Hepatic venous blood has been thought to play some role as a vasoactiv
e agent in the development of pulmonary arteriovenous fistulas in pati
ents with congenital heart disease. During the last 15 years, we have
observed pulmonary arteriovenous fistulas in 3, and systemic arteriove
nous fistulas in 2, patients from our 16 cases of left isomerism. Duri
ng the same period, neither pulmonary nor systemic arteriovenous fistu
las were detected among 50 patients with right isomerism. Pulmonary ar
teriovenous fistulas had developed in the absence of surgery in I of t
he patients. Both pulmonary and systemic fistulas were detected in an
another patient, in whom the hepatic venous blood bypassed the pulmona
ry circulation. The level of somatostatin, which is known to reduce sp
lanchnic blood flow was high in the systemic venous blood of this pati
ent. Although the mechanism of development of the fistulas has yet to
be clarified, we should be aware that not only pulmonary, but also sys
temic arteriovenous fistulas can be found in patients with left isomer
ism, even prior to any surgical intervention.