Background Anomalous origin of the left pulmonary artery (ALPA) from the ascending aorta (AA) is a rare congenital heart malformation. Aim To give some of our embryological considerations of ALPA from the AA. Methods We present a patient with ALPA from the AA, right-sided aortic arch, right-sided ductus arteriosus (DA), and aberrant left subclavian artery (LSCA). Results The distal end of ALPA was cut off, the proximal end was sutured, and the distal end was directly anastomosed to the left wall of the main pulmonary artery (MPA). Conclusion The failure of migration and differentiation of cardiac neural crest cells at the fourth and sixth archs result in unilateral arch agenesis or failure of detachment of the left sixth arch from the aortic sac, which form ALPA the AA.

Background: This study explores the strategy and effect of emergency surgical treatment for total anomalous pulmonary venous connection (TAPVC). Methods: From March 2009 to February 2020, 78 patients with TAPVC underwent emergency surgical correction. There were 51 males and 27 females. The median age was 39.5 days, and the median weight was 4.0 kg. The preoperative percutaneous oxygen saturation was 80.8±4.5%. Results: Of the cases investigated, seven died during the perioperative period, 16 had delayed chest closure, 19 had early pulmonary vein obstruction, two had secondary tracheal intubation, one had a brain complication, and one had third-degree atrioventricular block. Low weight, younger age, cardiopulmonary bypass time, and aortic cross-clamp time were identified as risk factors for early mortality. During the follow-up from four to 137 months, 12 cases did not respond to follow up. Ten patients died within one to six months after discharge. One patient underwent reoperation due to pulmonary vein obstruction. The longer hospital stays after operation and intensive care unit time were identified as risk factors for late mortality. Conclusions: Emergency surgery for severe TAPVC patients after admission had achieved good results in the near future. Prenatal diagnosis should be strengthened to save more patients. The higher late mortality rate indicates that such patients should strengthen post-discharge management to reduce the occurrence of post-discharge deaths.

Abstract Background Subaortic stenosis (SAS) was a rare congenital heart disease of left ventricular outflow tract (LVOT), ranging from “isolated” lesions to “tunnel” or “diffuse” lesions. We conducted a retrospective study to describe the characteristics of patients with different lesions and analyze the risk factors for reoperation. Methods In this study, we examined a single-center retrospective cohort of SAS patients undergoing resection from 2010 to 2019. Patients were classified as simple lesion group (n = 37) or complex lesion group (n = 28). Demographics, perioperative findings, and clinical data were analyzed. Results The surgical effect of the two groups was significantly lower than that before the operation (p <0.05). The median age at operation was 6(3-11.8) years. There was no operative mortality. In complex lesion group, extracorporeal circulation time (CPB time), aortic cross clamp time (ACC time), mechanical ventilation time and ICU stay time were longer. The median follow-up period was 2.8 years (range 1-3.8), with two late death. Six patients (9.2%) required reoperation due to restenosis or severe aortic insufficiency. The freedom from reoperation rates at 5 years was 66.7% for simple lesion but only 52.3% for complex lesion (p = 0.036). Conclusions Although the lesions include many forms, subaortic stenosis resection was still satisfactory. However, the reoperation after initial surgical treatment was not infrequent, especially in patients with complex lesion.

Backgroud: Surgical results of functional single ventricle (FSV) patients with totally anomalous pulmonary venous connection (TAPVC), have a poor outcome. We retrospectively analyzed our 10-year surgical clinical experience and risk factors of mortality of these patients. Methods: Between March 2008 and August 2018, 43 consecutive patients with FSV and TAPVC underwent initial surgical palliation and TAPVC repair or not. The median body weight and age were 12 (range 5-44) kg and 32 (range 2-256) months, respectively. Among these cases, there are 19 cases of supracardiac TAPVC, 22 of intracardiac type, and 2 of mixed type. 12 patients need to perform TAPVC repair during initial surgical palliation (supracardiac in 10 and mixed type in 2). Results: Overall survival at 1 and 5 years were 89.5% and 83.3%, respectively. In TAPVC repair group and non-TAPVC repair group, overall survival after the initial surgical palliation were 58.3 and 87.1% at 1 year, 40.0% and 87.1% at 3 years, respectively. Cox univariate analysis detected that preoperative pulmonary vein obstruction (PVO) (p=0.047) and concomitant TAPVC repair (p=0.007) were risk factors for mortality, and multivariable analysis indicated concomitant TAPVC repair as the only factor (p=0.033). Conclusions: The mid-term results of surgical results of FSV associated with TAPVC, especially for patients who need to concomitant TAPVC repair, remain poor. Preoperative PVO is identified as risk factor that increases mortality of these patients.

Background Ebstein’s anomaly (EA) is a kind of congenital heart disease, which is currently widely treated by cone reconstruction. However, prediction of postoperative recovery is still challenging. Methods A retrospective analysis was performed on EA cases undergoing cone reconstruction from January 2010 to January 2016. Univariate and multivariate logistic regression analyses were performed, with postoperative adverse events defined as dependent variable and pre- and intra-operative parameters defined as independent variables. Predictive capacity of preoperative SPO2 and Great Ormond Street (GOS) score was evaluated using areas under the curve of receiver operating characteristic (ROC). Results Preoperative SPO2 was 95.7 ± 5.20%. Cardiopulmonary bypass, aortic cross-clamp, postoperative mechanical ventilation, and hospitalization time were 101.7 ± 28.26 min, 60.9 ± 18.04 min, 16 hours (8, 22), and 8 days (7, 11), respectively. The incidence of total postoperative adverse events including low cardiac output syndrome, mechanical ventilation more than 3 days, postoperative hospitalization more than 2 weeks, postoperative re-intubation, extracorporeal membrane oxygenation assistance, and death was 13.1% (n=13). Low pre-operative SPO2 (P=0.001, OR=0.834), GOS score (P=0.021, OR=0.368), and cardiopulmonary bypass time (P=0.034, OR=1.021) were risk factors for adverse events. Multivariate logistic regression analysis showed that low preoperative SPO2 (P=0.002, OR=0.846) and GOS score (P=0.043, OR=0.577) were independent risk factors for adverse events. The areas of SPO2 and GOS score under the ROC curve were 0.764 and 0.740, respectively. Conclusions Low pre-operative SPO2 and GOS score were predictors of adverse events after cone reconstruction, and SPO2 was more convenient and objective than GOS score.