title: A management trial for Duchenne cardiomyopathy.
abstract: The physiatrist can now be instrumental in prolonging the survival of individuals with neuromuscular disease by using respiratory muscle aids. As a result, morbidity and mortality from cardiomyopathy are likely to increase for patients with generalized myopathies. One hundred consecutive patients with dystrophin-deficient muscular dystrophy and a mean age of 17.2 yr (range, 5-41) satisfied criteria for having dilated cardiomyopathy (DCM) and received digitalis and diuretics. Nine of the 14 patients were symptom-free, despite left ventricular ejection fractions (LVEFs) of 25-40%. The five patients with symptomatic heart failure had severe ventricular dilatation, with LVEFs < 25%. Two of the five patients died of heart failure within 1 yr. For the remaining three patients, we evaluated the addition of the angiotensin-converting enzyme (ACE) inhibitor enalapril and, subsequently, the use of beta-blockers to the therapeutic regimen. Addition of these medications, never before attempted in the management of cardiomyopathy associated with generalized myopathic disease, complemented each other in relieving symptoms and reversing signs of congestive heart failure and DCM. We conclude that the combination of ACE inhibitor and beta-blocker deserves further exploration for inclusion in any management regimen for the treatment of muscular dystrophy-associated cardiomyopathy.
authors.affiliation: Department of Pediatrics, Sapporo University of Medicine, Hokkaido, Japan.
authors.forename: Y
authors.initials: Y
authors.lastname: Ishikawa
authors.forename: J R
authors.initials: JR
authors.lastname: Bach
authors.forename: Y
authors.initials: Y
authors.lastname: Ishikawa
authors.forename: R
authors.initials: R
authors.lastname: Minami
mesh_terms: D000293:Adolescent; D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use*; D000328:Adult; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use*; D009320:Atrial Natriuretic Factor / Q000097:blood; D017298:Bisoprolol / Q000627:therapeutic use*; D002311:Cardiomyopathy, Dilated / Q000150:complications* / Q000188:drug therapy*; D002648:Child; D002675:Child, Preschool; D004232:Diuretics / Q000627:therapeutic use; D004334:Drug Administration Schedule; D004359:Drug Therapy, Combination; D004656:Enalapril / Q000627:therapeutic use*; D006333:Heart Failure / Q000150:complications / Q000188:drug therapy; D006334:Heart Function Tests; D006801:Humans; D009136:Muscular Dystrophies / Q000150:complications*
chemical_list: D000319:Adrenergic beta-Antagonists; D000806:Angiotensin-Converting Enzyme Inhibitors; D004232:Diuretics; D004656:Enalapril; D009320:Atrial Natriuretic Factor; D017298:Bisoprolol