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Clinical implication of maximal voluntary ventilation in myotonic muscular dystrophy

 Mi Ri Suh  ;  Dong Hyun Kim  ;  Jiho Jung  ;  Bitnarae Kim  ;  Jang Woo Lee  ;  Won Ah Choi  ;  Seong-Woong Kang 
 MEDICINE, Vol.98(18) : e15321, 2019 
Journal Title
Issue Date
Adult ; Carbon Dioxide/analysis ; Female ; Forced Expiratory Volume/physiology ; Humans ; Hypercapnia/physiopathology ; Lung/physiopathology ; Male ; Maximal Respiratory Pressures/methods ; Maximal Voluntary Ventilation/physiology ; Middle Aged ; Myotonic Dystrophy/classification ; Myotonic Dystrophy/complications ; Myotonic Dystrophy/physiopathology ; Neuromuscular Diseases/epidemiology ; Neuromuscular Diseases/physiopathology ; Polysomnography/methods ; Predictive Value of Tests ; Pressure ; Prospective Studies ; Respiratory Function Tests/methods ; Retrospective Studies ; Vital Capacity/physiology
Patients with myotonic muscular dystrophy type 1 (DM1) tend to exhibit earlier respiratory insufficiency than patients with other neuromuscular diseases at similar or higher forced vital capacity (FVC). This study aimed to analyze several pulmonary function parameters to determine which factor contributes the most to early hypercapnia in patients with DM1.We analyzed ventilation status monitoring, pulmonary function tests (including FVC, maximalvoluntary ventilation [MVV], and maximal inspiratory and expiratory pressure), and polysomnography in subjects with DM1 who were admitted to a single university hospital. The correlation of each parameter with hypercapnia was determined. Subgroup analysis was also performed by dividing the subjects into 2 subgroups according to usage of mechanical ventilation.Final analysis included 50 patients with a mean age of 42.9 years (standard deviation = 11.1), 46.0% of whom were male. The hypercapnia was negatively correlated with MVV, FVC, forced expiratory volume in 1 second (FEV1), and their ratios to predicted values in subjects with myotonic muscular dystrophy type 1. At the same partial pressure of carbon dioxide, the ratio to the predicted value was lowest for MVV, then FEV1, followed by FVC. Moreover, the P values for differences in MVV and its ratio to the predicted value between ventilator users and nonusers were the lowest.When screening ventilation failure in patients with DM1, MVV should be considered alongside other routinely measured parameters.
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1. College of Medicine (의과대학) > Dept. of Rehabilitation Medicine (재활의학교실) > 1. Journal Papers
Yonsei Authors
Kang, Seong Woong(강성웅) ORCID logo https://orcid.org/0000-0002-7279-3893
Kim, Dong Hyun(김동현)
Suh, Mi Ri(서미리)
Lee, Jang Woo(이장우)
Choi, Won Ah(최원아) ORCID logo https://orcid.org/0000-0003-0403-8869
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