Thallium-201 Single Photon Emission Computed Tomography (SPECT) in Patients With Duchenne's Progressive Muscular Dystrophy

Nishimura, Taiichi; Yanagisawa, Atsuo; Sakata, Hitomi; Sakata, Konomi; Ishihara, Tadayuki; Yoshino, Hideaki; Ishikawa, Kinya

doi:10.1253/jcj.65.99

Cited by 31 publications

(21 citation statements)

References 28 publications

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“…Examinations are carried out at rest and after pharmacological or physical stress [15, 25, 26, 27]. Myocardial fatty acid metabolism can be assessed using the radio-iodinated branched fatty acid 15-( p -iodophenyl)-3(R,S)-methyl-penta-decanoic acid (BMIPP) [28].…”

Section: Examinations To Assess Cardiac Involvementmentioning

confidence: 99%

“…In 7 DMD patients, thallium scintigraphy revealed perfusion defects predominantly in the posterolateral segments. At autopsy, severe transmural fibrosis and severe fatty infiltration were common in the segments of the perfusion defects [27]. The initial, predominant affection of the posterobasal region, where the fibers are longitudinally directed, might be due to the fact that forces directed along the major axis of the left ventricle are greater to the posterior wall than to other regions [76].…”

Section: Duchenne Muscular Dystrophymentioning

confidence: 99%

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The Heart in Human Dystrophinopathies

2003

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Dystrophinopathies are due to mutations in the dystrophin gene on chromosome Xp21.1 and comprise the allelic entities Duchenne muscular dystrophy (DMD), Becker muscular dystrophy (BMD) and X-linked dilative cardiomyopathy (XLDCM). In all three entities, the heart is affected to various degrees, depending on the stage of the disease and the type of the mutation (cardiac involvement, CI). The pathoanatomic evidence of CI in dystrophinopathies is the replacement of myocardium by connective tissue or fat. In DMD/BMD, the left ventricular posterobasal and lateral walls are most extensively affected, sparing the right ventricle and the atrium. Degree and dynamics of CI vary among the three entities. In DMD/BMD, CI usually remains subclinical in the early stages of the disease. Typical initial manifestations of CI in DMD/BMD are sinus tachycardia, tall R1 in V1, prominent Q in I, aVL, V6 or in II, III, and aVF, increased QT dispersion and possibly autonomic dysfunction. Initially, echocardiography is normal or shows regional wall motion abnormalities in areas of fibrosis. With spreading of fibrosis, left ventricular dysfunction and ventricular arrhythmias additionally occur. In the final stages of the disease, systolic function may lead to heart failure and sudden death. Subclinical or clinical CI is present in about 90% of the DMD/BMD patients but is the cause of death in only 20% of the DMD and 50% of the BMD patients. XLDCM is a rapidly progressive, almost exclusively myocardial disorder, starting in teenage males as heart failure due to dilative cardiomyopathy (CMP), leading to death from intractable heart failure within 1–2 years after diagnosis. Therapy of arrhythmias and CMP in all three disorders follows the established cardiological recommendations. Due to its protective effect, ACE inhibitors are recommended already at the early stages of the disease. β-Blockers may be an additional option if indicated.

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Section: Examinations To Assess Cardiac Involvementmentioning

confidence: 99%

Section: Duchenne Muscular Dystrophymentioning

confidence: 99%

The Heart in Human Dystrophinopathies

2003

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“…Myocardial fat is seen in adults with normal hearts (1)(2)(3)(4)(5)(6)(7)(8)(9)(10) and in patients with healed myocardial infarction (MI) (8,9,(11)(12)(13)(14)(15)(16)(17)(18), arrhythmogenic right ventricular (RV) cardiomyopathy or dysplasia (ARVC) (2,4,(19)(20)(21)(22)(23)(24)(25)(26)(27)(28)(29)(30), cardiac lipoma (31,32), lipomatous hypertrophy of the interatrial septum (LHIAS) (33)(34)(35), tuberous sclerosis complex (TSC) (36,37), and other cardiomyopathies, such as dilated cardiomyopathy (DCM) (11,26,38) and muscular dystrophy (39)(40)(41). We frequently encounter myocardial fat in the normal heart and in healed MI at routine CT and MR imaging.…”

Section: Introductionmentioning

confidence: 99%

Myocardial Fat at Cardiac Imaging: How Can We Differentiate Pathologic from Physiologic Fatty Infiltration?

Kimura

Matsuo²,

Nakajima³

et al. 2010

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109

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Myocardial fat is often seen at cardiac computed tomography (CT) and magnetic resonance (MR) imaging of healthy adults and patients with myocardial diseases. Physiologic myocardial fat develops with aging and is commonly seen at CT in the anterolateral right ventricular (RV) free wall and RV outflow tract with normal or thickened RV myocardium and a normal-sized RV in elderly patients. Pathologic conditions with myocardial fat include healed myocardial infarction (MI); arrhythmogenic RV cardiomyopathy or dysplasia (ARVC); and others, such as cardiac lipoma, lipomatous hypertrophy of the interatrial septum, tuberous sclerosis complex, dilated cardiomyopathy, and cardiomyopathy with muscular dystrophy. In patients with healed MI, CT and MR imaging show fat in left ventricular myocardium that is of normal thickness or thin and follows the distribution of the coronary artery; CT often depicts fat in mostly subendocardial regions. In patients with ARVC, characteristic CT and MR imaging findings include a thin RV outflow tract and free wall caused by subepicardial fatty infiltration; fat in the RV moderator band, trabeculae, and ventricular septum; and RV enlargement and wall motion abnormality. Recognition of patient age, characteristic locations of myocardial fat, myocardial thickness, and ventricular size helps in differentiating physiologic and pathologic myocardial fat at cardiac imaging; findings of wall motion abnormality and late gadolinium enhancement at MR imaging help narrow the diagnosis.

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“…MRI is increasingly used to evaluate myocardial damage and ventricular function 10, 11. Pathological changes in progressive degeneration such as interstitial edema, fatty transformation, and fibrosis are shared between skeletal and cardiac muscle 12. The timing of disease in myocardium in relation to skeletal muscle of younger boys with DMD has not yet been delineated.…”

Section: Introductionmentioning

confidence: 99%

Upper arm and cardiac magnetic resonance imaging in Duchenne muscular dystrophy

Gaur

Hanna

Bandettini

et al. 2016

Ann Clin Transl Neurol

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We analyzed quantitative maps of T 1 and T 2 relaxation times and muscle fat fraction measurements in magnetic resonance imaging of the upper arm skeletal muscles and heart in ambulatory boys with Duchenne muscular dystrophy and age‐range‐matched healthy volunteer boys. The cardiac‐optimized sequences detected fatty infiltration and edema in the upper arm skeletal muscles but not the myocardium in these Duchenne muscular dystrophy boys who had normal ejection fraction. Imaging the heart and skeletal muscle using the same magnetic resonance imaging methods during a single scan may be useful in assessing relative disease status and therapeutic response in clinical trials of Duchenne muscular dystrophy.

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Thallium-201 Single Photon Emission Computed Tomography (SPECT) in Patients With Duchenne's Progressive Muscular Dystrophy

Cited by 31 publications

References 28 publications

The Heart in Human Dystrophinopathies

The Heart in Human Dystrophinopathies

Myocardial Fat at Cardiac Imaging: How Can We Differentiate Pathologic from Physiologic Fatty Infiltration?

Upper arm and cardiac magnetic resonance imaging in Duchenne muscular dystrophy

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