Normal left ventricular ejection fraction and mass but subclinical myocardial dysfunction in patients with Friedreich's ataxia. - Université de Rennes Accéder directement au contenu
Article Dans Une Revue European Heart Journal - Cardiovascular Imaging Année : 2012

Normal left ventricular ejection fraction and mass but subclinical myocardial dysfunction in patients with Friedreich's ataxia.

Chantal Dedobbeleer
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Myriam Rai
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Massimo Pandolfo
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Philippe Unger
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Résumé

AIMS: Myocardial involvement in Friedreich's ataxia (FRDA) is characterized by iron deposits, diffuse fibrosis, and focal necrosis. We hypothesized that subclinical left ventricular (LV) dysfunction may occur in 'FRDA patients who have normal LV ejection fraction (LVEF) and mass. METHODS AND RESULTS: Twenty patients homozygous for the GAA expansion in the frataxin gene (mean age: 35 ± 16 years) and twenty age- and sex-matched controls (mean age: 34 ± 15 years) were studied using conventional echocardiography and speckle-tracking imaging. The two groups did not differ in terms of the LVEF (68 ± 6 vs. 67 ± 6%, in patients and controls, respectively) or LV mass (91 ± 20 vs. 82 ± 17 g/m(2)). Global systolic longitudinal (-15.3 ± 2.1 vs. -17.5 ± 1.6%, P = 0.001) and circumferential (-19.5 ± 2.9 vs. -21.4 ± 2.6%, P = 0.034) strain, and peak LV twist (9.2 ± 3.3 vs. 11.7 ± 2.3°, P = 0.008) were significantly reduced in patients compared with controls. Indexed stroke volume was also significantly lower in patients (36 ± 5 vs. 43 ± 8 mL/m(2), P = 0.0012) and this decreased LV pump performance was associated with a concentric remodelling pattern (relative wall thickness: 0.47 ± 0.08 vs. 0.35 ± 0.05, P < 0.001). CONCLUSION: There is evidence of morphological and functional abnormalities in FRDA patients with normal LVEF and mass.

Dates et versions

hal-00881662 , version 1 (08-11-2013)

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Chantal Dedobbeleer, Myriam Rai, Erwan Donal, Massimo Pandolfo, Philippe Unger. Normal left ventricular ejection fraction and mass but subclinical myocardial dysfunction in patients with Friedreich's ataxia.. European Heart Journal - Cardiovascular Imaging, 2012, 13 (4), pp.346-52. ⟨10.1093/ejechocard/jer267⟩. ⟨hal-00881662⟩
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