BACKGROUND: Duchenne muscular dystrophy (DMD) is a hereditary neuromuscular disorder frequently associated with progressive cardiac dysfunction, and is one of the common causes of death in these children. Early diagnostic markers of cardiac involvement might help in timely intervention. In this study we compared the short term HRV measures of DMD children with that of healthy subjects. METHOD: One hundred and twenty-four genetically confirmed boys with DMD and 50 age matched controls were recruited. Error-free, electrocardiogram was recorded in all subjects at rest in the supine position. HRV parameters were computed in time and frequency domains. Time domain measures included standard deviation of NN interval (SDNN), and root of square mean of successive NN interval (RMSSD). Frequency domain consisted of total, low frequency and high frequency power values. Ratio of low frequency and high frequency power values (LF/HF) was determined using customized software. RESULTS: HRV parameters were significantly altered in DMD children as compared to healthy controls. Following parameters [mean (SD)] were reduced in DMD as compared to controls; RMSSD (in ms) [52.14 (33.2) vs 64.64 (43.2); p = 0.038], High frequency component (nu) [38.77 (14.4) vs 48.02 (17.1); p = 0.001] suggesting a loss of vagal tone. In contrast, measure of sympathovagal balance LF/HF [1.18 (0.87) vs 0.89 (0.79); p = 0.020] was increased in DMD group. CONCLUSION: In this cross sectional study we have demonstrated alteration in autonomic tone in DMD. Loss of vagal tone and an increase in sympathetic tone were observed in DMD children. Further prospective studies are required to confirm the utility of these measures as predictors of adverse cardiac outcome in DMD.
BACKGROUND:Duchenne muscular dystrophy (DMD) is a hereditary neuromuscular disorder frequently associated with progressive cardiac dysfunction, and is one of the common causes of death in these children. Early diagnostic markers of cardiac involvement might help in timely intervention. In this study we compared the short term HRV measures of DMDchildren with that of healthy subjects. METHOD: One hundred and twenty-four genetically confirmed boys with DMD and 50 age matched controls were recruited. Error-free, electrocardiogram was recorded in all subjects at rest in the supine position. HRV parameters were computed in time and frequency domains. Time domain measures included standard deviation of NN interval (SDNN), and root of square mean of successive NN interval (RMSSD). Frequency domain consisted of total, low frequency and high frequency power values. Ratio of low frequency and high frequency power values (LF/HF) was determined using customized software. RESULTS: HRV parameters were significantly altered in DMDchildren as compared to healthy controls. Following parameters [mean (SD)] were reduced in DMD as compared to controls; RMSSD (in ms) [52.14 (33.2) vs 64.64 (43.2); p = 0.038], High frequency component (nu) [38.77 (14.4) vs 48.02 (17.1); p = 0.001] suggesting a loss of vagal tone. In contrast, measure of sympathovagal balance LF/HF [1.18 (0.87) vs 0.89 (0.79); p = 0.020] was increased in DMD group. CONCLUSION: In this cross sectional study we have demonstrated alteration in autonomic tone in DMD. Loss of vagal tone and an increase in sympathetic tone were observed in DMDchildren. Further prospective studies are required to confirm the utility of these measures as predictors of adverse cardiac outcome in DMD.
Authors: Pamela Barraza-Flores; Tatiana M Fontelonga; Ryan D Wuebbles; Hailey J Hermann; Andreia M Nunes; Joe N Kornegay; Dean J Burkin Journal: Hum Mol Genet Date: 2019-08-15 Impact factor: 6.150
Authors: 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 Journal: Pediatr Cardiol Date: 2018-04-25 Impact factor: 1.655
Authors: Mayra Priscila Boscolo Alvarez; Talita Dias da Silva; Francis Meire Favero; Vitor Engrácia Valenti; Rodrigo Daminello Raimundo; Luiz Carlos Marques Vanderlei; David M Garner; Carlos Bandeira de Mello Monteiro Journal: PLoS One Date: 2017-01-24 Impact factor: 3.240
Authors: Michael Taylor; John Jefferies; Barry Byrne; Joao Lima; Bharath Ambale-Venkatesh; Mohammad R Ostovaneh; Raj Makkar; Bryan Goldstein; Rachel Ruckdeschel Smith; James Fudge; Konstantinos Malliaras; Brian Fedor; Jeff Rudy; Janice M Pogoda; Linda Marbán; Deborah D Ascheim; Eduardo Marbán; Ronald G Victor Journal: Neurology Date: 2019-01-23 Impact factor: 9.910
Authors: M U Sujan; M Raghavendra Rao; Ravikiran Kisan; Hulegar A Abhishekh; Atchayaram Nalini; Trichur R Raju; T N Sathyaprabha Journal: J Neurosci Rural Pract Date: 2016 Jan-Mar