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Korean J Pediatr 2018 February;61(2) :59-63.
Published online 2018 February 15.       
Cardiac function associated with home ventilator care in Duchenne muscular dystrophy
Sangheun Lee1, Heeyoung Lee1, Lucy Youngmin Eun1, Seung Woong Gang2
1Division of Pediatric Cardiology, Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea
2Department of Rehabilitation Medicine, Yonsei University College of Medicine, Seoul, Korea
Corresponding Author: Lucy Youngmin Eun ,Tel: +82-2-2019-3350, Fax: +82-2-3461-9473, Email:
Copyright © 2018 by The Korean Pediatric Society
Purpose: Cardiomyopathy is becoming the leading cause of death in patients with Duchenne muscular dystrophy because mechanically assisted lung ventilation and assisted coughing have helped resolve respiratory complications. To clarify cardiopulmonary function, we compared cardiac function between the home ventilator-assisted and non–ventilator-assisted groups.
Methods: We retrospectively reviewed patients with Duchenne muscular dystrophy from January 2010 to March 2016 at Gangnam Severance Hospital. Demographic characteristics, pulmonary function, and echocardiography data were investigated.
Results: Fifty-four patients with Duchenne muscular dystrophy were divided into 2 groups: home ventilator-assisted and non–ventilator-assisted. The patients in the home ventilator group were older (16.251.85 years) than those in the nonventilator group (14.731.36 years) (P=0.001). Height, weight, and body surface area did not differ significantly between groups. The home ventilator group had a lower seated functional vital capacity (1,038620.41 mL) than the nonventilator group (1,455603.12 mL). Mean left ventricular ejection fraction and fractional shortening were greater in the home ventilator group, but the data did not show any statistical difference. The early ventricular filling velocity/late ventricular filling velocity ratio (1.70.44) was lower in the home ventilator group than in the nonventilator group (2.020.62). The mitral valve annular systolic velocity was higher in the home ventilator group (estimated , 1.06; standard error, 0.48). Patients with Duchenne muscular dystrophy on a ventilator may have better systolic and diastolic cardiac functions.
Conclusion: Noninvasive ventilator assistance can help preserve cardiac function. Therefore, early utilization of noninvasive ventilation or oxygen may positively influence cardiac function in patients with Duchenne muscular dystrophy.
Keywords: Cardiac function test | Mechanical ventilators | Duchenne muscular dystrophy
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The evolution of electrocardiographic changes in patients with Duchenne muscular dystrophies  2017 June;60(6)
Myocardial atrophy in children with mitochondrial disease and Duchenne muscular dystrophy  2014 May;57(5)
A Case of Xp21 Contiguous Gene Deletion Syndrome with Hyperglycerolemia, Congenital Adrenal Hypoplasia and Duchenne Muscular Dystrophy  2001 January;44(1)
A Clinical Study on Duchenne Muscular Dystrophy  1992 May;35(5)
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