Normal left ventricular ejection fraction and mass but subclinical myocardial dysfunction in patients with Friedreich's ataxia
| Autor: | Erwan Donal, Massimo Pandolfo, Philippe Unger, Myriam Rai, Chantal Dedobbeleer |
|---|---|
| Přispěvatelé: | Service de cardiologie et maladies vasculaires [Rennes] = Cardiac, Thoracic, and Vascular Surgery [Rennes], CHU Pontchaillou [Rennes], Service de cardiologie et maladies vasculaires, Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Hôpital Pontchaillou-CHU Pontchaillou [Rennes] |
| Rok vydání: | 2011 |
| Předmět: |
Male
Statistics as Topic 030204 cardiovascular system & hematology MESH: Stroke Volume Ventricular Function Left MESH: Ventricular Function Left Strain 0302 clinical medicine Health Status Indicators Prospective Studies Twist Prospective cohort study Ultrasonography Subclinical infection MESH: Statistics Nonparametric Ejection fraction Ventricular Remodeling biology General Medicine Stroke volume MESH: Friedreich Ataxia MESH: Case-Control Studies Cardiology Female [SDV.IB]Life Sciences [q-bio]/Bioengineering medicine.symptom Cardiology and Cardiovascular Medicine Adult medicine.medical_specialty MESH: Myocardium Ataxia MESH: Ventricular Remodeling Statistics Nonparametric 03 medical and health sciences [SDV.MHEP.CSC]Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system MESH: Health Status Indicators Internal medicine medicine Humans Radiology Nuclear Medicine and imaging Systole Ventricular remodeling Ventricular function MESH: Statistics as Topic MESH: Humans business.industry Myocardium Stroke Volume MESH: Adult medicine.disease MESH: Male MESH: Prospective Studies Friedreich ataxia Case-Control Studies Frataxin biology.protein business MESH: Female Ventricular remodelling 030217 neurology & neurosurgery |
| Zdroj: | European Heart Journal-Cardiovascular Imaging European Heart Journal-Cardiovascular Imaging, Oxford UP, 2012, 13 (4), pp.346-52. ⟨10.1093/ejechocard/jer267⟩ |
| ISSN: | 2047-2412 2047-2404 |
| DOI: | 10.1093/ejechocard/jer267 |
| Popis: | International audience; 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. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|