Cardiac involvement in myotonic muscular dystrophy (Steinert's disease): a prospective study of 25 patients

Journal Article · Wed Oct 31 23:00:00 EST 1984 · Am. J. Cardiol.; (United States)

DOI:https://doi.org/10.1016/S0002-9149(84)80147-2· OSTI ID:5855783

Perloff, J K; Stevenson, W G; Roberts, N K; Cabeen, W; Weiss, J

The presence, degree and frequency of disorders of cardiac conduction and rhythm and of regional or global myocardial dystrophy or myotonia have not previously been studied prospectively and systematically in the same population of patients with myotonic dystrophy. Accordingly, 25 adults with classic Steinert's disease underwent electrocardiography, 24-hour ambulatory electrocardiography, vectorcardiography, chest x-rays, echocardiography, electrophysiologic studies, and technetium-99m angiography. Clinically important cardiac manifestations of myotonic dystrophy reside in specialized tissues rather than in myocardium. Involvement is relatively specific, primarily assigned to the His-Purkinje system. The cardiac muscle disorder takes the form of dystrophy rather than myotonia, and is not selective, appearing with approximately equal distribution in all 4 chambers. Myocardial dystrophy seldom results in clinically overt ventricular failure, but may be responsible for atrial and ventricular arrhythmias. Since myotonic dystrophy is genetically transmitted, a primary biochemical defect has been proposed with complete expression of the gene toward striated muscle tissue, whether skeletal or cardiac. Specialized cardiac tissue and myocardium have close, if not identical, embryologic origins, so it is not surprising that the genetic marker affects both. Cardiac involvement is therefore an integral part of myotonic dystrophy, targeting particularly the infranodal conduction system, to a lesser extent the sinus node, and still less specifically, the myocardium.

• Research Organization: Univ. of California, Los Angeles
• OSTI ID: 5855783
• Journal Information: Am. J. Cardiol.; (United States), Journal Name: Am. J. Cardiol.; (United States) Vol. 54:8; ISSN AJCDA
• Country of Publication: United States
• Language: English

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62 RADIOLOGY AND NUCLEAR MEDICINE
BETA DECAY RADIOISOTOPES
BETA-MINUS DECAY RADIOISOTOPES
BODY
BODY AREAS
CARDIOVASCULAR DISEASES
CARDIOVASCULAR SYSTEM
CHEST
DIAGNOSIS
DIAGRAMS
DISEASES
ELECTROCARDIOGRAMS
ELECTROMAGNETIC RADIATION
GENES
HEART
HOURS LIVING RADIOISOTOPES
INTERMEDIATE MASS NUCLEI
IONIZING RADIATIONS
ISOMERIC NUCLEI
ISOMERIC TRANSITION ISOTOPES
ISOTOPE APPLICATIONS
ISOTOPES
LABELLED COMPOUNDS
MUSCLES
MYOCARDIUM
NUCLEI
ODD-EVEN NUCLEI
ORGANS
PATIENTS
RADIATIONS
RADIOISOTOPES
TECHNETIUM 99
TECHNETIUM ISOTOPES
TRACER TECHNIQUES
X RADIATION
YEARS LIVING RADIOISOTOPES