The vectorcardiogram in Duchenne's progressive muscular dystrophy

Ronan, James A.; Perloff, Joseph K.; Bowen, Patrick J.; Mann, Oscar

doi:10.1016/0002-8703(72)90172-x

Cited by 31 publications

(4 citation statements)

References 17 publications

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“…A distinctive electrocardiographic pattern, consisting of tall R waves over V1 with R/S ratio exceeding I and deep but narrow Q waves over leads I, aV1, V5, and V6, has been consistently reported in 80-90% of patients with Duchennes dystrophy' 6 and was seen in most of our patients, including the two whose hearts were studied for histologic and ultrastructural characteristics. Although these ECG abnormalities may closely simulate a myocardial infarction pattern, there are certain differences.…”

Section: Histologic and Ultrastructural Features Of Control Heartssupporting

confidence: 66%

“…ELECTROCARDIOGRAPHIC (ECG) ABNORMAL-ITIES in patients with Duchenne's progressive muscular dystrophy are well defined1 but their genesis, although the subject of much interest, is still unclear.5' 6 Frankel and Rosser,7 on the basis of histologic findings in cardiac tissue, recently suggested that these changes result from a generalized cardiomyopathy characterized by a peculiar distribution of fibrosis and other degenerative changes. Thus far, a comparable subcellular pattern of degenerative changes has not been reported.…”

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confidence: 99%

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An ultrastructural basis for electrocardiographic alterations associated with Duchenne's progressive muscular dystrophy.

et al. 1978

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SUMMARY Electrocardiographic abnormalities were identified in 63 (84%) of 75 patients with Duchenne's progressive muscular dvstrophy. A tall R wave over V, with an abnormal R/S ratio was seen in 64% of the patients, a deep and narrow Q wave > 4 mm over leads 1, V, and V8 in 44%, sinus tachycardia in 32% and right axis deviation in 16%. Other ECG abnormalities included an abnormal PV1 index in 14% of patients and a short P-R interval in 6%.Ultrastructural characteristics of the heart were determined for two patients with characteristic electrocardiographic abnormalities. Common to both hearts was a total loss of thick as well as thin myofilaments, which gave a "moth-eaten" appearance to the myofiber. This feature, combined with preservation of the transverse tubular system, formed the most characteristic ultrastructural find-ELECTROCARDIOGRAPHIC (ECG) ABNORMAL-ITIES in patients with Duchenne's progressive muscular dystrophy are well defined1 but their genesis, although the subject of much interest, is still unclear.5' 6 Frankel and Rosser,7 on the basis of histologic findings in cardiac tissue, recently suggested that these changes result from a generalized cardiomyopathy characterized by a peculiar distribution of fibrosis and other degenerative changes. Thus far, a comparable subcellular pattern of degenerative changes has not been reported. Our purpose in this communication is (i) to describe the ultrastructural characteristics of myocardial cells in patients with Duchenne's muscular dystrophy, (ii) to compare the changes in cardiac muscle with those in skeletal muscle, and (iii) to speculate as to the role of these myocardial changes in the genesis of electrocardiographic abnormalities in patients with this disease. Materials and MethodsStandard 12-lead electrocardiograms were obtained for 75 patients with Duchenne's progressive muscular dystrophy during follow-up observations at the Clinic for Muscular Disorders at St. Jude Children's Research Hospital. All patients were males ranging in age from 5 to 18 years with a median of 11.5 years. In each patient, the diagnosis of muscular dystrophy was established on the basis of clinical, biochemical and muscle biopsy findings. None of the patients had evidence of upper or lower respiratory tract infection or congestive heart failure at the time the electrocardiograms were obtained. A pansystolic blowing murmur, grade III/IV, was noted in four patients. In each of these pa- 1122ing and was seen most consistently in the posterobasal area of the left ventricle. Alterations of Z-band material; accumulation of mitochondria, occasionally containing electron-dense bodies and showing loss or discontinuity of cristae; dilatation of sarcoplasmic reticulum with striking ectasia of cisternae; depletion of glycogen particles; a paucity of lipoid or lipochrome granules; and the absence of virus-like particles were other consistent ultrastructural features. Comparison of skeletal and cardiac muscle disclosed identical subcellular changes. These observations support the contenti...

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Section: Histologic and Ultrastructural Features Of Control Heartssupporting

confidence: 66%

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confidence: 99%

An ultrastructural basis for electrocardiographic alterations associated with Duchenne's progressive muscular dystrophy.

et al. 1978

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“…'^The reason(s) for this distinctive localization are unknown, but the implication is that the {X)sterobasa] and contiguous left ventricular walls differ from the rest of ventricular myocardium, causing dystrophin-deficient myofibers in that region to be more vulnerable (sensitized) to calcium injury and myocardial dystrophy. Three observations are hypothetically relevant to this presumed regional difference in myocardial susceptibility to injury: (1) inhibitory cardiac receptors with vagal afferents activated by intracoronary injection of nicotine or veratridine are preferentially distributed in the inferoposterior wail of the canine left ventricle^^; (2) contraction or wall stress patterns may be peculiar to the infra-atrial portion of the left ventricW^; and (3) based on preliminary data in a canine model, localization of myocardial dystrophy may coincide with areas enriched with transient outward ionic currents (see below).…”

Section: Specialized Cardiac Tissuesmentioning

confidence: 99%

Cardiac Electrophysiology in Duchenne Muscular Dystrophy: From Basic Science to Clinical Expression

Perloff

Moı̈se

Stevenson

et al. 1992

Cardiovasc electrophysiol

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Cardiac Electrophysiology in Duchenne Muscular Dystrophy. Duchenne muscular dystrophy is an X‐linked recessive neuromuscular disorder that involves striated muscle fibers (skeletal, cardiac), smooth muscle Fibers (vasculature), and nervous system (neurons of central brain and cortex). The Duchenne dystrophy gene has been identified on the Xp21 locus of the short arm of the X chromosome. Dystrophin, the protein product of the gene, is present on and limited to myogenic cells in every tissue tested except the central nervous system, and is absent or nearly so in Duchenne dystrophy. However, the function of dystrophin and the pathogenesis of Duchenne dystrophy remain unclear. There is convincing morphological (gross, histologic, ultrastructural) and metabolic evidence (positron emission tomography) that myocardial involvement is found initially in the posterobasal left ventricular wall (cardiac phenotype). This regional localization of myocardial dystrophy is believed to account for the distinctive 12‐lead scalar electrocardiogram (ECG). Abnormalities of cardiac rhythm and conduction have been reported in Duchenne muscular dystrophy, but the pathogenesis of the electrophysiologic disturbances has not been established. Two variables are relevant to cardiac electrophysiologic involvement in Duchenne dystrophy: (1) the small vessel coronary arteriopathy (medial hypertrophy, luminal narrowing); and (2) abnormalities that might originate in the specialized cardiac tissues. The role of the coronary arteriopathy is presently speculative, and it is not currently known whether dystrophin is normally present on the cell membrane of normal cardiac specialized tissues, and if so, whether those tissues are dystrophin deficient in Duchenne dystrophy. Animal models (mouse, cat, dog) have shed light on the pathogenesis of skeletal muscle and myocardial involvement, less so on involvement of cardiac specialized tissues. The determinants of calcium homeostasis in dystrophin‐deficient myogenic cells (skeletal or cardiac) is unclear, and the relationship between dystrophin deficiency, calcium homeostasis, cellular response and phenotypic expression in skeletal muscle and myocardium is imperfectly understood. The purpose of this report is to bring together current genetic, molecular biological, biochemical, histopathological, and clinical information from human Duchenne dystrophy and from animal models bearing on the electrophysiologic expressions of the neuromuscular disease. (J Cardiovasc Electrophysiol, Vol. 3, pp. 394–409, August 1992)

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“…In our present state of knowledge, we may be justified in inferring from these results that the decrease in the leftward and inferior QRS forces results mainly from myofibrillar loss in the left ventricular free wall, and that progressive reduction of the Rx amplitude and abrupt reduction of the Ry amplitude are useful for assessing the extent of cardiac involvement. The duration of the QRS complex was slightly prolonged in the advanced stages.76) Ronan et al 24) also reported prolongation of the QRS complex. Abnormalities in the conducting system of the heart and marked myocardial involvement are considered to be a cause of such prolongation of the QRS complex.…”

Section: Investigations By Electrocardiography and Vectorcardiographymentioning

confidence: 90%

Cardiac Involvement in Progressive Muscular Dystrophy of the Duchenne Type.

Ishikawa

1997

Jpn Heart J

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SUMMARYDuchenne's progressive muscular dystrophy (DMD) is a genetic muscle disorder that causes degeneration and atrophy of the systemic and cardiac muscle. The disease is manifested early in childhood, and most of patients die by age 20 years of respiratory failure or heart failure. The cardiac involvement in DMD is characterized pathologically by degeneration and fibrosis of the myocardium, centering around the posterolateral wall of the left ventricle. Functionally, an abnormal electrocardiogram, valve motion, wall thickness, and wall motion are observed. Furthermore, abnormalities in plasma levels of atrial natriuretic peptide and autonomic function are also demonstrated.In this review, the cardiac involvements in DMD in the following aspects are described: 1) Electrocardiogram; a) high-frequency notches on the QRS complexes, b) amplitude of QRS complexes, c) late potential, d) arrhythmias, e) heart rate variability, f) a 10-year follow-up study, 2) Echocardiographic findings,

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The vectorcardiogram in Duchenne's progressive muscular dystrophy

Cited by 31 publications

References 17 publications

An ultrastructural basis for electrocardiographic alterations associated with Duchenne's progressive muscular dystrophy.

An ultrastructural basis for electrocardiographic alterations associated with Duchenne's progressive muscular dystrophy.

Cardiac Electrophysiology in Duchenne Muscular Dystrophy: From Basic Science to Clinical Expression

Cardiac Involvement in Progressive Muscular Dystrophy of the Duchenne Type.

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