Adrenomedullin is a novel hypotensive peptide originally isolated from huma
n pheochromocytoma. Accumulating evidence suggests the possible involvement
of adrenomedullin in the physiology of the pulmonary circulation and the p
athophysiology of hypoxaemia. The aim of the present study was to investiga
te the pathophysiological significance of adrenomedullin in hypoxaemia caus
ed by congenital cyanotic heart disease. Subjects were 16 patients with con
genital cyanotic heart disease aged 0.8-10 years (Group C) and 12 age-match
ed control subjects (patients with coronary artery dilatation after Kawasak
i disease; Group N). Plasma adrenomedullin concentrations were measured, us
ing radioimmunoassay, in femoral venous, pulmonary arterial and pulmonary v
enous blood obtained during cardiac catheterization. Plasma adrenomedullin
concentrations in Group C were significantly (3-fold) higher than those in
Group N at all sampling sites. In Group C, plasma adrenomedullin concentrat
ions in pulmonary venous blood were significantly lower than those in pulmo
nary arterial blood. Pulmonary uptake of adrenomedullin in Group C was sign
ificantly greater than that in Group N. Patients with congenital cyanotic h
eart disease showed elevated plasma adrenomedullin concentrations and an in
creased uptake of adrenomedullin in the pulmonary circulation, which may ac
t to dilate pulmonary vessels and increase pulmonary blood flow to alleviat
e hypoxaemia. Intrinsically increased adrenomedullin levels may function as
a compensatory mechanism for hypoxaemia in congenital cyanotic heart disea
se.