Widespread electroanatomic alterations of right cardiac chambers in patients with myotonic dystrophy type 1.
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.
Dello Russo
Antonio
A
Institute of Cardiology, Department of Cardiovascular Medicine, Catholic University of the Sacred Heart, Rome, Italy.
Pelargonio
Gemma
G
Parisi
Quintino
Q
Santamaria
Matteo
M
Messano
Loredana
L
Sanna
Tommaso
T
Casella
Michela
M
De Martino
Giuseppe
G
De Ponti
Roberto
R
Pace
Manuela
M
Giglio
Vincenzo
V
Ierardi
Carolina
C
Zecchi
Paolo
P
Crea
Filippo
F
Bellocci
Fulvio
F
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*