0430: Assessment of systolic and diastolic features in light chain amyloidosis: an echocardiographic and cardiac magnetic resonance study

2016 
Background Cardiac involvement in systemic light-chain amyloidosis (AL) is characterized by 2D-echocardiography (TTE) normal or slithly decreased left ventricular (LV) ejection fraction and typically a diastolic dys-function with left atrial (LA) enlargement. To assess cardiac involvement, the Mayo Clinic staging (MC) using NTproBNP and troponin, has been validated and allows risk stratification of patients into 3 groups with different outcomes. Cardiac magnetic resonance (CMR) assesses accurately chambers size and function. We aimed to compare by TTE and by CMR respectively: features of LV systolic and diastolic function and by CMR, morphological functional parameters namely LV myocardial late gadolinium enhancement (LGE) and indexed max LA volume (LAVi) and emptying fraction (LAEF). Methods and results Forty-two consecutive patients (66±10 years, 57% males) in sinus rhythm with confirmed systemic AL, underwent simultaneously TTE and CMR within 24 hours. LAEF was calculated after assessing the maximal and minimal LAVi (by area/length formula) in CMR using 4 and 2 chambers views. Diastolic parameters and 2D-LV global longitudinal strain (GLS) obtained by TTE were stratified according to LAEF, to LAVi and to the presence or not of LGE. Patients in MC stage III had the worse TTE and CMR parameters. LV GLS (–10.1±3.1 vs. –17.3±3.7, p Conclusion In systemic AL, reduced LV GLS is associated with presence of LGE while impaired LV filling pressures are rather related to decreased LA emptying fraction. Multimodality imaging in patients with AL may allow better and complementary assessment of LV hemodynamics. Abstract 0430 - Table LA Emptying fraction CMR Maximal LAVICMR LGE >17.5% p 2 >44 ml/m 2 p – + p Mitral E/A ratio 2.98±1.82 0.89±0.28 1.51±1.4 2.22±1.8 0.2 1.35±1.3 2.43±1.9 0.055 DT TTE (ms) 158±45 217±56 190±65 183±53 0.7 219±51 145 ±39 Lateral E/e’ 16.9±6.8 12.1±6.4 14±7 14.8±7 0.8 13.6±7.4 15.7±6.4 0.4 GLS –10.1±3.1 –17.3±3.7 –14.2±5 –12.7±4.6 0.4 –16.5±5.2 –10.8±2.8
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