Background. We describe the hemodynamic response to initiation and wit
hdrawal of inhaled nitric oxide (NO) in infants with pulmonary hyperte
nsion after surgical repair of total anomalous pulmonary venous connec
tion. Methods. Between January 1, 1992, and January 1, 1995, 20 patien
ts underwent repair of total anomalous pulmonary venous connection. Ni
ne patients had postoperative pulmonary hypertension and received a 15
-minute trial of inhaled NO at 80 parts per million. Five of these pat
ients received prolonged treatment with NO at 20 parts per million or
less. Results. Mean pulmonary artery pressure decreased from 35.6 +/-
2.4 to 23.7 +/- 2.0 mm Hg (mean +/- standard error of the mean) (p = 0
.008), and pulmonary vascular resistance decreased from 11.5 +/- 2.0 t
o 6.4 +/- 1.0 U . m(2) (p = 0.03). After prolonged treatment with NO,
pulmonary artery pressure increased transiently in all patients when N
O was discontinued. Conclusions. After operative repair of total anoma
lous pulmonary venous connection, inhaled NO selectively vasodilated a
ll patients with pulmonary hypertension. Withdrawal of NO after prolon
ged inhalation was associated with transient rebound pulmonary hyperte
nsion that dissipated within 60 minutes. Appreciation of rebound pulmo
nary hypertension may have important implications for patients with pu
lmonary hypertensive disorders when interruption of NO inhalation is n
ecessary or when withdrawal of NO is planned.