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.

[Evaluation of blood flow and the cross-sectional area of internal jugular vein in Japanese multiple sclerosis and neuromyelitis optica patients].

Zamboni et al proposed a new hypothesis for the pathomechanisms of multiple sclerosis (MS): chronic cerebrospinal venous insufficiency (CCSVI). Using Doppler ultrasound and venograms, they found severe extracranial venous stenosis in MS patients. They suggested that a venous obstruction in the neck caused a reflux back into the brain, which led to edema and demyelination. We examined the blood flow and the cross-sectional area of the internal jugular veins using Doppler ultrasound (Vivid 7 PRO, GE Health Japan, Tokyo) in 17 MS (8 males and 9 females; 20-58 years of age, median 38 years) and 11 neuromyelitis optica (NMO) Japanese patients (1 male and 10 females; 23-60 years of age, median 44 years). Nine of the 11 NMO patients were seropositive for anti-aquaporin4 antibodies. We did not find any obstruction or stenosis of the internal jugular veins in any patient. Other disorders such as bilateral internal and external jugular venous ligation or radical neck dissection, which result in venous stasis, are not known causes of demyelination in the central nervous system. Our data also does not support the hypothesis of CCSVI theory, despite the fact that our study was limited to a small group of patients and the examination was performed only using Doppler ultrasound.