Congenital heart disease(CHD) ranks the first in birth defects.Some children with CHD have other non-cardiac malformations, and the perioperative mortality and adverse events are higher in children with CHD.One of the main causes of this problem is that the risk assessment system and treatment plan for CHD with non-cardiac malformations are not perfect.This article summarizes the progress and epidemiology, diagnosis and treatment by reading the literature related to this disease, stratifies the severity of the disease, and summarizes the latest surgical program, and provides evidence for the treatment of patients with CHD and non-cardiac malformations.

Objective:To explore the safety and feasibility of the simultaneous combined operation in children with congenital heart disease complicated with non-cardiac malformation.Methods:A total of 72 children undergoing combined surgery or simple heart surgery in the Department of Cardiac Surgery, Children′s Hospital, Capital Institute of Pediatrics from January 2017 to January 2019 were enrolled.According to the severity of the disease, patients in the combined operation group (group A) and the simple heart surgery group (group B) were separately subdivided into the low risk group (group L) and the high risk group (group H). There were 36 children in group A, with the age ranging from 1.5 to 168.0 months old (median: 18.0 months). There were 36 cases in group B, with the age ranging from 1.0 to 170.0 months old (median: 19.0 months). Patients in groups A and B were sent to the cardiac intensive care unit(ICU) after operation.The cardiopulmonary bypass (CPB) time, aortic clamping (ACC) time, tracheal intubation time, intensive care unit (ICU) retention time, brain natriuretic peptide (BNP), alanine aminotransferase (ALT) and creatinine (Cr) were recorded.Besides, the cardiac output index (CI), cardiac circulation efficiency (CCE), maximum pressure gradient (dp/dt), lactic acid (Lac), blood glucose (Glu), inotropic score (IS) were also recorded at the time of returning to ICU (T0), 4 hours after operation (T1), 8 hours after operation (T2), 12 hours after operation (T3), 24 hours after operation (T4) and 48 hours after operation (T5), respectively.Results:(1) Intra-group comparison in group A: the age [(39.9±37.0) months], height [(94.1±20.1) cm] and weight [(14.4±6.7) kg] of children at low risk (group L-A) were significantly higher than those at high risk (group H-A) [(7.5±3.7) months, (68.1±6.4) cm, (7.8±2.2) kg] (all P<0.01). The CPB time [(37.0±23.6) min], ACC time [(19.1±13.4) min], endotracheal intubation time [(7.1±4.7) h], ICU retention time [(1.1±0.3) d] and BNP 24 hours after operation [(2 257.3±952.0) ng/L] in group L-A were significantly lower than those in group H-A [(84.7±28.4) min, (41.9±30.7) min, (71.0±67.6) h, (8.7±5.7) d and (5 327.2±992.9) ng/L] (all P<0.01). Glu, IS, CI, CCE were significantly different between patients at low risk and patients at high risk ( P<0.05). At the time of T0-T5, the Glu( F=4.43, P<0.05) and IS ( F=26.99, P<0.01)of group L-A were lower than those of group H-A, and the CI ( F=18.39, P<0.01)and CCE ( F=5.28, P<0.05) of group L-A were higher than those of group H-A.(2) Comparison between groups A and B: there was no significant difference in age, height, weight, CPB time, ACC time, hemodynamic parameters, arterial blood gas parameters and postoperative clinical indexes between patients at high risk or patients at low risk in group A and group B (all P>0.05). Conclusions:(1) For the patients at low risk, hemodynamics remains stable after the combined operation.The combined operation does not increase the endotracheal intubation time and ICU retention time, so it is safe and feasible.(2) For the patients at high risk, hemodynamics is also stable after the combined operation.However, their IS is higher than that of patients at low risk at any time point, and the incidence of postoperative adverse events is higher than that of patients at low risk.It is necessary to evaluate the condition and operation plan of the children before operation.

Objective:To explore the tricuspid annular plane systolic excusion(TAPSE) in children with left-to-right shunt after congenital heart disease surgery and to understand the early systolic function of right heart in thesepatient.Methods:From June 2018 to December 2018, a prospective study was conducted in 20 infants after repair of left-to-right shunt congenital heart disease, including 10 males(50%) and 10 females(50%) , aged from 1 to 12 months, with a median of 4.5(2.0, 6.8) months, a body mass of 3.0-9.0 kg with median of 6.0(3.7, 7.7) kg.On the first postoperative day, blood was taken from central venous for N-terminal pro-B-type natriuretic peptide(NT pro-BNP) test, TAPSE and left ventricular ejective fraction(LVEF) was measured by echocardiography.The effects of aortic occlusion time, cardiopulmonary bypass time, preoperative pneumonia and preoperative heart failure on TAPSE were compared. The relationship between TAPSE and heart rate, systolic pressure, central venous pressure, vasoactive drug score, endotracheal intubation time, detention time in intensive care unit, NT pro-BNP and LVEF after operation was analysed.Results:The aortic cross-clamping time was 15-87 minutes, with median 31(28, 50) minutes. The cardiopulmonary bypass time was 35-117 minutes, with an average of(68±22)minutes. The time of tracheal intubation was 4-117 hours, with an average of(50±35) hours. The stay time in CICU was 1-14 days, with a median of 5(2, 7) days.The LVEF was 0.18-0.66, with median 0.53(0.42, 0.57). The TAPSE was 2.0-10.0 mm, with an average of(5.2±2.0) mm. On the first day after operation, NT pro-BNP was 1 548-35 000 pg/ml, with an average of(9 446±8 130) pg/ml.TAPSE was negatively correlated with postoperative intubation time( r=-0.576, P= 0.007) and detention time in ICU( r=-0.765, P=0.000), and positively correlated with postoperative LVEF( r=0.461, P=0.041)( P<0.05). TAPSE was negatively correlated with heart rate( r=-0.303, P=0.193), central venous pressure( r=-0.425, P=0.062), vasoactive drug score( r=-0.418, P=0.067) and NT Pro BNP( r=-0.348, P=0.132), and positively correlated with systolic pressure( r=0.146, P=0.54), but there was no statistical significance in each item.Compared with patients with TAPSE≥5mm, the detention time and tracheal intubation time were longer than those TAPSE<5 mm, the central venous pressure and NT-pro BNP was higher than those TAPSE<5 mm( P<0.05), the difference was statistically significant, other indicators had no significant difference. Conclusion:It is simple and feasible to measure TAPSE by echocardiography in children after operation with left-to-right shunt congenital heart disease.TAPSE decreased postoperatively suggested that the function of right ventricle decreased at the early stage after surgery, and with left ventricle systolic function decreased, which eventually led to the increase of NT pro-BNP, the need for higher doses of vasoactive drug support, longer tracheal intubation time and the stay time in CICU.Attention should be paid to the right heart function of children after congenital heart surgery.

Objective:To explore the feasibility of simultaneous combined operation for congenital heart disease with other malformations and to summarize the experience of operation and the ways to avoid risks.Methods:From May 2015 to December 2019, the clinical data of 44 children undergoing simultaneous combined operation in our hospital were collected, and the data of 44 children who were matched with the children undergoing combined operation in the same period were collected as the research objects, which were divided into high-risk group(17 cases)and low-risk group(27 cases). To compare and analyze the early hemodynamic indexes and other hospitalization indexes of different groups of children undergoing simultaneous operation and staged operation, so as to evaluate whether the scheme of simultaneous combined operation is more optimized.Results:All the children in the same period successfully underwent combined operation, among which 4 cases had postoperative complications and 1 case died out of hospital, all of them were children in high-risk group, and the other children were cured and discharged. Compared with the staging group, there was no significant difference in general data and early postoperative hemodynamic indexes of children in the same period group, but the cost of operation and anesthesia was lower, and the average hospitalization time was shortened by about 5 days for each person, with statistical significance. Compared with the low-risk group, the children in the high-risk group were significantly lower in age and weight, complicated in deformity, longer in operation time, lower in early postoperative cardiac output, stable in hemodynamics after operation, but higher in inotropicscore score(IS). Postoperative endotracheal intubation time, ICU time and overall hospitalization time were prolonged, and the overall cost was more( P<0.05) The incidence of postoperative adverse events was higher. Conclusion:Simultaneous combined operation for children with congenital heart disease with other malformations is generally safe and feasible., Staging is safe for children in high-risk group, and if simultaneous combined surgery is unavoidable, the condition must be assessed individually and a detailed treatment plan must be developed to avoid surgical risks.