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Families With Myotonic Dystrophy With and Without Cardiac Involvement

Families With Myotonic Dystrophy With and Without Cardiac Involvement Abstract • Study of 30 patients with myotonic dystrophy and 17 unaffected family members in a total of 18 families by echocardiography and ECG, including noninvasive His-bundle recording, appeared to show two phenotypes of myotonic dystrophy: those with and those without substantial cardiac involvement. Besides the 29% incidence of asymptomatic mitral valve prolapse common to many neuromuscular diseases, 20% of families had progressive involvement of the cardiac conduction system. It is important to recognize these families in order to treat patients with cardiac pacemakers who are rapidly progressive or symptomatic. If one patient with myotonic dystrophy is found to have myotonic heart disease with cardiac block or arrhythmia, it should be expected in other family members with myotonic dystrophy. (Arch Intern Med 1983;143:2134-2136) References 1. Griffith TW: On myotonia. Q J Med 1911;5:229-249. 2. Spillane JD: The heart in myotonia atrophica. Br Heart J 1951;13:343-349.Crossref 3. Fisch C: The heart in dystrophia myotonica. Am Heart J 1951;41:525-538.Crossref 4. Orndahl G, Thulesius O, Sverker E, et al: The heart in myotonic disease. Acta Med Scand 1964;176:479-491.Crossref 5. Kuhn VE: Entwicklung eines Linkeschenkelblocks bei myotonischer Dystrophie. Schweiz Med Wochenshr 1960;90:1160-1161. 6. Prystowsky EN, Pritchett ELC, Gallagher JJ, et al: The natural history of the cardiac conduction system disease in myotonic dystrophy as determined by serial electrophysiologic studies. Circulation 1979;60:1360-1364.Crossref 7. Kennel AJ, Titus JL, Merideth J: Pathologic findings in the atrioventricular conduction in myotonic dystrophy. May Clin Proc 1974;49:838-842. 8. Thomson AMP: Dystrophia cordis myotonica studied by serial histology of the pacemaker and conducting system. J Pathol 1968;96:285-295.Crossref 9. Clements SD, Colmers RA, Hurst JW: Myotonia dystrophica: ventricular arrhythmias, intraventricular conduction abnormalities, atrioventricular block and Stokes-Adams successfully treated with permanent transvenous pacemaker. Am J Cardiol 1976;37:933-935.Crossref 10. Griggs RC, Davis RJ, Anderson DC, et al: Cardiac conduction in myotonic dystrophy. Am J Med 1975;59:37-42.Crossref 11. Winters SJ, Schreiner B, Griggs RC, et al: Familial mitral valve prolapse and myotonic dystrophy. Ann Intern Med 1976;85:19-22.Crossref 12. Walton JN, Gardner-Medwin D: Progressive muscular dystrophy and the myotonic disorders , in Walton JSN (ed): Disorders of Voluntary Muscle . London, Churchill Livingstone, 1981, chap 14. 13. Gottdiener JS, Hawley RJ, Gay JA, et al: Left ventricular relaxation, mitral valve prolapse, and intracardiac conduction in myotonia atrophica: Assessment by echocardiography and non-invasive His bundle recording. Am Heart J 1982;104:77-85.Crossref 14. Helfant RH, Scherlag BJ: His bundle electrocardiography: The atrioventricular and intraventricular conduction system . New York, Medcom, 1974, chap 1. 15. Cook AW, Bird TD, Spencer AM, et al: Myotonic dystrophy, mitral-valve prolapse, and stroke. Lancet 1978;335-336. 16. Motta J, Guilleminault C, Billingham M, et al: Cardiac abnormalities in myotonic dystrophy: Electrophysiologic and histopathologic studies. Am J Med 1979;67:467-473.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Internal Medicine American Medical Association

Families With Myotonic Dystrophy With and Without Cardiac Involvement

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Publisher
American Medical Association
Copyright
Copyright © 1983 American Medical Association. All Rights Reserved.
ISSN
0003-9926
eISSN
1538-3679
DOI
10.1001/archinte.1983.00350110120025
Publisher site
See Article on Publisher Site

Abstract

Abstract • Study of 30 patients with myotonic dystrophy and 17 unaffected family members in a total of 18 families by echocardiography and ECG, including noninvasive His-bundle recording, appeared to show two phenotypes of myotonic dystrophy: those with and those without substantial cardiac involvement. Besides the 29% incidence of asymptomatic mitral valve prolapse common to many neuromuscular diseases, 20% of families had progressive involvement of the cardiac conduction system. It is important to recognize these families in order to treat patients with cardiac pacemakers who are rapidly progressive or symptomatic. If one patient with myotonic dystrophy is found to have myotonic heart disease with cardiac block or arrhythmia, it should be expected in other family members with myotonic dystrophy. (Arch Intern Med 1983;143:2134-2136) References 1. Griffith TW: On myotonia. Q J Med 1911;5:229-249. 2. Spillane JD: The heart in myotonia atrophica. Br Heart J 1951;13:343-349.Crossref 3. Fisch C: The heart in dystrophia myotonica. Am Heart J 1951;41:525-538.Crossref 4. Orndahl G, Thulesius O, Sverker E, et al: The heart in myotonic disease. Acta Med Scand 1964;176:479-491.Crossref 5. Kuhn VE: Entwicklung eines Linkeschenkelblocks bei myotonischer Dystrophie. Schweiz Med Wochenshr 1960;90:1160-1161. 6. Prystowsky EN, Pritchett ELC, Gallagher JJ, et al: The natural history of the cardiac conduction system disease in myotonic dystrophy as determined by serial electrophysiologic studies. Circulation 1979;60:1360-1364.Crossref 7. Kennel AJ, Titus JL, Merideth J: Pathologic findings in the atrioventricular conduction in myotonic dystrophy. May Clin Proc 1974;49:838-842. 8. Thomson AMP: Dystrophia cordis myotonica studied by serial histology of the pacemaker and conducting system. J Pathol 1968;96:285-295.Crossref 9. Clements SD, Colmers RA, Hurst JW: Myotonia dystrophica: ventricular arrhythmias, intraventricular conduction abnormalities, atrioventricular block and Stokes-Adams successfully treated with permanent transvenous pacemaker. Am J Cardiol 1976;37:933-935.Crossref 10. Griggs RC, Davis RJ, Anderson DC, et al: Cardiac conduction in myotonic dystrophy. Am J Med 1975;59:37-42.Crossref 11. Winters SJ, Schreiner B, Griggs RC, et al: Familial mitral valve prolapse and myotonic dystrophy. Ann Intern Med 1976;85:19-22.Crossref 12. Walton JN, Gardner-Medwin D: Progressive muscular dystrophy and the myotonic disorders , in Walton JSN (ed): Disorders of Voluntary Muscle . London, Churchill Livingstone, 1981, chap 14. 13. Gottdiener JS, Hawley RJ, Gay JA, et al: Left ventricular relaxation, mitral valve prolapse, and intracardiac conduction in myotonia atrophica: Assessment by echocardiography and non-invasive His bundle recording. Am Heart J 1982;104:77-85.Crossref 14. Helfant RH, Scherlag BJ: His bundle electrocardiography: The atrioventricular and intraventricular conduction system . New York, Medcom, 1974, chap 1. 15. Cook AW, Bird TD, Spencer AM, et al: Myotonic dystrophy, mitral-valve prolapse, and stroke. Lancet 1978;335-336. 16. Motta J, Guilleminault C, Billingham M, et al: Cardiac abnormalities in myotonic dystrophy: Electrophysiologic and histopathologic studies. Am J Med 1979;67:467-473.Crossref

Journal

Archives of Internal MedicineAmerican Medical Association

Published: Nov 1, 1983

References