Paradoxical systemic air embolism (PAE) occurring as a complication of righ
t-to-left intracardiac shunting during evaluation and treatment of pulmonar
y hypertension (PEI) has not been previously reported. We report four cases
of PH-associated PAE recently encountered at our center. Two patients with
PH experienced transient neurologic deficits during agitated-saline contra
st echocardiography (ASCE), and a patent foramen ovale was subsequently dia
gnosed in both patients. Two patients with Eisenmenger's syndrome (ES), whi
le receiving epoprostenol via multilumen catheters, experienced transient n
eurologic deficits while flushing the unused port of the catheter, No patie
nt experienced permanent neurologic deficits. We conclude that ASCE poses a
risk for PAE in patients with PH and clinically silent, previously undetec
ted, right-to-left intracardiac shunts, and that multilumen catheters used
for long-term epoprostenol therapy in ES carry a risk of PAE.