title: Cardiac management of ventilator-assisted individuals with Duchenne muscular dystrophy.
abstract: As life expectancy of patients with Duchenne muscular dystrophy (DMD) has increased to the 5th decade, in part due to improved ventilatory support, cardiomyopathy is projected to increase as a cause of death. International guidelines recommend an annual assessment of cardiac function and initiation of appropriate pharmacological treatment. We conducted an audit of the cardiac management in patients with DMD requiring ventilatory support and reported a case series of the collated cardiac investigations. Patients with DMD requiring ventilatory support were included in the study. The date of the last electrocardiogram (ECG), echocardiogram (ECHO), cardiology review and pharmacological management were retrieved from the medical records. If an annual cardiac assessment had not been performed this was requested and the latest ECGs and ECHO reports were collated. A total of 30 patients with DMD (29 males, mean (SD) age of 30 (7) years) met the inclusion criteria. Although there was ECG and ECHO documentation in 24 and 21 individuals, respectively, it was only recent in 10 and 6 individuals. In all, 60% of patients had been assessed by a cardiologist, but only 10% within the last year. Over half of the patients failed to attend their new appointments. From the available results, 18 of the 19 patients had an abnormal ECG, 11 of the 16 patients had left ventricular (LV) impairment and 55% of patients had a change in prescription following cardiac investigations. There is a need for a coordinated cardiorespiratory approach towards adult patients with DMD. Over a third of patients had normal LV function suggesting that cardiomyopathy is not inevitable in this group.
authors.affiliation: 1Department of Respiratory Medicine, West Park Healthcare Centre, Toronto, Ontario, Canada.
authors.forename: Lauren
authors.initials: L
authors.lastname: O'Brien
authors.forename: Robert
authors.initials: R
authors.lastname: Varadi
authors.forename: Roger S
authors.initials: RS
authors.lastname: Goldstein
authors.forename: Rachael A
authors.initials: RA
authors.lastname: Evans
mesh_terms: D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use; D000328:Adult; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use; D009202:Cardiomyopathies / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology; D004232:Diuretics / Q000627:therapeutic use; D004452:Echocardiography / Q000706:statistics & numerical data*; D004562:Electrocardiography / Q000706:statistics & numerical data*; D005260:Female; D006801:Humans; D008297:Male; D008485:Medical Audit; D020388:Muscular Dystrophy, Duchenne / Q000150:complications* / Q000628:therapy; D010342:Patient Acceptance of Health Care; D017410:Practice Guidelines as Topic; D012121:Respiration, Artificial; D013148:Spironolactone / Q000627:therapeutic use; D018487:Ventricular Dysfunction, Left / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology; D055815:Young Adult
chemical_list: D000319:Adrenergic beta-Antagonists; D000806:Angiotensin-Converting Enzyme Inhibitors; D004232:Diuretics; D013148:Spironolactone
keywords: Duchenne muscular dystrophy; cardiomyopathy; noninvasive ventilation; respiratory failure; ventilator assisted