Two hundred and seventy five pregnancies in patients with congenital heart disease during 1980-1996 were analyzed retrospectively. Maternal and perinatal outcome was compared in 251 pregnancies of women with acyanotic and 24 pregnancies of women with cyanotic heart disease. Congenital heart disease was diagnosed during the index pregnancy in 26.1% of patients and the majority (88.4%) were in NYHA classes 1 and 2. Atrial septal defect (27.7%) was the most common lesion in women with acyanotic heart disease and the majority with cyanotic heart disease had Eisenmenger syndrome, 13 of 21 (61.9%). Sixty pregnancies occurred in patients with surgically corrected lesions (acyanotic, 56; cyanotic, 4). The incidences of abortions (8.3%), stillbirths (13.6%) and small for gestational age (SGA) (36.4%) were higher in cyanotic heart disease compared to acyanotic heart disease (stillbirth, 0.8%; SGA, 6.9%). There was a statistically significant difference in mean maternal age, mean gestational age and mean birth-weight in the surgically corrected and noncorrected lesions in both acyanotic and cyanotic heart disease. There was 1 maternal death in a woman with Eisenmenger syndrome.