Is heart rate variability a valid parameter to predict sudden death in patients with Beckers muscular dystrophy?

Ammendola, E.; Russo, V.; Politano, L.; Santangelo, L.; Calabarò, R.
November 2006
Heart;Nov2006, Vol. 92 Issue 11, p1686
Academic Journal
The article discusses a study which examined the prognostic value of heart rate variability (HRV) for sudden death in patients with Beckers' muscular dystrophy (BMD) and dilated cardiomyopathy. Patients with BMD were characterized by a lower HRV in the frequency and time domains. BMD patients also showed higher mean heart rate than in control group. HRV was found to be a reliable index to assess sympathovagal balance and can be used to predict arrhythmic risk in BMD patients.


Related Articles

  • Characterization of deletion breakpoints in patients with dystrophinopathy carrying a deletion of exons 45—55 of the Duchenne muscular dystrophy (DMD) gene. Miyazaki, Daigo; Yoshida, Kunihiro; Fukushima, Kazuhiro; Nakamura, Akinori; Suzuki, Kayo; Sato, Toshiyuki; Takeda, Shin'ichi; Ikeda, Shu-ichi // Journal of Human Genetics;Feb2009, Vol. 54 Issue 2, p127 

    Deletion of exons 45–55 (del45–55) in the Duchenne muscular dystrophy gene (DMD) has gained particular interest in the field of molecular therapy, because it causes a milder phenotype than DMD, and therefore, may represent a good candidate for the goal of a multiple exon-skipping...

  • Reliable detection of early myocardial dysfunction by tissue Doppler echocardiography in Becker muscular dystrophy. Meune, C.; Pascal, O.; Bécane, H. M.; Héloire, F.; Christoforou, D.; Laforet, P.; Eymard, B.; Gueret, P.; Leturcq, F.; Recan, D.; Devaux, J. Y.; Weber, S.; Duboc, D. // Heart;Aug2004, Vol. 90 Issue 8, p947 

    Becker muscular dystrophy (BMD) is an X linked mutation of the dystrophin gene characterised by skeletal muscle dystrophy and progressive heart failure which frequently leads to a fatal outcome. Tissue Doppler echocardiography allows accurate quantification of regional myocardial function and...

  • Brain natriuretic peptide is not predictive of dilated cardiomyopathy in Becker and Duchenne muscular dystrophy patients and carriers. van Westrum, Steven Schade; Dekker, Lukas; de Haan, Rob; Endert, Erik; Ginjaar, Ieke; de Visser, Marianne; van der Kooi, Anneke // BMC Neurology;2013, Vol. 13 Issue 1, p1 

    Background: Cardiomyopathy is reported in Duchenne and Becker muscle dystrophy patients and female carriers. Brain Natriuretic peptide (BNP) is a hormone produced mainly by ventricular cardiomyocytes and its production is up regulated in reaction to increased wall stretching. N-terminal-proBNP...

  • Dystrophic cardiomyopathy: amplification of cellular damage by Ca2+ signalling and reactive oxygen species-generating pathways. Jung, Carole; Martins, Adriano S.; Niggli, Ernst; Shirokova, Natalia // Cardiovascular Research;Mar2008, Vol. 77 Issue 4, p766 

    Aims: Cardiac myopathies are the second leading cause of death in patients with Duchenne and Becker muscular dystrophy, the two most common and severe forms of a disabling striated muscle disease. Although the genetic defect has been identified as mutations of the dystrophin gene, very little is...

  • Cardiac involvement in patients with Becker muscular dystrophy: new diagnostic and pathophysiological insights by a CMR approach.  // Journal of Cardiovascular Magnetic Resonance (BioMed Central);2008, Vol. 10, p1 

    Background : Becker-Kiener muscular dystrophy (BMD) represents an X-linked genetic disease associated with myocardial involvement potentially resulting in dilated cardiomyopathy (DCM). Early diagnosis of cardiac involvement may permit earlier institution of heart failure treatment and extend...

  • Transgenic models of cardiac arrhythmias and sudden death. Remme, Carol Ann // Frontiers in Physiology;Mar2013, p1 

    The article discusses the transgenic models of cardiac arrhythmias and sudden death. It notes that cardiac arrhythmias are associated with a wide range of clinical problems such as benign symptomatic heart rhythm alterations. It states that atrial and ventricular arrhythmias are acquired mostly...

  • Muscular dystrophies: key elements for everyday diagnosis and management. Palladino, Alberto; Nigro, Gerardo; Politano, Luisa // Cardiogenetics;2013, Vol. 3 Issue 1, p49 

    Muscular dystrophies are a heterogeneous group of inherited disorders that share similar clinical features and dystrophic changes on muscle biopsy, associated with progressive weakness. Weakness may be noted at birth or develop in late adult life. In recent years, cardiac involvement has been...

  • Heparin.  // Reactions Weekly;2/13/2016, Vol. 1588 Issue 1, p136 

    The article presents the case study of a 61-year old man who developed heparin-induced thrombocytopenia(HIT) while being treated with heparin and the medical history included dilated myocardiopathy associated with Becker muscular dystrophy.

  • Heart rate variability in children with hypertrophic cardiomyopathy. Butera, G.; Bonnet, D.; Kachaner, J.; Sidi, D.; Villain, E. // Heart;Feb2003, Vol. 89 Issue 2, p205 

    Discusses the results of a study evaluating the prognostic value of heart rate variability in children with hypertrophic cardiomyopathy. History of palpitations; Time domain analysis; Sudden death.


Read the Article


Sign out of this library

Other Topics