[Myocardial involvement in carrier states for Duchenne muscular dystrophy. A rare cause of supraventricular arrhythmia].
METHODS
Two women, both aged 54 years, were admitted because of supraventricular arrhythmias of recent onset. Patient 2 was also in heart failure. Male family members of both patients were known to have Duchenne's muscular dystrophy, of which one had died.


METHODS
The electrocardiogram of patient 1 demonstrated atrial fibrillation. Patient 2 had a raised serum creatine kinase concentration and increased pulmonary marking in the chest radiogram. Patient 1 had normal findings on left heart catheterization, but immunohistochemical analysis of a myocardial biopsy revealed dystrophin mosaic with 20% dystrophin-negative fibres. Patient 2 had a reduced ejection fraction and 80% dystrophin-negative fibres.


METHODS
Myocardial involvement in the carrier state for Duchenne's muscular dystrophy having been demonstrated in both women, patient 1 received antihypertensive treatment while patient 2, who was in cardiac failure, was given diuretics, ACE-inhibitor and beta-receptor blockers.


CONCLUSIONS
Cardiomyopathy in carriers of Duchenne's muscular dystrophy is a rare cause of supraventricular arrhythmias. The cause can be confirmed by immunochemical analysis of an endomyocardial biopsy.
Medizinische Klinik, Krankenhauses des Landkreises Starnberg.
A
A
Rüchardt
H
H
Eisenlohr
H
H
Lydtin
D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use; D000889:Anti-Arrhythmia Agents / Q000627:therapeutic use; D000959:Antihypertensive Agents / Q000627:therapeutic use; D001281:Atrial Fibrillation / Q000209:etiology; D003402:Creatine Kinase / Q000097:blood; D004232:Diuretics / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D016189:Dystrophin / Q000032:analysis; D004562:Electrocardiography; D004656:Enalapril / Q000627:therapeutic use; D005260:Female; D006321:Heart / Q000503:physiopathology; D006333:Heart Failure / Q000209:etiology; D006579:Heterozygote; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008790:Metoprolol / Q000627:therapeutic use; D008875:Middle Aged; D009136:Muscular Dystrophies / Q000150:complications / Q000235:genetics*; D009206:Myocardium / Q000473:pathology*; D009529:Nicardipine / Q000627:therapeutic use; D010375:Pedigree; D013617:Tachycardia, Supraventricular / Q000188:drug therapy / Q000209:etiology*
D000806:Angiotensin-Converting Enzyme Inhibitors; D000889:Anti-Arrhythmia Agents; D000959:Antihypertensive Agents; D004232:Diuretics; D016189:Dystrophin; D004656:Enalapril; D009529:Nicardipine; D003402:Creatine Kinase; D008790:Metoprolol