Heart Involvement in Progressive Muscular Dystrophy

Storstein, O; Kinge, Finn Olav

doi:10.1111/j.1600-0447.1961.tb01062.x

Cited by 14 publications

(5 citation statements)

References 10 publications

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“…Evidence of cardiac pathology in muscular dystrophy has been reviewed by several authors (see Gailani, Danowski, and Fisher, 1958;Lowenstein et al, 1962;Storstein, 1962;Solti, Zadory, and Bekeny, 1963;Storstein, 1964 Zimmerman, 1969). Particularly in the Duchenne type of X-linked muscular dystrophy the most consistent ECG abnormality is the presence of tall R waves in the right praecordial lead (Weisenfeld and Messinger, 1952;Schott et al, 1955;Gailani et al, 1958;Manning and Cropp, 1958;Skyring and McKusick, 1961;Gilroy et al, 1963;Perloff et al, 1966 andJedrzejowska-Kulakowska et al, 1968;Slucka, 1968;de los Arcos, Cascos, and de Rabago, 1969).…”

Section: Discussionmentioning

confidence: 99%

Abnormalities of the electrocardiogram in hereditary myopathies.

Emery¹

1972

Journal of Medical Genetics

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Section: Discussionmentioning

confidence: 99%

Abnormalities of the electrocardiogram in hereditary myopathies.

Emery¹

1972

Journal of Medical Genetics

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“…It has generally been difficult to associate the accounts of heart disease with specific types of systemic myopathy since the term "progressive muscular dystrophy" has usually been applied as an overall designation without attempting to subdivide patients into the various clinical groups." [10][11][12][13][14][15][16][17][18][19] Furthermore, the different forms of muscular dystrophy resemble each other histologically.9' 20 Biopsy, therefore, cannot be depended upon to separate one from the other. Reviews have been limited by employing retrospective analyses of hospital case records'0 or by depending upon published reports of incompletely classified cases.'…”

mentioning

confidence: 99%

The Cardiomyopathy of Progressive Muscular Dystrophy

1966

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A prospective study was undertaken in order to examine the histories, physical signs, phonocardiograms, electrocardiograms, vectorcardiograms, X-rays, serum enzymes, pulmonary ventilatory findings, and hemodynamics in a group of 55 patients selected solely on the basis of one of the major types of progressive muscular dystrophy (table 9). The classification included: (1) the classic Duchenne type, (2) the limb girdle type, (3) the facioscapulohumeral type, and (4) a group of patients in whom clinical distinction between benign Duchenne dystrophy and limb girdle dystrophy with pseudohypertrophy could not be made. No patient had recognized heart disease prior to selection. Follow-up periods ranged from a few months to 5 years. The pertinent literature was extensively reviewed. In all groups clinical evidence of heart disease had to be interpreted in the context of the influence of the somatic musculoskeletal defects on "cardiac" symptoms, cardiovascular physical signs, and radiological appearance of the heart. On the other hand, convincing evidence of intrinsic heart disease-subtle to overt-was found in each of the major categories of progressive muscular dystrophy. Detection of cardiomyopathy was simplest and most frequent in the classic Duchenne group because of the highly characteristic features of the scalar electrocardiogram which might direct attention to the systemic myopathy prior to its clinical recognition. However, the presence of heart disease in patients with forms of dystrophy other than the classic Duchenne deserves particular emphasis. Evidence of cardiomyopathy in these latter forms varied from subtle quadruple gallop rhythms in the limb girdle and facioscapulohumeral groups to cardiomyopathic death in the benign Duchenne and limb girdle-pseudohypertrophic group. Currently available information on the heart in muscular dystrophy has as a rule depended upon retrospective studies, upon reviews of published reports of incompletely classified cases, upon discussions of individual cardiovascular features, or upon observations of individual types of dystrophy. The present prospective study was undertaken to obviate some of these shortcomings. Comprehensive assessment of a large and carefully classified group of dystrophic subjects has provided additional information regarding the clinical spectrum of dystrophic cardiomyopathy.

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“…Measurement of pulmonary arterial pressure during oxygen breathing has not been carried out in these patients. One should expect it to fall as it commonly does in patients with heart failure (12). In this way there will be a fall in the work of the right ventricle.…”

Section: Discussionmentioning

confidence: 96%

The Cause of Arterial Hypoxemia in Acute Myocardial Infarction

Storstein¹,

Rasmussen²

1968

Journal of Internal Medicine

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The mechanism of arterial hypoxemia, commonly found in patients with acute myocardial infarction, has been studied in 24 patients with polarographic determination of arterial pO2 before and after ten minutes of pure oxygen breathing. Most of the patients have a reduction in arterial pO2 on air breathing and do not attain normal values for pO2 on oxygen breathing. Patients in shock or with pulmonary congestion show more severely reduced values of pO2 both on air breathing and on oxygen breathing. Calculation of the so‐called physiological shunt on air breathing and the so‐called anatomical shunt on oxygen breathing demonstrates that most of the reduction in arterial pO2 is due to disturbance of the ventilationperfusion relationships in the lungs, but a considerable amount of the reduction in pO2 is caused by shunting of pulmonary arterial blood through non‐ventilated parts of the lungs.

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Heart Involvement in Progressive Muscular Dystrophy

Cited by 14 publications

References 10 publications

Abnormalities of the electrocardiogram in hereditary myopathies.

Abnormalities of the electrocardiogram in hereditary myopathies.

The Cardiomyopathy of Progressive Muscular Dystrophy

The Cause of Arterial Hypoxemia in Acute Myocardial Infarction

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