Objective:To analyze the clinical features and outcomes of paroxysmal supraventricular tachycardia (PSVT) in neonates without structural heart disease.Methods:A retrospective study was conducted on PSVT neonates without structural heart disease who were treated and followed up at the Women and Children's Hospital, Qingdao University, from January 2019 to June 2022. Clinical data, including the prenatal history of PSVT, the time at first onset of PSVT after birth, anti-arrhythmic treatment, and follow-up outcomes, were collected and analyzed. These patients were divided into two groups based on the presence and absence of fetal PSVT history. Differences in the clinical data between the two groups were compared, including the time at first onset of PSVT, the proportion of patients with persistent tachycardia at initial diagnosis, and hospitalization frequency. Statistical analysis was performed using t-test, Mann-Whitney U test, or Pearson's Chi-square test. Results:A total of 72 neonates with PSVT were included, with an average gestational age at delivery of (38.8±1.8) weeks and an average birth weight of (3 260±330) g. There were 26 (36.1%) cases with a prenatal history of PSVT, while 46 (63.9%) cases without. The median time at the first onset of PSVT after birth was 2.1 (0.3-13.7) d. Anti-arrhythmic drugs used for the patients included propafenone (44 cases, 61.1%), amiodarone (28 cases, 38.9%), and cedilanid (14 cases, 19.4%). There were 44 cases (61.1%) received single drug therapy, 26 (36.1%) receiving dual therapy, and only two (2.8%) receiving triple therapy. Prophylactic drugs were administered to 38 patients (52.8%) for six months, and 20 (27.8%) for 12 months. Fourteen cases (19.4%) still exhibited tachycardia during follow-up and continued their drug therapy. No major illnesses or deaths occurred in the 72 patients during a 12-month follow-up. Compared with the patients without a history of fetal PSVT, those with a history of fetal PSVT had an earlier onset of PSVT after birth [0.2 d (0.0-0.7 d) vs. 12.0 d (2.2-15.0 d), Z=－4.83, P<0.001], a high rate of persistent tachycardia at first diagnosis [76.9% (20/26) vs. 39.1% (18/46), χ2=4.76, P=0.029], more hospitalizations [4.0 times(3.0-7.0 times) vs. 1.0 times (1.0-1.0 times), Z=－3.52, P<0.001], and longer duration of preventive anti-arrhythmic treatment [12.0 months (10.5-21.0 months) vs. 6.0 months (3.0-6.0 months), Z=－4.17, P<0.001]. Conclusion:Attention should be given to PSVT screening in neonates without structural heart disease, particularly for those with a history of fetal PSVT, who tend to have an early onset of PSVT after birth, persistent tachycardia at first diagnosis with high rates of recurrence and require longer preventive anti-arrhythmic treatment.

Objective:To review the clinical outcomes following perinatal multidisciplinary diagnosis and treatment of fetal D-transposition of great arteries (D-TGA).Methods:This retrospective analysis involved 37 fetuses (two fetuses were one of the twins) with D-TGA that were diagnosed by prenatal ultrasound at the Women and Children's Hospital, Qingdao University from January 2016 to December 2020. All the subjects received perinatal multidisciplinary diagnosis and treatment, from the Departments of Fetal Medicine, Genetics, Obstetrics, Ultrasonography, Pediatric Cardiology, Neonatology, etc., and the outcomes were described and summarized.Results:The detection rate of D-TGA was 0.059% (37/62 413), among which intact ventricular septum with D-TGA accounted for 56.8% (21/37) and ventricular septal defect with D-TGA for 43.2% (16/37). All the 37 cases were observed with normal nuchal translucency and four of them were at high risk in fetal Down syndrome screening. All the 31 cases who received non-invasive cell-free fetal DNA screening had normal results and two of 26 cases who received amniocentesis for karyotype analysis and chromosome microarray analysis were abnormal. In terms of pregnancy outcome, 19 pregnancies (51.4%) were terminated, of which 10 cases were terminated for medical reasons and others for non-medical reasons, and 18 cases gave birth to alive body (48.6%, 18/37). Postnatal ultrasound re-examination of one neonate revealed D-TGA with ventricular septal defect, patent ductus arteriosus, and bicuspid pulmonary valve malformation and severe hypoxia and acidosis occured. The patient was discharged after withdrawing treatment and was lost to follow-up. The other 17 neonates all underwent successful surgical treatment with a mean age of (10.2±6.0) d and length of hospital stay of (26.3±9.3) d. Postoperative follow-up (3.3±1.2) years showed all with good cardiac function.Conclusion:Perinatal multidisciplinary diagnosis and treatment of D-TGA can improve the success rate of postnatal treatment and prognosis.

Objective:To explore the value of current indications for fetal pulmonary valvuloplasty (FPV) by summarizing the postnatal diagnosis, treatment, and prognosis of fetuses with pulmonary atresia with intact ventricular septum (PA/IVS) and right ventricular hypoplasia (RVH).Methods:This prospective study was conducted at the Heart Center of Women and Children's Hospital, Qingdao University from September 2018 to March 2021, which included pregnant women who were (1) with fetal PA/IVS and RVH; (2) unable to receive FPV due to fetal position or gestational age despite the indications; (3) given integrated pre- and postnatal management. Prenatal fetal echocardiography assessment, postnatal diagnosis, treatment, and follow-up were summarized using Wilcoxon matched-pair signed-rank test.Results:A total of 35 singleton pregnant women were diagnosed with fetal PA/IVS and RVH by ultrasonic cardiogram and admitted during the study period. Among the 28 fetuses meeting the FPV indications, 18 underwent FPV, while the other 10 did not due to inappropriate fetal position or gestational age. After excluding four terminated pregnancies, the rest six cases were enrolled. The median gestational age at the initial prenatal fetal echocardiography diagnosis was 28.9 weeks (28.3-30.4 weeks). Compared with the initial evaluation, the fetal right ventricular to left ventricular length/diameter ratio [0.8 (0.6-0.9) vs 0.6 (0.5-0.8)] and tricuspid regurgitation velocity [4.7 m/s (3.2-5.1 m/s) vs 4.1 m/s (3.3-4.8 m/s)] were increased, while tricuspid valve Z value [－0.8(－1.6-0.8) vs 0.4 (－0.3-1.9)] and single-ventricular predictive score [0.5 (0.0-2.0) vs 2.0 (1.0-3.0)] were decreased when re-evaluated six weeks later ( T were－2.21, 2.00,－2.20, and 2.00; all P<0.05). All of the six fetuses were born alive with a median gestational age of 38.9 weeks (37.3-40.1 weeks). The median weight was 3 425 g (3 100-4 160) g after being transferred to cardiac intensive care unit. The median age was 12.5 d (0.0-20.0 d) at the first surgical intervention. The median follow-up duration was 15 months (11.8-18.5 months). At initial diagnosis, the single-ventricular predictive score was 1-2 points in four fetuses, and =3 points in two fetuses. There was no death during follow-up. Four patients achieved anatomical biventricular circulation, one achieved clinical biventricular circulation, and one still needed further follow-up, with single-ventricular predictive score at initial diagnosis of 1-3, 3, and 2 points, respectively. Conclusions:The prognosis is good in fetuses with PA/IVS and RVH who have FPV indications but do not receive intrauterine intervention, which suggests that the current FPV indications may be too broad, and a more suitable FPV indication need to be further explored given the difficulty of implementing FPV.

Objective:To summarize the interim outcome and right heart development of pulmonary atresia with intact ventricular septum (PA-IVS) in children after fetal cardiac intervention (FCI).Methods:The clinical data of 5 live births underwent FCI from October 2018 to April 2019 in Women and Children′s Hospital, Qingdao University were analyzed retrospectively. The development of right ventricle (RV) and tricuspid valve (TV) in uterus after FCI, at birth, the age of 6 months, 1 year and 2 years, and the final outcome were assessed.Results:Five PA-IVS fetuses were included in this study. The first evaluation was performed at 24-26 weeks of gestational age, and the FCI was performed at 26-28 weeks of gestational age. During the follow-up of 6 weeks after FCI, the minimum diameter of tricuspid annulus increased from 0.85 cm to 0.92 cm, and the minimum Z-score of tricuspid annulus decreased from -0.03 to -1.62. The minimum values of TV/mitral valve annular diameter and RV/left ventricular length ratios of all fetuses increased from 0.57, 0.52 to 0.88, 0.82, respectively. The maximum tricuspid regurgitation velocity decreased from 4.60 m/s to 3.64 m/s. No severe hemodynamic change was found in any of the fetuses. All 5 fetuses were born alive. Three cases underwent percutaneous balloon pulmonary valvuloplasty (PBPV) and stent implantation for ductus arteriosus. Two cases received PBPV alone. At follow-up (26 to 32 months), obvious development of TV was observed 6 months to 1 year after birth in 5 cases with the growth rate ranging from 19.64% to 40.00%. Meanwhile, the RV development was relatively slow at 6 months with the growth rate ranging from 9.41% to 21.42%. There were individual differences in RV development at 2 years. The growth and development of all children were equal to healthy children of the same age with the body mass index less than 18.4 kg/m 2. At the last follow-up, all children had a transcutaneous oxygen saturation of greater than 0.95, three became biventricular circulation and two had circulation approximation to biventricular circulation with almost closed stent. Conclusions:The findings support the potential of development of right ventricular and tricuspid valve for fetuses with PA-IVS underwent FCI. All fetuses underwent FCI received intervention after birth, and biventricular circulation can be realized finally. The development of right ventricular and tricuspid valve is not proportional. In utero, the right ventricle develops rapidly, and the development of tricuspid valve is more advantageous after birth.

Pulmonary artery denervation (PADN) can block local sympathetic nerve of pulmonary artery, reduce the hemodynamic parameters of pulmonary arterial hypertension(PAH), attenuate pulmonary vascular remodeling, right ventricular hypertrophy and fibrosis, thus improving cardiac function.Early basic experiment has determined the position of sympathetic nerve of pulmonary artery in pulmonary endarterium and confirmed the safety and effectiveness of PADN in the animal model of PAH.PADN may play a role by inhibiting execssive activation of the sympathetic nervous system and renin-angiotension-aldosterone system.PADN has been applied to adult clinical research, and has achieved a good clinical effect.On this basis, the possibility of applying PADN to children′s PAH is being explored preliminarily.

Objective:To summarize the experience of arterial duct (AD) stenting in children with ductus-dependent hypoplastic right heart syndrome (HRHS).Methods:Seven children including 4 cases of pulmonary atresia with intact ventricular septum (PA-IVS) with HRHS and 3 cases of critical pulmonary stenosis (CPS)-IVS with HRHS underwent AD stenting in Qingdao Women and Children′s Hospital between January 2012 and January 2019. During the same period, 9 patients of PA-IVS with HRHS received Blalock Taussig (B-T) shunt. Two groups of children on the operation time, hospital stay time, intensive care time and mortality were compared. T test or Mann-Whitney U test was used for comparison between the two groups. Results:There was no significant difference in the age (18 (7-100) vs. 17 (1-142) d, U=31.000, P>0.05) and weight ((3.8±1.1) vs. (3.7±1.3) kg, t=0.272, P>0.05) between the AD stenting group and the B-T group.The operation time ((108±7) vs. (160±49) min, t=-4.304), intensive care time ((3.4±1.0) vs. (6.3±4.5) d, t=-8.692) and total hospitalization time ((10.3±1.0) vs. (26.3±1.0) d, t=-7.822) in the AD stenting group were differed significantly compared with the B-T group (all P<0.05). The transcutaneous oxygen saturation improved significantly (0.723±0.125 vs. 0.926±0.005, t=-6.044, P<0.05) after AD stenting. The diameter of AD stent ranged from 3.5 to 4.0 mm, and the length of AD stent was 16-21 mm. There were no complications such as vascular injury, acute thrombus, catheter spasm and death in the AD stenting group. The mortality of children in the B-T group was 3 in 9 cases. Three cases in the AD stenting group received pulmonary valvulotomy and bilateral Glenn operation at 6, 9 and 9 months after AD stenting, respectively. Conclusions:AD stenting is a feasible, effective, safe and minimally invasive procedure for children with ductus-dependent HRHS. It can even be used as an alternative to B-T shunt.

Persistent pulmonary hypertension of the newborn (PPHN) is a serious disease that endangers the life of a newborn.Currently,extracorporeal membrane oxygenation is an important component of life support for PPHN patients who is ineffective of the conventional treatment.In recent years,the domestic related reports have gradually increased.This paper summarizes the related research results at home and abroad in recent years,and expounds its application progress.

Objective Transesophageal echocardiography (TEE) guided, minimally invasive perventricular device occlusion of ventricular septal defects ( VSDs) without cardiopulmonary bypass ( CPB) has been applied in multiple centers. We reported experiences and the mid-term results. Methods Four hundred and thirty-two cases from 4 cardiac centers were involved in the study. There were 235 males and 197 females, aged from 3 months to 15 years, with a body weight varying from 4.0 to 26.0 kg. Three hundred and fifty-one patients had perimembranous VSDs, 57 had intracristal or supracristal VSDs and 24 had muscular VSDs (17 had multiple muscular VSDs). The diameter of the VSD ranged from 3 to 12 (5.3 ±1.6 ) mm.For those with perimembranous or muscular VSDs, a 3 to 5 cm inferior sternotomy was made, but for those with intracristal or supracristal VSDs, a 2 to 3 cm incision was made parastemally through the left third intercostal space. Being monitored and guided with TEE, the device was deployed to occlude the VSD through the puncture at the free wall of the right ventricle. TEE was used for assessing the residual shunting, the left and right ventricular outlet tracts, valvular function and for detecting any arrhythmia, The devices would be released if the heart rhythm was normal, as well as the residual shunting and valvular regurgilalion were not detected. Results The procedure was completed successfully in 417 cases(96.5% ) and converted to traditional surgical closure with CPB in the other 15 cases(3.5% ). Concentric devices were used in 238 cases(57.1% )and eccentric devices were used in 179 patients(42.9% ). Successful procedures finished in less than 90 minutes, and the deployment and evaluation of the devices were completed in 5 to 60 (18. 2 ± 8.6) minutes. No residual shunt and detectable aortic or tricuspid insufficiency and arrhythmia was observed. Patients were extubated within 2 hours and discharged 3 to 5 days after the operation. During fellow-up period from 3 months to 2 years, no clinically significant complications occurred. Conclusion The minimally invasive device closure of VSD under TEE guidance without CPB is proved to be a simple, safe and effective treatment for a considerable number of children with VSD. Its use in the clinical practice should be encouraged.

Objective To observe the availability and safety of ventricular septal defect (VSD) occluder in infants and young children with large patent ductus arteriosus (PDA) associated with severe pulmonary hypertension.Methods Five patients (1 male and 4 fomale) of large PDA aged 5 months to 3 years,weighted from 5.1 to 15 kg,body surface area (BSA) 0.37-0.58 m2 underwent transcathter intervention with concentric VSD occluders from June 2008 to May 2009.Arterial ducta were tube-like and their diameters were 5.7 to 8.5 mm,with ulmonary vascular resistance from 4.8 to 5.7 Wood Unit,Qp/Qs 3.4-4.6.Three patients were given Bosentan after intervention.Results The large PDAs were successfully closed with VSD occluders,including 1 concentric perimembranous VSD occluder and 4 muscular VSD occluders.They all discharged 4 to 5 days with hidrosis and weight improved.Echocardiogram indicated VSD occluder was stable,no residue shunt and no stricture of left pulmonary artery and descending aorta were found.According to tricuspid and pulmonary regurgitation,pulmonary arterial pressure decreased differently and returned to normal after 6 months follow-up.Conclusion VSD occluder is available and effective to close large PDA associated with severe pulmonary hypertension in inrants and young children,but more cases and long-term follow-up are necessary.