Objective To evaluate the outcome of surgical repair of interrupted aortic arch (IAA) combined with anomalies. Methods We retrospectively analyzed the clinical data of 48 patients with IAA combined with anomalies undergoing one-stage biventricular repair in Shanghai Children's Medical Center from November 2006 to April 2016. There were 25 males and 23 females with a median age of 29 d (range, 8 to 91 d) and a mean weight of 3.80±0.67 kg. All patients underwent end-to-end anastomosis with patch augmentation, and relief of left ventricular outflow tract obstruction (LVOTO) was performed in 11 patients. Results In IAA children with anomalies, 39 (81.3%) suffered noncomplex lesions and 9 (18.8%) complex lesions. Mean follow-up was 72.1±19.7 months for 38 patients. There were 6 in-hospital deaths and 3 patients died during follow-up. The early survival rate was 87.5%, 5-year rate 83.3% and 10-year rate 81.3%. Reintervention was required in 10 patients, including 8 with subsequent LVOTO and 2 with anastomotic stenosis. Conclusion End-to-end anastomosis with patch augmentation is effective for IAA.
ObjectiveTo characterize surgical outcomes of supra-cardiac total anomalous pulmonary venous connection (TAPVC), investigate risk factors for postoperative death, and explore informed LASSO machine learning methods to solve "small sample size problem" in research of rare congenital heart diseases.MethodsA retrospective analysis of 241 patients with supra-cardiac TAPVC who underwent surgical repair in Guangdong Provincial People's Hospital from 2009 to 2019 was conducted, including 179 males and 62 females with a median surgical age of 71 (33, 232) d.Detailed clinical data of the postoperative death-related factors were extracted. Univariable Cox proportional hazard models were used to initially screen potential risk factors for postoperative death. Factors with P≤0.05 were retained. To solve the limitation of small sample size and the "P>n" problem, we proposed a novel LASSO method for conducting multivariable Cox regression analysis that was capable of bringing in findings of related studies to improve analysis power and to reduce false-negative findings.ResultsUnivariable Cox analyses showed several potential clinical risk factors, among which highly significant factors (P<0.001) included: surgical weight≤2.5 kg (HR=16.00), main pulmonary artery diameter (HR=0.78), prolonged cardiopulmonary bypass time (HR=1.21), aortic block time (HR=1.28), and postoperative ventilator-assisted time (HR=1.13/d). LASSO multivariable analysis revealed that independent risk factors for postoperative death included cardiopulmonary bypass time (aHR=1.308/30 min), age (aHR=0.898), postoperative ventilator-assisted time (aHR=1.023/d), weight≤2.5 kg (aHR=2.545), right vertical venous return (aHR=1.977), preoperative pulmonary venous obstruction (aHR=1.633) and emergency surgery (aHR=1.383).ConclusionOur proposed informed LASSO method can use previous studies' results to improve the power of analysis and effectively solve the "P>n" and small sample size limitation. Cardiopulmonary bypass time, surgical age, postoperative ventilator-assisted time, weight, right vertical venous return, preoperative pulmonary venous obstruction, and emergency surgery are risk factors for postoperative death of supra-cardiac TAPVC.
ObjectiveTo quest the risk factors of poor prognoses in children with tetralogy of Fallot (TOF) during perioperative period and evaluate its clinical application values.MethodsA retrospective analysis of the clinical data of 119 children who underwent one-stage correction of TOF in Guangdong Provincial People's Hospital from September 2016 to January 2019. The cohort includes 75 males and 44 females, with ages ranging from 3.2-137.1 (13.2±1.4) months and weights ranging from 4.6-21.0 (8.3±0.2) kg. Perioperative poor prognosis was defined as duration of mechanically assisted ventilation >48 h or secondary intubation, vasoactive-inotropic score (VIS) within 48 h >40, postoperative length of stay >14 d, and the occurrence of the major adverse events. Major adverse events were defined as early death, malignant arrhythmia, low cardiac output syndrome, non-fatal cardiac arrest, postoperative reintervention, diaphragm paralysis, and other clinical complications. Univariate and multivariate logistic analyses were used to analyze the correlation between risk factors and poor prognoses.ResultsThere was 1 perioperative death, and 9 with major adverse events. Variables selected by Least Absolute Shrinkage and Selection Operator (LASSO) included 2 preoperative variables (McGoon index, aortic root diameter index) and 4 intra-operative variables [left-right direction of bicuspid pulmonary valve, total length of right ventricular outflow tract (RVOT) incision index, pulmonary valve with commissurotomy, and minimum temperature in cardiopulmonary bypass (CPB)]. Univariate and multivariate logistic analyses were used to the above factors, respectively. The variables with statistical significance (P≤0.05) were McGoon index, aortic root diameter index, left-right direction of bicuspid pulmonary valve, and minimum temperature in CPB. A nomogram was established based on the above factors, and the results showed that the left-right direction of bicuspid pulmonary valve was more risky than the tricuspid pulmonary valve and the anterior-posterior direction of bicuspid pulmonary valve. The lower the McGoon index, the higher aortic root diameter, and the lower temperature in CPB, the higher risk of poor prognostic events in children with TOF.ConclusionThe left-right direction of the pulmonary bicuspid valve has a higher risk of poor prognosis than the tricuspid pulmonary valve and the anterior-posterior direction of bicuspid pulmonary valve. With the smaller McGoon index and the larger aortic root diameter, the risk of poor prognoses in children with TOF is higher. The temperature in CPB being lower than medium-low temperature obviously relates to the high incidence of poor prognostic events, which can be used as an auxiliary reference standard for decision-making in pediatric TOF surgery in the future.
ObjectiveTo compare the benefits and drawbacks of primary patch expansion and pericardial tube right ventricular-pulmonary artery connection in patients diagnosed with pulmonary atresia with ventricular septal defect (PA/VSD). MethodsA retrospective study was conducted on the patients who were diagnosed with pulmonary atresia with ventricular septal defect (PA/VSD) and underwent primary right ventricular-pulmonary artery connection surgery at our center between January 2010 and December 2020. The patients were categorized into two groups based on the type of right ventricular-pulmonary artery connection: the whole pericardial tube right ventricular-pulmonary artery connection group (pericardial tube group), and the patch expansion right ventricular-pulmonary artery connection group (patch expansion group). The patients' general clinical data and cardiopulmonary bypass data were documented, and the cardiac CT data obtained before and after the surgery were compared with the clinical and imaging data. ResultsFinally 51 patients were collected, including 31 males and 20 females, with a median age of 12.57 (4.57, 49.67) months. There were 19 patients in the pericardial tube group, with a median age of 17.17 (7.33, 49.67) months, and 32 patients in the patch expansion group with a median age of 8.58 (3.57, 52.72) months. In both groups, the diameter, McGoon index, and Nakata index were significantly increased after treatment (P<0.001). However, the pericardial tube group required a longer occlusion time (P<0.001). The rate of reoperation was high, with 72.5% of patients requiring further surgery. Specifically, 26 (81.3%) patients in the patch expansion group and 12 (63.2%) patients in the pericardial tube group finally completed radical resection. There was no statistical difference observed in the long-term cure rate or mortality between the two groups. Conclusion In patients with PA/VSD, the use of patch expansion or pericardial tube right ventricular-pulmonary artery connection as the initial palliative treatment strategy can promote the development of pulmonary vessels and provide a favorable basis for subsequent radical operations. However, compared to pericardial tube, patch expanded right ventricular-pulmonary artery connection is a simpler procedure to perform and retains some of the intrinsic pulmonary artery development potential, thus making it a preferred procedure.