The medical records from 20 pregnant women with pregnancy preservation who underwent only cardiac surgery in our hospital from January 2012 to December 2022 were retrospectively analyzed. The patients were divided into 2 groups according to the fetal outcome: fetal survival group and fetal loss group. Eleven patients were included in fetal survival group and 9 patients in fetal loss group. The overall fetal mortality rate was 45%. In fetal loss group, 2 cases died during operation, and the other 7 cases died at 26 (29) days after operation. The valvular disease and infective endocarditis were the most common heart diseases diagnosed in fetal survival group, while type A aortic dissection in fetal loss group. There were 2 cases and 6 cases with pulmonary hypertension, 2 cases and 3 cases with a history of cardiac surgery, and 3 cases and 5 cases underwent emergency surgery in fetal survival group and in fetal loss group, respectively. Four pregnant women underwent deep hypothermic circulatory arrest in fetal loss group. One patient with type A aortic dissection died 15 days after cardiac surgery, with an overall maternal mortality rate of 5% in fetal loss group. The incidence of adverse events after maternal cardiac surgery was 25%, all of which occurred in fetal loss group. In conclusion, the adverse fetal outcomes may be related to type A aortic dissection, pulmonary hypertension, recardiac surgery, emergency surgery, deep hypothermic circulatory arrest, adverse events after cardiac surgery, and long-term related factors after surgery in pregnant women with pregnancy preservation undergoing cardiac surgery alone.

Objective:To study the clinical and genetic characteristics of Wiskott-Aldrich syndrome (WAS) in neonates.Methods:From January 2016 to August 2022, neonates with WAS admitted to the neonatal department of our hospital were studied.Their clinical features, laboratory findings, genetic characteristics and clinical outcomes were retrospectively analyzed.Results:A total of 11 neonates(all male) were included. The mothers of 3 neonates had thrombocytopenia during pregnancy. The presenting symptoms included isolated bloody stool (4 cases), jaundice (3 cases), bloody stool with petechiae, bloody stool with hematemesis, cough and fever(1 case each). Eczema appeared from 6 d to 3 months after birth and in 6 cases during the neonatal period. None of the 11 cases had serious infection during the neonatal period. 9 cases had infection from 8 d to 5 months and 2 cases had not been infected until the last follow-up. Genetic sequencing showed four frameshift variants(c.30dupC, c.205dupT, c.1340_1343dupC and c.673_674delA), four nonsense variants(c.37C>T, c.295C>T, c.889C>T and c.823G>T) and three missense variants(c.134C>T, c.397G>A and c.341T>C). Pedigree verification of variants found 10 cases were inherited from their mothers and 1 case was de novo variant.Conclusions:WAS is characterized by bloody stool and eczema in the neonatal period, mostly without serious infections and lacking specific manifestations. Genetic screening for early identification of unexplained thrombocytopenia in male newborns should be performed as early as possible.

Objective:To study the clinical and laboratory characteristics of neonatal isolated sulfite oxidase deficiency (ISOD).Methods:An infant with neonatal ISOD admitted to our hospital was retrospectively analyzed. Using key words "isolated sulfite oxidase deficiency", "SUOX gene", "Infant, newborn", databases including CNKI, Wanfang database, National library and literature center of science and technology, China science paper online, PubMed, Web of Science and EMBASE (up to January 2021) were searched and literature review was conducted. The clinical manifestations, laboratory results, treatment and prognosis were analyzed.Results:Our patient was a full-term male infant with eye movement disorder, refractory seizures, feeding difficulties, increased muscle tone, developmental retardation and microcephaly. Urine sulfite paper-strip test was positive. Uric acid was normal. Whole exon sequencing (WES) revealed SUOX c.475G>T and c.1201A>G compound heterozygous mutations. Cranial MRI showed multiple encephalomalacia and brain atrophy at 5-month of age. The infant died at 8-month. In the literature review, a total of 29 articles and 32 cases of neonatal ISOD were found. 87.5% of the cases developed symptoms within 1-week after birth. All had convulsive seizures. Some of them had feeding difficulties, muscle tone changes, developmental retardation, microcephaly and ectopia lentis. Cranial imaging showed white matter cystic lesions and brain atrophy. Laboratory examination showed elevated urinary sulfite and S-sulfocysteine. Uric acid and xanthine/hypoxanthine were normal. Blood homocysteine was decreased. 23 cases received genetic testing and all of them had SUOX mutations. The treatment was mainly symptomatic relief and supportive treatment. During follow-up, 15 cases died, 13 cases survived and 4 cases were unknown. All the surviving children had drug-resistant convulsions and developmental retardation.Conclusions:Neonatal ISOD may present with refractory convulsions, feeding difficulties and developmental retardation. Cystic white matter changes and brain atrophy may be seen on cranial imaging. Elevated urinary sulfites, decreased blood homocysteine and normal uric acid are important clues for diagnosis. Genetic testing is helpful for early diagnosis.

Objective:To investigate the clinical significance of changes of serum Clara cell secretory protein(CC16) and pulmonary surfactant protein A(SP-A) in neonates with acute respiratory distress syndrome(ARDS).Methods:The data of 30 neonates with ARDS who needed mechanical ventilation in neonatal intensive care unit of Xi′an Children′s Hospital from January 2016 to November 2018 were collected as observation group, including 12 cases in mild group, 10 cases in moderate group and 8 cases in severe group.The data of healthy newborns during the same period were taken as control group.The serum levels of CC16 and SP-A were detected by ELISA.The serum levels of CC16 and SP-A among different groups were compared.Results:The levels of serum CC16 and SP-A in ARDS group were (59.35±3.67)mg/L and(75.38±6.27)mg/L respectively, (11.26±1.32)mg/L and(18.15±2.69)mg/L in healthy group.The difference was significant( P<0.05). And the differences of serum CC16 and SP-A levels among different degree ARDS groups were significant( P<0.05). The levels of serum CC16 in mild, moderate and severe subgroup were(38.27±16.01)mg/L, (51.25±15.63)mg/L, (84.76±13.12)mg/L and SP-A were(47.02±7.18)mg/L, (73.12±7.98)mg/L, (96.45±12.50)mg/L, which increased with disease severity. Conclusion:Serum CC16 and SP-A are increased and correlated with the severity of neonatal ARDS, which may be used as the index for evaluating the severity of neonatal ARDS in the future.

Objective    To identify the risk factors for coagulopathy after Stanford type A acute aortic dissection (AAD) repair to offer evidence for improvement of patients' prognosis. Methods    We retrospectively analyzed the clinical data of 95 patients undergoing Stanford type A AAD repair in Beijing Anzhen Hospital between January 2013 and December 2014. Patients with thromboelastography-coagulation index (TEG-CI) ≤–3 after surgery were allocated to a coagulopathy group (n=17, average age 48.70 years), whereas patients with TEG-CI >–3 after surgery were allocated to a control group (n=78, average age 46.80 years). Multivariate analysis was used to identify risk factors for coagulopathy after surgery. Results    Seventeen patients suffered from coagulopathy after surgery. Patients in the coagulopathy group had larger amount of fluid drainage than that in the control group (P=0.008). Risk factors for postoperative coagulopathy were activated partial thromboplastin time (APTT) at the end of surgery ( OR=0.011, 95% confidence interval 0.001 to 0.021, P=0.035), fibrinogen degradation products (FDP) at the end of surgery (OR=0.004, 95% confidence interval 0.001 to 0.007, P=0.022) and platelet count (×109/L) at the end of surgery (OR=–0.002, 95% confidence interval –0.003 to 0.000, P=0.049). The lower risk of postoperative coagulopathy was related to the platelet count at the end of surgery up to 137.00 × 109/L. Conclusion    Postoperative coagulopathy could be related to the clinical and experimental variables. In a   representative sample of Chinese adults undergoing Stanford type A AAD surgery, APTT, FDP and platelet count at the end of surgery are independent risk factors associated with postoperative coagulopathy. Adding haemostatic, such as fibrinogen and prothrombinase complex, is good for improving the recovery of coagulation function to reduce bleeding and postoperative blood transfusion, as well as adding platelet, plasma and other coagulation factors after AAD surgery.

Objective To observe the evolution of astrocytes,GDNF,BDNF and Jak-STAT signal pathway after spinal cord ischemia-reperfusion injury in rabbits.Methods Spinal cord ischemia was induced by means of balloon occlusion of the infrarenal aorta for 22 minutes in 54 male New Zealand white rabbits.We assigned rabbits to 9 groups (n =6),one sham group,eight operation groups.The operation process in the sham group was the same as the operation group except the ischemia reperfusion of the spinal cord.At 0 h,1 h,2 h,3 h,8 h,24 h,48 h and 72 h after reperfusion,animals were sarcrificed and the spinal cord was removed for histologic,immunohistochemical study and western blotting.Results Normal neurons were decreased with the extension of reperfusion time.Levels of GFAP increased at 3 h and reached a peak at 48 h after reperfusion.GDNF was increased reaching two peaks after injury,the first peak was at 3 h,the second was at 72 h.BDNF level was increased and peaked at 24 h after reperfusion.The expression of p-STAT3 showed a biphasic pattern which peaked at 1h and 48 h.GFAP,GDNF,BDNF were rare and the level of p-STAT3 could be neglected in sham group.Conclusion Spinal cord ischemia-reperfusion injury could induce the activation of astrocytes,the expression of GDNF,BDNF and the activation of JakSTAT signal pathway.They showed different expression rules in this study.

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.

Objective: To observe the activation of microglia and the changing rule of inflammatory cytokine as IL-6, IL-10 and nuclear factor-κB (NF-κB) in experimental rabbits after spinal cord ischemia reperfusion (SCIR) injury in order to provide theoretical basis for post-conditioning time. Methods: Rabbit SCIR injury model was established by thoracic aorta balloon occlusion. 54 New Zealand male adult white rabbits were divided into 9 groups: Sham group (the animals received balloon implantation without occlusion), SCIR-0h group (reperfusion was conducted at 0 hour of spinal cord ischemia), SCIR-1h, -2h, -3h, -8h, -24h,-48h and -72h groups. n=6 in each group. The number of normal and apoptosis neurons, the levels of Iba-1, IL-6, IL-10 and NF-κB in spinal tissue were examined and compared among different groups respectively. Results: The number of normal neuron was decreasing with the extended reperfusion time, TUNEL-positive neuron began to increasing in SCIR-8h group and the peak was reached in SCIR-24h group. The expression of Iba-1 began to elevating in SCIR-2h group and the peak was obtained in SCIR-8h group; NF-κB began to rising in SCIR-3h group and the peak was observed in SCIR-8h group; both IL-6 and IL-10 arrived the peak in SCIR-24h group. The expressions of NF-κB, IL-6 and IL-10 were positively related to Iba-1 level. Conclusion: Microglia activation had dynamic changes in experimental SCIR rabbits and the expression levels of NF-κB, IL-6 and IL-10 were positively to microglia activation; post-conditioning time at front and back to microglia activation may reduce neuron injury.

Objective To investigate the perinatal outcome , risk factors and long-term outcome of pregnancy complicated with pulmonary arterial hypertension (PAH) and congenital heart diseases (CHD). Methods Clinical data of 110 pregnant women who were diagnosed as PAH-CHD were retrospectively analyzed in the Department of Obstetrics and Gynecology and Surgical Intensive Care Unit at Beijing Anzhen Hospital from 2004 to 2013.The survival and treatment status were followed up .Results 110 subjects consisted of 11 mild PAH, 33 moderate and 66 severe ones .The incidences of deterioration in New York Heart Association ( NYHA ) classes (≥2 ) during pregnancy , respiratory failure , pulmonary hypertension crisis and arrhythmia were 25.5% (28/110),7.3% (8/110),10.0% (11/110),10.0% (11/110) respectively.Among them, the difference of deterioration in NYHA classes (≥2) during pregnancy among the three groups was statistically significant .A total of 8 ( 7.3%) maternal deaths occurred during hospitalization , all of whom were severe PAH cases .Multivariate analysis showed that pulmonary artery systolic pressure was a risk factor of perioperative death (OR=1.042, P=0.005).There were 55 cases (50.0%) of term delivery, and 35 cases (31.8%) of iatrogenic abortion.The proportion of term delivery in the severe PAH group was significantly lower . The proportion of iatrogenic abortion and small for gestational age infant ( SGA ) were higher in severe group .The incidence of neonatal malformations was 8.0%(6/75).The follow-up rate was 61.8%(63/102).Sudden death was reported in a parturient a few days after discharge .The remaining 62 patients survived during follow-up, while 53 patients (85.5%) were functional class ( FC ) Ⅰ -Ⅱ, 9 ( 14.5%) were FC Ⅲ -Ⅳ at follow-up.The cardiac function deterioration during pregnancy was not significantly correlated with long-term deterioration (P =0.767). Conclusions Perinatal mortality and the incidence of maternal and fetal adverse events were high in pregnancy with PAH-CHD.Pulmonary artery systolic pressure is a major risk factor for perioperative mortality in pregnant women .PAH-CHD woman had good overall outcome after puerperium .

Objective To measure the effect of prostaglandin E1 (PGE1) lipid microsphere on cardiac haemodynamics and oxygen metabolism during the perioperative period in the infants with ventricular septal defect(VSD)and severe pulmonary artery hypertension (PAH).Methods Forty infants [(7.1 ± 3.3) years old] with VSD and severe PAH who underwent surgery under cardiopulmonary bypass were involved in the study.They were divided into 2 groups averagely:control group (20 cases) and experimental group (20 cases).All the patients were continuously intravenous pumping of nitroglycerin or PGE1 during the perioperative period.The effect of PGE1 on cardiac haemodynamics and oxygen metabolism between the 2 groups were measured during 72 hours postoperatively.Results The statistical analysis demonstrated that the values trend of mean arterial blood pressure (mABP),mean pulmonary arterial pressure (mPAP),mPAP/mABP,pulmonary vascular resistance index (PVRI),left ventricular stroke work index (LVSWI) were affected during 72 h postoperative period (P ＜0.05).The mABP at 48 h,LVSWI at 48 h,72 h in experimental group were significantly higher than those in control group (all P ＜0.05).The mPAP at 8 h,48 h,PVRI at 72 h and pulmonary arterial wedge pressure (PAWP)at 12-48 h in experimental group were significantly lower than that in control group (all P ＜ 0.05).Compared to postoperative period,mABP at 12 h,72 h,mPAP at 4-12 h,48 h were increased significantly in control group (P ＜ 0.05) ; mABP and LVSWI at 8-72 h,right ventricular stroke work index at 48 h,72 h and cardiac index at 72 h were significantly increased (P ＜0.05),while PVRI and PAWP at 72 h,mPAP/mABP at 24-72 h were significantly decreased in experimental group (P ＜ 0.05).There were no significant differences in the values of oxygen metabolism between both groups (P ＞0.05).Conclusions LipoPGE1 can significantly decrease the pulmonary arterial pressure,which can enhance cardiac function and decrease the duration of intubation after surgery.