Myocardial metabolism, perfusion, wall motion and electrical activity in Duchenne muscular dystrophy

Technical Report · Fri Jan 01 04:00:00 EST 1982

OSTI ID:6456061

Perloff, J K; Henze, E; Schelbert, H R

The cardiomyopathy of Duchenne's muscular dystrophy originates in the posterobasal left ventricle and extends chiefly to the contiguous lateral wall. Ultrastructural abnormalities in these regions precede connective tissue replacement. We postulated that a metabolic fault coincided with or antedated the subcellular abnormality. Accordingly, regional left ventricular metabolism, perfusion and wall motion were studied using positron computed tomography and metabolic isotopes supplemented by thallium perfusion scans, equilibrium radionuclide angiography and M-mode and two-dimensional echocardiography. To complete the assessment, electrocardiograms, vectorcardiograms, 24 hour taped electrocardiograms and chest x-rays were analyzed. Positron computed tomography utilizing F-18 2-fluoro 2-deoxyglucose (FDG) provided the first conclusive evidence supporting the hypothesis of a premorphologic regional metabolic fault. Thus, cardiac involvement in duchenne dystrophy emerges as a unique form of heart disease, genetically targeting specific regions of ventricular myocardium for initial metabolic and subcellular changes. Reported ultrastructural abnormalities of the impulse and conduction systems provide, at least in part, a basis for the clinically observed sinus node, intraatrial, internodal, AV nodal and infranodal disorders.

• Research Organization: California Univ., Los Angeles (USA). Lab. of Biomedical and Environmental Sciences
• DOE Contract Number: AC03-76SF00012
• OSTI ID: 6456061
• Report Number(s): DOE/SF/00012-T15; ON: DE83005566
• Country of Publication: United States
• Language: English

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59 BASIC BIOLOGICAL SCIENCES
62 RADIOLOGY AND NUCLEAR MEDICINE
BETA DECAY RADIOISOTOPES
BETA-PLUS DECAY RADIOISOTOPES
BIOMEDICAL RADIOGRAPHY
BODY
CARDIOVASCULAR DISEASES
CARDIOVASCULAR SYSTEM
COMPUTERIZED TOMOGRAPHY
COUNTING TECHNIQUES
DIAGNOSIS
DIAGNOSTIC TECHNIQUES
DIAGRAMS
DISEASES
DYNAMIC FUNCTION STUDIES
ELECTROCARDIOGRAMS
ELEMENTS
EMISSION COMPUTED TOMOGRAPHY
FLUORINE 18
FLUORINE ISOTOPES
HEART
HOURS LIVING RADIOISOTOPES
ISOTOPES
LIGHT NUCLEI
MEDICINE
METABOLISM
METALS
MORPHOLOGICAL CHANGES
MUSCLES
MYOCARDIUM
NUCLEAR MEDICINE
NUCLEI
ODD-ODD NUCLEI
ORGANS
PATHOLOGICAL CHANGES
PATHOLOGY
POSITRON COMPUTED TOMOGRAPHY
RADIOISOTOPE SCANNING
RADIOISOTOPES
RADIOLOGY
SCINTISCANNING
THALLIUM
TOMOGRAPHY
ULTRASTRUCTURAL CHANGES