Skip to main content
NCBI home page
Clinical Cardiology logoLink to Clinical Cardiology
. 2010 Mar 16;33(3):126–131. doi: 10.1002/clc.20712

Evaluation of Biventricular Functions With Tissue Doppler Imaging in Patients With Myotonic Dystrophy

Tolga Ozyigit 1, Beste Ozben 2,✉, Huseyin Oflaz 3, Piraye Serdaroglu 4
PMCID: PMC6652946  PMID: 20235214

Abstract

Background:

Myotonic dystrophy (MD) is characterized by myotonia with dystrophic involvement of the muscles. Cardiac involvement is usually not evident in the early stages of MD.

Hypothesis:

We investigated biventricular functions by tissue Doppler imaging (TDI) in MD patients with no overt cardiac involvement to explore the value of TDI in the early detection of myocardial dysfunction.

Methods:

A total of 21 MD patients (15 male, age: 32.2 ± 12.3 yrs) and 21 healthy controls (13 male, age: 32.2 ± 7.8 yrs) were included. In addition to conventional echocardiography, pulsed Doppler and TDI were performed including measurement of myocardial performance index (MPI); peak systolic (Sm) and early (Em) and atrial (Am) diastolic myocardial velocities at the basal mitral and tricuspid annulus.

Results:

All patients and controls had normal ejection fraction. Transmitral E peak velocity was significantly lower while both deceleration time of E velocity and isovolumic relaxation time were significantly longer in MD patients (P = 0.007, P = 0.001, and P < 0.001, respectively). Sm, Em and Am peak velocities were significantly lower in MD patients in all segments except for Em of the mitral anterior annulus and Am of the tricuspid lateral annulus. Both left and right ventricular MPI were significantly higher in MD patients (P < 0.001 and P = 0.013, respectively).

Conclusion:

There are changes in myocardial systolic and diastolic functions in MD patients although they have no overt heart failure. Myocardial tissue velocities and MPI are useful in identifying subclinical biventricular involvement in these patients. Copyright © 2010 Wiley Periodicals, Inc.

Full Text

The Full Text of this article is available as a PDF (117.3 KB).

References

  • 1. Mathieu J, Allard P, Potvin L, et al. A 10‐year study of mortality in a cohort of patients with myotonic dystrophy. Neurology 1999; 52: 1658–1662. [DOI] [PubMed] [Google Scholar]
  • 2. New nomenclature and DNA testing guidelines for myotonic dystrophy type 1 (DM1). The International Myotonic Dystrophy Consortium (IDMC). Neurology 2000; 54: 1218–1221. [DOI] [PubMed] [Google Scholar]
  • 3. Pelargonio G, Dello Russo A, Sanna T, et al. Myotonic dystrophy and the heart. Heart 2002; 88: 665–670. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 4. Rakocević‐Stojanović V, Grujić M, Seferović P, et al. Myotonic dystrophy and cardiac disorders. Panminerva Med 2000; 42: 257–261. [PubMed] [Google Scholar]
  • 5. Mammarella A, Paradiso M, Antonini G, et al. Natural history of cardiac involvement in myotonic dystrophy (Steinert's disease): a 13‐year follow‐up study. Adv Ther 2000; 17: 238–251. [DOI] [PubMed] [Google Scholar]
  • 6. Kennel AJ, Titus JL, Merideth J. Pathologic findings in the atrioventricular conduction system in myotonic dystrophy. Mayo Clin Proc 1974; 49: 838–842. [PubMed] [Google Scholar]
  • 7. Nguyen HH, Wolfe JT III, Holmes DR Jr, et al. Pathology of the cardiac conduction system in myotonic dystrophy: a study of 12 cases. J Am Coll Cardiol 1988; 11: 662–671. [DOI] [PubMed] [Google Scholar]
  • 8. Thornton C. The myotonic dystrophies. Semin Neurol 1999; 19: 25–33. [DOI] [PubMed] [Google Scholar]
  • 9. Sonaglioni G, Curatola L, Bollettini G, et al. Echocardiographic findings in dystrophia myotonica (Steinert's disease). G Ital Cardiol 1984; 14: 551–556. [PubMed] [Google Scholar]
  • 10. Fragola PV, Caló L, Luzi M, et al. Doppler echocardiographic assessment of left ventricular diastolic function in myotonic dystrophy. Cardiology 1997; 88: 498–502. [DOI] [PubMed] [Google Scholar]
  • 11. Bu'Lock FA, Sood M, De Giovanni JV, et al. Left ventricular diastolic function in congenital myotonic dystrophy. Arch Dis Child 1999; 80: 267–270. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 12. Parisi M, Galderisi M, Sidiropulos M, et al. Early detection of biventricular involvement in myotonic dystrophy by tissue Doppler. Int J Cardiol 2007; 118: 227–232. [DOI] [PubMed] [Google Scholar]
  • 13. Rakocević‐Stojanović V, Pavlović S, Seferović P, et al. Pathohistological changes in endomyocardial biopsy specimens in patients with myotonic dystrophy. Panminerva Med 1999; 41: 27–30. [PubMed] [Google Scholar]
  • 14. Sutherland GR, Bijnens B, McDicken WN. Tissue Doppler echocardiography: historical perspective and technological considerations. Echocardiography 1999; 16: 445–453. [DOI] [PubMed] [Google Scholar]
  • 15. Brook JD, McCurrach ME, Harley HG, et al. Molecular basis of myotonic dystrophy: expansion of a trinucleotide (CTG) repeat at the 3′ end of a transcript encoding a protein kinase family member. Cell 1992; 68: 799–808. [DOI] [PubMed] [Google Scholar]
  • 16. Nagueh SF, Middleton KJ, Kopelen HA, et al. Doppler tissue imaging: a noninvasive technique for evaluation of left ventricular relaxation and estimation of filling pressures. J Am Coll Cardiol 1997; 30: 1527–1533. [DOI] [PubMed] [Google Scholar]
  • 17. Nagueh SF, Mikati I, Kopelen HA, et al. Doppler estimation of left ventricular filling pressure in sinus tachycardia. A new application of tissue Doppler imaging. Circulation 1998; 98: 1644–1650. [DOI] [PubMed] [Google Scholar]
  • 18. Garcia MJ, Thomas JD, Klein AL. New Doppler echocardiographic applications for the study of diastolic function. J Am Coll Cardiol 1998; 32: 865–875. [DOI] [PubMed] [Google Scholar]
  • 19. Sundereswaran L, Nagueh SF, Vardan S, et al. Estimation of left and right ventricular filling pressures after heart transplantation by tissue Doppler imaging. Am J Cardiol 1998; 82: 352–357. [DOI] [PubMed] [Google Scholar]
  • 20. Tekten T, Onbasili AO, Ceyhan C, et al. Novel approach to measure myocardial performance index: pulsed‐wave tissue Doppler echocardiography. Echocardiography 2003; 20: 503–510. [DOI] [PubMed] [Google Scholar]
  • 21. Vinereanu D, Bajaj BP, Fenton‐May J, et al. Subclinical cardiac involvement in myotonic dystrophy manifesting as decreased myocardial Doppler velocities. Neuromuscul Disord 2004; 14: 188–194. [DOI] [PubMed] [Google Scholar]
  • 22. De Ambroggi L, Raisaro A, Marchianó V, et al. Cardiac involvement in patients with myotonic dystrophy: characteristic features of magnetic resonance imaging. Eur Heart J 1995; 16: 1007–1010. [DOI] [PubMed] [Google Scholar]
  • 23. Tokgozoglu LS, Ashizawa T, Pacifico A, et al. Cardiac involvement in a large kindred with myotonic dystrophy. Quantitative assessment and relation to size of CTG repeat expansion. JAMA 1995; 274: 813–819. [PubMed] [Google Scholar]
  • 24. Fung KC, Corbett A, Kritharides L. Myocardial tissue velocity reduction is correlated with clinical neurologic severity in myotonic dystrophy. Am J Cardiol 2003; 92: 177–181. [DOI] [PubMed] [Google Scholar]
  • 25. Gorcsan J III, Deswal A, Mankad S, et al. Quantification of the myocardial response to low‐dose dobutamine using tissue Doppler echocardiographic measures of velocity and velocity gradient. Am J Cardiol 1998; 81: 615–623. [DOI] [PubMed] [Google Scholar]
  • 26. Titcolor Nagueh SF, Titcolor Bachinski LL, Titcolor Meyer D, et al. Tissue Doppler imaging consistently detects myocardial abnormalities in patients with Titcolor hypertrophic cardiomyopathy and provides a novel means for an early diagnosis before and independently of hypertrophy. Circulation. 2001; 104: 128–130. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 27. Tei C. New non‐invasive index for combined systolic and diastolic ventricular function. J Cardiol 1995; 26: 135–136. [PubMed] [Google Scholar]
  • 28. Tei C, Ling LH, Hodge DO, et al. New index of combined systolic and diastolic myocardial performance: a simple and reproducible measure of cardiac function—a study in normals and dilated cardiomyopathy. J Cardiol 1995; 26: 357–366. [PubMed] [Google Scholar]
  • 29. Poulsen SH, Jensen SE, Nielsen JC, et al. Serial changes and prognostic implications of a Doppler‐derived index of combined left ventricular systolic and diastolic myocardial performance in acute myocardial infarction. Am J Cardiol 2000; 85: 19–25. [DOI] [PubMed] [Google Scholar]
  • 30. Lindqvist P, Mörner S, Olofsson BO, et al. Ventricular dysfunction in type 1 myotonic dystrophy: Electrical, mechanical, or both? Int J Cardiol 2009; doi:10.1016/j.ijcard.2009.03.084. [DOI] [PubMed] [Google Scholar]

Articles from Clinical Cardiology are provided here courtesy of Wiley

RESOURCES