Objective:To summarize the effect of one and a half ventricle repair in the treatment of Ebstein’s Anomaly.Methods:The data of 149 patients diagnosed with Ebstein’s Anomaly and received with surgical treatment in Pediatric Cardiac Surgery Department of Beijing Anzhen Hospital affiliated to Capital Medical University from January 2010 to December 2018 were continuously collected, and the survival rate, reoperation rate and tricuspid regurgitation were followed up in the medium-long term.Results:There were 68 males and 81 females, with a median age of 5.58 years. Patients were divided into Biventricular repair group and one and a half ventricle repair group. The operative age was significantly younger in the one and a half ventricle repair group (4.15 years vs. 6.71 years, P=0.019). There were also significant differences in patiens’ body length[(107.70±31.28)cm vs. (123.20±35.22)cm, P=0.014]and body weight[(19.69±12.22)kg vs. (29.65±20.41)kg, P=0.001], between the two groups, which may be related to the severity of the disease and the need for early surgical intervention. However, there was no significant difference between the two groups in preoperative cyanosis, hemoglobin level, cardiac function, arrhythmia, and common cardiac malformations such as atrial septal defect. Notably, the proportion of preoperatively complicated pulmonary stenosis (10.81% vs. 1.79%, P=0.016) and right ventricular dysplasia (16.22% vs. 3.57%, P=0.008) was significantly higher in the one and a half ventricle repair group. In intraoperative and postoperative indicators, as one and a half ventricle repair under the collateral circulation, extracorporeal circulation time was slightly longer [(125.51±37.35)min vs. (100.44±25.24)min, P<0.001], and other indicators such as aortic cross-clamp time, endotracheal intubation time, length of hospital stay, and mid-term follow-up results, including mid-term mortality, reoperation rate, cardiac function and valvular regurgitation, there was no significant difference between the two groups. However, the proportion of hospital mortality in half ventricular therapy group was slightly higher, which may be related to the poor right heart function and postoperative recovery difficulties. Conclusion:Good follow-up results have been achieved in the treatment of two surgical therapy. Patients with right ventricular dysplasia and pulmonary artery stenosis should be paid more attention to. Pulmonary artery pressure and pulmonary vascular development should be evaluated before surgery to make preparations for one and a half ventricle repair.

Objective:To improve the understanding of special type of atrioventricular septal defect (AVSD) and reduce clinical missed diagnosis and misdiagnosis by investigating its echocardiographic features.Methods:Echocardiographic images of patients with special type of AVSD diagnosed by echocardiography and confirmed by surgery in Pediatric Cardiovascular Center, Beijing Anzhen Hospital, Capital Medical University from November 2011 to November 2021 were retrospectively analyzed. Depending on the presence of ventricular septal defect(VSD), special type of AVSD were divided into two types, namely AVSD with intact ostium primum and AVSD with intact ostium primum and ventricular septum. The echocardiographic features of special type of AVSD were summarized.Results:Twenty-five cases of special type of AVSD were diagnosed by echocardiography and 13 cases were confirmed by surgery, including 12 cases with intact ostium primum and 1 case with intact ostium primum and ventricular septum. There were 5 cases without surgical indications, and 1 case was kept under observation due to small VSD. One patient with complex malformation only underwent pulmonary artery banding operation without treating intracardiac malformation. The other 4 cases of perimembrane VSD and 1 case of partial AVSD were misdiagnosed as special type of AVSD by echocardiography. All patients had mild to severe left atrioventricular regurgitation before surgery, and most patients remained left atrioventricular regurgitation of varying degrees after left atrioventricular valve formation. Characteristic sonographical findings included: ①The ostium primum was continuous and there was no ostium primum defect. ②The attachment points of the left and right atrioventricular valves were at the same level. ③The left atvioventricular valve was a trileaflet left atrioventricular valve and attached to the crest of the ventricular septum, forming the so-called "anterior mitral valve leaflet cleft". ④The two groups of papillary muscles of the left ventricle changed the normal anterolateral and posterolateral alignment, but showed a anteriorly and posteriorly relationship (counterclockwise transposition). ⑤In the presence of VSD, an inlet ventricular septal defect would be present. ⑥Color Doppler showed varying degrees of regurgitation signals from the left atrioventricular valve during systolic period. ⑦Other complex deformities may be combined with special type of AVSD.Conclusions:Echocardiography can make a definite diagnosis for special types of AVSD. The prognosis of this malformation is closely related to the left atrioventricular valve, and the shaping effect depends on the development of left atrioventricular valve and regurgitation.

Objective:To analyze the characteristics and surgical outcomes of Shone′s syndrome in children, and to explore the surgical treatment strategy and technical key.Methods:Retrospective study.Children with Shone′s syndrome treated in the Pediatric Heart Center, Beijing Anzhen Hospital, Capital Medical University from May 2013 to June 2019 were retrospectively analyzed for their baseline characteristics and surgical data.The data were compared by Student t-test. Results:A total of 22 children with Shone′s syndrome were recruited, involving 15 males (68.2%) and 7 females (31.8%). There were 6 cases (27.3%) of complete form of Shone′s syndrome and 16 cases (72.7%) of incomplete form.No deaths were reported.The postoperative mitral valve velocity [(149.7±38.2) cm/s vs.(234.9±34.0) cm/s, t=7.341, P<0.05], left ventricular outflow tract velocity [(202.0±105.0) cm/s vs.(328.6±120.3) cm/s, t=6.575, P<0.05] and aortic arch coarctation velocity [(186.1±60.9) cm/s vs.(347.9±100.8) cm/s, t=7.630, P<0.05]were significantly lower than those of preoperative levels.There were no complications occurred at 1-year follow-up, and 91.7% of the patients were followed up for 3 years, and 80.2% were followed up for 5 years without complications, 2 cases needed reoperation. Conclusions:Surgical treatment of Shone′s syndrome achieved satisfactory outcomes.Early diagnosis and early intervention are beneficial to children with Shone′s syndrome, although they need to be followed up and have the risk of reoperation in the long term.

Objective:Analyze the effect of intracardiac method and upturning method in the treatment of infracardiac total anomalous pulmonary venous connection(TAPVC), to explore the surgical method of infracardiac TAPVC.Methods:From July 2011 to August 2019, 20 patients with infracardiac TAPVC were treated, including 12 cases with upturning method and 8 cases with intracardiac method. The cardiopulmonary bypass time, aortic cross-clamp time, delayed thoracic closure, ICU time, mechanical ventilation time, postoperative days and anastomotic flow rate were compared between the two groups.Results:There was no significant difference in cardiopulmonary bypass time, aortic cross-clamp time, delayed thoracic closure, ICU time and mechanical ventilation time between the two groups. The postoperative hospital stay in upturning group was significantly lower than that in intracardiac group [(14.7±2.9)days vs.(16.1±6.2)days, P<0.05], and the postoperative anastomotic velocity > 120 cm/s in intracardiac group was significantly less than that in upturning group(1 case vs. 7 cases, P<0.05). Two patients died in upturning group, but there was no significant difference compared with the intracardiac group. Conclusion:There is no significant difference between the two methods in the treatment of subcardiac TAPVC. The authors think that the exposure of the upturning methods is difficult, and the distortion of the anastomosis may be hidden trouble. The in situ anastomosis of the intracardiac method is not easy to make mistakes.

Objective    To analyze the clinical characteristics and surgical treatment results of cardiac tumors. Methods    Clinical data of 28 patients with cardiac tumors, who underwent cardiac surgery in our hospital from 2013 to 2019 were retrospectively reviewed. There were 12 males and 16 females. The median age was 11.5 years, ranging 1 month to 69 years; and the median weight was 39.0 (4.8-100.0) kg. All patients underwent tumor resection under cardio-pulmonary bypass with general anesthesia, and postoperative pathological analysis was performed. Results    There were 27 patients with primary cardiac tumors and 1 patient with secondary cardiac tumor. Among the primary cardiac tumors, there were 16 myxomas, 5 rhabdomyomas, 3 inflammatory myofibroblastomas, 2 fibroids and 2 hemangiomas. The secondary cardiac tumor was chondrosarcoma. A total of 28 cardiac tumors occurred in 29 locations, including 14 in the left atrium, 3 in the right atrium, 1 in the left ventricle, 7 in the right ventricle, 2 in the mitral and 2 in the tricuspid. Cerebral embolism occurred in 6 patients, peripheral vascular embolism in 3 patients, and syncope in 3 patients. There was no death during the perioperative period and in the long term follow-up. Complete resection was performed in 25 patients and partial resection was performed in 3 patients. During the follow-up, 2 patients with myxoma received reoperation for recurrence. Among the 3 patients with partial resection, residual tumor tissue disappeared in 2 patients, and in the other patient, there was no change in the size of the residual tumor tissue during the follow-up compared to that at discharge. Conclusion    Cardiac surgery is an effective method for patients with primary benign cardiac tumors. However, whether the cardiac tumor tissue can be completely removed depends on the anatomical location of the tumor during the operation. If there are systemic embolism events and syncope, the possibility of cardiac tumors should be considered.

Objective    To evaluate the mid-term results of surgical treatment for functional single ventricle associated with total anomalous pulmonary venous connection. Methods    We reviewed the clinical data and follow-up results of 12 patients in our hospital who underwent both single ventricle series palliation and total anomalous pulmonary venous connection correction from 2008 to 2018. There were 6 males and 6 females at age of 2.3 (1-21) years. Univariable and multivariable Cox proportional hazard regression methods were performed. The Kaplan-Meier method was used to estimate the survival rate. Results    All patients were successfully removed from extracorporeal circulation. The cardiopulmonary bypass time was 113.8±42.5 min. The myocardial block time was 57.7±31.7 min. There were 3 in-hospital deaths and 4 late deaths. The causes of death in hospital were pulmonary hypertension, pulmonary hemorrhage, and hypoxemia. During follow-up, the cause of death was heart failure. The survival rate at 1 year and 3 years was 58.3%(95%CI 27% to 80%) and 40% (95%CI 13% to 65%), respectively. Median follow-up was 48.3 months (range: 1 to 118 months). Conclusion    Functional single ventricle combined with complete pulmonary venous drainage is a serious clinical disease, with poor therapeutic effect and high overall mortality.

Non-coding RNA refers to a class of RNAs that cannot encode proteins, and they play a very important role in regulating cellular activities. Pulmonary arteryhypertension is a group of diseases characterized by progressive elevation of pulmonary vascular pressure. Its pathogenesis is complex and its influencing factors are numerous. The study found that non-coding RNA, as a transcription product that does not participate in translational functions, plays an important role in the pathogenesis of patients with pulmonary arteryhypertension. With regard to the more well-studied and relatively mature circRNAs, lncRNAs, and miRNAs in non-coding RNAs, this article review the pathophysiological processes involved in the formation of pulmonary arteryhypertension.

Objective    To explore the anatomical characteristics and surgical strategies of atrioventricular septal defect (AVSD) with left ventricular outflow tract (LVOT) stenosis. Methods    The clinical data of 11 AVSD patients with LVOT stenosis who underwent surgeries in our hospital from 2008 to 2019 were retrospectively analyzed, including 6 males and 5 females with a median age of 15.0 (7.6-22.0) years. Results    There were 3 patients of complete AVSD and 8 patients of partial AVSD. Subaortic stenosis resulted from discrete subaortic membrane in 3 patients, diffused subaortic membrane in 4 patients, hypertrophied muscle bundles in 3 patients and distorted valve frame in 1 patient. Among these patients, 5 patients underwent LVOT stenosis and AVSD repairs simultaneously for the first time, 5 patients underwent LVOT stenosis repair for the second time and 1 patient for the third time. No postoperative death occurred. The postoperative LVOT flow velocity decreased dramatically after LVOT stenosis repair compared with preoperative one [449.0 (393.0, 507.5) cm/s vs. 212.0 (183.0, 253.5) cm/s, P<0.05]. Conclusion    Surgical results of AVSD combined with LVOT stenosis are satisfactory, but the restenosis should be paid attention to via long-term follow-up.

Objective:The study aimed to evaluate the effect of systemic-pulmonary shunt(SPS) on the infants with tetralogy of Fallot(TOF) with hypoplasia pulmonary arteries.Methods:Among 949 infants less than three-year-old who underwent surgical intervention between January 2010 and December 2015, 853 infants underwent one-stage primary repair(group Ⅰ), whereas 96 infants underwent SPS(group Ⅱ). Among them, 546 were males and 403 were females. The average age and average weight at operation were 13 months and 9.4 kg, respectively. 20.8% infants had clinical symptoms before operation. Perioperative and follow-up parameters were assessed including age, weight, sex, transcutaneous oxygen saturation(SPO 2), preoperative clinic symptom, ratio of McGoon, NAKATA index, Z score of pulmonary annulus, left ventricular end diastolic diameter(LVEDD). Results:There were 28(3.0%) early postoperative deaths in the whole group. And no significant difference in mortality was found between group Ⅰ(2.9%) and group Ⅱ(3.1%). Compared with group Ⅰ, age and weight were significantly lower in patients in group Ⅱ[(0.95±0.56)years old vs.(1.09±0.59)years old] and[(8.6±1.8)kg vs.(9.5±2.1)kg], respectively. Patients in group Ⅱ had a lower SPO 2(0.75±0.09 vs. 0.85±0.09) and was more prone to appear hypoxic spell(30.2% vs. 19.7%) than patients in group Ⅰ. Patients in group Ⅱ had a more poor development in pulmonary artery. The ratio of McGoon, NAKATA index and Z score of pulmonary annulus in group Ⅱ were significantly less than that in group Ⅰ[1.4±0.3 vs. 1.9±0.5, (124±43)mm 2/m 2 vs.(222±88)mm 2/m 2 and-4.3±2.6 vs.-2.3±2.1], respectively. There was no difference of extubation time bewteen two groups. SPO 2 rose significantly from 0.75 to 0.83 after SPS. And after SPS, the diameter of main pulmonary artery, the ratio of McGoon, NAKATA index and Z score of pulmonary annulus increased significantly from 6.4 mm, 1.42, 126.4 mm 2/m 2 and -4.3 to 9.2 mm, 1.83, 212.6 mm 2/m 2 and -2.4, respectively. Moreover, LVEDD also increased significantly from 21.2 mm to 24.5 mm after SPS. Conclusion:SPS is safe and effective for little infants with anoxic spell and hypoplasia pulmonary arteries. SPS can improve oxygenation significantly and delay the age for radical surgery. SPS also promote the growth of the pulmonary arteries, pulmonary valve annulus and left ventricular, and it facilitates the preservation of pulmonary valve annulus and reduce the rate of transannular patching in a portion of infants.

Objective:This study aims to review two surgical techniques results of the reoperation for left atrioventricular valve(LAVV) failure in repaired atrioventricular septal defect.Methods:All consecutive patients with repaired atrioventricular septal defect(AVSD) who underwent redo-LAVV surgery from 2005 to 2019 were included. Patients with single ventricles, banding, atrial isomerism, and complex associated anomalies were excluded. Univariate analysis included repair and replacement. Data analyzed included number and year of primary AVSD and redo-LAVV operation, morphology of AVSD, mortality, and reoperation, early and long-term survival.Results:There were a total of 28 patients including 7 boys(25%) with age of 114.5 months(63.0-194.5 months). The mean body weight was 28.55 kg(15.5-55.9 kg). There were 11(39.3%) patients with complete AVSD and 17(60.7%) with partial AVSD, and 21(75%) patients with LAVV valvuloplasty(LAVVP), 7(25%) patients with LAVV replacement(LAVVR). The aortic cross clamp time was significantly longer in patients who underwent LAVVR compared to LAVVP[LAVVR 94 min(79-107)min vs. LAVVP 66 min(45-83 min), P<0.05]. The technique of cleft closure with LAVVP 18(87.5%) more than LAVVR 2(28.6%), P<0.05. Conclusion:The postoperative left atrio-ventricular valve(LAVV) regurgitation is the main reason of the reoperation. The technique of cleft closure with LVVR-Repair is favorable. At follow-up, survivors with re-LVVR-Repair have high rates with numerous operations.