794-1 Outcome of Pregnancy in Women with Complex Pulmonary Atresia

To define the outcome of pregnancy in patients (pts)with complex pulmonary atresia (PA); i.e. PA with VSD and congenital systemic to pulmonary collaterals, we reviewed data on 76 such pts ≥16 yrs old, seen at the Mayo Clinic between 1980–92. There were 62 pts who were advised against or had not attempted pregnancy, no pts reported infertility. Fourteen pts had 24 pregnancies resulting in 10 live births, (including one twin pregnancy). There was one neonatal death due to abruptio placenta at 27 wks. Seven deliveries (including the twins; i.e. 8 births), were vaginal and 2 were by cesarean section. Six pregnancies were terminated due to unoperated maternal PA. Miscarriage occurred in 9/18 (50%) of uninterrupted pregnancies in 7 pts (3 in pts without prior cardiac surgery, 4 in pts after palliation, and 2 after complete repair).Six pts had successful pregnancies; 2 unoperated pts had 3 deliveries (including twins; i.e. 4 births), 2 palliated pts had 3 deliveries, and 2 pts had 2 successful and 2 unsuccessful pregnancies after complete repair. Two pts had preterm labor, 1 due to toxemia (twin pregnancy). One pregnancy was complicated by high RV pressure (from conduit obstruction), and 1 by CHF requiring hospitalization for the last month of pregnancy (no prior surgery). The mean maternal Hb in pts with successful pregnancy was 14.9±1.3 gms vs 18.3±2.1 gms in pts who terminated pregnancy (p=0.01) and 16.4±2.2 gms in pts with unsuccessful pregnancies (p=NS).No pregnancy related maternal deaths occurred. The overall mean birth weight was 2.2 kg; 2.0±0.7 kg for 7 offspring of cyanotic pts vs 3.1±0.3 kg for 2 offspring of pts post complete repair (p=0.08). None of the offspring had congenital heart disease.ConclusionPregnancy in pts with complex PA can be successfully carried out but there is an increased risk of fetal loss even without maternal hypoxia (miscarriage rate 50%). Pre-pregnancy assessment of functional class, pulmonary pressures, ventricular and conduit function and degree of cyanosis should be established