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

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.

Assessment of cardiac involvement in myotonic muscular dystrophy by T1 mapping on magnetic resonance imaging

“…However, two recent studies, did not observe a difference in LV mass, end diastolic and end-systolic volume, and ejection fraction of DM patients compared to control subjects. 13, 14 Such discrepancies may be due to variations in disease severity among study samples. The current study was in line with previous studies by showing no significant difference in LV mass and ejection fraction in DM patients compared to control subjects.…”

Myotonic dystrophy and the heart: A systematic review of evaluation and management

“…Tissue Doppler Lower longitudinal and radial systolic velocities (S′), longer time to peak systolic velocity Systolic velocities correlate inversely with age, QRS duration and severity of neurologic manifestations, but not with number of CTG repeats [13,48] Lower early diastolic velocity (E′) and atrial velocity (A′) even with normal LV systolic function [7] Lower E′ and E′/A′ ratios and higher E/E′ at lateral tricuspid annulus [7] Spectral Doppler Lower peak transmitral early (E) velocities, lower peak atrial (A) velocities, prolongation of the deceleration time of the E wave and increased isovolumic relaxation time compared to healthy controls [2,7,13,23,86] Correlation between parameters of diastolic dysfunction with age and duration of neurological symptoms [2] Speckle tracking Significantly lower LV peak strain rate and lower mean strain rate Early diastolic dysfunction detected by a lower E/A strain rate [87] Lower global longitudinal strain compared to controls, despite normal ejection fraction Higher radial strain in patients with MD [88] Right atrial speckle tracking analysis correlates with regions of low voltage on electroanatomical mapping [34] Myocardial performance index MPI (isovolumic relaxation time + isovolumic contraction time / ejection time) of LV and RV are higher in patients with MD than normal subjects [86] High discriminating ability to distinguish between the hearts of MD patients and those without MD [87] rate of progression does not correlate with the initial HV interval on serial EP studies [68]. New conduction disease on the surface ECG is strongly associated with progressive HV prolongation at repeat EP study.…”

Outcome measures frequently used to assess muscle strength in patients with myotonic dystrophy type 1: a systematic review

“…Twenty-four studies assessed the cardiac muscle strength indirectly [34][35][36][37][38][39][40][41][42][43][44][45][46][47][48][49][50][51][52][104][105][106]112,113].…”

“…Twenty-two studies used echocardiography [34][35][36][37][39][40][41][42][43][44][45][46][47][48][49][50][51][52]105,106,112,113] and three used cardiac magnetic resonance [38,104,106] for the indirect evaluation of cardiac muscle strength. Main findings of the measures used to assess cardiac muscle strength for each study can be found in table 1 and Figure 2A.…”

“…Left ventricular ejection fraction (n=21) [34][35][36][37][39][40][41][42][43][44][45][46][47][49][50][51][52]105,106,112,113], left ventricular shortening fraction (n=4) [39,46,51,52], left ventricular global longitudinal strain (n=3) [43,44,106], endocardial shortening fraction (n=2) [47,48] and stroke volume (n=1) [46] were evaluated (table 1). The range of left ventricular ejection fraction values obtained was 56%-70% for patients with DM1 and 61%-77% for controls [34][35][36][37][39][40][41][42][43][44][45][46][47][49][50][51]…”