P993Atrial function in cardiac amyloidosis: a 2d-strain echocardiography and cardiac MRI study

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Purpose: Familial amyloid polyneuropathy (FAP), an autosomal dominant disease characterized by the systemic deposition of transthyretin, can involve atria. Cardiac magnetic resonance (CMR) by delayed enhancement (DE) permits to identify atrial damage due to transthyretin deposition. Nowadays, two dimensional strain echocardiography (SE) enables to quantify, non-invasively and accurately, left atrial (LA) function (reservoir, conduit andcontraction). The purpose of the study is to assess LA function in atrial amyloidosis.Methods: 18 patients affected by FAP were enrolled. All patients underwent traditional transthoracic echocardiogram (TTE), SE imaging and DE-CMR. TTE (LA volume and E/E') and SE parameters were obtained on apical views (4-and 2-chambers). On the same day, all patients underwent DE-CMR in order to evaluate atrial deposition of transthyretin. According to DE-CMR, 2 groups were identified: 9 FAP patients with atrial involvement (FAP atrial group, 59±15 years) and 9 FAP patients without atrial involvement (FAP No-atrial group, 57±14 years). SE was performed off-line, using a dedicated software (MyLab Desk), by placing at the edge of left atrial endocardium several points; there were identified three (lower, middle and apical) segments, respectively, for each (septal, lateral, lower and anterior) atrial wall. Several SE-derived parameters were considered: global peak atrial longitudinal strain (PALS,%) to assess atrial reservoir function; conduit volume (CV, ml) as indication of conduit function; minimal atrial volume (MAV, ml) and atrial active empting fraction (AAEF,%), calculated as ((CV – MAV)/ CV)*100), to assess atrial contraction.Results: TTE showed similar LA volumes between FAP atrial group (84±39ml) and FAP No-atrial group (74±24 ml). E/E' was significantly impaired in patients with atrial involvement (FAP atrial group 15±6 vs FAP No-atrial group 6±1, p<0,001). FAP atrial group showed a significant worsening of LA function respect to FAP No-atrial group; particularly, reservoir function was reduced (PALS: 25±9 vs 49±18 %, p<0.01), as well as conduit function (CV: 75±24 vs 45±15 ml, p<0,01); similarly contraction function was impaired in FAP atrial group (MAV: 48±19 vs 23±9 ml, p<0,01; AAEF: 37±9 vs 53±12 %, p<0,01) respect to FAP No-atrial group. There was also a good correlation between E/E' and PALS values (R:0,66; p<0,001), and between E/E' and AAEF (R=0,61; p< 0,01). There was a lower correlation between E/E' and strain-derived LA volume values.Conclusion: LA Reservoir, conduit and contraction are impaired in patients with atrial amyloidosis.

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