A comparative study of thallium-201 single-photon emission computed tomography and electrocardiography in duchenne and other types of muscular dystrophy

doi:10.1016/0002-9149(88)91076-4

The American Journal of Cardiology

Volume 61, Issue 10, 1 April 1988, Pages 836-843

https://doi.org/10.1016/0002-9149(88)91076-4 Get rights and content

Abstract

Single-photon emission computed tomography (SPECT) using thallium-201 was compared with 12-lead electrocardiography (ECG) in patients with Duchenne (29), facioscapulohumeral (7), limb-girdle (6) and myotonic (5) dystrophies, by dividing the left ventricular (LV) wall into 5 segments. SPECT showed thallium defects (37 patients, mostly in the posteroapical wall), malrotation (23), apical aneurysm (5) and dilatation (7). ECG showed abnormal QRS (36 patients), particularly as a posterolateral pattern (13). Both methods of assessment were normal in only 7 patients. The Duchenne type frequently showed both a thallium defect (particularly in the posteroapical wall) and an abnormal QRS (predominantly in the posterolateral wall); the 3 other types showed abnormalities over the 5 LV wall segments in both tests. The percent of agreement between the 2 tests was 64, 66, 70, 72 and 72 for the lateral, apical, anteroseptal, posterior and inferior walls, respectively. The 2 tests were discordant in 31 % of the LV wall, with SPECT (+) but ECG (−) in 21% (mostly in the apicoinferior wall) and SPECT (−) but ECG (+) in 10% (mostly in the lateral wall). Some patients showed large SPECT hypoperfusion despite minimal electrocardiographic changes. ECG thus appeared to underestimate LV fibrosis and to reflect posteroapical rather than posterolateral dystrophy in its posterolateral QRS pattern. In this disease, extensive SPECT hypoperfusion was also shown, irrespective of clinical subtype and skeletal involvement.

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2010, International Journal of Cardiology
Citation Excerpt :
In this study, the incidence of segmental LV systolic dysfunction was significantly higher in the subjects with DMD than in any of the other groups. In particular, myocardial damage was frequently seen in the LV posterior-wall in all MD types, but especially in DMD, an observation which is similar to previous reports [5–9]. We also estimated the correlations between TTE parameters and plasma ANP and BNP levels, especially in DMD.
To examine the usefulness of plasma atrial (ANP) and brain natriuretic peptide (BNP) levels and transthoracic echocardiogram (TTE) findings in predicting left ventricular (LV) dysfunction in muscular dystrophies (MD).
135 MD subjects (83 Duchenne MD (DMD), all males, mean age 22 ± 7 years, 20 Becker MD (BMD), all males, 45 ± 16 years, 21 limb-girdle MD (LGMD), 14 males, 52 ± 13 years, and 11 facioscapulohumeral MD (FSHD), all males, 58 ± 13 years) underwent TTE and measurement of BNP and ANP.
In DMD, TTE revealed asynergy of the LV posterior-wall (72%), interventricular septum (IVS) (29%) and LV apex-wall (24%); in BMD subjects: TTE revealed asynergy of LV posterior-wall (50%) and IVS (25%); in LGMD and FSHD subjects: TTE revealed asynergy of LV posterior-wall (33 and 27%, respectively). LV end-diastolic (LVDdI) and end-systolic diameter index (LVDsI) were significantly larger in DMD than FSHD, and LV ejection fraction (LVEF) was significantly lower in DMD than LGMD and FSHD. In DMD, when LVEF was > = 35%, BNP and ANP levels remained low, but when LVEF < 35%, BNP and ANP levels showed steep increase. Relationship between LVEF and BNP or ANP levels was curvilinear (R² = 0.69 and 0.55, respectively, p < 0.001) using following equation: BNP = 673491×EF^− 2.897, ANP = 5716.6 × EF^− 1.422, respectively. When LVDdI > = 40 mm/m² or LVDsI > = 35 mm/m², LVEF < = 35%, plasma BNP and ANP levels increased steeply and the relationship between LVDdI, LVDsI, or LVEF and plasma BNP or ANP levels was curvilinear.
In DMD, LVEF was significantly lower, asynergy of LV posterior-wall was more frequent and BNP and ANP levels were dramatically higher when LVDdI > = 40 mm/m², LVDsI > = 35 mm/m² or LVEF < 35%.
Differentiation of diagnosis and prognoses of non-coronary arterial primary myocardial diseases with left ventricular focal myocardial thinning evaluated by multislice computed tomography
2010, International Journal of Cardiology
Citation Excerpt :
Also in cardiac sarcoidosis, thinned sites are commonly in IVS [1–3] but sometimes occur at other sites such as the inferior wall. As outlined in this study, in subjects with muscular dystrophy, reduced wall motion of LV posterior wall is frequently observed [13–17]. However, diagnosis of muscular dystrophy has been performed on the basis of neurological clinical findings and increment of serum CPK.
This study sought to differentiate diagnosis and prognoses of non-coronary arterial primary myocardial diseases with focal left ventricular myocardial (LVM) thinning evaluated by multislice-CT. Based on the presence of fibro-fatty change and asynergy in LVM, we sought to understand the clinical significance of detection of non-coronary arterial focal LVM thinning.
766 consecutive subjects (419-male, 59 ± 18 years) underwent enhanced ECG-gated multislice-CT. 12 subjects (8 male, mean age 58 years) with non-coronary arterial primary diseases and evidence of focal LVM thinning were selected. Non-coronary arterial primary diseases exhibiting focal LVM thinning were defined as follows: In comparison with the adjoining reference normal area, focal LVM thinning in end-diastole was less than half that of the adjoining reference normal area.
On transthoracic echocardiography, LV sizes of the 12 subjects tended to be slightly enlarged and the mean LV ejection fraction (46.8%) was slightly below the normal range. There are many non-coronary arterial primary diseases which exhibit focal LVM thinning. In this study, we constructed a differential flowchart in which estimation of final diagnosis, treatment and prognosis for such subjects can usefully be based on the presence of fibro-fatty change and asynergy in thinned LVM sites by MSCT. Detection of the presence of fibro-fatty change and asynergy in thinned LVM sites may indicate the need for implantation of implantable cardioverter defibrillator or cardiac pacemakers.
A differential flowchart for those subjects using the presence of fibro-fatty change and asynergy in thinned LVM sites by MSCT may be useful to differentiate prognoses of such subjects.
Clinical, genetic, and cardiac magnetic resonance imaging findings in primary desminopathies
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Citation Excerpt :
Additionally, in patients undergoing chemotherapy with cardiotoxic agents such as anthracyclines, which can cause dilatative cardiomyopathy, an early increase of myocardial hyperenhancement was predictive of future worsening of LV function [40]. In patients with Duchenne myopathy and other forms of muscular dystrophy diffuse hypoperfusion on thallium SPECT – which can be assumed to be a correlate of dystrophy-related myocardial fibrosis – correlates with adverse prognosis [41]. One patient (1-5) in our study group displayed focal hypertrophy of the lateral LV wall associated with intramyocardial hyperenhancement of the anterolateral and lateral segments, already at the age of 18.
We report the clinical, genetic and cardiac magnetic resonance imaging (MRI) findings in 11 German patients with heterozygous E245D, D339Y, R350P and L377P desmin mutations and without cardiac symptoms. Clinical evaluation revealed a marked variability of skeletal muscle, respiratory and cardiac involvement even between patients with identical mutations, ranging from asymptomatic to severely affected. While echocardiography did not show any pathological findings in all 11 patients, cine MRI revealed focal left ventricular hypertrophy in 2 patients and MR delayed enhancement imaging displayed intramyocardial fibrosis in the left ventricle in 4 patients indicating early myocardial involvement. Our data argue against distinct genotype-phenotype correlations and suggest that comprehensive cardiac MRI is superior to conventional echocardiography for the detection of early and clinically asymptomatic stages of cardiomyopathy in desminopathy patients. Therefore, cardiac MRI may serve as a screening tool to identify patients at risk, which might benefit from early pharmacological and/or interventional (e.g. implantable cardioverter-defibrillator devices) therapy.
Long-term benefits of deflazacort treatment for boys with Duchenne muscular dystrophy in their second decade
2006, Neuromuscular Disorders
We compare the clinical course of 74 boys 10–18 years of age with Duchenne muscular dystrophy (DMD) treated (40) and not treated (34) with deflazacort. Treated boys were able to rise from supine to standing, climb stairs and walk 10 m without aids, 3–5 years longer than boys not treated. After 10 years of age, treated boys had significantly better pulmonary function than boys not treated and after15 years of age, 8 of 17 boys not treated required nocturnal ventilation compared with none of the 40 treated boys. For boys over 15 years of age, 11 of 17 boys not treated required assistance with feeding compared to none of the treated boys. By 18 years, 30 of 34 boys not treated had a spinal curve greater than 20° compared to 4 of 40 treated boys. By 18 years, 7 of 34 boys not treated had lost 25% or more of their body weight (treated 0 of 40) and 4 of those 7 boys required a gastric feeding tube. By 18 years, 20 of 34 boys not treated had cardiac left ventricular ejection fractions less than 45% compared to 4 of 40 treated boys and 12 of 34 died in their second decade (mean 17.6±1.7 years) primarily of cardiorespiratory complications. Two of 40 boys treated with deflazacort died at 13 and 18 years of age from cardiac failure. The treated boys were significantly shorter, did not have excessive weight gain and 22 of 40 had asymptomatic cataracts. Long bone fractures occurred in 25% of boys in both the treated and not treated groups. This longer-term study demonstrates that deflazacort has a very significant impact on health, quality of life and health care costs for boys with DMD and their families, and is associated with few side effects.
Effects of deflazacort on left ventricular function in patients with Duchenne muscular dystrophy
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Depressed myocardial fatty acid metabolism in patients with muscular dystrophy
2001, Neuromuscular Disorders
Myocardial involvement is frequently associated with various types of muscular dystrophy and Thallium-201 scintigraphy can show regional myocardial perfusion abnormalities in patients with muscular dystrophy. Myocardial fatty acid metabolism can now be imaged using a radioiodinated branched fatty acid (¹²³I-BMIPP). The present study evaluates myocardial fatty acid metabolism in muscular dystrophy. Twenty-eight patients underwent ¹²³I-BMIPP(BMIPP) and Thallium dual single photon emission tomography. Regional uptake of both tracers was visually analyzed. We also assessed electrocardiography and echocardiography. The results showed that the BMIPP uptake compared to Thallium was smaller in 57% of all patients. BMIPP SPECT images revealed abnormalities in four of eight patients with a normal electrocardiogram. Abnormal BMIPP uptake with normal regional wall motion was evident in nine patients. The size of the region with defective BMIPP uptake was larger than that of asynergic areas detected by echocardiography in 11 patients. In conclusion, muscular dystrophy involves depressed myocardial fatty acid metabolism in larger extent of region than that in perfusion or mechanical abnormality.

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A comparative study of thallium-201 single-photon emission computed tomography and electrocardiography in duchenne and other types of muscular dystrophy

Abstract

Am Heart J

Am J Med

Am J Cardiol

Am J Cardiol

Am J Cardiol

Cardiac involvement in progressive muscular dystrophy

Am Heart J

The cardiomyopathy of progressive muscular dystrophy

Circulation

The electrocardiogram in Duchenne progressive muscular dystrophy

Circulation

Echocardiographic evaluation of posterior left ventricular wall motion in muscular dystrophy

Circulation

Alterations in regional myocardial metabolism, perfusion, and wall motion in Duchenne muscular dystrophy studied by radionuclide imaging

Circulation

Single-photon transaxial computed tomography of the heart in normal subjects and in patients with infarction

J Nucl Med

Clinical application of the digital gamma camera with twin opposed large rectangular detectors. (1) Speciality and performance of the system

Kaku Igaku

“Circumferential profiles:” a new method for computer analysis of thallium-201 myocardial perfusion images

J Nucl Med

The unipolar precordial and extremity electrocardiogram in normal infants and children

Circulation