Objective: To observe and analysis the changes of hemodynamic parameters and outcomes of pregnant women combined with congenital heart disease-pulmonary arterial hypertension (CHD-PAH). Methods: A prospective analysis of hemodynamic parameters of patients combined with CHD-PAH in Beijing Anzhen Hospital from January 2015 to December 2017 was carried out. Total 99 cases were divided into 4 groups based on systolic pulmonary arterial pressure (SPAP): mild group (SPAP 25-49 mmHg, 1 mmHg=0.133 kPa), moderate group (SPAP 50-79 mmHg), severe group (SPAP≥80 mmHg) and Eisenmenger syndrome group. Results: (1) SPAP of all groups increased during gestation, and the growth rates had no statistical differences (P>0.05). The SPAP in each group decreased after termination but was still higher than the early pregnancy's SPAP. (2) CHD-PAH patients in moderate [2 cm(2) (1-5 cm(2)), 1.0 cm (-2.0 - 2.0 cm)], severe [5 cm(2) (3-9 cm(2)), -3.5 cm (-6.0 - 1.0 cm)] or Eisenmenger syndrome [5 cm(2) (3-6 cm(2)), -1.0 cm (-5.5 - 2.0 cm)] group had larger right atrium area and smaller left ventricular end-diastolic diameter than patients in mild group [1 cm(2) (-1 - 4 cm(2)), 3.0 cm (1.0-6.0 cm)], the differences were statistically significant (all P<0.05). (3) Each group's SPAP/brachial arterial-systolic (BSAP) pressure continously increased during gestation. The SPAP/BSAP only decreased in patients in mild or moderate group after termination. However, the SPAP/BSAP kept rising in patients in severe group or Eisenmenger syndrome group. (4) CHD-PAH patients in severe or Eisenmenger syndrome group had worse cardiac function, in which the rates of Ⅲ-Ⅳ cardiac function were 61%(14/23) and 12/17, compared with patients in mild [8% (2/26)] and moderate [12%(4/33)] group, the differences were statistically significant (all P<0.05). (5) The rates of medicine treatment, cesarean section were higher, the weeks of gestational termination were earlier and the newborn's weights were lower in patients in severe or Eisenmenger syndrome group, the differences were statistically significant (all P<0.05). Conclusions: (1) SPAP keeps rising during gestation in all patients with CHD-PAH, and the SPAP decreases after termination but it's still higher than the early pregnancy's SPAP. This suggests that pregnancy would exacerbate these patients' heart problems, the risk should be fully considered before and during pregnancy. (2) Patients with severe CHD-PAH or combined with Eisenmenger syndrome have lager right atrium area, smaller left ventricular end-diastolic diameter, worse cardiac function, and bigger changes of hemodynamic, the incidence of adverse outcomes of mothers and children is also high, pregnancy should be avoided.