Depressed myocardial fatty acid metabolism in patients with muscular dystrophy.

Myocardial involvement is frequently associated with various types of muscular dystrophy and Thallium-201 scintigraphy can show regional myocardial perfusion abnormalities in patients with muscular dystrophy. Myocardial fatty acid metabolism can now be imaged using a radioiodinated branched fatty acid (123I-BMIPP). The present study evaluates myocardial fatty acid metabolism in muscular dystrophy. Twenty-eight patients underwent 123I-BMIPP(BMIPP) and Thallium dual single photon emission tomography. Regional uptake of both tracers was visually analyzed. We also assessed electrocardiography and echocardiography. The results showed that the BMIPP uptake compared to Thallium was smaller in 57% of all patients. BMIPP SPECT images revealed abnormalities in four of eight patients with a normal electrocardiogram. Abnormal BMIPP uptake with normal regional wall motion was evident in nine patients. The size of the region with defective BMIPP uptake was larger than that of asynergic areas detected by echocardiography in 11 patients. In conclusion, muscular dystrophy involves depressed myocardial fatty acid metabolism in larger extent of region than that in perfusion or mechanical abnormality.