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223 Adverse functional significance of delayed enhancement on cardiac MRI in primary systemic amyloidosis
Journal of Cardiovascular Magnetic Resonance volume 10, Article number: A84 (2008)
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.
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.
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.
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).
Delayed enhancement, present in 74% of AL patients, is associated with significant diastolic dysfunction and may be important for noninvasive evaluation of primary amyloidosis.
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Migrino, R.Q., Phillips, S.A., Bright, M. et al. 223 Adverse functional significance of delayed enhancement on cardiac MRI in primary systemic amyloidosis. J Cardiovasc Magn Reson 10 (Suppl 1), A84 (2008). https://doi.org/10.1186/1532-429X-10-S1-A84
- Left Ventricular Ejection Fraction
- Diastolic Function
- Delay Enhancement
- Plasma Cell Dyscrasia