| Objective:Congenital pulmonary airway malformation(CPAM)is one of the common congenital pulmonary cystic diseases,which can be complicated by mediastinal shift,fetal hydrops,postnatal dyspnea and even death.The pathogenesis and etiology of CPAM are unclear.Previous studies have shown that congenital pulmonary airway malformation-volume ratio(CVR)has a certain predictive value for fetal prognosis,but its prediction threshold for adverse prognosis is still controversial at home and abroad.This study mainly explored the CVR threshold of poor prognosis and the risk factors of fetal CPAM and built a risk prediction model,in order to provide reference for prenatal consultation and perinatal management.Methods:1.Analysis of risk factors of congenital pulmonary airway malformation(CPAM)and building prediction modelThe birth information of the children diagnosed with CPAM in Shanxi Children’s Hospital from January 2016 to October 2022 and healthy children with the same age and birth time in the community during the same period and the pregnancy information of their parents were collected.Independent risk factors of fetal CPAM were obtained by univariate analysis and multivariate Logistic regression analysis.The selected independent risk factors were imported into Rstudio software to establish a nomogram prediction model.Receiver operating characteristics(ROC)area under the curve(AUC)was used to evaluate the differentiation of the nomogram prediction model.Hosmer-Lemshow test was used to evaluate the calibration degree of the model.The 1000 times Bootstrap repeated sampling method was used to verify it’s effectiveness.SPSS25.0 software was used for statistical analysis,and P<0.05 was considered statistically significant.2.Study on congenital pulmonary airway malformation-volume ratio(CVR)thresholds of risk factors for fetal prognosis in CPAM fetusesThe prenatal ultrasound data of 278 fetuses diagnosed with CPAM from January 2012 to October 2022 in Shanxi Children’s Hospital were retrospectively analyzed,and the pregnancy outcome was followed up by telephone.The patients were grouped according to the poor prognosis risk factors(mediastinal shift,fetal hydrops,and postnatal dyspnea).The CVR and prognosis were compared between the fetal hydrops group and the non-hydrops one,the mediastinal shift group and the non-mediastinal shift group,and the postnatal dyspnea group and the non-dyspnea group,respectively.The ultrasonic CVR first detected and diagnosed as CPAM was defined as the primary CVR,and the peak value of CVR during pregnancy was defined as the maximum CVR.The thresholds of risk factors for poor fetal prognosis were studied for the primary CVR and the maximum CVR with statistical differences between groups..SPSS25.0 software was used for statistical analysis,and P<0.05 was considered statistically significant.Results:1.Analysis of risk factors of congenital pulmonary airway malformation(CPAM)and building prediction model(1)General information: A total of 161 fetuses in the CPAM group were collected.The median gestational age of the live births was 274(267,280)days,and the average birth weight was(3.24±0.44)kg.In the control group,191 patients had a median gestational age of 273(266,280)days and an average birth weight of(3.24 ± 0.40)kg.There were no significant differences in gestational age and birth weight between the two groups(P>0.05).(2)Univariate analysis showed that there were significant differences in toxic substance exposure history during pregnancy,medication history in early pregnancy,family history of hypertension,family history of diabetes,bacterial and/or viral infection in early pregnancy,and active/passive smoking between case group and control group(P<0.05).(3)Multivariate Logistic regression analysis showed that history of toxic substance exposure during pregnancy(OR=2.300,95%CI: 1.330-3.980),medication history in early pregnancy(OR=2.253,95%CI:1.039-4.882)and family history of hypertension(OR=8.833,95%CI: 2.386-32.708)were independent risk factors for CPAM(P<0.05).(4)Three independent risk factors selected by multivariate Logistic regression analysis were used as variables to establish a nomogram prediction model.The AUC under ROC curve was 0.681(95%CI:0.624～0.737,P<0.001).Hosmer-Lemshow test showed no statistical significance in the deviation between predicted value and actual value(χ~2=2.938,P=0.568).The calibration curve obtained was close to the standard curve,and the mean absolute error of the calibration curve was 0.024,indicating that the model had good stability in clinical prediction.2.Study on congenital pulmonary airway malformation-volume ratio(CVR)thresholds of risk factors for prognosis in CPAM fetuses(1)General data:A total of 278 cases of CPAM were collected.The median gestational age of CPAM diagnosed by prenatal ultrasound was 23.86(22.71,24.57)weeks,136 lesions were on the left,137 on the right,and 5 were bilateral.There were 9 cases with fetal hydrops(9/278,3.2%)and 80 cases with mediastinal shift(80/278,28.8%).There were 35 cases(35/278,12.5%)with CVR>1.6,including 6(6/35,17.1%)with fetal hydrops,24(24/35,68.6%)with mediastinal shift,5(5/35,14.3%)with missing outcome data,and 14(14/30,46.7%)with termination of pregnancy.In the remaining 16 cases,3(3/16,18.8%)were born with dyspnea,16(16/16,100.0%)survived,and 1(1/16,6.3%)of the surviving fetuses was performed intramuscular steroid hormone injection during the second trimester.Of the 278 cases,142 had complete outcome data,including 8 with fetal hydrops(8/142,5.6%),46 with mediastinal shift(46/142,32.4%),29 with termination of pregnancy(29/142,20.4%),13 with dyspnea after birth(13/113,11.5%).Two cases(2/113,1.8%)died,including 1 with fetal hydrops,2 with mediastinal shift,and 2 with dyspnea after birth.142 cases were divided into two groups according to the presence or absence of fetal hydrops,mediastinal shift,and dyspnea after birth.(2)Comparison between groups:1)Comparison of CVR and prognosis between fetal hydrops group and non-hydrops groupThere were significant differences in the maximum CVR value and the number of dyspnea after birth between the hydrops group and the non-hydrops group(P<0.05).There were no significant differences in the number of pregnancies terminated,deaths,mediastinal shift,the primary CVR value,the gestational age at the primary CVR and the maximum CVR between the two groups(P>0.05).2)Comparison of CVR and prognosis between mediastinal shift group and non-mediastinal shift groupCompared with the group without mediastinal shift,the group with mediastinal shift had a significantly earlier gestational age at achieving the primary CVR and the maximum CVR,a significantly higher primary CVR and the maximum CVR,and a significantly higher number of infants with dyspnea after birth(P<0.05).There were no significant differences in the number of pregnancies terminated,fetal hydrops and deaths between the two groups(P>0.05).3)Comparison of CVR and prognosis between dyspnea group and non-dyspnea group after birthThere were significant differences in the number of cases with fetal hydrops,mediastinal shift and death between the dyspnea group and the non-dyspnea group(P<0.05).There was no significant difference in gestational age at primary CVR and maximum CVR,primary CVR and maximum CVR between the two groups(P>0.05).(3)Threshold studies were performed on the CVR with statistically significant differences between the above groups :1)CVR threshold for predicting fetal hydrops: The AUC for predicting the maximum CVR for fetal hydrops was 0.880(95%CI: 0.756～1.000,P<0.001),and the threshold was1.67(sensitivity 0.857,specificity 0.898,positive predictive value 0.240,negative predictive value 0.994).2)CVR thresholds for predicting mediastinal shift: The AUC of primary CVR and maximum CVR for predicting fetal mediastinal shift was 0.807(95%CI: 0.746-0.869,P<0.001)and 0.830(95%CI: 0.770-0.891,P<0.001),respectively.The thresholds were0.44(sensitivity 0.925,specificity 0.620,positive predictive value 0.485,negative predictive value 0.955)and 1.00(sensitivity 0.754,specificity 0.787,positive predictive value 0.597,negative predictive value 0.884),respectively.Conclusions:1.Exposure to toxic substances during pregnancy,medication history in early pregnancy and family history of hypertension are independent risk factors for the development of CPAM in the fetus,and the prediction of CPAM risk model based on these risk factors is good and stable.It is suggested that the mother should avoid exposure to radiation,formaldehyde and other chemical substances during pregnancy,strengthen self-protection in early pregnancy,and follow doctor’s advice to choose safe and effective medicines for the fetus when suffering from disease,so as to reduce the risk of CPAM in the fetus.2.With the progress of intrauterine intervention and neonatal lung surgery techniques,most of the CPAM fetuses with CVR>1.6 have good prognosis.Clinicians should not hastily recommend termination of pregnancy to give these fetuses a chance of survival.The maximum CVR was of great value in predicting fetal edema,and the optimal threshold was 1.67.Both the primary CVR and the maximum CVR could predict the mediastinal shift well,and the optimal thresholds were 0.44 and 1.0,respectively.CPAM fetuses with a primary CVR of less than 0.44 and maximum CVR of less than 1.0 had a lower risk of poor prognosis.Neither the primary nor the maximum CVR may be an appropriate predictor of dyspnea,and the CVR may be used for prenatal advice in combination with the presence of fetal hydrops and /or mediastinal shift. |
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