To explore whether FK506 has anti-vascular remodeling effect and its mechanism on prenatal administration of tacrolimus (FK506) in rats with experimental pathological model of pulmonary arterial hypertension (PAH) associated with congenital diaphragmatic hernia (CDH).

Seventeen healthy, adult specific pathogen free (SPF)-grade Sprague Dawley (SD) rats were selected in this study, all of which were 8 weeks old, with 12 females and 5 males, respectively. The CDH rat model was made with nitrofen. After a successful pregnancy, they were randomly divided into FK506 group (n=3), CDH group (n=3), CDH+ FK506 group (n=3) and control group (n=3). The development of lung tissue in each group were observed. The vessel wall thickness of the pulmonary arteries was observed by elastic van gieson (EVG) staining, immunofluorescent double staining of α-SMA and CD31 for the detection of neo-vascularization. The expression levels of BMPR2, p-Smad1 and p-Smad5 in fetal rats lungs were determined using Western blotting. The study was performed with the approval of the Ethics Committee of Sichuan Provincial People′s Hospital, University of Electronic Science & Technology of China [Approval No.2022(19)].

①The overall comparison of lung weight and lung weight/body weight of 4 groups showed statistically significant differences (H=81.25, 106.98; both P<0.001), while the overall comparison of body weight of 4 groups showed no statistical significance (P>0.05). EVG staining showed that the percentage of pulmonary artery media thickness (MT%) and alveolar area (S%) in 4 groups were statistically significant differences (F=13.26, P=0.006; F=37.48, P<0.001). Further, EVG staining showed that MT% in CDH group was greater than that in control group (P=0.001), The MT% of pulmonary artery in CDH+ FK506 group was lower than that in CDH group (P=0.002). ③Overall comparison of the proliferation indices of α-SMA and CD31-positive cells among 4 groups, respectively, showed statistically significant differences (F=33.76, 9.180; P<0.05). Further, The differences between CDH group and CDH+ FK506 group and control group were statistically significant (P<0.05). ④Western blotting analysis showed that the overall comparison of relative expression levels of BMPR2 and p-SMAD1 proteins in fetal rats of 3 groups had statistically significant differences (F=11.45, 10.94; P<0.05), while the overall comparison of the relative expression levels of p-SMAD5 protein in fetal rats of 3 groups did not show statistically significant difference (F=0.01, P>0.05). Further, the relative expression levels of BMPR2 and p-Smad1 in CDH group were lower than those in control group (P=0.049, 0.018), and the relative expression levels of BMPR2 and p-Smad1 in CDH+ FK506 group were higher than those in CDH group (P=0.010, 0.023).

Prenatal administration of FK506 can reduce pulmonary vascular remodeling in Nitrofen-induced CDH rat model, it has beneficial effects on alleviating lung hypoplasia in congenital diaphragmatic hernia.

To explore the clinical manifestation, genetic characteristics, treatment and outcome of neonatal tuberous sclerosis complex related epilepsy (TRE).

A newborn(child 1) of tuberous sclerosis complex (TSC) with frequent epileptic seizure in neonatal period admitted to Tongji Hospital, Tongji Medical College of Huazhong University of Science and Technology in August, 2021 was chosen in this study. Retrospective analysis was made on the family history, clinical manifestation, laboratory results, head imaging and molecular biology data, diagnosis, treatment and prognosis. The clinical features of neonatal TRE were summarized based on literature review of TRE related studies retrieved in domestic and foreign databases. The study followed the requirements of the World Medical Association Declaration of Helsinki revised in 2013. The guardians informed consent to the diagnosis and treatment of the child 1.

① Child 1 was a male newborn and admitted at age of 28 d due to seizure occurred on the 10 d after birth and lasted for more than half month.His cranial CT and MRI showed multiple nodules under the cortex of bilateral frontal parietal lobes and subependymal of bilateral lateral ventricles. Electroencephalogram detected focal seizure. Whole exome sequencing revealed a heterozygous splicing mutation (c.3884-1G>A) in the TSC2 gene of child 1, and it was a suspicious pathogenicity variation originated from his mother. He was improved after antiepileptic treatment of more than 3 kinds of anti-seizure medication (ASM). However, he still had recurrent seizures and was slightly behind normal children of the same age in development. He was treated with surgical treatment of epilepsy at age of 4 months 25 days, and epilepsy was controlled and intelligence was normal followed up to the age of 11 months. ② Literature review results: a total of 18 pieces of literature related to neonatal TRE, including 41 cases from 41 families plus child 1. The median onset age of 39 children was 5 d after birth, and 59.0% had the first seizure in the first week after birth. Among the 41 cases, 68.3% were focal seizures and 66.7% were intractable seizures. 18 cases(43.9%)had accepted gene detection, with TSC1 gene mutations accounting for 16.7% and TSC2 gene mutations accounting for 72.2%. Among 35 cases who were followed up, 57.1% showed mental retardation compare to children of the same gender and age.

Most neonatal TRE was early onset, focal seizure and refractory. Surgical treatment may be helpful to drugs resistant epilepsy. Children with this disease should be followed up for a long time, paying attention to whether there is any neurological system lesion.

To explore clinical characteristics, diagnosis and treatment results of a child with mild Gitelman syndrome (GS), and review related literature, so as to provide reference for clinical diagnosis of children with GS.

One girl (patient-1) with mild GS who visited People′s Hospital of Wuchuan County on August 25, 2021 was selected as research subject. Her clinical data were retrospectively analyzed, including medical history, clinical manifestations, laboratory tests and genetic test results, treatment and follow-up results. Literature of children aged 0 to 18 years with a confirmed diagnosis of GS caused by SLC12A3 gene mutations were retrieved with " Gitelman syndrome" " child" and " SLC12A3" etc. as keywords in CNKI, Wanfang service platform and PubMed database, and the search time was set from January 2018 to December 2022. The initial clinical manifestations of children with GS were summarized. The procedure followed in this study was in accordance with regulations of the Ethics Committee of People′s Hospital of Wuchuan County, and was reviewed and approved by the Ethics Committee (Approval No.02-2022).

①Patient-1 was a girl of 11 years and 8 months old who admitted to case collected hospital with " recurrent abdominal pain and low serum K⁺ concentration for 7⁺ years". Her condition began at age of 4, with abdominal pain as primary clinical manifestation, combined or non-combined with diarrhea. There was no abdominal distention, occasional mild fatigue, without symptoms of limb convulsions, numbness, excessive sweating, increased thirst, polyuria, or enuresis, and with long-term hypokalemia. Physical examination at admission showed that abdomen was soft with no palpable masses throughout. The results of relevant auxiliary examinations at admission showed low serum K⁺ concentration which was 3.13 mmol/L, while serum Mg²⁺, Ca²⁺, Na⁺, and Cl^- concentrations were normal, and result of renal ultrasound was also normal. Her condition was recurrent, without a clear cause for low serum blood K⁺ concentration, and treatment effect on serum K⁺ concentration by potassium supplementation was poor. Her result of genetic testing showed SLC12A3 gene compound heterozygous mutations: c.1000C>T(p.Arg334Trp) and c. 1034C>T(p.Ser345Phe), inherited from her father and mother, respectively. Based on her clinical manifestations and genetic testing results, she was diagnosed as GS. After oral treatment by potassium chloride (KCl), spironolactone and enalapril, her gastrointestinal symptoms disappeared and serum K⁺ concentration recovered to normal level when followed up to May 2022. ②Literature review results: a total of 26 pieces of relevant research literature involving 58 cases of GS children were retrieved. Among them, 6 children initially presented with gastrointestinal symptoms at the onset of GS, 2 cases were initially admitted with other conditions and were later diagnosed with GS through result of genetic testing. Initial symptoms in the other 48 cases included low serum K⁺ concentrations, short stature, infections, fatigue, muscle pain, seizures, and hyperthyroidism.

So far, there are few literature has been reported on GS children with slightly low serum K⁺ concentration whose main symptoms were gastrointestinal discomfort. Due to their atypical clinical manifestations, they are prone to be missed diagnosis and misdiagnosis. Genetic testing serves as a crucial method for confirming diagnosis of children with GS. Early diagnosis and treatment can improve their quality of life.

To explore value of non-invasive hemodynamic monitoring by bioelectrical impedance analysis (NHM-BIA) in predicting neonatal sepsis (NS) complicated with cardiac dysfunction (CD).

A total of 152 children with NS who were admitted to neonatal intensive care unit (NICU) of Affiliated Children′s Hospital of Xi′an Jiaotong University from January 2017 to November 2020 were selected as research subjects. They were divided into study group (n=53, complicated with CD) and control group (n=99, without CD) according to whether they were complicated with CD or not by retrospective analysis method. The gender, gestational age at birth, weight at birth, proportion of critically ill neonates, serum N-terminal pro-brain natriuretic peptide (NT-proBNP), high sensitive cardiac troponin (hs-cTn), and creatine kinase isoenzyme MB (CK-MB), atrial and ventricular diameter and ejection fraction, as well as heart rate, stroke volume index (SI), cardiac output (CO), cardiac index, stroke volume variability (SVV), flow time of corrected (FTC), systemic vascular resistance (SVR), systemic vascular resistance index (SVRI), index of contractility (ICON), systolic time ratio (STR) of two groups were compared by independent-samples t test, Mann-Whitney U test and chi-square test. Multivariate unconditional logistic regression analysis was used to analyze the influencing factors of NS complicated with CD in neonates. Receiver operating characteristics (ROC) curves of different indexes of NHM-BIA to predict NS complicated with CD were drawn, and area under the curve (AUC) was calculated. According to principle of maximum Youden index, the best critical value of different indexes for predicting NS complicated with CD were determined. The procedures followed in this study met the standards formulated by the Ethics Review Committee of Affiliated Children′s Hospital of Xi′an Jiaotong University and has been approved by it (Approval No. 20210034). There were no significant differences in general clinical data of neonates with NS between two groups, such as gender composition ratio, gestational age and weight at birth (P>0.05).

①The proportion of critically ill neonates and serum levels of NT-proBNP and hs-cTn in study group were significantly higher than those in control group, and the differences were statistically significant (P<0.05). ②Heart rate, SVV, SVR, SVRI and STR of neonates with NS in study group were significantly higher than those in control group, while SI, cardiac index, FTC, and ICON were significantly lower than those in control group, and all differences were statistically significant (P<0.05). ③Multivariate unconditional logistic regression analysis showed that high serum levels of NT-proBNP and hs-cTn were independent risk factors for NS neonates complicated with CD (OR=1.001, 1.095; 95%CI: 1.000-1.001, 1.047-1.146, all P<0.001), while high cardiac index was an independent protective factor for NS neonates complicated with CD (OR=0.075, 95%CI: 0.022-0.253, P<0.001). ④AUC of serum levels of NT-proBNP and hs-cTn, cardiac index and three indexes combined to predict of NS neonates complicated with CD were 0.814 (95%CI: 0.734-0.894, P<0.001), 0.858 (95%CI: 0.784-0.931, P<0.001), 0.925 (95%CI: 0.784-0.931, P<0.001), and 0.975 (95%CI: 0.942-1.000, P<0.001), respectively. According to principle of maximum Youden index, the best critical values of serum levels of NT-proBNP and hs-cTn and cardiac index for predicting CD in neonates with NS were 13 355 ng/L, 74 ng/L, and 2.74 L/(min·m²), the sensitivities of serum levels of NT-proBNP and hs-cTn, cardiac index and three indexes combined were 77.4%, 58.5%, 84.9%, and 81.1%, respectively, and the specificities were 73.6%, 95.4%, 93.1%, and 96.6%, respectively.

NHM-BIA can effectively monitor hemodynamic changes in neonates with NS complicated with CD. Cardiac index combined with serum levels of NT-proBNP and hs-cTn has high predictive value in NS neonates complicated with CD.

To summarize the clinical characteristics of patient with haploinsufficiency of A20 (HA20) complicated with systemic lupus erythematosus (SLE).

A case of HA20 complicated with SLE (patient 1) was treated in Xi′an Children′s Hospital in June 2019 was selected in the study. The clinical case data of patient 1 were analyzed retrospectively, and the relevant literature of patients with HA20 complicated with SLE in domestic and foreign databases were retrieved in order to summarize the clinical characteristics of the disease. This study was approved by the Ethics Committee of Xi′an Children′s Hospital (Approval No. 20230048). The guardians were informed consent of the diagnosis and treatment of patient 1.

① The patient 1 was a girl, 5 and a half years old, the main manifestations were pustulosis, fever and joint pain, accompanied by oral ulcer, enlarged liver, spleen and lymph nodes, and short stature. She had a history of hepatosplenomegaly and thrombocytopenia. Her laboratory examinations results showed proteinuria, autoimmune hemolytic anemia, decreased of serum complement (C)3, positive antinuclear antibody (1∶1 000), and positive antism antibody. Renal puncture pathological examination showed mild mesangial proliferative lupus nephritis type Ⅱ (LN-Ⅱ). Gene detection showed that the heterozygous variation of TNFAIP3 gene c. 547 (exon4) C>T, which was a pathogenic mutation and came from her father. She was treated with hormone, cyclosporine, cyclophosphamide and mycophenolate mofetil successively. During the treatment, after hormone reduction, rash occurred again, serum C3 decreased and urine protein increased. Up till now, she has no rash and other manifestations, serum C3 decreased slightly and urine protein is negative. She was final diagnosed with HA20, SLE and LN-Ⅱ. ② Literature review results: There were 14 HA20 complicated with SLE patients retrieved (patients 2-15), the analysis of 15 cases including patient 1 showed that: there were 2 males and 13 females, and the age of onset ranges from 2 months to 29 years old, and 12 patients had the damage of kidney, only 2 patients had genital ulcer. The treatments of these 15 cases was as follows, 12 patients were treated with hormone and 10 patients were treated combined with biological agents. After the treatments, the symptoms of most patients could be controlled, and some patients had repeated symptoms.

The incidence rate of HA20 complicated with SLE is low, and its clinical manifestations are not typical. For SLE children with early onset and poor treatment effect, gene detection is conducive to early diagnosis and treatment for patient of the disease.

To investigate changes and significance of fatty acid-binding protein (FABP)-4 in serum and placental tissues and related lipid protein expression of high-fat induced pregnant rats.

A total of 50 Wistar female rats and 16 Wistar male rats were combined in cages at night according to the ratio of female to male of 1∶1 after 7 days of adaptive feeding. After successful fertilization, 40 pregnant rats were selected and divided into high-fat group (n=20, high-fat diet) and control group (n=20, ordinary diet) by random number table method. Serum FABP-4 and blood lipid levels, including total cholesterol (TC), triglyceride (TG), high density lipoprotein (HDL), low density lipoprotein (LDL), very low density lipoprotein (VLDL) and arteriosclerosis index Twere measured by automatic biochemical analyzer between 2 groups. The placental area and weight were measured in pregnant rats. Placental lipid deposition, trophoblast infiltration, and spiral artery changes were observed by hematoxylin-eosin (HE) staining. The mRNA relative expression levels of FABP-4, lipid drop related protein(perilipin) A, B, C and D in placental tissues were determined by real time-quantitative polymerase chain reaction (RT-PCR). The disposal of animals during the experiments was in accordance with the requirements of animal ethics.

①The placenta weight and placenta area of high-fat group were higher than those in control group, and there were statistical differences (t=-4.44, -1.93; P=0.043, 0.018). ②The serum levels of FABP-4、TC、TG of pregnant rats in high-fat group were higher than those in control group, while serum level of HDL was lower, and the above differences were statistically significant (t=-11.06, -17.81, -15.28, 2.48; P=0.024, 0.010, 0.010, 0.002). ③The serum level of FABP-4 was positively correlated with serum TC and TG levels (r=0.827, 0.818; P<0.001) and negatively correlated with HDL level (r= 0.426, P=0.006). ④The results on placenta tissue by HE staining showed that there was no obvious adipocyte infiltration in the placental tissue of control group, the vascular structure was intact, and there was no inflammatory cell invasion. In the placental tissue of pregnant rats in the high-fat group, there were obvious vacuolar adipocytes infiltrating, the vascular intima fibrous thickened lumen stenosis and cellline-like necrosis of the tube wall around the cytoplasm and arterioles, and the infiltration of lipid and inflammatory cells were observed. ⑤RT-PCR results showed that the relative expression levels of FABP-4, perilipin A, B and C mRNA in placental tissues of high-fat group were significantly lower than those in control group, and the differences between 2 groups were statistically significant (t=10.72, 18.68, 9.21, 55.39; P=0.009, 0.001, 0.001, 0.001).

During pregnancy, high-fat intake in Wistar rats can down-regulate the expression of placenta-related lipoproteins, resulting in abnormal fatty acid transport in placenta and the placental lipid deposition, arterial changes, triggering inflammatory responses, abnormal placental function or structure, and ultimately leading to adverse perinatal outcomes; FABP-4, as a special adipokines, is involved in the lipid metabolism of placental trophoblast cells, and it may be used as a new means to evaluate placental function through its dynamic monitoring;

To explore the improvement effect of budesonide suspension for aerosol inhalation combined with dyclonine mucilage smearing with tracheal catheter on pharyngeal discomfort in patients undergoing gynecological laparoscopic surgery and tracheal intubation under general anesthesia.

A total of 200 patients undergoing tracheal intubation under general anesthesia during gynecological laparoscopy in the People′s Hospital of Luzhai were enrolled as the research objects from January 2021 to January 2023. According to random number table method, they were divided into observation group (n=100, budesonide suspension for aerosol inhalation combined with dyclonine mucilage smearing with tracheal catheter) and control group (n=100, dyclonine mucilage smearing with tracheal catheter). By prospective research methods, the operation time, tracheal intubation time and occurrence of pharyngeal discomfort after extubation in both groups were observed. Immediately after extubation (T0), and at 1, 6, 24, 48 and 72 h after extubation (T1, T6, T24, T48, T72), pharyngeal symptoms (sore throat, foreign body sensation in throat, hoarseness) were scored by visual analogue scale (VAS). The mucosal responses of pharynx and larynx were evaluated by scores of the World Health Organization (WHO) pharyngeal and laryngeal mucosal responses. The scores of pharyngeal and laryngeal mucosal responses of two groups at the 6 time points (T0, T1, T6, T24, T48, T72) were compared by double-factor repeated measure ANOVA. The intra-group and inter-group comparison of VAS scores of three kinds of throat symptoms in two groups at the 6 time points were conducted by Mann-Whitney U test. This study was approved by the Ethics Committee of the People′s Hospital of Luzhai (Approval No. 202012KD002). Patients and their families gave informed consent to the treatment and signed informed consent forms.

① There were no significant differences in VAS scores of sore throat and hoarseness between two groups at T0 (P>0.05). The VAS score of pharyngeal foreign body sensation at T0 in observation group was lower than that in control group, and the difference was statistically significant (P<0.05). ② The VAS scores of sore throat, foreign body sensation in throat and hoarseness at T1, T6, T24, T48 and T72 in observation group were lower than those in control group, respectively, and the differences were statistically significant (all with P<0.05). ③ There were significant differences in inter-group comparison of VAS scores of three kinds of throat symptoms at T0, T1, T6, T24, T48 and T72 in observation group, respectively, (all with P<0.001), which were the same in control group. ④ There were significant differences in scores of pharyngeal and laryngeal mucosal response of two groups at T0, T1, T6, T24, T48 and T72, including inter-group principal effect, inter-group with time interaction effect and time principal effect (F_inter-group=626.46, P<0.001; F_{inter-group × time}=12.02, P<0.001; F_time=436.81, P<0.001). ⑤ The incidence of pharyngeal discomfort in observation group was lower than that in control group, and the difference was statistically significant (22.0% vs 39.0%, χ²=6.82, P=0.009).

Budesonide suspension for aerosol inhalation combined with dyclonine mucilage smearing with tracheal catheter can reduce pharyngeal discomfort in patients undergoing tracheal intubation under general anesthesia during gynecological laparoscopy surgery.

To explore the genetic etiology of fetuses with short femur length (FL) at the genome-wide level by chromosome microarray analysis (CMA) technique.

Seventy-three pregnant women and their fetuses who had short FL by prenatal ultrasonography and underwent invasive prenatal diagnosis at Guangzhou Women and Children′s Medical Center from January 2019 to December 2020 were enrolled in the study. According to whether they were combined with other obvious structural developmental abnormalities, they were divided into isolated group (n=56) and complex group (n=17). Chromosomal karyotyping and CMA were performed, and results were analyzed by applying CHAS software and related bioinformatics methods. The procedures followed in this study were in accordance with the ethical standards set by the Human Experimentation Committee of Guangzhou Women and Children′s Medical Center, and were approved by this ethical committee (Approval No. [2019] 11600). Written informed consents were obtained form all pregnant women.

① Of these 56 fetuses in isolated group, 6 were combined with ultrasound soft index abnormalities; of these 17 fetuses in complex group, 6 were combined with neurological malformations, 6 with cardiovascular malformations, and 6 with malformations of other skeletal systems. ②The CMA results of 73 fetuses showed that 10 cases contained pathogenic/probably pathogenic copy number variants (CNV), with a total pathogenicity detection rate of 13.7% (10/73), the variants of uncertain significance (VUS) detection rate of 2.7% (2/73), and a benign CNV detection rate of 83.6% (61/73). ③Of the 44 short FL fetuses that underwent both chromosomal karyotyping and CMA testing, 34 were in isolated group and 10 were in complex group. Comparison of the rates of chromosomal karyotype abnormality (10.0%) and abnormal detection by CMA technique (30.0%) in the complex group showed no statistically significant difference (χ²=2.08, P=0.149). ④None of the 73 fetuses were found to contain the known pathogenic locus of FGFR3 gene. Of the 63 CMA-negative fetuses, 7 fetuses were highly suspected of having long-bone dysplasia due to prenatal ultrasonography (long bones of the limbs <3% of the children of the same gestational age), and further whole exome sequencing (WES) testing showed that the COL1A1 mutation (c.2519C>T; p. P840L) was detected in 1 fetus as VUS. ⑤Follow-up showed that 31 (42.5%) fetuses were delivered at term, 15 (48.4%) live births showed a growth rate of long below the 10th percentile of the same-age child in infancy, and other developmental assessments did not show any obvious abnormalities.

CMA has a certain application value in prenatal diagnosis of fetuses with short FL and has a higher detection rate than chromosomal karyotyping. Therefore, it is suggested that CMA should be used as a first-line technique in the prenatal diagnosis of fetuses with short FL on prenatal ultrasound examination, especially when combined with other structural abnormalities.

To analyze ultrasonographic characteristics and concomitant malformations and prognosis of absent pulmonary valve syndrome (APVS).

From November 2010 to December 2020, a total of 26 fetuses (fetus 1-26) diagnosed as APVS in Beijing Anzhen Hospital, Capital Medical University were selected in the study, and they were enrolled into APVS group. Meanwhile, other 78 healthy fetuses who prenatal examination at the same hospital and fetal age match with APVS group were enrolled into control group. Fetal age at diagnosis, cardiothoracic ratio, cardiac axis, presence of combined intra- and extracardiac malformations and heart failure, ventricular septal defect (VSD) size, aortic ride-through rate, lung area, aortic valve annular diameter, pulmonary valve annular diameter, pulmonary artery trunk and branch diameter, left atrial internal diameter, left ventricular internal diameter, right atrial internal diameter, right ventricular internal diameter, aortic valve flow velocity and pulmonary valve flow velocity were collected and compared between 2 groups. Further, 4 fetuses with copy number variant sequencing (CNV-seq) results were collected. The influencing factors of fetal APVS diagnosis and identification were analyzed by multivariate unconditional logistic regression analysis. The procedure followed in this study conformed to the standards formulated by the Ethics Committee of Beijing Anzhen Hospital, Capital Medical University, and was approved by the Ethics Committee (Approval No. 2023135X). Written informed consents were obtained from pregnant mother of all fetuses.

①Among 26 cases of APVS fetuses, 17 cases (17/26, 65.4%) were tetralogy of Fallot (TOF)-type APVS, 4 cases (4/26, 15.4%) were isolated APVS, and 5 cases (5/26, 19.2%) were complicated with heart malformation.② Echocardiography of 26 APVS cases showed stenosis of pulmonary valve ring, absence or dysplasia of pulmonary valve leaves, and tumor-like expansion of pulmonary trunk and left and right branches. Examination result of color doppler flow imaging(CDFI)showed severe regurgitation of fetal pulmonary valve in 26 cases, and pulsed-wave Doppler (PW) measured increased blood flow velocity of fetal pulmonary valve in 23 cases. ③Among 26 APVS fetuses, 1 case was stillborn, 18 cases were induced to terminate pregnancy, and 12 cases of 19 cases were confirmed by autopsy. One of them was misdiagnosed as pulmonary valve stenosis by fetal echocardiography. Seven cases lost follow-up. ④ There were significant differences in fetal heart axis, cardiothoracic ratio, right ventricular diameter, aortic annulus diameter, pulmonary annulus diameter, left pulmonary artery diameter, right pulmonary artery diameter, aortic valve velocity and pulmonary valve velocity between two groups (P<0.05). ⑤Multivariate unconditional logistics regression analysis showed that gestational age (OR=0.538, 95%CI: 0.295-0.984, P=0.044), right ventricular diameter (OR=2.772, 95%CI: 1.024-7.511, P=0.045), cardiac axis (OR=1.331, 95%CI: 1.101-1.608, P=0.003), and cardiothoracic ratio (OR=2.084, 95%CI: 1.082-4.017, P=0.028) were influencing factors to identify the existence of fetal APVS.

Fetal APVS is a rare conic malformation. Pulmonary valve absence or hypoplasia, pulmonary valve ring stenosis, pulmonary artery trunk or branch tumor-like dilation are typical ultrasound features. Combined with the parameters of cardiac axis, right ventricle and cardiothoracic ratio, it will improves the prenatal ultrasound diagnosis rate. Fetal APVS affects the development of left lung. The prenatal ultrasound diagnosis of fetal APVS provides important information for prenatal prognosis consultation and timely treatment after birth.