Utility of real-time three-dimensional echocardiography for Duchenne muscular dystrophy with echocardiographic limitations.
Duchenne muscular dystrophy (DMD) is strongly associated with a unique form of dilated cardiomyopathy. Cardiac complications are the leading cause of death in DMD; thus, longitudinal assessments and early intervention for cardiac dysfunction are necessary to improve prognosis. Two-dimensional echocardiography, which is routinely used for cardiac assessment, has some limitations for quantitative analyses in DMD patients with thoracic deformities and regional wall motion abnormalities in the left ventricle. Recently, real-time three-dimensional echocardiography has emerged as a feasible tool for cardiac assessment in various cardiac diseases. The aim of this study was to examine the utility of this technology in DMD. We evaluated left ventricular ejection fraction (LVEF), a major parameter of left ventricular function, in 17 male DMD patients. LVEF values measured by real-time three-dimensional echocardiography were compared with those determined by two established nuclear cardiology methods: "the first-pass method of radionuclide angiocardiography" and "quantitative electrocardiogram-gated single-photon emission computed tomography". A good correlation was observed for LVEF values, particularly between real-time three-dimensional echocardiography and "the first-pass method of radionuclide angiocardiography" (r=0.90, p<0.05). Thus, real-time three-dimensional echocardiography can provide an accurate measurement of LVEF in DMD patients with echocardiographic limitations.
Department of Pediatric Neurology, National Hospital Organization Utano Hospital, Kyoto, Japan. Electronic address: r-suzuki@unh.hosp.go.jp.
Rie S
RS
Tsuburaya
Department of Cardiology, National Hospital Organization Utano Hospital, Kyoto, Japan.
Hiroshi
H
Uchizumi
Department of Radiology and Clinical Physiology, National Hospital Organization Utano Hospital, Kyoto, Japan.
Michio
M
Ueda
Department of Radiology and Clinical Physiology, National Hospital Organization Utano Hospital, Kyoto, Japan.
Yutaka
Y
Demura
Department of Pediatric Neurology, National Hospital Organization Utano Hospital, Kyoto, Japan.
Souichi
S
Mukaida
Department of Neurology, National Hospital Organization Utano Hospital, Kyoto, Japan.
Shinji
S
Sudou
Department of Pediatric Neurology, National Hospital Organization Utano Hospital, Kyoto, Japan.
Kaori
K
Irahara
Department of Pediatric Neurology, National Hospital Organization Utano Hospital, Kyoto, Japan.
Naoko
N
Sakai
Department of Pediatric Neurology, National Hospital Organization Utano Hospital, Kyoto, Japan.
Kazuhiro
K
Shiraishi
D000293:Adolescent; D000328:Adult; D055426:Cardiac-Gated Single-Photon Emission Computer-Assisted Tomography; D019560:Echocardiography, Three-Dimensional / Q000379:methods*; D005240:Feasibility Studies; D015637:Gated Blood-Pool Imaging; D006801:Humans; D008297:Male; D020388:Muscular Dystrophy, Duchenne / Q000000981:diagnostic imaging*; D016277:Ventricular Function, Left; D055815:Young Adult
Dilated cardiomyopathy; Duchenne muscular dystrophy; First-pass method of radionuclide angiocardiography; Quantitative electrocardiogram-gated single-photon emission computed tomography (QGS); Real-time three-dimensional echocardiography