The pyrophosphate heart scintigram in children with progressive muscular dystrophy.

A pyrophosphate heart scintigram was obtained in 16 boys with progressive muscular dystrophy Duchenne. All of them showed pathological ECG findings and high plasma levels of CK, AST, ALT and LD. In 4 patients the scintigram was distinctly positive and in further 3 it reached borderline values. The remaining 9 boys had normal scintigraphic findings. Those with a positive heart scintigram had very high plasma levels of the enzymes under study which was suggestive of current progression of the disease. There was, however, no relation between heart scintigraphy and the affliction of the skeletal muscles expressed by means of an index.

The sensitivity of scintigraphic myocardial imaging by the use of 99mTc-labelled pyrophosphate in the diagnosis of cardiomyopathy of various etiology.

A total of 10 patients were examined. In five children who suffered from secondary cardiomyopathy accompanying progressive muscular dystrophy scintigraphic examination of the myocardium was made using 99mTc-labelled pyrophosphate. In two cases the scan was distinctly positive, in three cases negative. Five men with poorly defined primary nonobstructive cardiomyopathy were examined too. The scan was positive in three cases and negative in two cases. The positivity of the pyrophosphate scan of the heart is therefore not pathonomonic of ischaemic damage. An increased accumulation of pyrophosphate in the myocardium in cardiomyopathy indicates an acute phase of the disease.