title: Widespread electroanatomic alterations of right cardiac chambers in patients with myotonic dystrophy type 1.
abstract: BACKGROUND
  Conduction disturbances and arrhythmias characterize the cardiac feature of myotonic dystrophy type 1 (MD1); a myocardial involvement has been suggested as part of the cardiac disease. The aim of our study was to investigate the underlying myocardial alterations using electroanatomic mapping (CARTO) and their possible correlation with genetic and neurological findings.
  
  
  RESULTS
  Right atrial and ventricular CARTO maps were obtained in 13 MD1 patients. Thirteen age-matched patients with paroxysmal supraventricular tachycardia and normal heart served as controls. Unipolar voltage (UNI-v), bipolar voltage (BI-v) amplitudes, bipolar potential duration (Bi-dur), and atrial propagation time (A-pt) were measured. UNI-v and BI-v in interatrial septum, anterolateral atrial wall, and right ventricle outflow tract were lower in MD1 patients than controls (P < 0.001). Bi-dur and A-pt were longer in MD1 patients than controls (P < 0.001, P = 0.046, respectively). A significant relationship was documented between CTG triplets and the percentage of Bi-v <0.5 mV in the atrial anteroseptal region (r = 0.6, P = 0.02).
  
  
  CONCLUSIONS
  Altered electroanatomic patterns are present in the right cardiac chambers in MD1 patients. Widespread myocardial alterations, not necessarily limited to the conduction system, may support the presence of a cardiac myopathy as part of the disease.
authors.affiliation: Institute of Cardiology, Department of Cardiovascular Medicine, Catholic University of the Sacred Heart, Rome, Italy.
authors.forename: Antonio
authors.initials: A
authors.lastname: Dello Russo
authors.forename: Gemma
authors.initials: G
authors.lastname: Pelargonio
authors.forename: Quintino
authors.initials: Q
authors.lastname: Parisi
authors.forename: Matteo
authors.initials: M
authors.lastname: Santamaria
authors.forename: Loredana
authors.initials: L
authors.lastname: Messano
authors.forename: Tommaso
authors.initials: T
authors.lastname: Sanna
authors.forename: Michela
authors.initials: M
authors.lastname: Casella
authors.forename: Giuseppe
authors.initials: G
authors.lastname: De Martino
authors.forename: Roberto
authors.initials: R
authors.lastname: De Ponti
authors.forename: Manuela
authors.initials: M
authors.lastname: Pace
authors.forename: Vincenzo
authors.initials: V
authors.lastname: Giglio
authors.forename: Carolina
authors.initials: C
authors.lastname: Ierardi
authors.forename: Paolo
authors.initials: P
authors.lastname: Zecchi
authors.forename: Filippo
authors.initials: F
authors.lastname: Crea
authors.forename: Fulvio
authors.initials: F
authors.lastname: Bellocci
mesh_terms: D001145:Arrhythmias, Cardiac / Q000150:complications / Q000503:physiopathology*; D018780:Body Surface Potential Mapping; D005260:Female; D006325:Heart Atria / Q000503:physiopathology*; D006329:Heart Conduction System / Q000503:physiopathology*; D006352:Heart Ventricles / Q000503:physiopathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009223:Myotonic Dystrophy / Q000150:complications / Q000503:physiopathology*