C. Michel et al., REPORT OF A PATIENT WITH SYNDROME-X DUE TO EXCESSIVE ADENOSINE EFFECT- MYOCARDIAL MIGRAINE WITHOUT MYOCARDIAL-ISCHEMIA, Canadian journal of cardiology, 11(4), 1995, pp. 339-344
A 53-year-old female presented with disabling chest pain. The pain had
most of the characteristics of ischemic pain; however, the results of
the initial clinical investigation were consistent with the diagnosis
of syndrome X. That is, her treadmill exercise test was positive but
her coronary angiogram was normal A dipyridamole-thalium; test resulte
d in severe chest pain, marked ST abnormalities, but no evidence of an
y focal reduction in flow. A dipyridamole stress echocardiogram reveal
ed that left ventricular function was entirely normal during the dipyr
idamole-induced pain and ST segment abnormalities, making ischemia an
unlikely cause and for either. To attempt to account for this paradox,
the hypothesis was generated that both the pain and ST segment abnorm
alities were due to a primary abnormality of adenosine metabolism rath
er than secondary to ischemia. Accordingly, adenosine-MIBI scans were
done with and without pretreatment with aminophylline Infusion adenosi
ne virtually immediately resulted in crushing chest pain and profound
ST abnormalities again without any evidence of focal abnormalities of
MIBI estimated flow. By contrast, administration of adenosine after pr
etreatment with aminophylline failed to produce either chest pain or S
T abnormalities. Moreover, long term therapy with aminophylline almost
entirely relieved the symptoms which had been so distressing. This ca
se indicates that there is a subset of patients with syndrome X - in w
hich faults in adenosine metabolism result in excessive adenosine accu
mulation or effect and this results,; in turn, in adenosine-induced is
chemic-like chest pain and adenosine-induced ST abnormalities. There i
s, however, no actual ischemia of the myocardium. Given the known effe
cts of adenosine on coronary flow, the problem in this subset of patie
nts appears to be equivalent to an attack of myocardial migraine and b
lockers of adenosine action might be of help to other patients with a
similar pathophysiology for their chest pain.