Obscure cardiomyopathies with coronary artery changes |
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| Authors: | E Varnauskas B Ivemark S Paulin B Rydén |
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| Affiliation: | 1. Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA;2. Computer Science and Artificial Intelligence Lab, MIT, USA;3. Department of Population Health Sciences, German Centre for Neurodegenerative Diseases (DZNE), Germany;4. Athinoula A. Martinos Center for Biomedical Imaging, Department of Radiology, Massachusetts General Hospital, Charlestown, MA, USA;5. Program in Medical and Population Genetics, Broad Institute of MIT and Harvard, Cambridge, MA, USA;6. Department of Clinical Sciences Lund, Neurology, Lund University, Lund, Sweden;7. Department of Clinical Sciences Lund, Radiology, Lund University, Lund, Sweden;8. Department of Radiology, Neuroradiology, Skåne University Hospital, Malmö, Sweden;9. Department of Neurology, University of Maryland School of Medicine and Veterans Affairs Maryland Health Care System, Baltimore, MD, USA;10. Institute of Neuroscience and Physiology, the Sahlgrenska Academy at University of Gothenburg, Gothenburg, Sweden;11. Institute of Biomedicine, the Sahlgrenska Academy at University of Gothenburg, Gothenburg, Sweden;12. Department of Neurology, Neurovascular Research Group (NEUVAS), IMIM-Hospital del Mar (Institut Hospital del Mar d''Investigacions Mèdiques), Universitat Autonoma de Barcelona, Barcelona, Spain;13. Department of Neurosciences, Experimental Neurology and Leuven Research Institute for Neuroscience and Disease (LIND), KU Leuven - University of Leuven, Leuven, Belgium;14. VIB, Vesalius Research Center, Laboratory of Neurobiology, Department of Neurology, University Hospitals Leuven, Leuven, Belgium;15. Department of Neurology and Rehabilitation Medicine, Skåne University Hospital, Lund, Sweden;p. Department of Neurology, Mayo Clinic, Jacksonville, FL, USA;q. Department of Neurology, Miller School of Medicine, University of Miami, Miami, FL, USA;r. Department of Neurology, Clinical Division of Neurogeriatrics, Medical University Graz, Graz, Austria;s. Institute of Cardiovascular Research, St Peter''s and Ashford Hospitals, Royal Holloway University of London (ICR2UL), Egham, UK;t. Department of Neurology, Jagiellonian University Medical College, Krakow, Poland;u. Stroke Division, Australia and Department of Neurology, Austin Health, Florey Institute of Neuroscience and Mental Health, Heidelberg, Australia;v. Department of Neurology and Rehabilitation Medicine, University of Cincinnati College of Medicine, Cincinnati, OH, USA;w. Department of Radiology, University of Cincinnati College of Medicine, Cincinnati, OH, USA;x. Division of Endocrinology, Diabetes and Nutrition, Department of Medicine, University of Maryland School of Medicine, Baltimore, MD, USA;y. Center for Human Genetic Research, Massachusetts General Hospital, Boston, MA, USA;z. Departments of Neurology and Public Health Sciences, University of Virginia, Charlottesville, VA, USA |
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| Abstract: | A series of patients with suspected cardiomyopathy were investigated by coronary angiography. Thirteen of them had coronary arteriograms suggesting more or less extensive coronary involvement of the type demonstrated by markedly straight and narrow branches of both main coronary arteries. One of these patients had established Friedreich's disease and another had three sisters affected by this neurologic disease. The coronary angiographie findings in these 2 patients were indistinguishable from those of the remaining 11.A special histopathologic examination in 1 of the 5 deceased patients (all from the group of 11) disclosed widely distributed medial changes in the medium-sized and small coronary arteries and fibrotic involvement of the myocardium of the same appearance as described in cases of Friedreich's disease or other heritable diseases. Thus a possibility exists that a similar coronary arteriopathy is responsible for the angiographie pattern of all patients in the present series.Various types of cardiac arrhythmias, occurring paroxysmally or persistently, conduction disturbances, and in a few cases cardiac failure as well as chest pain, were the main causes of symptoms, except in 3 patients who were free of symptoms. No conclusive correlation could be established between electrocardiographic abnormalities (including ST-T changes in a few patients) and the extent and distribution of the coronary involvement on the arteriograms. The arrhythmias were, with one exception, resistant to the usual therapy. Hemodynamic (in 4 patients) and other laboratory investigations were unrewarding concerning the pathogenesis.Five of the 13 patients died (4 in ventricular fibrillation and 1 in congestive heart failure) during a relatively short follow-up period. |
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