Antonio Dello Russo, Lucia De Luca, Cesare Santini*, Loredana Messano, Giuseppe De Martino, Vincenzo Giglio*, Fortunato Mangiola*, Enzo Ricci*, Antonello Damiani*, Gaetano Antonio Lanza, Fulvio Bellocci.
Cardiology Division, Department of Medicine, State University of New York, Health Science Center and Veterans Affairs Medical Center, Brooklyn, USA Istituto di Cardiologia, Universita Cattolica,
*Centro per le Malattie Neuromuscolari, Unione Italiana Lotta alla Distrofia Muscolare, Rome, Italy
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Duchenne muscular dystrophy is a recessive sex-linked hereditary
familial disorder, characterized by degeneration, atrophy and weakness of skeletal and cardiac muscle cells1.
The clinical features of the disease appear in early childhood and Duchenne muscular dystrophy patients
become wheelchair-bound before 13 years of age, with most of them dying approximately at 20 years of age
owing to respiratory and/or heart failure2-4. The frequent presence of tachycardia, sweating and chills suggests
early neuroautonomic involvement in these patients5. Recently, heart rate variability has largely been used to
assess cardiac autonomic activity in several diseases, including diabetes mellitus, ischemic heart disease, and
congestive heart failure6-8. Heart rate variability has also been studied assessed in Duchenne muscular
dystrophy patients, showing an imbalance in cardiac autonomic tone characterized by a decreased
parasympathetic activity with prevalence of sympathetic activity9,10. Whether the autonomic dysfunction
is just a consequence of a mechanical myocardial dysfunction or depends on other factors is not known. In
this study we investigated cardiac autonomic activity and its relationship with left ventricular systolic function
in a group of Duchenne muscular dystrophy patients.
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