title: Cardiopulmonary response to exercise and cardiac assessment in patients with turner syndrome.
abstract: Turner syndrome (TS) is a chromosomal disorder; however, little is known about the exercise tolerance of patients with this syndrome. The aim of the present study was to measure the maximal aerobic capacity and cardiac function using cardiopulmonary exercise testing and lung function tests and to evaluate the cardiac parameters using echocardiography in patients with TS and control subjects. A total of 50 women with TS (mean age 21.3 ± 8.5 years) and 56 age-matched controls (mean age 21.1 ± 3.7 years) were enrolled from the Pediatric Department of "Sapienza" University of Rome and underwent cardiopulmonary exercise testing, lung function testing, and echocardiography. The maximal oxygen uptake was lower in the patients with TS than in the controls (28.4 ± 4.0 vs 35.6 ± 6.2 ml/min/kg; p <0.0001). Also, the forced expiratory volume in 1 second, expressed as a percentage of the predicted value, was greater in the patients with TS than in the controls (116.2 ± 15.2% vs 102.8 ± 4.8%, p <0.0001). The patients with TS had a smaller left ventricle than did the controls. Tissue Doppler imaging revealed subclinical systolic and diastolic dysfunction in the left ventricle in those with TS but not in the controls. The left ventricular mass index was greater in the patients with TS than in the controls (38.6 ± 9.3 vs 27.2 ± 4.5 g/m(2.7), p <0.0001). In conclusion, the patients with TS had a lower maximal aerobic capacity and exercise tolerance than did the controls. The anatomic and functional cardiac aspects were peculiar to those with TS and might represent a specific cardiac phenotype.
authors.affiliation: Department of Pediatrics, Sapienza University of Rome, Rome, Italy. giancarlo.tancredi@uniroma1.it
authors.forename: Giancarlo
authors.initials: G
authors.lastname: Tancredi
authors.forename: Paolo
authors.initials: P
authors.lastname: Versacci
authors.forename: Anna Maria
authors.initials: AM
authors.lastname: Pasquino
authors.forename: Anna Chiara
authors.initials: AC
authors.lastname: Vittucci
authors.forename: Ida
authors.initials: I
authors.lastname: Pucarelli
authors.forename: Marco
authors.initials: M
authors.lastname: Cappa
authors.forename: Corrado
authors.initials: C
authors.lastname: Di Mambro
authors.forename: Bruno
authors.initials: B
authors.lastname: Marino
mesh_terms: D000293:Adolescent; D001794:Blood Pressure / Q000502:physiology*; D002306:Cardiac Volume / Q000502:physiology; D004452:Echocardiography; D015150:Echocardiography, Doppler; D054459:Elasticity Imaging Techniques; D004562:Electrocardiography; D005080:Exercise Test; D005260:Female; D006330:Heart Defects, Congenital / Q000000981:diagnostic imaging / Q000235:genetics / Q000503:physiopathology*; D006339:Heart Rate / Q000502:physiology*; D006801:Humans; D007621:Karyotyping; D008176:Lung Volume Measurements; D010100:Oxygen / Q000097:blood*; D012815:Signal Processing, Computer-Assisted; D014424:Turner Syndrome / Q000000981:diagnostic imaging / Q000235:genetics / Q000503:physiopathology*; D018487:Ventricular Dysfunction, Left / Q000000981:diagnostic imaging / Q000503:physiopathology; D055815:Young Adult
chemical_list: D010100:Oxygen