Maternal And Fetal Outcomes Of Pregnant Women With Pulmonary Arterial Hypertension Associated With Congenital Heart Disease In Different Clinical Subtypes

Objective: To investigate the peripartum maternal and fetal outcomes and clinical characteristics of pregnant women of pulmonary arterial hypertension associated with congenital heart disease(PAH-CHD)with different clinical subtypes,analyze the independent influencing factors of major adverse cardiac events(MACE),assessing the predictive value of clinical indicators for MACE and provide a basis for clinicians to diagnose and treat pregnant patients with PAH-CHD.Methods:A total of 64 pregnant patients with PAH-CHD who were admitted and delivered in Sheng Jing Hospital Affiliated to China Medical University from February 2015 to February 2022 were retrospectively collected and analyzed.(1)According to the clinical classification of PAH-CHD by the European Society of Cardiology(ESC)and the European Respiratory Society(ERS),the admitted patients were divided into pulmonary arterial hypertension associated with left-to-right shunt(L-R-PAH)group of 30 cases,pulmonary arterial hypertension associated with Eisenmenger syndrome(ES-PAH)group of 19 cases,pulmonary arterial hypertension after defect correction(CD-PAH)group of 15 cases.According to whether MACE occurred or not,the patients were divided into 29 cases with MACE and 35 cases without MACE.(2)The etiological classification of the cases was analyzed,and the basic information characteristics,perinatal maternal and infant outcomes(MACE,perinatal complications,etc.),clinical indicators(delivery mode,anesthesia method,gestational age of delivery,BNP,CK,CK-MB,LVEF,s PAP,NYHA cardiac function classification,etc.)of the three groups were compared,and the independent influencing factors of MACE were analyzed by univariate and multivariate Logistic regression methods.And the ROC curve was used to evaluate the predictive value of clinical indicators for MACE.Results: A total of 64 pregnant patients with PAH-CHD were included in the study,and they were divided into L-R-PAH group,30 cases,ES-PAH group,19 cases,and CD-PAH group,15 cases.(1)The main causes of PAH-CHD were classified as atrial septal defects(50.0%)and ventricular septal defects(28.1%);the L-R-PAH group was dominated by atrial septal defect(83.3%),ventricular septal defect accounted for the largest proportion in the ES-PAH group(47.4%)and CD-PAH group(40.0%).(2)The basic information of admission was compared among the three groups,the overall average age was 31.40±4.46 years old,there was a statistically significant difference in the gestational age at admission(P<0.05),among which the ES-PAH group was significantly lower than the CD-PAH group,and there was no statistical difference between the L-R-PAH group and the other two groups(P>0.05),and there was no statistical difference in the other basic information characteristics(age,gravidity,parity,regular obstetric examination,conception method,etc.)(P>0.05).(3)The perinatal maternal outcomes of the three groups were compared,and the results showed that there were significant differences in the MACE,cardiac death,heart failure,pulmonary hypertension crisis,and malignant arrhythmia among the three groups(P<0.05).Pairwise comparison,the ES-PAH group and the L-R-PAH group had statistical differences in the MACE,pulmonary hypertension crisis and malignant arrhythmia events(P<0.05),which were all significantly higher in the ES-PAH group than L-R-PAH group;ES-PAH group and CD-PAH group had statistical differences in the composition ratio of cardiac death,heart failure,and pulmonary hypertension crisis(P<0.05),all of which were higher in ES-PAH group compared with CD-PAH group;L-R-PAH group and CD-PAH group had no statistical difference in various maternal outcomes(P>0.05).(4)The perinatal outcomes of the three groups were compared,and there were significant differences in neonatal weight,overall complications,fetal growth restriction,neonatal respiratory distress syndrome,and respiratory failure among the three groups(P<0.05).In both of the ES-PAH and L-R-PAH groups,as well as the ES-PAH and CD-PAH groups,there were significant differences in neonatal weight,overall complications,fetal growth restriction,neonatal respiratory distress syndrome,and respiratory failure.The neonatal body weight was lower in the ES-PAH group than in the L-R-PAH and CD-PAH groups respectively,overall complications,fetal growth restriction,neonatal respiratory distress syndrome,respiratory distress in ES-PAH group were higher than that in L-R-PAH and CD-PAH groups respectively,there was no statistical difference between L-R-PAH and CD-PAH groups in various neonatal outcome events(P>0.05).(5)The clinical indicators of the three groups were compared,and there were significant differences in gestational age of delivery,anesthesia method,postpartum ICU,ICU hospital stay,CK-MB,s PAP,s PAP classification,and NYHA cardiac function classification(P<0.05).There was a moderate positive correlation between cardiac function classification and s PAP class(r=0.538,P<0.001).(6)Univariate Logistic regression results showed that cardiac function grade,BNP and s PAP were risk factors for MACE(P<0.05).Multivariate Logistic regression results showed that cardiac function classification was an independent risk factor for MACE(OR=33.259,95%CI:3.919～282.272,P<0.05).(7)The ROC curve was used to evaluate the predictive value of cinical index,BNP,s PAP,and cardiac function classification had predictive significance(P<0.05),in which cardiac function classification had the highest predictive value(AUC=0.924).The combination of three indicators had the highest predictive value in predicting MACE(AUC=0.952).Conclusion: The peripartum maternal and fetal outcomes of pregnant women with PAH-CHD in different clinical subtypes were not the same.Among them,the ES-PAH group had the highest incidence of MACE,perinatal complications and so on,which was the worst.There was no statistical difference in the maternal and infant outcomes between the L-R-PAH group and the CD-PAH group.NYHA functional classification was an independent risk factor for MACE.Cardiac function classification,BNP and s PAP had significance in predicting the outcome of MACE,cardiac function classification had the highest predictive value for single index prediction,while the combination prediction of the three indexes had the highest predictive value in predicting the outcome of MACE.