The development of pulmonary arteriovenous malformations is a well-known co
mplication after Fontan operations, and may result in significant morbidity
due to increasing arterial desaturation. We compared the use of bubble con
trast echocardiography and pulmonary angiography in detecting such malforma
tions. We also examined which anatomical and haemodynamic variables were as
sociated with their development. Our study includes 20 patients who had und
ergone modified Fontan procedures, 10 with atriopulmonary and 10 with total
cavopulmonary connections, in Gothenburg between 1980 and 1991. All patien
ts underwent cardiac catheterisation and pulmonary angiography. Bubble cont
rast echocardiography was performed at the same time, with injection of agi
tated polygelin colloid solution (Haemaccel, Hoechst) into the right and le
ft pulmonary arteries, respectively. Transoesophageal echocardiography was
used to detect the appearance of bubble contrast in the pulmonary venous at
rium. The aim was also to evaluate the role of hepatic venous blood. Of the
20 patients, 9 (45%) had a positive contrast echocardiography study, compa
red with only 2 (10%) detected by pulmonary angiography. Patients with posi
tive contrast echocardiography had a significantly lower arterial oxygen sa
turation than those with negative studies, both at rest (88% vs 95%, p < 0.
01) and during exercise testing (78% vs 89%, p = 0.01). Bubble contrast ech
ocardiography is much more sensitive in detecting pulmonary arteriovenous m
alformations than pulmonary angiography. By injecting echo contrast into th
e right and left pulmonary arteries, the method can be made highly selectiv
e. Pulmonary arteriovenous malformations develop much more frequently in pa
tients with the Fontan circulation than previously reported.