P827Speckle tracking, compared with strain Doppler, allows to assess hidden right ventricular dysfunction in asymptomatic first-degree relatives of arrhythmogenic right ventricular cardiomyopathy (ARVC)


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Abstract

Purpose:According to modifications of criteria of ARVC, proposed to facilitate clinical diagnosis in first-degree relatives, who often have incomplete expression of the disease, the diagnosis of familial ARVC is based on one of the following findings: either mild global dilatation or reduction in RV ejection fraction (EF) with normal LV or mild segmental dilatation of the RV or regional RV hypokinesis. The potential utility of Strain-Strain rate (S-SR) Doppler and two-dimensional(2D) to quantitatively assess RV, LV and RA(right atrium) function in asymptomatic family members of ARVC, with apparently normal RV, was evaluated.Methods:80 subjects were studied:40 first degree ARVC relatives with normal RV at standard echocardiography and 40 healthy controls. By E9-GE LV EF, LV diameters and volumes, RV dimension, fractional area change (FAC%) and RVOT fractional shortening (RVOTfs%), RA volume were measured. By DTI velocity of early (E') and late (A') diastolic and systolic wave (S) at tricuspidal annulus were measured. Longitudinal systolic RV S-SR Doppler and 2D S-SR in apical 4 and 2-chambers views were measured at level of RV free wall segments, all LV segments and RA wall. Circumferential and radial systolic LV 2D S-SR were measured in middle short axis view. By 3D echocardiography with volumetric probe we measured RA and RV volumes.Results: No significant differences were found between relatives and controls for RV dimensions (1,9±0,3vs2±0,3cm), RVFAC (50±12vs51±11%) and RVOTfs (64,8±13vs 65,3±14%), RA max volumes by 2D(39±8,5vs37±7,5ml;index:20,3±4,5vs 8,7±6ml/mq) and by 3D(52±9,6vs±51±13ml;index27,4±5,6vs28±10ml/mq), 3D RV end-diastolic (31±10,5vs33±11ml/mq) and end-systolic Volume(15±4vs16±6ml/mq), RV S-SR Doppler, LV longitudinal(SR=1,49±0,45 vs-1,53±0,49 S-1,S=19,59±4,1vs 20,59±4,47%), circonferential(SR=-1,59±0,4vs-1,62±0,4 S-1;S=-20,8±5vs-21±5,1%), radial(SR=1,56±0,29vs1,58±0,3 S-1;S= 45,9±9vs 46,3±9,2%) S and SR and for RA S-SR between ARVC relatives and controls. E'/A' ratio at tricuspidal annulus was inverted in 32/40 ARVC relatives. In 28/40(70%) family members (normal RV M-2D-3D parameters) RV systolic 2D S(-18,5±4,8%vs -26,6±8,1%,p< 0.002) and SR(-1,54±0,4 vs -2,37±0,51 S-1,p< 0.002) were significantly lower than in controls.Conclusions: 2D Speckle Tracking shows early RV dysfunction in asymptomatic ARVC relatives, when standard and 3D echocardiography doesn't show any impairment and may have potential clinical value in the objective quantitative assessment of regional hypokinesia, improving diagnostic sensitivity. Instead, S-SR Doppler is less reproducible and has more limitations.

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