Two-dimensional speckle tracking echocardiography for early detection of myocardial damage in young patients with Fabry disease

SACCHERI C; CIANCIULLI T; LAX J; GAGLIARGI J; CACERES G; QUARIN A; KISINOVSKY; ROZENFELD PA; REISIN R

ECHOCARDIOGRAPHY (MOUNT KISCO, N.Y.)

WILEY-BLACKWELL PUBLISHING, INC

Lugar: Londres; Año: 2013 vol. 30 p. 1069 - 1077

0742-2822

Fabry disease is characterized by left ventricular hypertrophy (LVH). Conventional echocardiography isnot sensitive enough to perform the preclinical diagnosis To assess whether longitudinal myocardialstrain of the left ventricle (LV), using speckle tracking, is useful to detect early myocardial involvement inFabry disease. Forty-four patients with FD who were diagnosed with genetic testing were prospectivelyincluded and were compared to a sex-matched control group. They were divided into three groups: 22with LVH (Group I), 22 without LVH (Group II), and 22 healthy volunteers (Group III). LV longitudinalstrain was measured from the apical views. An ANOVA test was used for multiple comparisons forvariables with a normal distribution, and a Kruskal?Wallis test was used for variables with non-Gaussiandistribution. Longitudinal LV strain was different in the three groups: it was 15% in at least onesegment in all Group I patients, in 50% of patients of Group II and in no patient of Group III. Seventypercent of the segments with abnormal strain in Group II were located in the basal regions (32/46).These ndings show that the presence of at least one strain value 15% demonstrates subclinicalmyocardial dysfunction in patients with preclinical Fabry disease. Longitudinal myocardial LV strainmeasured with speckle tracking is a useful tool to detect early myocardial involvement in young patientswith Fabry disease. This information allows the detection and treatment of myocardial dysfunction atan early stage, which is of high clinical importance