years) with biopsy-proven AL were grouped into DE+ (N = 17, 74%) and DE- (N = 6, 26%). Left ventricular ejection fraction (LVEF) and diastolic function (early diastolic filling rate DFR and early diastolic filling volume 1/3 FF on MRI, and ratio of early transmitral inflow to mitral annular velocity E/Em and deceleration time DT on echo) were compared. Automated simple linear curve fitting of early (first 1/3) diastolic filling curve was used to calculate DFR (slope of curve) and 1/3 FF was filling volume of first 1/3 of diastole.

Results

LVEF (DE+ vs. DE-: 67.3 ± 17 vs. 68.8 ± 12%, p = NS) was similar. However, DFR (1.7 ± 2 vs. 5.4 ± 3% enddiastolic volume/% cardiac cycle, p = 0.04, Figure 1) and 1/3 FF (19.8 ± 13 vs. 36.3 ± 23% enddiastolic volume, p = 0.03) were lower in DE+ patients. E/Em was higher in DE+ patients (17.4 ± 9 vs. 7.9 ± 3, p = 0.02) while DT was shorter (219 ± 62 vs. 288 ± 64 ms, p = 0.03). There was a nonsignificant trend towards reduced survival in DE+ subjects (304 ± 273 vs. 560 ± 280 days, p = 0.07, Figure 2).

Figure 1

The functional significance of delayed enhancement in primary systemic amyloidosis is not defined. We found amyloid patients with delayed enhancement on MRI have significant diastolic dysfunction compared to those without.

Figure 2

Conclusion

Delayed enhancement, present in 74% of AL patients, is associated with significant diastolic dysfunction and may be important for noninvasive evaluation of primary amyloidosis.