Objective:To summarize the characteristics of pregnant women with critical congenital heart disease, and to explore continuous, integrated, multidisciplinary management for this segment of population.Methods:The clinical records of pregnant women with severe congenital heart disease with a history of intensive care who were treated in Guangdong Provincial People′s Hospital from January 1, 2008 to December 31, 2020 were retrospectively analyzed.Results:(1) A total of 132 cases were included, including 128 pregnant women [gestational age (28.0±8.8) weeks] and 4 puerpera cases (6-32 days postpartum), 63.6% (84/132) from economic underdeveloped rural areas, and 78.0% (103/132) by the municipal hospital, irregular prenatal examination accounted for 59.1% (78/132). The main type of congenital heart disease was shunt lesion (55.3%, 73/132). 90.9% (120/132) with mWHO risk classification stage Ⅳ were assigned to it. The main cardiovascular complication was pulmonary hypertension (64.4%, 85/132). 46.2% (61/132) of the patients had been diagnosed with congenital heart disease before pregnancy, and 70.5% (93/132) of the patients had not received any treatment before pregnancy. (2) All patients received obstetric-led, multidisciplinary care. The rescue success rate was 96.2% (127/132), and no serious obstetric complications occurred. The mortality within 24 hours after discharge was 3.8% (5/132). 16.7% (22/132) underwent cardiac surgery during pregnancy, of which 77.3% (17/22) continued their pregnancy beyond 34 weeks. Totally, the delivery week was (30.5±8.6) weeks, and the main mode was cesarean section (71.2%, 94/132). The average weight of 99 live births (including 1 twin pregnancy) was (2 167±698) g. Preterm birth, fetal growth restriction, and congenital malformations were the main fetal comorbidities.Conclusions:Pregnant women with severe congenital heart disease mainly come from areas with underdeveloped economic and medical levels. Later disease intervention, pregnancy retention despite of clear pregnancy contraindications are the distinctive features, which leaded to a significant increase of incidence of maternal and fetal complications, and an increase of the consumption of medical resources. Multidisciplinary active treatment and cardiac surgery during pregnancy could relatively improve maternal and fetal pregnancy outcomes.

Objective:To investigate the feasibility of echocardiography-guided transcatheter closure of atrial septal defect (ASD) during pregnancy and summarize the multidisciplinary treatment experience for such women.Methods:A retrospective analysis was performed on three women receiving echocardiography-guided secondum ASD closure during pregnancy in Guangdong Provincial People's Hospital from January 2018 to October 2021. Perioperative and perinatal multidisciplinary treatment and prognosis were described.Results:All three patients underwent cardiac ultrasonography due to abnormal electrocardiogram during routine prenatal examination and were diagnosed with secondum ASD. Progressive cardiac dysfunction was found during close follow-ups and all cases met the criteria for ASD closure during pregnancy after multidisciplinary evaluation. Echocardiography-guided ASD closure was successfully performed in all patients. Pulmonary arterial pressure was significantly reduced and the cardiac function was stable after the operation. All patients delivered vaginally at term without complications such as miscarriage, premature birth, postpartum hemorrhage or fetal growth restriction and had their cardiac function recovered during postpartum follow-up.Conclusions:Echocardiography-guided ASD closure during pregnancy is technically feasible. Good maternal and fetal outcomes can be obtained through multidisciplinary and close monitoring and treatment during perioperative and perinatal periods.

Objective:To explore the efficacy and safety of open cardiac operation and interventional therapy in pregnant patients and describe the feto-neonatal and maternal outcomes.Methods:A retrospective study of 39 cases of women undergoing open cardiac operation or interventional therapy during pregnancy was conducted in Guangdong Provincial People′s Hospital from Jan. 2014 to Oct. 2019.Results:The age of 39 pregnant women with gestational heart disease was (30±6) years old (21-43 years old). Among them, 37 cases were single and 2 cases were twin pregnancy. Modified World Health Organization (mWHO) pregnancy risk classification were all level Ⅳ. There were 22 women receiving cardiac operation under cardiopulmonary bypass during pregnancy, 14 patients undergoing percutaneous balloon mitral valvuloplasty, 2 patients accepting percutaneous balloon pulmonary valvuloplasty, and 1 case receiving atrial septal defect occluder with ultrasound guidance. Three were no maternal deaths during and after the operation. One patient had an inevitable abortion. Four fetuses died in the uterine after open cardiac surgery. There patients chose termination of the pregnancy after cardiac operation. There were 31 live birth, in which 7 cases were preterm live birth and 24 patients were term live birth. The total number of newborns were 33. Two fetuses suffered neonatal intracranial hemorrhage and died after birth. Thirty-one fetuses were alive and born without any abnormity.Conclusion:For pregnant women with high risk of cardiovascular disease and classified as mWHO pregnancy risk level Ⅳ, cardiopulmonary bypass and interventional therapy during pregnancy could be used as an alternative for better materal and fetal outcomes.

Objective To retrospectively analyze the diagnosis and perinatal management of fetal congenital heart disease ( CHD) in our hospital during the past eight years.Methods A retrospective analysis of 358 cases of CHD diagnosed by fetal echocardiography prenatal and/or postnatal was done in Guangdong General Hospital from Jan 2006 to Dec 2013,the delivery conditions of these fetus were analyzed.Results Incidence of CHD was 1.85％(358/19338), 297 cases were prenatal diag-nosed, 61 cases were postnatal diagnosed.In the prenatal diagnosed group, 60.6％(180/297) were complex CHD.In the postnatal diagnosed group, 90.2％(55/61) were simple CHD.In the complex CHD, 96.8％(180/186) were prenatal diag-nosed.In the prenatal diagnosed group, the survival rate was 68.35％(203/297), of which the simple CHD was 93.16％(109/117), the complex CHD was 52.22％(94/180).The vaginal delivery rate(46.3％ vs 42.6％) in the prenatal diag-nosed group was higher than the postnatal diagnosed group, the difference was significant(P<0.001).Conclusion Through standardized screening and diagnosis methods, most of the complex CHD and part of simple CHD can be prenatal diagnosed . Except for obstetric indications, vaginal delivery was suggested for all of CHD, delivery way did not affect the prognosis.Most of postnatal diagnosed CHD were simple CHD, the prognosis was good.Integrated pattern of prenatal diagnosis and postnatal treatment was completely feasible .

Objectives: Two cases who underwent fetal pulmonary valvuloplasty (FPV) for pulmonary atresia with intact ventricular septum (PA-IVS) or critical pulmonary stenosis with intact ventricular septum (CPS-IVS) successfully were reported. The aim of the report was to explore the criteria for case selection, the technical essentials of FPV, and the postpartum outcome of the fetus. Methods: One case with PA-IVS and the other with CPS-IVS were enrolled in September 2016 and February 2017 in Guangdong General Hospital, and both cases were diagnosed with severe right ventricular dysplasia and tricuspid regurgitation by fetal echocardiogram. Parameters of right ventricle development and hemodynamics from echocardiography included tricuspid/mitral annulus (TV/MV), right ventricle/left ventricle long-axis (RV/LV), pulmonary/aortic annulus (PV/AV), tricuspid inflow duration/cardiac cycle, degree of tricuspid regurgitation (TR), blood flow direction of arterial duct and ductus venosus. Multidisciplinary team including the maternal-fetal cardiology, pediatric cardiology, cardiac surgery, obstetrics, neonatology and anesthesiology was summoned to discuss the indications and timing of PFV. Two cases underwent ultrasound-guiding trans-abdominal PFV at the 28 weeks of gestational age. Echocardiography was performed to observe the opening and closing of the pulmonary valve, and to evaluate the development of right ventricle and improvement in hemodynamics every 2-4 weeks until delivery. Results: From the technical perspective, pulmonary balloon valvuloplasty was successfully performed in these two cases. The opening of pulmonary valve improved in these two cases at 2-4 weeks after FPV. However, an obvious restenosis was detected in the first case at 5-8 weeks after FPV. In the first case, the echocardiography parameters including TV/MV, RV/LV, PV/AV and tricuspid inflow duration/cardiac cycle increased from 0.56, 0.42, 0.85,0.26 to 0.59, 0.51, 0.87, 0.32 at 5-8 weeks after FPV, respectively. However, the direction of blood flow through the arterial duct was still reverse. In the second case, TV/MV, RV/LV, PV/AV and tricuspid inflow duration/cardiac cycle ratio increased from 0.70, 0.63, 0.91,0.35 to 0.80, 0.80, 0.97, 0.42 at 5-8 weeks after FPV, respectively. The direction of blood flow through the arterial duct changed to bidirectional. Both fetuses were born alive. The first case underwent pulmonary valve commissurotomy and modified Blalock-Taussig shunt on the 8^th day after delivery and received follow-up for 6 months. The strategy for the next-step therapy was still pending. The second case underwent transcutaneous pulmonary balloon valvuloplasty on the 19^th day after delivery and received follow-up for 3 months. The opening of pulmonary valve improved obviously and the cardiac function was normal in the second case. Conclusions FPV is safe and effective for fetus during the second and third trimester of pregnancy, and FPV is beneficial for the development of fetal ventricle, valve and large artery. In addition, FPV may help to avoid the postnatal surgery for isolated single ventricle, improve fetal heart failure and prevent fetal death.

Objective To investigate the morbidity, diagnostic profile and perinatal outcome of pregestational diabetes mellitus (PGDM) in 15 hospitals in Guangdong province. Methods A total of 41338 women delivered in the 15 hospitals during the 6 months,195 women with PGDM(PGDM group) and 195 women with normal glucose test result(control group)were recruited from these tertiary hospitals in Guangdong province from January 2016 to June 2016. The morbidity and diagnostic profile of PGDM were analyzed. The complications during pregnancy and perinatal outcomes were compared between the two groups. In the PGDM group, pregnancy outcomes were analyzed in women who used insulin treatment (n=91) and women who did not (n=104). Results (1)The incidence of PGDM was 0.472%(195/41338). Diabetes mellitus were diagnosed in 59 women (30.3%, 59/195) before pregnancy, and 136 women (69.7%,136/195) were diagnosed as PGDM after conceptions. Forty-six women (33.8%) were diagnosed by fasting glucose and glycohemoglobin (HbA1c) screening. (2) The maternal age, pre-pregnancy body mass index (BMI), prenatal BMI, percentage of family history of diabetes, incidence of macrosomia, concentration of low density lipoprotein were significantly higher in PGDM group than those in control group (all P<0.05). Women in PGDM group had significantly higher HbA1c concentration((6.3±1.3)% vs (5.2±0.4)%), fasting glucose [(6.3±2.3) vs (4.8±1.1) mmol/L], oral glucose tolerance test(OGTT)-1 h glucose((12.6±2.9) vs (7.1± 1.3) mmol/L)and OGTT-2 h glucose [(12.0±3.0) vs (6.4±1.0) mmol/L] than those in control group (P<0.01). (3)The morbidity of preterm births was significantly higher (11.3% vs 1.0%, P<0.01), and the gestational age at delivery in PGDM group was significantly smaller [(37.6±2.3) vs (39.2±1.2) weeks, P<0.01]. Cesarean delivery rate in the PGDM group (70.8% vs 29.7%) was significantly higher than the control group (P<0.01). There was significantly difference between PGDM group and control in the neonatal male/female ratio (98/97 vs 111/84, P=0.033). The neonatal birth weight in PGDM group was significantly higher((3159±700) vs (3451±423) g, P<0.01). And the incidence of neonatal hypoglycemia in the PGDM group was higher than the control group (7.7% vs 2.6%, P=0.036).(4)In the PGDM group, women who were treated with insulin had a smaller gestational age at delivery [(36.9±2.9) vs (37.9±2.5) weeks, P<0.01], and the neonates had a higher neonatal ICU(NICU)admission rate (24.2% vs 9.6% , P<0.01). Conclusions The morbidity of PGDM in the 15 hospitals in Guangdong province is 0.472%. The majority of PGDM was diagnosed during pregnancy; HbA1c and fasting glucose are reliable parameters for PGDM screening. Women with PGDM have obvious family history of diabetes and repeated pregnancy may accelerate the process of diabetes mellitus. Women with PGDM have higher risk for preterm delivery and neonatal hypoglycemia. Unsatisfied glucose control followed by insulin treatment may increase the need for NICU admission.

Objective To summarize the clinical experience of surgical intervention for cardiac neoplasm in a fetus . Methods A 32-year-old pregnant woman was admitted to our hospital for complaint of fetal cardiac neoplasm .A separated het-erogenic cardiac occupying lesion was identigied at right atrium of the fetus by echocardiography , whose size is 2.85 cm ×2.25 cm, but the pathogenic origin still remained uncertain, maybe originate from ether pericardium or atrium.The annulus of tri-cuspid valve was compressed nearly 50% with the presence of amount of pericardial effusion.The fetal heart rate decreased at some fetal position resulting in the compression to the heart.So an Ex-utero Intrapartum Therapy(EXIT) procedure was per-formed under the supply of placenta at the 32 weeks of pregnancy.Cesarean section was performed with intact umbilicus and fe-tal circulation by obstetricians.Consequently, the median sternotomy of this fetus and pericardiotomy were performed , with 30 ml clear pericardial effusion drained .The tumor was confirmed to be giant right atrial neoplasm after the intraoperative explora-tion.Considering on the high risk of the cardiopulmonary bypass and limited time for EXIT , the giant atrial neoplasm was left alone with delayed sternum closure after the effectively decompression of the heart .The omphalotomy was successfully per-formed after the EXIT surgery.The neoplasm resection and the repair for its defect on right atrium were performed with cardiop-ulmonary bypass 2 days later.Results Convalesce of this mother was quite good after cesarean resetion .Hemodynamics of the premature baby was satisfatory after the resection of right atrial neoplasm which pathological report was benign hemangioma . Conclusion Via multiple disciplines collaboration , EXIT intervention for fetus is feasible and safe under adequate prepara-tion.

Objective To explore the effects of delivery classification scale for fetal cardiac disease on the prenatal and postnatal integrated treatment strategies.Methods Delivery classification scale for fetal cardiac disease included:grade Ⅰ,no hemodynamics instability; grade Ⅱ,ducted-dependent lesions,stable hemodynamics anticipated;grade Ⅲ,possibility or likelihood of hemodynamic instability; IMPACT(delivery immediately heart intervention) level,hemodynamic instability is anticipated at separation from placental circulation.During August 2006 to May 2010,a retrospective study of 46 cases of prenatal diagnosis of congenital heart disease and delivery in one cardiac center was taken,in which 33 in grade Ⅰ,9 in grade Ⅱ,4 in grade Ⅲ,and no IMPACT.Results Thirty-nine boys and 7 girls were born at (38.0 ± 1.4) weeks of gestation and had consistent fetal diagnoses of mainly cardiac abnormalities with postnatal screen.Thirteen neonates underwent cardiac intervention within one week after birth with one death,including 2 in grade Ⅰ,7 in grade Ⅱ,4 in grade Ⅲ,of them 1 death.Seven infants including 5 in grade Ⅰ and 2 in grade Ⅱ underwent cardiac intervention with one death.The remaining 26 children in grade Ⅰ had uneventfully outcomes,in which 7 cases of surgical operation,17 cases of interventional therapy,2 cases of spontaneous healing.Conclusion Delivery classification scale for fetal cardiac disease should have some guiding significance for early treatment strategies and could enhance closely integration of prenatal diagnosis and postnatal treatment.The most fetuses in grade Ⅰ need not undergo cardiac interventions in neonatal stage.However,early cardiac intervention for fetuses in grade Ⅱ and Ⅲ should be carried out postnatally with the help of neonatologists.

Objective To evaluate the impact of carvedilol and metoprolol on left ventricular (LV)dyssynchrony in idiopathic dilated cardiomyopathy(IDC).Methods In this study,we randomly assigned 65 IDC patients from January 2009 to June 2011 to re-ceive carvedilol or metoprolol succinate.All patieats were divided in to carvedilol group(n=33)and metoprolol group(n=32)Echo-cardiographic measurements and N-terminal pro-brain natriuretic peptide levels were obtained at baseline and 6 month after thera-py.Then long-term follow up the survival rate of patients were observed.Results In carvedilol group,reduction in LVEDS and in-crease in LVEF was higher compared to metoprolol group.Also improvement in LV dyssynchrony achieved and survival rate with carvedilol was higher than metoprolol.However,Improvements in LV mechanical dyssynchrony was similar in two groups.Im-provements in LV mechanical dyssynchrony achived with both drugs were accompanied by reduction in NT-pro-BNP levels in both carvedilol and metoprolol groups(P >0.05).Conclusion Carvedilol is an effective drug improves the intraventricular dyssynchro-nyfor for IDC patients with left ventricular dyssynchrony,and could increasea the survival rate.

Objective To summarize primary experiences of integrated fetal diagnosis and postnatal treatment for the transposition of the great arteries (TGA). Methods Five fetus were diagnozed as TGA at(28.4 ±4.4) weeks of gestation via fetal echocardiography. The mean age of the pregnant women was (28.4 ±3.0) years old. Delivers were taken by caesarean at (36.5 ±1.8) weeks of gestation. The body weight of neonates was (2468 ±442) grams. All neonates were transported to the department of neonatology and re-checked by echocardiography. 3 cases were TGA with intact ventricular septum, 2 cases were TGA with ventricular septal defect. Prostaglandin and mechanical ventilation were applied if oxygen saturation was lower. The standard arterial switch procedure was performed under cardiopulmonary bypass with moderate hypothermia. Results The mean age for patients at surgery was (9. 0 ±6. 2) days ( ranged 2-19 days). Three neonates survived, 2 preterm neonates died. One with body weight 1770 g, 2 days after operation was died of sudden heart arrest and failure of resuscitation. Another was treated by mechanical ventilation and prostaglandin after delivery and underwent operation at the second postnatal day, the neonate appeared low cardiac output and high serum lactate postoperatively and died in the third day. Conclusion The integrated fetal diagnosis and postnatal treatment for TGA could prevent severe cyanosis and emergency transportation after parturition. The lack of any link in the cooperation among the multidisciplinary team could affect the benefits for the TGA neonates provided by prenatal diagnosis.