Right ventricular dilatation score: a new assessment to right ventricular dilatation in adult patients with repaired tetralogy of Fallot.

BACKGROUND: Patients with repaired tetralogy of Fallot (rTOF) experience long-term chronic pulmonary valve regurgitation resulting in right ventricular (RV) dilatation. According to current guidelines, the evaluation of patients with rTOF for RV dilatation should be based on cardiac magnetic resonance (CMR). However, for many asymptomatic patients, routine CMR is not practical. Our study aims to identify screening methods for CMR based on echocardiographic data, with the goal of establishing a more practical and cheap method of screening for severity of RV dilatation in patients with asymptomatic rTOF. METHODS: Thirty two rTOF patients (mean age, 21(10.5) y, 21 males) with moderate to severe pulmonary regurgitation (PR) were prospectively recruited. Each patient received CMR and echocardiogram examination within 1 month prior to operation and collected clinical data, and then received echocardiogram examination at discharge and 3-6 months post-surgery. RESULTS: RV moderate-severe dilatation was defined as right ventricular end-diastolic volume index (RVEDVI) ≥ 160 ml/m2 or right ventricular end-systolic volume index (RVESVI) ≥ 80 ml/m2 in 15 of 32 patients (RVEDVI, 202.15[171.51, 252.56] ml/m2, RVESVI, 111.99 [96.28, 171.74] ml/m2). The other 17 (RVESDI, 130.19 [117.91, 139.35] ml/m2, RVESVI = 67.91 [63.35, 73.11] ml/m2) were defined as right ventricle mild dilatation, i.e., RVEDVI < 160 ml/m2 and RVESVI < 80 ml/m2, and the two parameters were higher than normal values. Compared with the RV mild dilatation group, patients of RV moderate-severe dilatation have worse cardiac function before surgery (right ventricular ejection fraction, 38.92(9.19) % versus 48.31(5.53) %, p < 0.001; Left ventricular ejection fraction, 59.80(10.26) versus 66.41(4.15), p = 0.021). Patients with RV moderate-severe dilatation faced longer operation time and more blood transfusion during operation (operation time, 271.53(08.33) min versus 170.53(72.36) min, p < 0.01; Intraoperative blood transfusion, 200(175) ml versus 100(50) ml, p = 0.001). Postoperative RV moderate-severe dilatation patients have poor short-term prognosis, which was reflected in a longer postoperative hospital stay (6.59 [2.12] days versus 9.80 [5.10] days, p = 0.024) and a higher incidence of hypohepatia (0[0] % versus 4[26.7] %, p = 0.023). Patients with RV dilatation score > 2.35 were diagnosed with RV moderate-severe dilatation (AUC = 0,882; Sensitivity = 94.1%; Specificity = 77.3%). CONCLUSIONS: RV moderate-severe dilatation is associated with worse preoperative cardiac function and short-term prognosis after PVR in rTOF patients with moderate to severe PR. The RV dilatation score is an effective screening method. When RV dilatation score > 2.35, the patient is indicated for further CMR examination and treatment.

Pulmonary artery reconstruction and correction of tetralogy of Fallot combined with the absence of the mediastinal left pulmonary artery.

OBJECTIVES: Tetralogy of Fallot (TOF) is the most common deformity combined with the unilateral absence of the mediastinal pulmonary artery (UAMPA), and its treatment strategy remains controversial. In this study, we analyzed the effect of bilateral pulmonary reconstruction in patients with TOF combined with UAMPA. METHODS: This was a single-center, retrospective review of 1713 patients with TOF between January 2009 and November 2021. Overall, eight patients were diagnosed with TOF combined with UAMPA. Among them, seven underwent surgery: three underwent one-stage TOF correction with bilateral pulmonary artery reconstruction; three patients underwent bilateral pulmonary artery reconstruction, followed by two-stage TOF correction after several months; and one patient underwent two procedures of left pulmonary artery reconstruction, and the ventral septal defect remained open. The left pulmonary arteries were reconstructed with a Goretex conduit in three cases, direct anastomosis in two cases, and the modified autologous tissue extension technique in two cases. RESULTS: All seven patients survived during the postoperative follow-up and showed good cardiac function and normal oxygen saturation of >97%. During follow-up echocardiography, we noted that the left pulmonary arteries reconstructed with a Goretex conduit or direct anastomosis had thrombosis or stenosis. However, those reconstructed using the modified autologous tissue extension technique was unobstructed. CONCLUSIONS: In patients with TOF and UAMPA, if there is a pulmonary artery confluence in the affected hilum, it is feasible to implement bilateral pulmonary artery reconstruction for one-stage TOF correction. The use of the pulmonary artery extension technique and autologous tissue for bilateral pulmonary reconstruction could reduce the incidence of anastomotic stenosis.

Characteristics of in-hospital mortality of congenital heart disease (CHD) after surgical treatment in children from 2005 to 2017: a single-center experience.

BACKGROUND: To evaluate trends in the in-hospital mortality rate for pediatric cardiac surgery procedures between 2005 and 2017 in our center, and to discuss the mortality characteristics of children's CHD after thoracotomy. METHODS: This retrospective data were collected from medical records of children underwent CHD surgery between 2005 and 2017. RESULTS: A total of 19,114 children with CHD underwent surgery and 444 children died, with the in-hospital mortality was 2.3%. Complex mixed defect CHD had the highest fatality rate (8.63%), left obstructive lesion CHD had the second highest fatality rate (4.49%), right to left shunt CHD had the third highest mortality rate (3.51%), left to right shunt CHD had the lowest mortality rate (χ2 = 520.3,P < 0.05). The neonatal period has the highest mortality rate (12.17%), followed by infant mortality (2.58%), toddler age mortality (1.16%), and preschool age mortality (0.94%), the school age and adolescent mortality rate was the lowest (χ2 = 529.3,P < 0.05). In addition, the fatality rate in boys was significantly higher than that in girls (2.77% versus 1.62%, χ2 = 26.4, P < 0.05). CONCLUSIONS: The mortality rate of CHD surgery in children decreased year by year. The younger the age and the more complicated the cyanotic heart disease, the higher the mortality rate may be.

Three-Dimensional Printing, Virtual Reality and Mixed Reality for Pulmonary Atresia: Early Surgical Outcomes Evaluation.

BACKGROUND: Single-stage unifocalisation for pulmonary atresia (PA) with ventricular septal defect (VSD) and major aortopulmonary collateral arteries (MAPCA) requires a high degree of three-dimensional (3D) anatomical imagination. A previous study has reported the application of a 3D-printed heart model with virtual reality (VR) or mixed reality (MR). However, few studies have evaluated the surgical outcomes of the 3D model with VR or MR in PA/VSD patients. METHODS: Three-dimensional (3D) heart models of five selected PA/VSD patients were derived from traditional imageology of their hearts. Using VR glasses, the 3D models were also visualised in the operating room. Both the 3D-printed heart models and preoperative evaluation by VR were used in the five selected patients for surgical simulation and better anatomical understanding. Mixed reality holograms were used as perioperative assistive tools. Surgical outcomes were assessed, including in-hospital and early follow-up clinical data. RESULTS: The use of these three new technologies had favourable feedback from the surgeons on intraoperative judgment. There were no in-hospital or early deaths. No reintervention was required until the last follow-up. Three (3) patients developed postoperative complications: one had right bundle branch block and ST-segment change, one had chest drainage >7 days (>40 mL/day) and one had pneumonia. CONCLUSION: The preoperative application of a 3D-printed heart model with VR or MR helped in aligning the surgical field. These technologies improved the understanding of complicated cardiac anatomy and achieved acceptable surgical outcomes as guiding surgical planning.

Early and midterm outcomes of a modified single-patch technique for repairing complete atrioventricular septal defect in children and adults.

OBJECTIVES: The "modified single-patch" technique (Nunn's technique) has become more widely adopted, especially in small infants with common atrioventricular septal defect (CAVSD). In this study, we retrospectively reviewed our surgical experiences of the "modified single-patch" technique with CAVSD patients, including newborns and adults, to reveal the early and midterm clinical results. METHODS: There were 233 cases diagnosed with CAVSD undergoing biventricular repair in our hospital from June 2009 to 2016. A total of 197 cases (84.5%) with the "modified single-patch" technique were enrolled in the final analysis, including type A in 106 cases (53.8%), type B in 13 cases (6.6%), type C in 15 cases (7.6%), and intermediate type in 63 cases (32%). Major associated cardiovascular malformations included five cases of unroofed coronary sinus syndrome with LSVC (UCS), five cases of coarctation of the aorta and one case of translocation of the great artery. The median age at operation of the studied 197 cases was 44.3 ± 103.3 months (ranging from 18 days to 58 years old), including two newborns. Detailed information on the "modified single-patch" procedure was described previously, and the other cardiac malformations were repaired simultaneously with surgical repair. RESULTS: There were 14 (7.1%) early deaths and 3 (1.5%) late deaths at 3 months and 1 year after the initial operation, respectively. The total mortality rate was 8.1%, and there was no significant difference in mortality among the different age groups (P = .291). Five patients (2.5%) underwent reoperation because of severe mitral insufficiency (MI), and another patient was reoperated for left ventricular outflow tract obstruction 3 years after the initial surgery. Long-term survival in the <3 months group was significantly lower than that in the group of older children (1-18 years old). Of the 180 survivors who were followed up for at least 1 year, the proportion of severe MI began to drop from 26.2% before the operation to 13.8% just 1-week postoperation and continued to decline to the lowest level, 3.4%, at 3 months postoperation. However, MI began to rebound at 3 months and reached 20.8% at 1-year postoperation. The rebound of MI was evident in the (3 months, 6 months) age group and the >18 years age group. However, compared to the preoperation situation, the proportion of severe tricuspid insufficiencies decreased each month from 24.8% to 0.0% until 3 months after the operation and remained at a relatively low level within 1 year after surgery. CONCLUSIONS: In our experience, age is not a limitation of the modified single-patch technique, and patients even in adults with surgical indications can apply this technique. In older children and adults, we should pay more attention to atrioventricular valve annuloplasty. Infants may suffer from severe residual left atrioventricular valve regurgitation, especially in the <3 months age group, so we should improve postoperative follow-up work during infancy.

Value of pulmonary annulus area index in predicting transannular patch placement in tetralogy of Fallot repair.

BACKGROUND: Precisely evaluating the need for transannular patch (TAP) placement is very important in the surgical treatment of tetralogy of Fallot. We hypothesized that the pulmonary annulus area index (PAAI, the pulmonary-to-aortic valve annulus cross-sectional area ratio) could be a useful and accessible predictor for TAP placement. METHODS: The medical records of patients who underwent tetralogy of Fallot repair between 1 January 2016 and 31 December 2017 were reviewed retrospectively. A total of 255 patients were included and categorized into two groups: patients who needed TAP placement and patients who did not. Various candidate predictors for TAP placement (PAAI, pulmonary annulus z-score, and velocity across the pulmonary annulus) were compared using receiver operating characteristic curves. The optimal cutoff for each predictor was assessed. RESULTS: Among the 255 patients included, 156 needed TAP placement (156/237, 65.8%). Both the PAAI (0.28 [0.20/0.34] vs 0.14 [0.09/0.19]; P < .0001) and z-score (-1.5 [-2.9, -0.4] vs -3.6 [-5.3/-2.6]; P < .0001) were smaller in the TAP group. The PAAI is a useful predictor of the pulmonary annulus z-score (AUC 0.830 vs 0.811, P = .19). Combination analysis of the PAAI and velocity across the pulmonary annulus (PV vmax ) showed better predictive value than the PAAI and z-score (AUC 0.860, sensitivity 89.7%, specificity 61.7%, P < .0001). CONCLUSIONS: Our results suggest that the PAAI is a useful and accessible predictor for TAP placement and can be applied readily and simply in clinical practice. A combination with the velocity across the pulmonary annulus could promote the accuracy of prediction.

Results of surgery on adults with functional single ventricle without prior cardiac surgery in childhood.

OBJECTIVES: Patients with unoperated functional single ventricle (FSV) rarely survive into adulthood with good functional status and there are few reports about surgical results of adult patients with FSV. This study retrospectively reviews our experience with surgery in adult patients with FSV. METHODS: From January 2008 to September 2017, 65 adult patients with FSV underwent surgery in our hospital. Twenty underwent Blalock-Taussig shunt or bidirectional Glenn procedures in other hospitals prior, and four were lost to follow-up. Finally, 41 patients were included in this study. RESULTS: The early postoperative mortality was 7.3% (3/41). Postoperative systemic arterial oxygen saturation (SpO2 , 83.7% ± 4.8%) was significantly higher than preoperative SpO2 (77.9% ± 10.1%, P < .01). The mean follow-up time was 3.9 ± 3.1 years (range 1-11 years). There was only one case of late mortality and massive hemoptysis was the cause of death. Right ventricular morphology and severe atrioventricular valve regurgitation (AVVR) were the risk factors of postoperative death for patients with pulmonary stenosis(PS). There was no death case in patients who received Fontan procedure. In a follow-up, we found the grade of AVVR was reduced and the grade of heart function (New York Heart Association) was improved. CONCLUSIONS: Adult patients with FSV can also undergo surgery. The mortality was acceptable and late results were satisfactory. After surgery, oxygen saturations increased, grades of AVVR decreased and the heart functions improved. Right ventricular morphology and severe AVVR were risk factors for patients with PS.

Perinatal and early postnatal outcomes for fetuses with prenatally diagnosed d-transposition of the great arteries: a prospective cohort study assessing the effect of standardised prenatal consultation.

BACKGROUND: The aim of this study was to explore perinatal and early postnatal outcomes in fetuses with prenatally diagnosed d-transposition of the great arteries and impacts of standardised prenatal consultation. METHODS: All fetuses with prenatally diagnosed d-transposition of the great arteries prospectively enrolled at South China cardiac centre from 2011 to 2015. Standardised prenatal consultation was introduced in 2013 and comprehensive measures were implemented, such as establishing fetal CHD Outpatient Consultation Service, performing standard prenatal consultation according to specifications, and establishing a multidisciplinary team with senior specialists performing in-person consultations. Continuous follow-up investigation was conducted. Perinatal and postnatal outcomes were compared before and after consultation including live birth, elective termination of pregnancy, spontaneous fetal death, stillbirths, referral for surgery, and survival. RESULTS: In all, 146 fetuses were enrolled with 41 (28%) lost to follow-up. Among 105 remaining fetuses, 29 (28%) were live births and 76 (72%) were terminated. After consultation, live birth rate was higher (50 versus 33%) and termination rate was lower (50 versus 76%), although there was no statistical significance. Excluding three live births without postnatal d-transposition of the great arteries, 65% (17/26) underwent arterial switch operation within 30 days. A total of three in-hospital deaths occurred and during the 10-month follow-up period, one death was observed. In one case, the switch procedure was performed at 13 months and the infant survived. Out of eight infants without arterial switch operation, two died. CONCLUSIONS: Live birth rate increased after consultation; however, termination remained high. Combining termination, patients without arterial switch operation, and operative mortality, outcomes of d-transposition of the great arteries infants can be improved. Standard consultation, multidisciplinary collaboration, and improved perinatal care are important to improve outcomes.

Risk Factors of Chylothorax After Congenital Heart Surgery in Infants: A Single-Centre Retrospective Study.

Background: Studies of chylothorax after congenital heart disease in infants are rare. Chylothorax has a higher incidence in infancy, but its risk factors are not well understood. Objective: The purpose of this study is to investigate the risk factors of chylothorax after congenital heart surgery in infants. Methods: This retrospective study included 176 infants who underwent congenital heart disease surgery at the Guangdong Cardiovascular Institute, China, between 2016 and 2020. According to the occurrence of chylothorax, the patients were divided into a control group (n = 88) and a case group (n = 88). Univariate and multivariate logistic regression were performed to analyse the incidence and influencing factors of chylothorax after congenital heart surgery in infants. Results: Between 2016 and 2020, the annual incidence rate fluctuated between 1.55% and 3.17%, and the total incidence of chylothorax was 2.02%. Multivariate logistic regression analysis showed that postoperative albumin (p = 0.041; odds ratio [OR] = 0.095), preoperative mechanical ventilation (p = 0.001; OR = 1.053) and preterm birth (p = 0.002; OR = 5.783) were risk factors for postoperative chylothorax in infants with congenital heart disease. Conclusion: The total incidence of chylothorax was 2.02% and the annual incidence rate fluctuated between 1.55% and 3.17% between 2016 and 2020. Premature infants, longer preoperative mechanical ventilation and lower albumin after congenital heart surgery may be risk factors for chylothorax. In addition, infants with chylothorax are inclined to be infected, need more respiratory support, use a chest drainage tube for longer and remain longer in hospital.

Outcome of surgical repair of anomalous left coronary artery from the pulmonary artery in a single-center experience.

BACKGROUND: This study aims to review the midterm results of surgical repair of anomalous left coronary artery from the pulmonary artery (ALCAPA) in our center and assess the postoperative cardiac function recovery and misdiagnosis. METHOD: Patients who underwent repair of ALCAPA between January 2005 and January 2022 at our hospital were retrospectively reviewed. RESULTS: A total of 136 patients underwent repair of ALCAPA, among which 49.3% were misdiagnosed before referral to our hospital. On multivariable logistic regression analysis, patients with low left ventricular ejection fraction (LVEF) (odds ratio = 0.975, p = 0.018) were at increased risk for misdiagnosis. The median age at surgery was 0.83 years (range, 0.08 to 56 years), and the median LVEF was 52% (range, 5% to 86%). The mortality rate was 6.6% (n = 9), and four patients underwent reintervention. The median postoperative recovery time of left ventricular function (LVF) was 10 days (1 to 692 days). Competing risk analysis revealed that a low preoperative LVEF (hazard ratio = 1.067, p < 0.001) and age younger than 1 year (hazard ratio = 0.522, p = 0.007) were risk factors for a longer postoperative recovery time of LVF. During the follow-up period, 91.9% (113/123) of the patients had no aggravation of mitral regurgitation. CONCLUSION: The perioperative and intermediate outcomes after ALCAPA repair were favorable, but preoperative misdiagnosis deserved attention, especially in patients with low LVEF. LVF normalized in most patients, but patients younger than 1 year and with low LVEF required longer recovery times.

Supracardiac total anomalous pulmonary venous connection type Ib: Morphology and outcomes.

BACKGROUND: Supracardiac total anomalous pulmonary venous connection is the most common subtype of total anomalous pulmonary venous connection. We aimed to describe the morphological spectrum of supracardiac total anomalous pulmonary venous connection and to identify risk factors for death and postoperative pulmonary venous obstruction. METHODS: From February 2009 to June 2019, 241 patients diagnosed with supracardiac-Ia (left-sided vertical vein, n = 185) or supracardiac-Ib (right-sided connection directly to superior vena cava, n = 56) total anomalous pulmonary venous connection underwent initial surgical repair at our institute. Cases with functionally univentricular circulations or atrial isomerism were excluded. Patients' postoperative survival was described by Kaplan-Meier curves. Cox proportional hazards models and competing risk regression models were used to identify clinical risk factors for death and postoperative pulmonary venous obstruction. RESULTS: There were 8 early deaths and 4 late deaths. The overall survivals at 30 days, 1 year, and 10 years were 97.1%, 94.8%, and 94.8%, respectively, in the supracardiac-Ia group (2.7%, 5/185) (hazard ratio, 4.8; P = .003). Five patients required reoperation for pulmonary venous obstruction, including 2 patients who required reintervention for superior vena cava syndromes (all in the supracardiac-Ib group). One patient required superior vena cava balloon dilation for superior vena cava syndromes. Multivariable analysis showed that the supracardiac-Ib group (12.5%, 7/56) had a significantly higher mortality rate than the supracardiac-Ia group (adjusted hazard ratio, 8.5, P = .008). Surgical weight less than 2.5 kg (adjusted hazard ratio, 10.8, P = .023), longer duration of cardiopulmonary bypass (adjusted hazard ratio, 1.15 per 10 minutes, P = .012), and supracardiac-Ib subtype (adjusted hazard ratio, 4.7, P = .037) were independent risk factors associated with death. The supracardiac-Ib subtype (adjusted hazard ratio, 4.8, P = .003) was an incremental risk factor associated with postoperative pulmonary venous obstruction. CONCLUSIONS: Morphological features of supracardiac total anomalous pulmonary venous connection, especially the supracardiac-Ib subtype, were risk factors associated with postoperative pulmonary venous obstruction and survival. Patients with unique anatomic subtypes might require more individualized surgical planning.

Surgical aortic valvuloplasty is a better primary intervention for isolated congenital aortic stenosis in children with bicuspid aortic valve than balloon aortic valvuloplasty.

OBJECTIVES: Surgical aortic valvuloplasty (SAV) and balloon aortic valvuloplasty (BAV) are two main treatments for children with isolated congenital aortic stenosis (CAS). We aim to compare the two procedures' midterm outcomes, including valve function, survival, reintervention, and replacement. METHODS: From January 2004 to January 2021, children with isolated CAS undergoing SAV (n = 40) and BAD (n = 49) at our institution were included in this study. Patients were also categorized into subgroups based on the aortic leaflet number(Tricuspid = 53, Bicuspid = 36) to compare the two procedures' outcomes. Clinical and echocardiogram data were analyzed to identify risk factors for suboptimal outcomes and reintervention. RESULTS: Postoperative peak aortic gradient (PAG) and PAG at follow-up in the SAV group were lower compared with the BAV group (p < 0.001, p = 0.001, respectively). There was no difference in moderate or severe AR in the SAV group compared with the BAV group before discharge (5.0% vs 12.2%, p = 0.287) and at the last follow-up (30.0% vs 32.7%, p = 0.822). There were no early death but three late deaths (SAV = 2, BAV = 1). Kaplan-Meier estimated survivals were 86.3% and 97.8% in SAV and BAV groups respectively at 10 years (p = 0.54). There was no significant difference in freedom from reintervention (p = 0.22). For patients with bicuspid aortic valve morphology, SAV achieved higher freedom from reintervention (p = 0.011) and replacement (p = 0.019). Multivariate analysis indicated that residual PAG was a risk factor for reintervention (p = 0.045). CONCLUSIONS: SAV and BAV achieved excellent survival and freedom from reintervention in patients with isolated CAS. SAV performed better in PAG reduction and maintenance. For patients with bicuspid AoV morphology, SAV was the preferred choice.

Bronchus compression is a predictor for reobstruction in coarctation with hypoplastic arch repair.

OBJECTIVES: The surgical treatment of coarctation of aorta with hypoplastic aortic arch (CoA/HAA) was challenging to achieve long-lasting arch patency. We reviewed early and late outcomes in our centre and identified predictors for arch reobstruction. METHODS: A retrospective analysis of medical records was performed to identify CoA/HAA patients who underwent primary arch reconstruction via median sternotomy between 2011 and 2020. Preoperative aortic arch geometry was analysed with cardiac computed tomographic angiography. Bedside flexible fibre-optic bronchoscopy was routinely performed after surgery in intensive care unit. RESULTS: There were 104 consecutive patients (median age 39.5 days) who underwent extended end-to-end anastomosis, extended end-to-side anastomosis and autograft patch augmentation. Early mortality was 3.8% and overall survival was 94.1% [95% confidence interval (CI) 89.6-98.8%] at 1, 3 and 5 years. Reobstruction-free survival was 85.1% (95% CI 78.4-92.3%) at 1 year, 80.6% (95% CI 73.1-88.9%) at 3 years and 77.4% (95% CI 69.2-86.6%) at 5 years. Preoperative aortic arch geometric parameters were not important factors for reobstruction. Nineteen patients (18.3%) were detected with left main bronchus compression (LMBC) on flexible fibre-optic bronchoscopy. Cardiopulmonary bypass time [P < 0.001, hazard ratio (95% CI): 1.02 (1.01-1.03)] and postoperative LMBC [P = 0.034, hazard ratio (95% CI): 2.99 (1.09-8.23)] were independent predictive factors on multivariable Cox regression analysis of reobstruction-free survival. CONCLUSIONS: Aortic arch can be satisfactorily repaired by extended end-to-end anastomosis, extended end-to-side anastomosis and autograft patch augmentation via median sternotomy in CoA/HAA. Cardiopulmonary bypass time and postoperative LMBC detected by flexible fibre-optic bronchoscopy are significant predictors for long-term arch reobstruction.

Epidemiological characteristics and trends in postoperative death in children with congenital heart disease (CHD): a single-center retrospective study from 2005 to 2020.

OBJECTIVES: To analyze the epidemiological characteristics and trends in death after thoracotomy in children with congenital heart disease (CHD). METHODS: The clinical data of children with CHD aged 0-14 years who died after thoracotomy in our hospital from January 1, 2005, to December 31, 2020, were retrospectively collected to analyze the characteristics of and trends in postoperative death. RESULTS: A total of 502 patients (365 males; 72.7%) died from January 1, 2005, to December 31, 2020, with an average of 31 deaths per year. For these patients, the median age was 2.0 months, the median length of hospital stay was 16.0 days, the median postoperative time to death was 5.0 days, and the median risk adjustment in congenital heart surgery-1 (RACHS-1) score was 3.0. 29.5% underwent emergency surgery, 16.9% had postoperative ECMO support, and 15.9% received postoperative blood purification treatment. In the past 16 years, the deaths of children with CHD under 1 year old accounted for 80.5% of all deaths among children with CHD aged 0-14 years, and deaths (349 cases) under 6 kg accounted for 69.5% of all deaths. Age at death, weight, and disease type were characterized by annual changes. CONCLUSIONS: The postoperative deaths of children with CHD mainly occurred in infants and toddlers who weighed less than 6.0 kg, and TGA and PA were the most lethal CHDs. The proportion of deaths has been increasing across the years among patients who are young, have a low body weight, and have complex cyanotic CHD.

Long-term outcomes of pulmonary atresia with ventricular septal defect by different initial rehabilitative surgical age.

Background: There is a lack of evidence guiding the surgical timing selection in pulmonary atresia with ventricular septal defect. This study aims to compare the long-term outcomes of different initial rehabilitative surgical ages in patients with pulmonary atresia with ventricular septal defect (PAVSD). Methods: From January 2011 to December 2020, a total of 101 PAVSD patients undergoing the initial rehabilitative surgery at our center were retrospectively reviewed. Receiver-operator characteristics curve analysis was used to identify the cutoff age of 6.4 months and therefore to classify the patients into two groups. Competing risk models were used to identify risk factors associated with complete repair. The probability of survival and complete repair were compared between the two groups using the Kaplan-Meier curve and cumulative incidence curve, respectively. Results: The median duration of follow-up was 72.76 months. There were similar ΔMcGoon ratio and ΔNakata index between the two groups. Multivariate analysis showed that age ≤6.4 months (hazard ratio (HR) = 2.728; 95% confidence interval (CI):1.122-6.637; p = 0.027) and right ventricle-to-pulmonary artery connection (HR = 4.196; 95% CI = 1.782-9.883; p = 0.001) were associated with increased probability of complete repair. The cumulative incidence curve showed that the estimated complete repair rates were 64% ± 8% after 3 years and 69% ± 8%% after 5 years in the younger group, significantly higher than 28% ± 6% after 3 years and 33% ± 6% after 5 years in the elder group (p < 0.001). There was no significant difference regarding the estimated survival rate between the two groups. Conclusion: Compared with those undergoing the initial rehabilitative surgery at the age >6.4 months, PAVSD patients at the age ≤6.4 months had an equal pulmonary vasculature development, a similar probability of survival but an improved probability of complete repair.

Surgical repair of unilateral absence of pulmonary artery in children with pulmonary hypertension: a single-center retrospective study.

Background: The early diagnosis of unilateral absence of pulmonary artery (UAPA) in children offers an opportunity for effective intervention. Due to the lack of clinical evidence, a consensus regarding surgical treatment has yet to be reported. The aim of this study is to evaluate the effectiveness and safety of pulmonary artery (PA) reconstruction with a "two-segment" technique to repair UAPA in patients with pulmonary hypertension. Methods: Intraoperatively, the ligamentum arteriosum connecting the innominate artery and distal PA was dissected and occluded. A conduit created by fresh autologous pericardium formed the first "segment" of the neo-PA. The second "segment" was a Gore vascular graft with integrated rings anastomosed between the proximal end of the pericardial conduit and the main pulmonary artery (MPA). Results: A total of five consecutive patients were included, and the absent PA was successfully reconstructed using the "two-segment" technique in all patients. Following revascularization, the direct measurement of the pressure in MPA during the operation showed that the average mean pulmonary artery pressure (mPAP) decreased from 31.3±16.0 to 16.8±4.2 mmHg (P=0.047). The average mPAP/radial mean arterial pressure (rMAP) ratio decreased from 0.59±0.27 preoperatively to 0.30±0.10 postoperatively (P=0.028). The mean follow-up period was 18.85±4.67 months. The median diameter of the reconstructed PA (pericardial segment) measured by transthoracic echocardiography (TTE) was 6.1 mm. One patient safely underwent a redo operation to repair relative stenosis in the neo-PA. Conclusions: Early PA reconstruction may effectively alleviate pulmonary hypertension in children with UAPA. The "two-segment" technique is safe and can facilitate potential redo pulmonary arterioplasty. Anticoagulation and antiplatelet therapy, as well as frequent follow-up, is required after the operation.

Anomalous left coronary artery from the pulmonary artery: Outcomes and management of mitral valve.

Objective: Use of concomitant mitral valve repair remains controversial in the anomalous left coronary artery from the pulmonary artery (ALCAPA) with mitral regurgitation (MR). This study aimed to evaluate postoperative mitral valve function and explore the indication for concomitant mitral valve repair. Materials and methods: The medical records of 111 patients with ALCAPA and MR who underwent ALCAPA surgery between April 2006 and November 2020 were reviewed. The patients were categorized into three groups for comparison, namely, group I consisted of 38 patients with trivial or mild MR who underwent ALCAPA repair only; group II consisted of 37 patients with moderate or severe MR who similarly had only surgery of the ALCAPA performed; and group III consisted of 36 patients who had concomitant mitral valve repair for moderate or severe MR. Result: Overall mortality was 7.2% (8 of 111). The mortality of group II (16.2%, 6 of 37) was higher than those of groups I (5.3%, 2 of 38) and III (0%, 0 of 36) (p = 0.027). All three patients who underwent mitral valve reintervention were in group II. At the last follow-up, none of the patients had more than moderate MR in group I. The percentage of patients with improved MR grade was 79.4% (27 of 34) in group III and 51.4% (19 of 37) in group II (p = 0.001). The multivariate logistic regression revealed that concomitant mitral valve repair (adjusted odds ratio = 4.492, 95% CI: 1.909-12.794; p < 0.001) was the major factor influencing MR grade improvement. Conclusion: The long-term outcomes after ALCAPA repair were favorable. For mild MR, ALCAPA repair only can be performed. For moderate and severe MR, we suggest concomitant mitral valve repair.

Complications and management of functional single ventricle patients with Fontan circulation: From surgeon's point of view.

Fontan surgery by step-wise completing the isolation of originally mixed pulmonary and systemic circulation provides an operative approach for functional single-ventricle patients not amenable to biventricular repair and allows their survival into adulthood. In the absence of a subpulmonic pumping chamber, however, the unphysiological Fontan circulation consequently results in diminished cardiac output and elevated central venous pressure, in which multiple short-term or long-term complications may develop. Current understanding of the Fontan-associated complications, particularly toward etiology and pathophysiology, is extremely incomplete. What's more, ongoing efforts have been made to manage these complications to weaken the Fontan-associated adverse impact and improve the life quality, but strategies are ill-defined. Herein, this review summarizes recent studies on cardiac and non-cardiac complications associated with Fontan circulation, focusing on significance or severity, etiology, pathophysiology, prevalence, risk factors, surveillance, or diagnosis. From the perspective of surgeons, we also discuss the management of the Fontan circulation based on current evidence, including post-operative administration of antithrombotic agents, ablation, pacemaker implantation, mechanical circulatory support, and final orthotopic heart transplantation, etc., to standardize diagnosis and treatment in the future.

End-diastolic forward flow in repaired tetralogy of Fallot: Mid-term outcomes from a single center.

Background: Pulmonary arterial end-diastolic forward flow (EDFF) following repaired tetralogy of Fallot (rTOF) is recognized as right ventricular (RV) restrictive physiology, which is closely related to poor prognosis. This study sought to review mid-term experience and investigate the risk factors of EDFF in the rTOF patients. Methods: From September 2016 to January 2019, 100 patients (age < 18 years old) who underwent complete tetralogy of Fallot (TOF) repair were enrolled and were divided into EDFF group (n = 52) and non-EDFF group (n = 48) based on the presence of postoperative EDFF. Elastic net analysis was performed for variable selection. Univariate and multivariate logistic analyses were used to analyze the correlation between risk factors and EDFF. Results: End-diastolic forward flow group had lower systolic blood pressure (P = 0.037), diastolic blood pressure (P = 0.027), and higher vasoactive-inotrope score within 24 h after surgery (P = 0.022) than non-EDFF group. Transannular patch (TAP) was an independent predictor of postoperative EDFF [P = 0.029, OR: 2.585 (1.102∼6.061)]. Patients were followed up for a median of 2.6 years [interquartile range (IQR) 1.6] after the first TOF repair. During follow-up, the prevalence of the EDFF was lower in those with pulmonary valve (PV) reconstructions than that in those undergoing patch enlargement without PV reconstructions in the primary TOF repair (P < 0.001). Conclusion: End-diastolic forward flow was associated with TAP. Patients with EDFF might have a transient hemodynamic instability in the early postoperative period. PV reconstructions in the TOF repair might reduce the incidence of EDFF in the mid-term follow-up.

Long-term outcomes of primary aortic valve repair in children with congenital aortic stenosis - 15-year experience at a single center.

Background: Studies on the long-term outcomes of children with congenital aortic stenosis who underwent primary aortic repair are limited. We reviewed the long-term outcomes of children who underwent aortic valve (AoV) repair at our center. Methods: All children (n = 75) who underwent AoV repair between 2006 and 2020 were reviewed. The Kaplan-Meier curve was used to demonstrate the survival estimates. The Cox proportional hazard model and competing risk regression model were used to identify risk factors for death, reintervention, adverse events, and replacement. Results: The median age at surgery was 1.8 (IQR, 0.2-7.7) years, and the median weight at surgery was 10.0 (IQR, 5.0-24.0) kg. Early mortality and late mortality were 5.3% (4/75) and 5.6% (4/71), respectively. Risk factors for overall mortality were concomitant mitral stenosis (P = 0.01, HR: 9.8, 95% CI: 1.8-53.9), low AoV annulus Z-score (P = 0.01, HR: 0.6, 95% CI: 0.4-0.9), and prolonged cardiopulmonary bypass time (P < 0.01, HR: 9.5, 95% CI: 1.7-52.1). Freedom from reintervention was 72.9 ± 0.10% (95% CI: 56.3-94.4%) at 10 years. Risk factors for occurrence of adverse event on multivariable analysis included preoperative intubation (P = 0.016, HR: 1.004, 95% CI: 1.001-1.007) and a low AoV annulus Z-score (P = 0.019, HR: 0.714, 95% CI: 0.540-0.945). Tricuspid AoV morphology was associated with a suboptimal postoperative outcome (P = 0.03). Conclusion: Aortic valve repair remains a safe and durable solution for children with congenital aortic stenosis. Concomitant mitral stenosis and aortic valve anatomy, including tricuspid valve morphology and smaller annulus size, are associated with poor early and long-term outcomes.