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

نویسندگان

  • Chantal Dedobbeleer
  • Myriam Rai
  • Erwan Donal
  • Massimo Pandolfo
  • Philippe Unger
چکیده

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.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Comparison of Cardiac Function in Young Patients with Thalassemia Intermedia and Healthy Individuals Using Echocardiography Method

Background: Cardiac dysfunction due to chronic anemia and hemosiderosis are the major causes of death among patients with thalassemia intermedia. This study was performed to compare the cardiac function in thalassemia intermedia patients with normal subjects by means of echocardiography. Materials and Methods: This was a case-control study performed on 22 patients affected by thalassemia int...

متن کامل

Prediction of Changes in Left Ventricular Ejection Fraction after Off-Pump Coronary Artery Bypass Grafting Surgery by Myocardial Perfusion Single-Photon Emission Computed Tomography

Introduction: Left ventricular ejection fraction (LVEF) is considered to be the single most important prognostic factor in patients with previous myocardial infarction. LVEF is not improved in all patients after coronary artery bypass grafting (CABG). This study aimed to assess the possibility of prediction of LVEF changes after CABG using myocardial perfusion gated signle photon emission compu...

متن کامل

Dipyridamole stress and rest gated 99mTc-sestamibi myocardial perfusion SPECT: left ventricular function indices and myocardial perfusion findings

  Introduction: We investigated the difference in left ventricular ejection fraction (LVEF) and end-systolic volume(ESV) measured by gated myocardial perfusion SPECT (GSPECT) in the post-dipyridamole stress and rest periods, and compared the results with the perfusion patterns found in the conventional non-gated tomograms. Methods: 297 consecutive patients were studie...

متن کامل

بررسی یافته های اکوکاردیو گرافیک قلبی در بیماران سیروز کبدی

Introduction: Cardiovascular abnormalities have been reported in liver cirrhosis (LC). In these patients, cardiac symptoms and physical signs occur as the liver functions worsen. Cirrhosis is associated with hyper dynamic circulation and beta-adrenergic system changes responsible for the cardiovascular abnormalities. The purpose of the present study was to explore the echocardiographic findi...

متن کامل

Echocardiographic Evaluation of left Ventricular Function and Geometry in Pediatric Patients with Kidney Transplantation

Extended abstract Echocardiographic Evaluation of Left Ventricular Function and Geometry in Pediatric Patients with Kidney Transplantation Cardiovascular disease (CVD) is an important, leading cause of mortality and morbidity in patients with chronic kidney disease (CKD) as well as in renal transplant recipients. Cardiovascular complications become more important in children because of the i...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:
  • European heart journal cardiovascular Imaging

دوره 13 4  شماره 

صفحات  -

تاریخ انتشار 2012