Pulmonary Rehabilitation: Pulmonary Rehabilitation |

Clinical Implication of Maximal Voluntary Ventilation in Myotonic Muscular Dystrophy FREE TO VIEW

Miri Suh, MD; Bitnarae Kim; Won-Ah Choi, MD; Seong-Woong Kang, MD
Author and Funding Information

Severance Hospital, Rehabilitation Institute of Neuromuscular Disease, Yonsei University College of Medicine, Seoul, Korea (the Republic of)

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):1124A. doi:10.1016/j.chest.2016.08.1233
Text Size: A A A
Published online

SESSION TITLE: Pulmonary Rehabilitation

SESSION TYPE: Original Investigation Poster

PRESENTED ON: Wednesday, October 26, 2016 at 01:30 PM - 02:30 PM

PURPOSE: Patients with myotonic muscular dystrophy (MMD) are reported to have early carbon-dioxide (CO2) retention compared to patients with other neuromuscular diseases with similar functional vital capacity (FVC). We analyzed several pulmonary function parameters of patients with MMD, and compared them with that of the patients with Duchenne muscular dystrophy (DMD) to seek the reason of early respiratory failure in the patients with MMD.

METHODS: We performed pulmonary function test including FVC, forced expiratory volume in 1 second (FEV1), and maximal voluntary ventilation (MVV) among the patients with MMD and Duchenne muscular dystrophy (DMD) who visited our clinic from May, 2015 until February, 2016. Paired t-test was performed to compare the FVC and MVV in the same group, linear regression was performed to find the correlation of each parameter within age, and ANCOVA was performed to compare the slope of decrement among the two groups.

RESULTS: We compared measured FVC and MVV, and also the FVC marked as ratio to normal predicted value (FVCPred) in 71 patients with DMD and 29 patients with MMD. Each showed significant decrement according to age. Patients with DMD showed lower average of FVC, FVCPred and MVV compared to the patients with MMD. However, slope of MVV/FVCPred according to age was showed positive value in the patients with DMD while it showed negative value in the patients with MMD. In other words, assuming that FVCPred is identical, MVV can be significantly lower in MMD compared with that of DMD.

CONCLUSIONS: Even with adequate FVC, patients with MMD may show relatively lower MVV compared with patients with DMD in similar FVC. Lower MVV in ratio to FVC can be the possible cause of early CO2 retention in patients with MMD.

CLINICAL IMPLICATIONS: Decline in MVV in ratio to FVC according to age is larger in the patients with MMD compared to patients with other neuromuscular disease such as DMD. This may implicate different pattern of respiratory muscle compromise in the patients with MMD, and this can be the possible reason of early CO2 retention shown in these patients.

DISCLOSURE: The following authors have nothing to disclose: Miri Suh, Bitnarae Kim, Won-Ah Choi, Seong-Woong Kang

No Product/Research Disclosure Information




Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s "Cited By" API will populate this tab (http://www.crossref.org/citedby.html).

Some tools below are only available to our subscribers or users with an online account.

Related Content

Customize your page view by dragging & repositioning the boxes below.

Find Similar Articles
CHEST Journal Articles
PubMed Articles
  • CHEST Journal
    Print ISSN: 0012-3692
    Online ISSN: 1931-3543