Early detection of biventricular involvement in myotonic dystrophy by tissue Doppler.
BACKGROUND
Myotonic dystrophy is associated with arrhythmias and risk of sudden death but also with symptoms of heart failure. Our study aimed to identify early biventricular dysfunction in asymptomatic patients with myotonic dystrophy by tissue Doppler.


METHODS
Thirty-six patients with myotonic dystrophy (M/F=20/16, mean age=36.4 years), asymptomatic for heart failure, and 36 age- and sex-matched healthy controls underwent Doppler echocardiography and pulsed tissue Doppler of lateral mitral annulus and of tricuspid annulus.


RESULTS
The two groups had similar body mass index, blood pressure, heart rate, cardiac mass and endocardial shortening. Standard Doppler showed significantly lower transmitral early (E) diastolic peak velocity, longer transmitral deceleration and isovolumic relaxation times and higher tricuspid inflow atrial peak velocity in myotonic dystrophy than in controls. Tissue Doppler of mitral annulus showed lower myocardial systolic velocity (p<0.02), lower early diastolic velocity (E(m)) (p<0.05) and atrial velocity (A(m)) (p<0.005), but no difference of E(m)/A(m) ratio. At tricuspid annulus, E(m) and E(m)/A(m) ratio were lower (p<0.02 and p<0.005, respectively). The ratio between tricuspid inflow E velocity and E(m), index of the degree of right ventricular filling pressure, was higher (p<0.001) than in controls. Tissue Doppler derived left ventricular and right ventricular measurements were all associated with the disease condition, independent of age and heart rate.


CONCLUSIONS
Tissue Doppler identifies subclinical biventricular involvement in myotonic dystrophy. Early left ventricular myocardial systolic and diastolic changes are evident. Right ventricular dysfunction, involving myocardial relaxation and right ventricular filling pressure, might be the arrhythmogenic substratum of these patients.
Parisi
Michele
M
Divisione di Cardioangiologia con UTIC, Dipartimento di Medicina e Clinica Sperimentale, Italy.
Galderisi
Maurizio
M
Sidiropulos
Milena
M
Fiorillo
Chiara
C
Lanzillo
Roberta
R
D'Errico
Arcangelo
A
Grieco
Michela
M
Innelli
Pasquale
P
Santoro
Lucio
L
de Divitiis
Oreste
O
D000328:Adult; D042241:Early Diagnosis; D004452:Echocardiography; D018619:Echocardiography, Doppler, Pulsed / Q000379:methods*; D005260:Female; D006801:Humans; D008297:Male; D008943:Mitral Valve / Q000000981:diagnostic imaging; D009223:Myotonic Dystrophy / Q000150:complications*; D012680:Sensitivity and Specificity; D014261:Tricuspid Valve / Q000000981:diagnostic imaging; D018754:Ventricular Dysfunction / Q000000981:diagnostic imaging* / Q000209:etiology*