Objective To investigate the effects of pregnancy on long-term outcomes of pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD).Methods Women with PAH-CHD who had undergone pregnancy under the care of Beijing Anzhen Hospital from 2004 to 2013 were retrospectively identified and 1∶1 matched to nulliparous PAH-CHD females (controls).Functional status and other clinical data were recorded for each group at baseline and follow-up.Results We successfully matched 40 pairs of pregnant and non-pregnant women with PAH-CHD.The patients were followed up for a mean of (6.5 ± 1.9) years,the outcomes of patients were documented during April 2016 to October 2016.No deaths occurred in either group during the study period.There were no statistically significant differences in long-term cardiac function between the two groups (Z =-1.41,P =0.16).After adjusting age,timing of follow-up,specific drug therapy and Eisenmenger's syndrome,pregnancy didn't have significant effect on the long-term deterioration of cardiac function in PAH-CHD patients (OR =1.32,95％ CI:0.33-5.37,P =0.70).Conclusion Pregnancy may not have significant effect on long-term cardiac function in PAH-CHD patients,but this conclusion needs to be confirmed by further studies.