Case Report: Right aortic arch with isolation of left brachiocephalic artery and ventricular septal defect.

Right aortic arch with isolation of left brachiocephalic artery is a rare congenital aortic arch anomaly. Herein, we reported a case of this rare anomaly with ventricular septal defect in a 9-month-old infant. We successfully reconstructed the islolated left brachiocephalic artery and repaired the ventricular septal defect in one stage.

Veno-arterial extracorporeal membrane oxygenation for respiratory and cardiac support in neonates: a single center experience.

Objective: Extracorporeal membrane oxygenation (ECMO) is an advanced life support that has been utilized in the neonate for refractory respiratory and circulatory failure. Striving for the best outcomes and understanding optimal surgical techniques continue to be at the forefront of discussion and research. This study presents a single-center experience of cervically cannulated neonatal patients on V-A ECMO, a description of our cannulation/decannulation techniques and our patient outcomes. Methods: Single center retrospective review of neonates who received neck V-A ECMO support from January 2012 to December 2022. The data and outcomes of the patients were retrospectively analyzed. Results: A total of 78 neonates received V-A ECMO support. There were 66 patients that received ECMO for respiratory support, the other 12 patients that received ECMO for cardiac support. The median duration of ECMO support was 109 (32-293) hours for all patients. During ECMO support, 20 patients died and 5 patients discontinued treatment due to poor outcome or the cost. A total of 53 (68%) patients were successfully weaned from ECMO, but 3 of them died in the subsequent treatment. Overall 50 (64%) patients survived to hospital discharge. In this study, 48 patients were cannulated using the vessel sparing technique, the other 30 patients were cannulated using the ligation technique. We found no significant difference in the rates of normal cranial MRI at discharge between survivors with and without common carotid artery ligation. Conclusion: We achieved satisfactory outcomes of neonatal ECMO in 11-year experience. This study found no significant difference in early neuroimaging between survivors with and without common carotid artery ligation. The long-term neurological function of ECMO survivors warranted further follow-up and study.

Experience of treating congenital complete atrioventricular block with epicardial pacemaker in infants and young children: a retrospective study.

BACKGROUND: This article summarizes the treatment experience for congenital complete atrioventricular block (CCAVB) in newborns and infants, and discusses the necessity and feasibility of treating CCAVB with permanent pacemaker implantation in this population. METHODS: In this study, the clinical data and follow-up results of nine children admitted at our center with CCAVB from January 2005 to March 2023 were retrospectively analyzed. Among them, two children received early implantation of permanent pacemakers (within 1 year of age), two children received non-early implantation (1 year or older), and the remaining five children received no pacemaker implantation. CCAVB diagnosis was confirmed by clinical symptoms and clinical examinations, including electrocardiography and echocardiography before surgery. After surgery, the pacing and sensing functions of the pacemaker were observed using electrocardiography, echocardiography, and pacing threshold monitoring. A comprehensive assessment of the treatment efficacy was conducted, encompassing improvements in clinical symptoms, growth and development, as well as the absence of any additional potential complications. The children who did not receive pacemaker implantation were followed up. RESULTS: Among the four children who successfully received pacemaker implantation, one child who received non-early implantation died. For the remaining three children, the threshold level, amplitude, impedance, and minute ventilation sensor function of the pacemaker were good during the follow-up period, with a heart rate at the pacing rate. The growth and development of the aforementioned patients who received pacemaker implantation demonstrated adherence to the percentile curve, and their motor and cognitive development remained unaffected. However, among the children who did not undergo pacemaker implantation, two experienced death, while three were lost to follow-up, thereby limiting the evaluation of their long-term outcomes. CONCLUSIONS: Early implantation of an epicardial pacemaker at an early stage in newborns and infants diagnosed with CCAVB can significantly improve clinical symptoms without affecting their growth and development. These data are in line with current literature and suggest that early implantation of an epicardial pacemaker in newborns and infants diagnosed with CCAVB but further studies are needed.

Clinical profile of congenital heart diseases detected in a tertiary hospital in China: a retrospective analysis.

Background: Congenital heart diseases (CHDs) are conditions that involve structural problems to the heart's structure existing at birth, with an incidence of approximately 8 per 1,000 live births globally. CHD is one of the leading causes of maternal, fetal, and neonatal morbidity and mortality worldwide. The present study sought to examine the clinical profiles of CHD patients and provide important implications for therapeutic interventions. Methods: This was a retrospective, observational, cohort study. The medical records of all CHDs patients aged between 0 and 18 years were collected from July 1, 2021 to June 30, 2022. Clinical profiles and demographic data were collected from cardiology and pediatric department registers for analysis. Results: Of the 265 children with CHDs, 201 were diagnosed with acyanotic CHD (ACHD), while 64 children had cyanotic CHD (CCHD). Based on the eleventh revision of the International Classification of Diseases (ICD-11), "congenital anomaly of a ventricle or the ventricular septum" was the most common CHD. The most common symptom was failure to thrive, accounting for 18.5% of all CHD cases. The most frequent symptom in ACHD was murmur (93.53%) and sweating (80.60%), whereas the most common symptom in CCHD was sweating (95.31%) and cyanosis (84.38%). Conclusions: This study retrospectively analyzed CHD clinical characteristics from children receiving care at the seventh center, which forms a proper basis for appropriate clinical treatments and further studies.

Window anastomosis technique for repair of supracardiac total anomalous pulmonary venous connection in infants.

Outcomes of operations for total anomalous pulmonary venous connection (TAPVC) have improved. However, postoperative pulmonary venous obstruction remains the most significant complication with high morbidity and mortality. We introduce a window anastomosis technique for repair of supracardiac TAPVC in infants. The mainstay of the surgical technique is to resect the anterior wall of the pulmonary vein confluence and part of the posterior wall of the left atrium to form a large and undistorted "window to window" anastomosis.

Ventricular septal intramyocardial dissection after cardiac tumor excision.

We reported a rare case: an infant underwent cardiac tumor resection, and postoperative transthoracic echocardiography revealed a cystic dissection located in the interventricular septum.

Pectoralis major muscle turnover flap reconstruction for treatment of deep sternal wound infection in infants and children.

OBJECTIVE: The aim of the study is to assess the therapeutic effect and applicability of pectoralis major muscle turnover flap (PMMTF) reconstruction for treatment of deep sternal wound infection (DSWI) after cardiac surgery in infants and children. METHODS: From March 2013 to October 2021, 23 patients with DSWI after cardiac surgery underwent PMMTF reconstruction. The data and outcomes of the patients were retrospectively analyzed. RESULTS: Twenty patients were treated with unilateral PMMTF reconstruction, and three patients were treated by bilateral PMMTF. All of the sternal wounds healed successfully. All patients survived and were discharged without evidence of infection. In a follow-up period, ranging from 15 to 83 months (mean 32.6 months), all patients demonstrated normal development with no limitations to limb movements. There were no signs of chronic sternal infection in all of them. CONCLUSION: PMMTF reconstruction is a simple, feasible, and effective treatment of DSWI after cardiac surgery in infants and children, with minimal developmental problems.

Prognostic Model to Predict Postoperative Adverse Events in Pediatric Patients With Aortic Coarctation.

Background: Postoperative adverse events remain excessively high in surgical patients with coarctation of aorta (CoA). Currently, there is no generally accepted strategy to predict these patients' individual outcomes. Objective: This study aimed to develop a risk model for the prediction of postoperative risk in pediatric patients with CoA. Methods: In total, 514 patients with CoA at two centers were enrolled. Using daily clinical practice data, we developed a model to predict 30-day or in-hospital adverse events after the operation. The least absolute shrinkage and selection operator approach was applied to select predictor variables and logistic regression was used to develop the model. Model performance was estimated using the receiver-operating characteristic curve, the Hosmer-Lemeshow test and the calibration plot. Net reclassification improvement (NRI) and integrated discrimination improvement (IDI) compared with existing risk strategies were assessed. Results: Postoperative adverse events occurred in 195 (37.9%) patients in the overall population. Nine predictive variables were identified, including incision of left thoracotomy, preoperative ventilation, concomitant ventricular septal defect, preoperative cardiac dysfunction, severe pulmonary hypertension, height, weight-for-age z-score, left ventricular ejection fraction and left ventricular posterior wall thickness. A multivariable logistic model [area under the curve = 0.8195 (95% CI: 0.7514-0.8876)] with adequate calibration was developed. Model performance was significantly improved compared with the existing Aristotle Basic Complexity (ABC) score (NRI = 47.3%, IDI = 11.5%) and the Risk Adjustment for Congenital Heart Surgery (RACHS-1) (NRI = 75.0%, IDI = 14.9%) in the validation set. Conclusion: Using daily clinical variables, we generated and validated an easy-to-apply postoperative risk model for patients with CoA. This model exhibited a remarkable improvement over the ABC score and the RACHS-1 method.

Tracheal development after left pulmonary artery reimplantation: an individual study.

Pulmonary artery sling (PA sling) often presents as a life-threatening condition requiring urgent surgical correction. We reported 32 cases of PA sling in children who were followed up postoperatively in the past 6 years. All patients with PA slings who were admitted to the hospital from January 2012 to December 2017 and underwent surgery were retrospectively analyzed. The mean age of the 32 patients at repair was 16.97 months (range, 15 days to 128 months). Six patients required ventilator assistance for respiratory failure. All children underwent left pulmonary artery (LPA) reimplantation (n = 32), and 3 patients needed reimplantation slide tracheoplasty (n = 3) due to ventilation weaning failure. Four patients died, 27 patients survived until discharge, and 18 patients were followed up. Pulmonary computed tomography imaging and echocardiography were performed in 18 patients who were followed up. After LPA reimplantation, the tracheal carina area was significantly enlarged compared to that preoperation (p = 0.0002). In this follow-up cohort study, 75% of the patients who underwent LPA reimplantation survived until discharge. The survivors had subsequently well-developed pulmonary arteries and tracheas.

Left superior pulmonary artery sling.

A pulmonary artery sling is formed when the left pulmonary artery originates from the right pulmonary artery and encircles the distal trachea, coursing between the trachea and esophagus to reach the hilum of the left lung. Pulmonary artery slings are often associated with distal tracheal narrowing, due to either intrinsic stenosis or secondary compression by the anomaly itself. We report a very rare case in which the left superior pulmonary artery originated from the right pulmonary artery and then acted as a sling, the left inferior pulmonary artery originated from the pulmonary trunk.

Resection of a large primary left ventricle tumour by cardiac autotransplantation in a 2-month-old infant: a case report.

BACKGROUND: Surgery is the fundamental method for the treatment of primary cardiac tumours. However, due to the inaccessibility of anatomy and the proximity of important structures, it is very difficult to completely resect tumours of the left atrium or left ventricle without damaging the normal tissues. Cardiac autotransplantation for the resection of cardiac tumours is carried out by taking out the heart from the body, resecting cardiac tumours, and then transplanting the heart back into the body. CASE SUMMARY: This article presents a successful case of cardiac autotransplantation for the complete resection of primary cardiac tumour in a 2-month-old infant and shares the noteworthy experience. DISCUSSION: Tumours located in the left atrium and left ventricle are difficult to be exposed because of their deep posterior location and proximity to important anatomical structures such as mitral valve and chordae tendineae. How to resect the tumours completely without damaging the normal tissues is a great challenge. This case proves that cardiac autotransplantation is a good solution for tumours that are difficult to be resected completely by orthotopic cardiac transplantation.

Management of pulmonary artery sling with tracheal stenosis: LPA re-implantation without tracheoplasty.

OBJECTIVES: Pulmonary artery sling (PA sling) is frequently associated with tracheal and/or bronchial stenosis. Most PA sling patients receive left pulmonary artery (LPA) re-implantation and tracheoplasty under the cardiopulmonary bypass, but the postoperative complications of tracheoplasty remain a great challenge. In this study, we reviewed 14 PA sling children who received surgery in our hospital, and tried to find out whether tracheoplasty could be avoided or not. METHODS: A total of 14 patients receiving surgery due to PA sling/tracheal stenosis were recruited. Complete tracheal ring was confirmed by fiberoptic bronchoscopy in all the patients preoperatively. The clinical outcome and the severity of trachea stenosis (tracheal diameter and length) were evaluated, and effectiveness of various managements was analyzed. RESULTS: Fourteen PA sling/tracheal stenosis children underwent surgical treatment. Three patients needed intubation and mechanical ventilation for severe respiratory symptoms preoperatively. Eight patients received LPA re-implantation alone to relieve the trachea compression, and slide tracheoplasty was performed in one patient for extubation failure who finally died of air leakage. Six patients received LPA re-implantation and tracheal intervention simultaneously. Three patients received slide tracheoplasty, and one was discharged after recovery. The remaining 3 patients received tracheal stent implantation, but finally died. The diameter/length (%) in the survivors without tracheal intervention was significantly higher than that in patients with tracheal intervention. CONCLUSIONS: Patients with PA sling undergoing LPA re-implantation achieved a good outcome. Diameter/length (%) may be a more reliable indicator used for determination of tracheal intervention in surgical management of PA sling.

Extracorporeal membrane oxygenation as a support for TGA/IVS with low cardiac output syndrome and pulmonary hemorrhage.

A 15-day-old neonate with complete transposition of the great arteries/intact ventricular septum was admitted with life-threatening hypoxemia and heart arrest. After successful resuscitation, heart beat recovered but blood lactate began to arise and maintained above 15 mmol/L 6 hours later. Emergency arterial switch operation was done at 20 hours after resuscitation. Planned extracorporeal membrane oxygenation support was employed postoperatively. The baby experienced severe pulmonary hemorrhage and severe hypoxemia after weaning from cardiopulmonary bypass, which were treated with extracorporeal membrane oxygenation support.

A combined ultrafiltration strategy during pediatric cardiac surgery: a prospective, randomized, controlled study with clinical outcomes.

OBJECTIVE: To investigate the clinical effects of a combined ultrafiltration strategy on the surgical treatment of pediatric patients with congenital heart diseases. DESIGN: A prospective, randomized, controlled study. SETTING: A single-institution study in an affiliated hospital of a university. PARTICIPANTS: Sixty-five pediatric patients who underwent open heart surgery with cardiopulmonary bypass (CPB) to treat congenital heart disease were enrolled. The participants were randomized into 2 groups: conventional + modified ultrafiltration (MUF) (CM) group and prime + zero-balanced + MUF (PZM) group. INTERVENTIONS: In the CM group (n = 33), conventional ultrafiltration was performed after removal of the aortic clamp, and MUF was performed after the completion of CPB. In the PZM group (n = 32), ultrafiltration was performed for the circuit prime solution, zero-balance ultrafiltration was performed after removal of the aortic clamp, and MUF was performed after the completion of CPB. MEASUREMENTS AND MAIN RESULTS: The blood gas parameters and tumor necrosis factor alpha content in the priming solution and perioperative blood samples were analyzed. Postoperative parameters, including mechanical ventilation time, respiratory indices, intensive care unit time, and hospital time, also were recorded. One hospital death occurred in each group. No severe complications occurred in either group. The lactic acid, glucose, and tumor necrosis factor alpha contents in the priming solution and perioperative blood samples were significantly lower in the PZM group compared with the CM group. The respiratory indices were statistically significantly better in the PZM group compared with the CM group in the early postoperative period. No significant differences were found between the 2 groups regarding the postoperative ventilation time, inotropic support, homologous blood transfusion, drainage, intensive care unit time, or postoperative hospital time. CONCLUSION: The combined use of ultrafiltration of prime solution, zero-balance ultrafiltration, and MUF strategy is associated with a modest improvement in pulmonary function compared with the combination of conventional and MUF strategies in the early postoperative period, but the principal clinical outcomes are similar.

Extracorporeal membrane oxygenation as a support for TGA/IVS with low cardiac output syndrome and pulmonary hemorrhage / Oxigenação extracorpórea por membrana como suporte para TGA/IVS com síndrome de baixo débito e hemorragia pulmonar

A 15-day-old neonate with complete transposition of the great arteries/intact ventricular septum was admitted with life-threatening hypoxemia and heart arrest. After successful resuscitation, heart beat recovered but blood lactate began to arise and maintained above 15 mmol/L 6 hours later. Emergency arterial switch operation was done at 20 hours after resuscitation. Planned extracorporeal membrane oxygenation support was employed postoperatively. The baby experienced severe pulmonary hemorrhage and severe hypoxemia after weaning from cardiopulmonary bypass, which were treated with extracorporeal membrane oxygenation support.

Um recém-nascido de 15 dias de idade, com transposição completa das grandes artérias/septo ventricular intacto, foi internado com hipoxemia com risco de vida e parada cardíaca. Após a reanimação bem sucedida, o coração voltou a bater, mas o lactato elevou-se e manteve-se acima de 15 mmol/L, 6 horas após. Operação de emergência para correção da transposição das grandes artérias foi realizada 20 horas após a ressuscitação. Suporte de oxigenação por membrana extracorpórea foi empregado no pós-operatório. O bebê apresentou hemorragia pulmonar e hipoxemia graves após desconexão da circulação extracorpórea, que foram tratadas com suporte da xigenação por membrana extracorpórea.

[Effects of pulmonary surfactant in treatment of acute lung injury after cardiac surgery in infants].

OBJECTIVE: Acute lung injury is a severe complication after cardiac surgery performed with cardiopulmonary bypass in infant patients. Pulmonary surfactant has been successfully used in treating neonatal respiratory distress syndrome for many years. This study focused on exploring the clinical efficacy of exogenous pulmonary surfactant in treating infant patients with acute lung injury after cardiac surgery with the use of extracorporeal circulation. METHOD: Twenty-three infants with ventricular septal defect (VSD) were enrolled in the study. None of the patients needed oxygen treatment nor ventilation before heart surgery. VSD repair operation was done under cardiopulmonary bypass, and acute lung injury was found postoperatively. Ten infant patients in the treatment group were given pulmonary surfactant by tracheal instillation during mechanical ventilating postoperatively. Thirteen patients in control group were randomly selected in the same period in hospital, and acute lung injury was diagnosed without pulmonary surfactant treatment after cardiopulmonary bypass. Blood samples were obtained from all the patients, and blood gas analysis was performed every 6 hours. Demographics (body weigh, age, gender, VSD diameter) and clinical characteristics (CPB time, oxygenation index, mean airway pressure, pH and PCO2) of all the patients were collected, and statistical analysis was done to compare the data between treatment and control group. RESULT: In the first 24 hours after heart surgery, compared with the control group, a more increased oxygenation index (from 89.36 ± 12.69 to 285 ± 16.51) was observed in treatment group, and it was from 93.71 ± 11.82 to 133 ± 19.62 in the control group. There was a significant difference in oxygenation index between the two groups (P < 0.05). At the same time, the MAP (from 17.5 ± 3.18 to 10.4 ± 3.37) of the patients in comparison with the control group (from 18.2 ± 2.63 to 13.8 ± 2.55), a more significantly decreased MAP was observed in the treatment group (P < 0.05). There was no significant difference in pH and PCO2 between the two groups. The ventilation time in cardiac care unit was shorter in treatment group (17.43 ± 9.12) h compared with the control group [(30.97 ± 14.85) h, P < 0.05]. ICU stay time of treatment group (3.90 ± 1.34) d was shorter than that of control group [(6.18 ± 1.90) d, P < 0.05].Two infants of the control group died, but none in treatment group died. CONCLUSION: In this study, a satisfactory curative effect was observed for the treatment of acute lung injury with PS intratracheal instillation after heart surgery under cardiopulmonary bypass in infant patients. It can reduce the duration of mechanical ventilation and cardiac care unit stay and improve prognosis. In addition, this study was a pilot study and the limited sample size was probably the cause of insufficient statistical power. Further study of larger scale is needed.

[Extracorporeal membrane oxygenation for severe acute respiratory and heart failure in a child with severe pneumonia].

OBJECTIVE: To report clinical application of Extracorporeal membrane oxygenation for severe acute respiratory and heart failure in a child with severe pneumonia. METHOD: A seven-year old male patient with severe pneumonia complicated with heart and lung function failure was admitted to PICU in 28th of December, 2008.Veno-artery access was set up via euthyphoria cannulation in operative incision. Blood was drained from the right atrium through a cannula introduced via femoral veins, and returned via femoral artery. The inter-surface of the ECMO equipment system was completely coated with heparin-coating technique. Anticoagulation was maintained with heparin to keep the activated clotting time (ACT) between 150 and 200 seconds and heparin usage dose was 10 U/(kg.h), mean blood flow was 1/2-2/3 of 80-120 ml/(kg.min) during ECMO assistant period. During ECMO, ventilator settings were gradually reduced to allow lung rest, i.e. peak inspiratory pressure less than 25 cm H2O (1 cm H2O=0.098 kPa), end expiratory pressure 8-10 cm H2O, rate 10-15 breaths per minute and FiO2 30%-40%. RESULTS: In management of ECMO, the incipient blood flow was set at 0.8 L/min, the radio of oxygen and blood flow was 1:1, FiO2 60%. After ten minutes of ECMO working, the blood oxygen saturation of radial artery increased from 40 mm Hg (1 mm Hg=0.133 kPa) to 177 mm Hg, Lac decreased from 3.5 mmol/L to 2.8 mmol/L. Four hours later, blood gas analysis of radial artery showed PaO2 202 mm Hg, PCO2 44 mm Hg, Lac 1.5 mmol/L, blood flow was set at 0.6 L/min, FiO2 60%, PaO2 kept above 150 mm Hg. 96 hours after ECMO supporting, the blood flow was set at 0.4 L/min [20 ml/(kg.min)], the results of blood gas analysis of radial artery was PaO2 190 mm Hg, PaCO2 36 mm Hg, SaO2 100%, Lac 0.9 mmol/L, then the child weaned off successfully from ECMO. Two days later, the child was successfully extubated. After two weeks treatment, the patient was discharged. The main complication associated with extracorporeal membrane oxygenation were bleeding. CONCLUSION: ECMO is an effective mechanical assistant therapy method for severe pulmonary and cardiac failure in a child.

Tissue-engineered heart valve on acellular aortic valve scaffold: in-vivo study.

The feasibility of constructing a tissue-engineered heart valve on an acellular porcine aortic valve leaflet was evaluated. A detergent and enzymatic extraction process was developed to remove the cellular components from porcine aortic valves. The acellular valve leaflets were seeded for 7 days in vitro with cells from canine arterial wall and endothelial cells. The constructs were implanted into the lumens of 6 canine abdominal aortas to assess the reconstruction of the valve leaflets. It was found that all cellular components had been removed from the porcine aortic valves. The valve leaflets were completely reconstructed at the end of the 10th week in vivo. Scanning electron microscopy showed that the valve leaflets were partially covered with endothelial cells. It was concluded that porcine aortic valves can be decellularized by the detergent and enzymatic extraction process and it is feasible to construct a tissue-engineered heart valve in vivo on an acellular valve scaffold.