Ventricular dysfunction in type 1 myotonic dystrophy: Electrical, mechanical, or both?

Lindqvist, Per; Mörner, Stellan; Backman, Christer; Lundblad, Dan; Forsberg, Håkan; Henein, Michael Y.

doi:10.1016/j.ijcard.2009.03.084

Cited by 25 publications

(23 citation statements)

References 30 publications

(29 reference statements)

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“…This is an unlikely explanation since our findings show an additional clear evidence for compromised LV subendocardial long axis function in patients with reduced HRV. This confirms the presence of intrinsic myocardial involvement with the amyloid pathology, despite being at a subendocardial level, and hence the dyssynchronous function, as reviously shown in other systemic diseases involving the myocardium [24], [25]. p…”

Section: Data Interpretationsupporting

confidence: 77%

Left ventricular dyssynchrony is associated with reduced heart rate variability in familial amyloidotic polyneuropathy

Zhao

Hörnsten

Lindqvist

et al. 2012

International Journal of Cardiology

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Suhr, O. et al. (2012) "Left ventricular dyssynchrony is associated with reduced heart rate variability in familial amyloidotic polyneuropathy" International Journal of Cardiology, 155 (2) Methods: Twenty-nine FAP patients and 29 healthy controls were studied using Doppler echocardiography. Patients' and controls' HRV was studied using power spectral analysis from 24-hour Holter ECG recordings. Results:In FAP patients, all HRV parameters were lower (p<0.01 for all) than in controls.Echocardiography showed normal LV systolic function in patients. Relative filling time (FT/RR) was shorter (p<0.01) and total isovolumic time (t-IVT) was longer (p<0.01) in patients than in controls. E/Em was higher (p<0.01), as was Tei index (p=0.02) as compared to controls. T-IVT and Tei index correlated with stroke volume (SV) (r=-0.54, p<0.05 and r=-0.44, p<0.05, respectively) in patients.HRV was reduced in 9/29 (31%) patients, who had shorter FT/RR (p<0.01), longer t-IVT (p<0.01), higher Tei index (p=0.05), A wave (p<0.01) and E/Em (p<0.05) than in subjects without reduced HRV. FT/RR and t-IVT correlated with HRV spectral parameters (p<0.05 for all). The correlation between t-IVT and SV was stronger in patients with reduced HRV (r=-0.80, p<0.01) than in those without. QRS duration was not different in the two subgroups of patients Conclusions:In a subset of patients with FAP, HRV was significantly reduced and appeared to be associated with shortened LV filling time and prolonged t-IVT, which reflect ventricular dyssynchrony, despite normal QRS. Thus, in addition to autonomic 2 disturbances in FAP, ventricular dyssynchrony is another factor associated with reduced HRV. Correction of such disturbed ventricular function by cardiac resynchronization therapy may control patients' symptom.

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Section: Data Interpretationsupporting

confidence: 77%

Left ventricular dyssynchrony is associated with reduced heart rate variability in familial amyloidotic polyneuropathy

Zhao

Hörnsten

Lindqvist

et al. 2012

International Journal of Cardiology

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“…29 There is only 1 recently published study which demonstrated higher LV MPI in these patients. 30 To our knowledge, our study is the first that also demonstrated significantly higher RV MPI in MD patients. Since the MPI does not depend on ventricular geometry and incorporates diastolic function in addition to systolic function, we suggest the use of MPI in addition to myocardial tissue velocities for detection of biventricular involvement in MD patients.…”

Section: Discussionsupporting

confidence: 50%

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

Özyiğit

Özben

Oflaz

et al. 2010

Clinical Cardiology

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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.

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“…9 Even when LV impairment is mild, there is often marked cardiac dyssynchrony. 10 Both electrical (broad QRS) and myocardial factors for dyssynchrony are implicated. 10 Dyssynchrony is associated with reduced exercise tolerance and a poorer prognosis.…”

Section: Lesson Of the Month (1)mentioning

confidence: 99%

“…10 Both electrical (broad QRS) and myocardial factors for dyssynchrony are implicated. 10 Dyssynchrony is associated with reduced exercise tolerance and a poorer prognosis. If detected early, cardiac resynchronisation therapy can potentially improve survival and reduce heart failure symptoms, as in conventional heart failure therapy.…”

Section: Lesson Of the Month (1)mentioning

confidence: 99%

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Myotonic dystrophy and out-of-hospital arrest

et al. 2010

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Ventricular dysfunction in type 1 myotonic dystrophy: Electrical, mechanical, or both?

Cited by 25 publications

References 30 publications

Left ventricular dyssynchrony is associated with reduced heart rate variability in familial amyloidotic polyneuropathy

Left ventricular dyssynchrony is associated with reduced heart rate variability in familial amyloidotic polyneuropathy

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

Myotonic dystrophy and out-of-hospital arrest

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