Cardiac function associated with home ventilator care in Duchenne muscular dystrophy

Abstract

PurposeCardiomyopathy 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.MethodsWe 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.ResultsFifty-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 (16.25±1.85 years) than those in the nonventilator group (14.73±1.36 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 (1,038±620.41 mL) than the nonventilator group (1,455±603.12 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 (1.7±0.44) was lower in the home ventilator group than in the nonventilator group (2.02±0.62). The mitral valve annular systolic velocity was higher in the home ventilator group (estimated β, 1.06; standard error, 0.48). Patients with Duchenne muscular dystrophy on a ventilator may have better systolic and diastolic cardiac functions.ConclusionNoninvasive 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.

ERS statement on paediatric long-term noninvasive respiratory support

“…Literature review (Online Table 5.4) Due to ethical constraints, the benefits on NIV have not been confirmed in randomized controlled trials and the published cohort studies mainly consist of a limited number of patients. NIV is associated with: o an increase in survival in patients with SMA type 1 [57,62,65,66,209] and Duchenne muscular dystrophy [71,210] o fewer hospitalizations in patients with SMA type 1 [57,61,62,64,209], and some NMD [211,212]); but no change in hospitalizations in other children with NMD [213] o improvement in SDB symptoms in patients with SMA type 2-3 [60], infantile Pompe disease [73], and other NMD [69,82,84] o improvement in nocturnal and daytime gas exchange in patients with juvenile Pompe disease [72], and NMD [82] o improvement in sleep quality/architecture and cyclic-alternating patterns in patients with SMA type 1-2 [60], SMA type 2 [214], and other NMD [69,82] o decrease in chest deformity in patients with SMA type 1 [58,63], and SMA type 1-3 [61] o transient improvement in predicted FVC and thoraco-abdominal asynchrony in patients with Duchenne muscular dystrophy [215] o improvement in cardiac function in patients with Duchenne muscular dystrophy [216] o improvement in QoL in children with NMD [217].…”

Association between pulmonary function and left ventricular volume and function in duchenne muscular dystrophy

“…Twenty-seven boys and adolescent and young adult males (median age 15 years; range, [9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25][26] were included in the study. Most of the participants had 3 or 4 clinic visits (median, 3; range, 1-10).…”

“…Lee and colleagues demonstrated that optimizing ventilatory support with NIV decreases the LV afterload, leading to an improved diastolic function and likely to a slowing of the progression of dilated cardiomyopathy. The linear mixed model was adjusted for the duration of NIV.…”

Is It Possible for Children in Duchenne Muscular Dystrophy to Preserve Cardiac Function with Medical Assistance?

“…Progression of cardiomyopathy is the major cause of mortality. Cardiomyopathy can occur at any age but often occurs around 14–15 years [ 12 , 13 ].…”

“…Baseline assessment of cardiac function is needed for patients with DMD at diagnosis, and annual cardiac assessment is recommended for patients with DMD older than 10 years. Poor treatment outcomes have been noticed in patients with DMD who fail to see a cardiologist after the onset of clinical symptoms of heart failure [ 2 , 11 , 12 , 13 ].…”