Widespread electroanatomic alterations of right cardiac chambers in patients with myotonic dystrophy type 1

Antonio Dello Russo, Gemma Pelargonio, Quintino Parisi, Matteo Santamaria, Loredana Messano, Tommaso Sanna, Michela Casella, Giuseppe De Martino, Roberto De Ponti, Manuela Pace, Vincenzo Giglio, Carolina Ierardi, Paolo Zecchi, Filippo Crea, Fulvio Bellocci

Research output: Contribution to journalArticlepeer-review


Introduction: 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. Methods and 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

Original languageEnglish
Pages (from-to)34-40
Number of pages7
JournalJournal of Cardiovascular Electrophysiology
Issue number1
Publication statusPublished - Jan 2006


  • Arrhythmias
  • Cardiomyopathy
  • Electroanatomic mapping
  • Myotonic muscular dystrophy

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Physiology


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