Skip to main content
British Heart Journal logoLink to British Heart Journal
. 1978 Nov;40(11):1262–1266. doi: 10.1136/hrt.40.11.1262

Noninvasive assessment of left ventricular function in myotonic muscular dystrophy.

A Venco, M Saviotti, D Besana, G Finardi, G Lanzi
PMCID: PMC483561  PMID: 718766

Abstract

In order to assess left ventricular function, measurements of left ventricular internal dimension and its rate of change have been made by echocardiography in 7 patients with myotonic dystrophy and the three children of one of them, who were clinically normal but had abnormal muscle biopsies. Electrocardiograms and systolic time intervals were also recorded in all. Only one patient had signs of overt heart disease and an abnormal electrocardiogram (type B WPW). Systolic time intervals were normal in all 7 patients. Five subjects had echocardiographic abnormalities, which were of minor degree except in the patient with overt heart disease who had considerable impairment of both systolic and diastolic left ventricular function. Another patient had abnormalities of both systolic and diastolic function; systolic abnormalities occurred alone in one patient and diastolic abnormalities alone in one relative. It is concluded that patients with myotonic dystrophy and no clinical signs of heart disease may have minor abnormalities of left ventricular function as shown by echocardiography. Echocardiography is more sensitive than systolic time intervals in detecting these abnormalities; both systolic and diastolic function abnormalities, alone or together, can occur. There seems to be no relation between involvement of skeletal and cardiac muscle.

Full text

PDF
1262

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Buchthal F. Electrophysiological signs of myopathy as related with muscle biopsy. Acta Neurol (Napoli) 1977 Jan-Feb;32(1):1–29. [PubMed] [Google Scholar]
  2. CANNON P. J. The heart and lungs in myotonic muscular dystrophy. Am J Med. 1962 May;32:765–775. doi: 10.1016/0002-9343(62)90166-3. [DOI] [PubMed] [Google Scholar]
  3. Chapelle M., Mensch B. Etude des variations du diamètre ventriculaire gauche chez l'homme par échocardiographie transthoracique. Arch Mal Coeur Vaiss. 1969 Nov;62(11):1505–1517. [PubMed] [Google Scholar]
  4. Church S. C. The heart in myotonia atrophica. Arch Intern Med. 1967 Feb;119(2):176–181. [PubMed] [Google Scholar]
  5. DeMaria A. N., Vera Z., Neumann A., Mason D. T. Alterations in ventricular contraction pattern in the Wolff-Parkinson-White syndrome. Detection by echocardiography. Circulation. 1976 Feb;53(2):249–257. doi: 10.1161/01.cir.53.2.249. [DOI] [PubMed] [Google Scholar]
  6. DeWIND L. T., JONES R. J. Cardiovascular observations in dystrophia myotonica. J Am Med Assoc. 1950 Sep 23;144(4):299–303. doi: 10.1001/jama.1950.02920040013004. [DOI] [PubMed] [Google Scholar]
  7. FEARRINGTON E. L., GIBSON T. C., CHURCHILL R. E. VECTORCARDIOGRAPHIC AND ELECTROCARDIOGRAPHIC FINDINGS IN MYOTONIA ATROPHICA. A STUDY EMPLOYING THE FRANK LEAD SYSTEM. Am Heart J. 1964 May;67:599–609. doi: 10.1016/0002-8703(64)90330-8. [DOI] [PubMed] [Google Scholar]
  8. FISCH C., EVANS P. V. The heart in dystrophia myotonica; report of an autopsied case. N Engl J Med. 1954 Sep 23;251(13):527–529. doi: 10.1056/NEJM195409232511306. [DOI] [PubMed] [Google Scholar]
  9. FISCH C. The heart in dystrophia myotonica. Am Heart J. 1951 Apr;41(4):525–538. doi: 10.1016/0002-8703(51)90020-8. [DOI] [PubMed] [Google Scholar]
  10. Gibson D. G., Brown D. Measurement of instantaneous left ventricular dimension and filling rate in man, using echocardiography. Br Heart J. 1973 Nov;35(11):1141–1149. doi: 10.1136/hrt.35.11.1141. [DOI] [PMC free article] [PubMed] [Google Scholar]
  11. HOLT J. M., LAMBERT E. H. HEART DISEASE AS THE PRESENTING FEATURE IN MYOTONIA ATROPHICA. Br Heart J. 1964 May;26:433–436. doi: 10.1136/hrt.26.3.433. [DOI] [PMC free article] [PubMed] [Google Scholar]
  12. Hishida H., Sotobata I., Koike Y., Okumura M., Mizuno Y. Echocardiographic patterns of ventricular contraction in the Wolff-Parkinson-White Syndrome. Circulation. 1976 Oct;54(4):567–570. doi: 10.1161/01.cir.54.4.567. [DOI] [PubMed] [Google Scholar]
  13. MILLER P. B. Myotonic dystrophy with electrocardiographic abnormalities. Report of a case. Am Heart J. 1962 May;63:704–707. doi: 10.1016/0002-8703(62)90017-0. [DOI] [PubMed] [Google Scholar]
  14. PAYNE C. A., GREENFIELD J. C., Jr Electrocardiographic abnormalities associated with myotonic dystrophy. Am Heart J. 1963 Apr;65:436–440. doi: 10.1016/0002-8703(63)90092-9. [DOI] [PubMed] [Google Scholar]
  15. PETKOVICH N. J., DUNN M., REED W. MYOTONIA DYSTROPHICA WITH A-V DISSOCIATION AND STOKES-ADAMS ATTACKS: A CASE REPORT AND REVIEW OF THE LITERATURE. Am Heart J. 1964 Sep;68:391–396. doi: 10.1016/0002-8703(64)90307-2. [DOI] [PubMed] [Google Scholar]
  16. Popp R. L., Wolfe S. B., Hirata T., Feigenbaum H. Estimation of right and left ventricular size by ultrasound. A study of the echoes from the interventricular septum. Am J Cardiol. 1969 Oct;24(4):523–530. doi: 10.1016/0002-9149(69)90495-0. [DOI] [PubMed] [Google Scholar]
  17. Rampulla C., Mandelli V., Venco A., Benza G. C., Beulcke G., Finardi G., Morpurgo M. Metodo di elaborazione statistica e di rappresentazione grafica di alcuni fattori che modificano i tempi della cardiodinamica ventricolare sinistra. G Ital Cardiol. 1973;3(1):41–51. [PubMed] [Google Scholar]
  18. Schmitt J., Schmidt C. l. Maladie de Steinert et myocarde. Expressivite totale du gène pour le tissu musculaire du coeur. J Genet Hum. 1975 Mar;23(1):59–64. [PubMed] [Google Scholar]
  19. Slatt B. Myotonia Dystrophia: A Review of 17 Cases. Can Med Assoc J. 1961 Jul 29;85(5):250–261. [PMC free article] [PubMed] [Google Scholar]
  20. Venco A., Gibson D. G., Brown D. J. Relation between apex cardiogram and changes in left ventricular pressure and dimension. Br Heart J. 1977 Feb;39(2):117–125. doi: 10.1136/hrt.39.2.117. [DOI] [PMC free article] [PubMed] [Google Scholar]
  21. Weissler A. M., Harris W. S., Schoenfeld C. D. Systolic time intervals in heart failure in man. Circulation. 1968 Feb;37(2):149–159. doi: 10.1161/01.cir.37.2.149. [DOI] [PubMed] [Google Scholar]
  22. Wolintz A. H., Sonnenblick E. H., Engel W. K. Stokes-Adams syndrome and atrial arrhythmias as the presenting symptoms of myotonic dystrophy, with response to electrocardioversion. Ann Intern Med. 1966 Dec;65(6):1260–1266. doi: 10.7326/0003-4819-65-6-1260. [DOI] [PubMed] [Google Scholar]

Articles from British Heart Journal are provided here courtesy of BMJ Publishing Group

RESOURCES