title: Early detection of biventricular involvement in myotonic dystrophy by tissue Doppler.
abstract: 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.
authors.affiliation: Divisione di Cardioangiologia con UTIC, Dipartimento di Medicina e Clinica Sperimentale, Italy.
authors.forename: Michele
authors.initials: M
authors.lastname: Parisi
authors.forename: Maurizio
authors.initials: M
authors.lastname: Galderisi
authors.forename: Milena
authors.initials: M
authors.lastname: Sidiropulos
authors.forename: Chiara
authors.initials: C
authors.lastname: Fiorillo
authors.forename: Roberta
authors.initials: R
authors.lastname: Lanzillo
authors.forename: Arcangelo
authors.initials: A
authors.lastname: D'Errico
authors.forename: Michela
authors.initials: M
authors.lastname: Grieco
authors.forename: Pasquale
authors.initials: P
authors.lastname: Innelli
authors.forename: Lucio
authors.initials: L
authors.lastname: Santoro
authors.forename: Oreste
authors.initials: O
authors.lastname: de Divitiis
mesh_terms: 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*