[Early detection of myocardial disease in young patients with Becker's muscular dystrophy asymptomatic from the cardiac point of view: value of myocardial doppler tissue imaging].
Becker's muscular dystrophy is an X-linked hereditary disorder characterised by progressive muscle weakness and possible cardiac disease. Cardiac involvement is assumed to be rare in young patients. Early diagnosis could lead to earlier treatment at an infra-clinical stage of the disease. The object of the study was to evaluate systolic and diastolic cardiac function of young patients with Becker's disease by echocardiography and using Doppler tissue imaging. Consecutive patients under 20 years of age with Becker's disease confirmed genetically were included and compared with paired normal subjects. Subendocardial and subepicardial myocardial velocities were obtained by Doppler tissue imaging and the corresponding velocity gradients were measured. Twelve patients were included (17.4 +/- 2.5 years). None of them had disabling muscle disease. No significant difference was observed from normal subjects with respect to: ventricular dimensions, wall thickness, fractional shortening, E/A ratio measured by transmitral Doppler. Nevertheless, patients with Becker's disease had lower systolic and diastolic intra-myocardial velocity gradients: 2.2 +/- 1.1 vs. 4.7 +/- 2.4 s(-1), p = 0.006, and 3.6 +/- 2.0 vs. 5.6 +/- 1.3 s(-1), p = 0.048, respectively, compared with the control group. These results show that myocardial disease is possible in patients with Becker's muscular dystrophy under the age of 20. Myocardial Doppler tissue imaging is a sensitive method for detecting these early abnormalities and should be recommended in the young patients.
Meune
C
C
Servoce de cardiologie, hôpital Cochin, université Paris V René Descartes, Paris, France. christophe.meune@cch.ap-hop-paris.fr
Wahbi
K
K
Bécane
H M
HM
Laforet
P
P
Eymard
B
B
Leturcq
F
F
Devaux
J Y
JY
Weber
S
S
Duboc
D
D
D000293:Adolescent; D000328:Adult; D001783:Blood Flow Velocity / Q000502:physiology; D009202:Cardiomyopathies / Q000175:diagnosis* / Q000000981:diagnostic imaging; D016022:Case-Control Studies; D042241:Early Diagnosis; D004452:Echocardiography; D015150:Echocardiography, Doppler; D004562:Electrocardiography; D006352:Heart Ventricles / Q000000981:diagnostic imaging; D006801:Humans; D008297:Male; D008943:Mitral Valve / Q000000981:diagnostic imaging; D020388:Muscular Dystrophy, Duchenne / Q000150:complications*; D009200:Myocardial Contraction / Q000502:physiology; D011446:Prospective Studies; D015635:Radionuclide Ventriculography; D013318:Stroke Volume / Q000502:physiology