Background::Due to the wide variety of morphology, size, and dynamics, selecting an optimal valve size and location poses great difficulty in percutaneous pulmonary valve implantation (PPVI). This study aimed to report our experience with in vitro bench testing using patient-specific three-dimensional (3D)-printed models for planning PPVI with the Venus P-valve. Methods::Patient-specific 3D soft models were generated using PolyJet printing with a compliant synthetic material in 15 patients scheduled to undergo PPVI between July 2018 and July 2020 in Central China Fuwai Hospital of Zhengzhou University.Results::3D model bench testing altered treatment strategy in all patients (100%). One patient was referred for surgery because testing revealed that even the largest Venus P-valve would not anchor properly. In the remaining 14 patients, valve size and/or implantation location was altered to avoid valve migration and/or compression coronary artery. In four patients, it was decided to change the point anchoring because of inverted cone-shaped right ventricular outflow tract (RVOT) ( n = 2) or risk of compression coronary artery ( n = 2). Concerning sizing, we found that an oversize of 2-5 mm suffices. Anchoring of the valve was dictated by the flaring of the in- and outflow portion in the pulmonary artery. PPVI was successful in all 14 patients (absence of valve migration, no coronary compression, and none-to-mild residual pulmonary regurgitation [PR]). The diameter of the Venus P-valve in the 3D simulation group was significantly smaller than that of the conventional planning group (36 [2] vs. 32 [4], Z = -3.77, P <0.001). Conclusions::In vitro testing indicated no need to oversize the Venus P-valve to the degree recommended by the balloon-sizing technique, as 2-5 mm sufficed.

Objective:To analyze the short and mid-term efficacy of aortic valvuloplasty with autopericardium on children with aortic valve diseases.Methods:A total of 26 children with aortic valve diseases (stenosis or regurgitation) who underwent aortic valvuloplasty with autopericardium in Fuwai Central China Cardiovascular Hospital from September 2017 to June 2021 were retrospectively analyzed.The short-term and mid-term follow-up data were collected.The maximum aortic valve pressure gradient, subaortic regurgitation area, left ventricular end-diastolic volume (LVEDV) and left ventricular ejection fraction (LVEF) were compared before and after operation.Paired t test was used to analyze the short-term and mid-term efficacy of aortic valvuloplasty with autopericardium on children with aortic valve diseases. Results:All 26 cases were successfully operated, and there were no deaths and serious complications during the follow-up period of (22.96±6.45) months.There was a significant difference between the preoperative and postoperative maximum aortic valve pressure gradient at 1 month ( t=7.85, P<0.05), 6 months ( t=6.43, P<0.05), 1 year ( t=6.16, P<0.05) and 2 years postoperatively ( t=4.22, P<0.05) in children with aortic stenosis or that combined with mild-to-moderate closure.The follow-up data of 9 children with simple aortic stenosis showed that there was a significant difference between the preoperative (8.87±3.57) cm 2 and postoperative aortic regurgitation area at 1 month ( t=6.85, P<0.05), 6 months ( t=5.13, P<0.05), 1 year ( t=6.62, P<0.05) and 2 years postoperatively ( t=5.41, P<0.05). The LVEDV of 26 children was significantly lower at 6 months[(63.54±27.61) mL], 1 year [(53.61±20.20) mL] and 2 years postoperatively [(64.39±17.78) mL] compared with that of preoperative level[(89.42±45.89) mL]( t=3.89, 4.67, 3.58, all P<0.05). The left ventricular pressure and volume decreased, the enlarged heart was narrowed down, and the geometry of the heart was restored.The LVEF of 26 patients also from (61.65±9.67)% before surgery increased to (67.88±4.69)% 6 months after surgery( t=3.68, P<0.05), and increased to (68.62±4.46)% 1 year after surgery( t=4.01, P<0.05), and increased to (67.55±3.09)% 2 years after operation( t=3.01, P<0.05), and the heart function was improved. Conclusions:Aortic valvuloplasty with autopericardium presents an effective short and mid-term efficacy on children with aortic valve diseases, which prevents or delays the aortic valve replacement.

Objective:To evaluate the medium-term clinical and hemodynamic outcomes of percutaneous pulmonary valve implantation (PPVI) using a domestic Venus-P self-expanding valve in the treatment of severe pulmonary regurgitation after Tetralogy of Fallot (TOF).Methods:Retrospective study.From December 2017 to December 2020, 13 TOF patients with (17.8±4.7) years old and (50.2±12.3) kg underwent PPVI using the Venus-P self-expanding valve in the Department of Children′s Heart Center, Zhengzhou University People′s Hospital were recruited.The mean valve size was (31.9±3.1) mm.All patients received the transannular patch surgery and developed severe pulmonary regurgitation.After PPVI, 13 patients were followed up for at least 12 months.The operation-related complications, improvement of valve and heart function and the durability and reintervention of the Venus-P self-expanding valve were analyzed.The right ventricular end-diastolic volume index (RVEDVi) before and after operation was compared by the paired t-test, and the New York Heart Association (NYHA) class was compared by the paired Wilcoxon signed rank sum test. Results:PPVI was successfully performed in all 13 patients without death.At 6 months post-PPVI, cardiac magnetic resonance imaging findings showed that RVEDVi was significantly reduced[(145.7±9.6) mL/m 2vs.(100.2±12.2) mL/m 2, P<0.05], and the NYHA class was significantly improved ( P<0.05). One patient presented moderate pulmonary valve regurgitation at 12 months postoperatively.No vegetation was found on echocardiography, and blood culture was negative in this case.Six patients did not have postoperative pulmonary valve regurgitation, and the remaining presented mild or less pulmonary regurgitation.One patient had sudden ventricular tachycardia on the 6 th day postoperatively, which was converted to sinus rhythm after synchronous electrocardiography.Only one case underwent appendectomy 7 months after operation due to acute appendicitis, and the remaining did not require valve-related reintervention after implantation.During the follow-up for (22.8±8.0) months, no patients had perivalve leakage, stent migration and rupture.Complications like embolization and coronary artery compression were not reported. Conclusions:PPVI using the Venus-P self-expanding valve is safe and effective in patients with severe pulmonary valve regurgitation after TOF surgery, showing an acceptable medium-term follow-up outcome.Studies with a large sample size and long follow-up period are still needed to validate our findings.

Objective:To discuss the treatment strategies and experiences of total anomalous pulmonary venous connection (TAPVC) and study the risk factors for postoperative pulmonary venous obstruction (PVO).Methods:Clinical data of 125 children with TAPVC in the Children Heart Center of Fuwai Central China Cardiovascular Hospital from January 2018 to December 2020 were included in this study and analyzed retrospectively.Of the 125 cases, 116 were treated surgically.They were divided into 2 groups according to whether PVO appeared after repair of TAPVC.The t-test or chi- square test was used to compare the clinical data of the 2 groups, and Logistic regression analysis was adopted to analyze the risk factors for postoperative PVO. Results:Among the 116 patients undergoing surgery, including 73 males (62.9%) and 43 females (37.1%), and according to Darling′s classification, there were 58 cases (50.0%) of supracardiac, 35 cases(30.2%)of intracardiac, 15 cases(12.9%) of infracardiac and 8 cases (6.9%) of mixed.The median age and body weight during surgery were 3.0 (1.2, 7.0) months and 5.0 (4.0, 6.8) kg.There were 13 deaths (11.2%), and recurrent PVO was observed in 18 patients (15.9%). Statistically significant diffe-rences were observed in terms of Darling′s classification (supracardiac 27.8% vs.56.6%, intracardiac 27.8% vs.31.6%, infracardiac 27.8% vs.10.5%, mixed 16.6% vs.5.3%, χ2=8.571, P=0.036) of PVO group and non-PVO group after operation preoperative PVO (83.3% vs.21.1%, χ2=25.293, P<0.01), with confluence (55.6% vs.81.1%, χ2=6.049, P=0.014), preoperative critical state (83.3% vs.31.6%, χ2=12.938, P<0.01), cardiopulmonary bypass time[ (128.800±47.254) min vs.(106.700±37.288) min, t=-2.094, P=0.039 ], delayed thoracic closure(27.8% vs.5.3%, χ2=6.227, P=0.013), and anastomotic velocity at 1 day after operation[1.0(0.9, 1.4) m/s vs.0.9(0.8, 1.1) m/s, Z=-2.004, P=0.045], anastomotic velocity at 1 week after operation[1.4 (1.3, 1.8) m/s vs.0.9 (0.7, 1.1) m/s, Z=-4.446, P<0.001], and mechanical ventilation time[121.0 (76.8, 246.9) h vs.91.5 (60.4, 135.9) h, Z=-1.989, P=0.047]. All of these data were included in the Logistic regression analysis.The results showed that preoperative PVO ( OR=797.179, 95% CI: 8.074-78 712.270, P=0.004) and increased anastomotic flow velocity at 1 week after operation ( OR=11 848.376, 95% CI: 23.746-5 912 017.803, P=0.003) were associated with postoperative PVO. Conclusions:Surgical correction in patients with TAPVC with a biventricular anatomy in this center is satisfactory.Preoperative PVO and early anastomotic flow velocity increase after operation are the high risk factors for postoperative PVO.

Objective:To investigate the changes and their clinical values of renal oxygen saturation(RrSO 2) and abdominal local oxygen saturation(A-rSO 2) in infants who underwent cardiac surgery. Methods:Thirty children with atrial septal defect or ventricular septal defect underwent cardiopulmonary bypass (CPB) in Henan People′s Hospital from April to August 2019 were randomly selected.There were 15 males and 15 females, aged 2-13 months, weighted 4.5-10.0 kg and American Society of Anesthesiologists(ASA) grade Ⅰ-Ⅲ.The probe of near-infrared spectroscopy(NIRS)was fixed at the body surface of the right kidney and 1 cm below the umbilicus.RrSO 2 and A-rSO 2 were continuously monitored during operation.The changes of parameters, including RrSO 2, A-rSO 2, mean arte-rial pressure(MAP), and nose temperature were recorded after anesthesia induction (T 0), cardiopulmonary bypass (T 1), 5 minutes after aortic blockade (T 2), the lowest temperature (T 3), 5 minutes after aortic opening (T 4), and 5 minutes after stopping cardiopulmonary bypass (T 5). CPB time, ascending aorta occlusion time and operation time were recorded as well.Meanwhile, perioperative complications such as acute renal injury (AKI) and gastrointestinal dysfunction were recorded.Relevant information, including the time of first eating after operation was recorded. Results:Totally, 30 children were enrolled in this study.The basic values of RrSO 2 and A-rSO 2 were (70.00±7.50)% and (70.70±11.29)%, respectively.Compared with T 0, the RrSO 2 and A-rSO 2 of patients decreased at T 1, gradually increased at T 2, T 3 and T 4, and returned to T 0 at T 5.There was no significant difference in RrSO 2 and A-rSO 2 at each observation point. Pearson correlation analysis displayed that there was a positive correlation between A-rSO 2 and RrSO 2 ( r=0.806, P<0.01). RrSO 2 and A-rSO 2 were positively correlated with MAP ( r=0.565, 0.605, all P<0.05), and negatively correlated with the nasopharynx temperature ( r=-0.365, -0.331; all P<0.05). Among them, 3 children(10%) suffered from AKI after operation.Compared with T 0, RrSO 2 values at T 1, T 2, T 3 and T 4 decreased significantly ( P<0.05). Postoperative gastrointestinal hysteresis occurred in 6 children(20%). The A-rSO 2 value in T 0-T 5 of children with gastrointestinal hysteresis was significantly lower than that of children without gastrointestinal hysteresis ( P<0.05). Conclusions:As a new noninvasive monitoring method of renal and intestinal function, NIRS has certain clinical guiding value in perioperative period of infantile congenital heart disease.

22q11.2 deletion syndrome (22q11.2DS) is the second leading chromosomal cause of congenital heart disease (CHD) after Down syndrome.In the past few decades, great progress has been made in describing the anatomical types, potential heredity, and pathogenesis of CHD complicated with 22q11.2DS in terms of improving the diagnosis and treatment of these diseases.However, cardiac malformation is still the leading cause of death in 22q11.2 DS.Further studies are needed to elucidate the genetic mechanism behind high phenotypic variability of CHD and to explore the multidisciplinary treatment for the improvement of the prognosis.22q11.2 gene detection in patients suffering from CHD may provide useful insights into early diagnosis and multidisciplinary treatment.

Atrial septal defect (ASD) is a congenital heart disease that causes blood communication between the left and right ventricles due to partial atrial septal tissue defects, accounting for about 13% of all heart malformations. Secondary ASD is the most common type of ASD and can generally be treated with minimally invasive closure. At present, the commonly used minimally invasive methods in clinical practice mainly include X-ray-guided percutaneous occlusion, transesophageal ultrasound-guided transthoracic occlusion and ultrasound-guided percutaneous occlusion. This review focuses on the basic research process of occluder materials, and advantages and disadvantages of three different surgical methods.

Objective:To summarize the clinical experience of changing the membranous pulmonary system during extracorporeal membrane oxygenation(ECMO) in infants after congenital heart disease opration with cardiopulmonary bypass.Methods:From January to September in 2019, 6 cases of congenital heart disease with cardio-pulmonary bypass in our hospital were analyzed retrospectively, whose membrane obstruction occurred during ECMO treatment and replaced successfully.The hemodynamics and blood gas before and after replacement of ECMO system were observed, and the experience was summarized.Results:Six patients(3 males and 3 females), aging from 1 to 3 months and weighing from 3.0 to 4.9 kg, were received VA-ECMO adjuvant therapy.The ECMO system replacement process was smooth and took 175-209 s. The hemodynamic of the children was stable.The ECMO support time was 134-249 h. After the improvement of cardiac systolic function, all children were successfully withdrawn and survived.Conclusion:The improved method of liquid replacement in ECMO system can make full use of the blood components in the original system and avoid the loss of blood tangible components.According to the plan of rapid replacement, the risk of replacement will not be increased.

Objective:To evaluate the efficacy of combination of intranasal dexmedetomidine and esketamine for preoperative sedation in pediatric patients with congenital heart disease.Methods:Fifty American Society of Anesthesiologists physical status Ⅱ or Ⅲ pediatric patients, aged 1-3 yr, undergoing elective cardiac surgery for left-to-right shunt type congenital heart diseases, were divided into dexmedetomidine group (group D, n=25) or dexmedetomidine combined with esketamine group (group DK, n=25) using a random number table method.Dexmedetomidine 3.9 μg/kg was intranasally delivered in group D. Dexmedetomidine 3.3 μg/kg combined with esketamine 2 mg/kg was intranasally administered in group DK.The Children′s Hospital of Wisconsin Sedation Scale score, SpO 2, HR, and pulmonary artery systolic pressure (PAP) were recorded before and at 30 min after administration, and the rate of decrease in SpO 2, HR and PAP after administration was calculated.The onset time of sedation and occurrence of adverse effects such as nausea and vomiting, bradycardia and respiratory depression during sedation were recorded. Results:Inadequate sedation and over-sedation were not observed in either group.Compared with group D, Children′s Hospital of Wisconsin Sedation Scale scores were significantly decreased at 30 min after administration, the onset time of sedation was shortened, and the decrease rate of HR was decreased in group DK ( P<0.05), and there were no significant changes in HR, SpO 2 and PAP before and after administration ( P>0.05). In group DK, nausea and vomiting occurred in 2 cases, but the symptoms were mild and no medication intervention was needed.No other adverse effects such as bradycardia and respiratory depression were found in either group. Conclusion:Combination of intranasal dexmedetomidine and esketamine can optimize the efficacy of preoperative sedation in pediatric patients with congenital heart disease, esketamine may induce nausea and vomiting, and the fasting time should be strictly controlled during sedation.

Objective:To summarize the efficacy of general anesthesia for percutaneous pulmonary valve implantation (PPVI).Methods:The clinical data of 6 patients underwent PPVI under general anesthesia in Children′s Heart Center of Henan Provincial People′s Hospital from December 2017 to January 2020 were retrospectively analyzed.Systolic blood pressure, diastolic blood pressure, heart rate, central venous pressure, SpO 2 and regional cerebral oxygen saturation were recorded before anesthesia induction (T 1), after anesthesia induction (T 2), before beginning of surgery (T 3), before pulmonary valve implantation (T 4), during pulmonary valve implantation (T 5), immediately after pulmonary valve implantation (T 6) and when the patients left the operating room (T 7). Right ventricular systolic pressure, diastolic pressure, pulmonary artery systolic pressure and diastolic pressure were recorded at T 4 and T 6.The development of related complications during operation and the cardiac, liver and kidney functions before and after operation were recorded.The postoperative extubation time, intensive care unit stay time and hospital stay time were recorded. Results:Six patients (3 males, 3 females), aged (16±4) yr, weighing (41±12) kg, were analyzed.Compared with the value at T 1-4 and T 6, 7, systolic blood pressure, diastolic blood pressure, heart rate, regional cerebral oxygen saturation and SpO 2 were significantly decreased at T 5 ( P<0.05). Compared with the value at T 1-5, central venous pressure was significantly decreased at T 6, 7 ( P<0.05). Compared with the value at T 4, right ventricular diastolic pressure was significantly decreased, and pulmonary artery diastolic pressure was increased at T 6 ( P<0.05). No anesthesia- and surgery-related serious complications occurred among the patients.One patient was transferred to the ward after extubation in the operating room, and 5 patients were transferred to the intensive care unit after operation.All 6 patients were discharged successfully and entered the follow-up stage. Conclusion:General anesthesia provides better efficacy when used for PPVI, and hemodynamic monitoring of pulmonary circulation and systemic circulation should be strengthened during pulmonary valve implantation to maintain circulation stable.