scholarly journals Duchenne Muscular Dystrophy (DMD) with Severe Cardiomyopathy

2018 ◽  
Vol 6 (2) ◽  
Author(s):  
Ji Hyoung Park ◽  
Kwang Ho Lee ◽  
Wi Kwang Wang ◽  
Hyun Kyo Lim
Keyword(s):  
Heart Rate ◽  
Cardiac Arrest ◽  
Mental Retardation ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Sudden Cardiac Arrest ◽  
Proper Function ◽  
Organ Perfusion ◽  
Severe Mental Retardation ◽  
Successful Resuscitation

Duchenne muscular dystrophy (DMD) is a progressive myopathy. The development of respiratory therapy has increased the life expectancy of DMD patients. This change has increased the chances of anesthesia administration in DMD patients with advanced cardiomyopathy. We report a severe cardiomyopathy case in a 14-year-old boy with DMD, adrenal insufficiency, and severe mental retardation, who experienced a sudden cardiac arrest with successful resuscitation. The patient underwent feeding gastrostomy surgery to relieve recurrent aspiration pneumonia, during which cardiac index and heart rate decreased. Cardiomyopathy has emerged as a new challenge in DMD patients; it is important to maintain end organ perfusion by proper function of the left ventricle.

Multifocal glial nodules in a case of Duchenne muscular dystrophy with severe mental retardation

1999 ◽  
Vol 19 (3) ◽  
pp. 322-327
Author(s):  
Kyoko Itoh ◽  
Kenji Jinnai ◽  
Kazuo Tada ◽  
Kiyokazu Hara ◽  
Hiroshi Itoh ◽  
...  
Keyword(s):  
Mental Retardation ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Severe Mental Retardation ◽  
Glial Nodules

Multifocal glial nodules in a case of Duchenne muscular dystrophy with severe mental retardation

1999 ◽  
Vol 19 (3) ◽  
pp. 322-327 ◽  
Author(s):  
Kyoko Itoh ◽  
Kenji Jinnai ◽  
Kazuo Tada ◽  
Kiyokazu Hara ◽  
Hiroshi Itoh ◽  
...  
Keyword(s):  
Mental Retardation ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Severe Mental Retardation ◽  
Glial Nodules

High concentration of middle chain fatty acid in a case of Duchenne muscular dystrophy with severe mental retardation

2012 ◽  
Vol 54 (1) ◽  
pp. 137-140 ◽  
Author(s):  
Hisashi Kawashima ◽  
Kiyoko Watanabe ◽  
Yasuyoshi Morishima ◽  
Hiroaki Ioi ◽  
Yasuyo Kashiwagi ◽  
...  
Keyword(s):  
Mental Retardation ◽  
Fatty Acid ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Chain Fatty Acid ◽  
Severe Mental Retardation ◽  
High Concentration

scholarly journals Autonomic modulation at rest and in response to postural change in adolescents with Duchenne muscular dystrophy: a cross-sectional study

2021 ◽  
Vol 79 (9) ◽  
pp. 766-773
Author(s):  
Mariana Viana Rodrigues ◽  
Mileide Cristina Stoco-Oliveira ◽  
Talita Dias da Silva ◽  
Celso Ferreira ◽  
Heloisa Balotari Valente ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Heart Rate ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Oxygen Saturation ◽  
Cross Sectional Study ◽  
Control Group ◽  
Autonomic Modulation ◽  
Postural Change ◽  
Cross Sectional

ABSTRACT Background: Analysis of autonomic modulation after postural change may inform the prognosis and guide treatment in different populations. However, this has been insufficiently explored among adolescents with Duchenne muscular dystrophy (DMD). Objective: To investigate autonomic modulation at rest and in response to an active sitting test (AST) among adolescents with DMD. Methods: Fifty-nine adolescents were included in the study and divided into two groups: 1) DMD group: adolescents diagnosed with DMD; 2) control group (CG): healthy adolescents. Participants’ weight and height were assessed. Lower limb function, motor limitations and functional abilities of the participants in the DMD group were classified using the Vignos scale, Egen classification and motor function measurement, respectively. The following variables were assessed before, during and after AST: systolic blood pressure (SBP), diastolic blood pressure (DBP), respiratory rate (f), oxygen saturation and heart rate (HR). To analyze the autonomic modulation, the HR was recorded beat-by-beat. Heart rate variability (HRV) indices were calculated in the time and frequency domains. Results: Differences in relation to groups were observed for all HRV indices, except LF/HF, oxygen saturation, HR and f (p < 0.05). Differences in relation to time and the interaction effect between group and time were observed for RMSSD, SD1, SD2, SD1/SD2, LFms2 and LFnu, HFun, SBP and DBP (p < 0.05). Differences in relation to time were also observed for the indice SDNN, FC and f (p < 0.05). Conclusions: Performing the AST promoted reduced autonomic modulation and increased SBP, DBP and HR in adolescents with DMD.

Abstract 697: Dyssynchrony in Duchenne Muscular Dystrophy Demonstrated by Tagged MRI Analysis is Independent of Ejection Fraction

Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
William M Gottliebson ◽  
Wojciech Mazur ◽  
Janaka Wansapura ◽  
D. W Benson ◽  
Kan N Hor
Keyword(s):  
Heart Rate ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Ejection Fraction ◽  
Cardiac Mri ◽  
Circumferential Strain ◽  
Coronary Perfusion ◽  
Peak Strain ◽  
Tagged Mri ◽  
Significant Difference

Patients with Duchenne Muscular Dystrophy (DMD), a dystrophinopathy, universally develop dilated cardiomyopathy, which is associated with abnormal myocardial strain, as well as heterogeneous development of fibrosis as demonstrated by pathology and more recently by cardiac MRI methods. We sought to determine the presence of systolic dyssynchrony in this population using cardiac MRI tagging methods. We analyzed tagged MRI images for the presence of dyssynchrony in 61 males (age 12.5 ± 4.4 y) with a dystrophin mutation undergoing clinical cardiac MRI. Tagged MRI images were analyzed using HARP® analysis of regional strain and strain-time curves. The mid-myocardial slice was specifically analyzed, by dividing it into 6 coronary perfusion regions. Dyssynchrony was defined by the presence of either of 2 previously published indexes modified for use with MRI data: 1) time difference of 1 st to last regional peak strain > 100 ms; 2) standard deviation of time differences to peak strain for each of the six regions > 33 ms. Additional indexes evaluated included heart rate, LV ejection fraction, mid-myocardial composite circumferential strain, and presence of delayed myocardial hyperenhancement (MDE). Among the 61 subjects analyzed, 28 (46%) exhibited dyssynchrony indexes 1 and 2, while 8 additional subjects met dyssynchrony index 2 but not index 1. Only 4 subjects, all of whom met both dyssynchrony criteria, had positive MDE and abnormal EF <55%. The regions of slowest activation were highly dispersed and not clustered to the areas of positive MDE. One additional subject with dyssynchrony by either of the critieria also had abnormal EF but did not have MDE. There was no statistically significant difference between mean EF (61 vs 62%), age (12.58 vs 12.63 yrs), heart rate (105 vs 108 bpm) or mid-myocardial composite circumferential strain (−13.3 vs −12.9%) between those subjects with dyssynchrony (by either criteria) and those without. DMD patients frequently exhibit systolic dyssynchrony even in the presence of normal EF. However, the dispersed nature of the dyssynchrony suggests that resynchronization therapy once EF becomes abnormal is unlikely to be of benefit in DMD cardiomyopathy.

Facioscapulohumeral muscular dystrophy with severe mental retardation and epilepsy

2007 ◽  
Vol 29 (4) ◽  
pp. 231-233 ◽  
Author(s):  
Yoshiaki Saito ◽  
Satoshi Miyashita ◽  
Atsushi Yokoyama ◽  
Hirofumi Komaki ◽  
Ayuki Seki ◽  
...  
Keyword(s):  
Mental Retardation ◽  
Muscular Dystrophy ◽  
Facioscapulohumeral Muscular Dystrophy ◽  
Severe Mental Retardation

Sudden Cardiac Arrest during Adenotonsillectomy in a Patient with Subclinical Duchenne's Muscular Dystrophy

1993 ◽  
Vol 72 (2) ◽  
pp. 130-131 ◽  
Author(s):  
Nick C. Benton ◽  
Robert A. Wolgat
Keyword(s):  
Cardiac Arrest ◽  
Muscular Dystrophy ◽  
General Anesthesia ◽  
Case Reports ◽  
Sudden Cardiac Arrest ◽  
Elevated Serum ◽  
Serum Creatine Kinase ◽  
Strong Family History ◽  
History Of ◽  
Duchenne's Muscular Dystrophy

We describe a four-year-old boy of Indian descent who had elective adenotonsillectomy for chronic sore throat and partial airway obstruction. About 10 minutes into the procedure, the patient suddenly developed cardiac asystole. After prolonged cardiac resuscitation, recovery was achieved. No permanent neurologic deficits resulted. The child was later found to have a strong family history of Duchenne's muscular dystrophy (DMD) and an elevated serum creatine kinase level documented since shortly after birth. We reviewed several case reports substantiating the risk for cardiac arrest during general anesthesia in DMD patients, and we concluded that DMD is a little-known risk for cardiac arrest during general anesthesia. The otolaryngologist must be aware of this potential complication, because tonsillectomy and adenoidectomy are commonly indicated for children at an age when DMD may be subclinical.

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