Pediatric Cardiology

, Volume 39, Issue 5, pp 869–883 | Cite as

Heart Rate Variability and Cardiopulmonary Dysfunction in Patients with Duchenne Muscular Dystrophy: A Systematic Review

  • Talita Dias da Silva
  • Thais Massetti
  • Tânia Brusque Crocetta
  • Carlos Bandeira de Mello Monteiro
  • Alex Carll
  • Luiz Carlos Marques Vanderlei
  • Carlie Arbaugh
  • Fernando Rocha Oliveira
  • Luiz Carlos de Abreu
  • Celso Ferreira Filho
  • John Godleski
  • Celso Ferreira
Review Article


Duchenne muscular dystrophy (DMD) is a genetic recessive disorder with progressive muscle weakness. Despite the general muscle wasting, degeneration and necrosis of cardiomyocytes have been the main causes of morbidity and death in individuals with DMD. Cardiac failure is generally preceded by disturbances in heart rate variability (HRV), and non-invasive measurement of the autonomic nervous system has been an important tool to predict adverse cardiovascular events. Hence, the application of HRV to study autonomic modulation in DMD individuals, and the establishment of correlations between HRV and heart/lung diseases, age, and mortality will have the potential to improve quality of life and life expectancy of individuals with DMD. In order to evaluate the state of the art in this field, we conducted a systematic search in Medline/PubMed and BVS (virtual library in health) databases. We selected 8 studies using pre-defined criteria and meta-analysis revealed decreased parasympathetic activity and increased sympathetic predominance in individuals with DMD as major observations. Moreover, there is a strong association between diminished HRV and myocardial fibrosis with DMD. These patterns are evident in patients at early-stage DMD and become more prominent as disease severity and age increase. Thus, data minning clearly indicates that HRV assessment can be used as a predictor for sudden death in individuals with DMD. The use of the HRV, which is inexpensive, ubiquitously available in clinics and hospitals, and a non-invasive analysis tool, can save lives and decrease the morbity in DMD by alerting care givers to consider autonomic nervous system intervention.


Duchenne muscular dystrophy Cardiomyopathy Heart rate variability Autonomic modulation 



Duchenne muscular dystrophy


Heart rate variability


Virtual library in health

Mean RR

Mean of the RR intervals


Square root of the mean of squared differences between successive beat intervals


Standard deviation of all normal RR interval


Standard deviation of the means of normal-to-normal heart periods obtained from all 5-min periods throughout the whole data series


Average of the standard deviations of all normal-to-normal intervals calculated from all 5-min periods of a 24-h recording period


Percentage of differences between RR intervals with an absolute value greater than 50 ms


Coefficient of variation of the RR interval


Partial pressure of carbon dioxide (CO2) in the arterial blood


Low frequency


High frequency


Very low frequency


Autonomic nervous system


Brain natriuretic peptide


Fractional shortening


Trans-thoracic echocardiography


Tissue doppler imaging


Magnetic resonance imaging


Cardiac magnetic resonance


Late gadolinium enhancement


Wall motion analysis


Quantitative muscle testing


Angiotensin-converting enzyme inhibitors


Preferred reporting items for systematic reviews and meta-analyses


Population intervention comparison outcome


Standard mean differences


Standard deviation



The authors thank CNPq (National Council for Scientific and Technological Development, process number 142280/2015-1), CAPES (Coordination for the Improvement of Higher Education Personnel, Process No. 99999.014604/2013-02) for support of this work.

Author Contributions

TDS, TM, FRO, and TBC participated in the acquisition of data and TDS, AC, CBMM, CA, LCA, LCMV, CFF, JG, and CF participated in the revision of the manuscript. TDS, TM, CBMM, CF determined the design, interpreted the data and all authors helped on draft the manuscript. All authors read and gave final approval for the version submitted for publication.

Compliance with Ethical Standards

Conflict of interest

All authors report no conflict of interest.

Informed Consent

The current study does not contain any data from individual persons.


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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Paulista School of MedicineFederal University of São PauloSão PauloBrazil
  2. 2.Department of Environmental HealthHarvard TH Chan School of Public HealthBostonUSA
  3. 3.Graduate Program in Rehabilitation Sciences, Faculty of MedicineUniversity of São PauloSão PauloBrazil
  4. 4.Faculty of Medicine of ABCSanto AndréBrazil
  5. 5.Sao Paulo State University - UNESPPresidente PrudenteBrazil
  6. 6.Stanford University School of MedicineStanfordUSA
  7. 7.School of Public HealthUniversity of São PauloSão PauloBrazil
  8. 8.Graduate Program in Medicine (Cardiology), Paulista School of MedicineFederal University of São PauloSão PauloBrazil

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