Objective: To explore the surgical strategy for Ebstein anomaly in children. Methods: From January 2003 to December 2015, a total of 141 cases of Ebstein anomaly were treated at Department of Pediatric Cardiothoracic Surgery, Shanghai Children′s Medical Center, School of Medicine, Shanghai Jiaotong University. There were 65 male and 76 female patients, with age of (6.9±1.6) years (ranging from 10 months to 15 years), weight of (19.6±4.7) kg (ranging from 6.5 to 59.0 kg). All patients were diagnosed by 2 dimensional Doppler echocardiography and the septal leaflet and posterior leaflet displaced downward from 1.0 to 5.0 cm. The tricuspid valve regurgitation (TR) were mild in 26 cases, moderate in 46 cases and severe in 69 cases. Tricuspid valvuloplasty were performed in 131 cases (94 cone reconstruction, 37 valve hoist), tricuspid valve replacement in 2 cases and tricuspid valve closed in 8 cases. Surgical strategy were divided into biventricular heart function in 77 cases, one and a half ventricular heart function in 56 cases, and single ventricular heart function in 8 cases. Results: Three patients were changed to one and a half ventricular repair from biventricular repair due to unstable hemodynamics in the early postoperative period. One case died in biventricular group. The complete atrioventricular block were occurred in 3 patients and pacemaker were applied. One hundred and forty cases discharged from hospital. There were mild TR in 118 cases, moderate in 14 cases and closed in 8 cases. One hundred and thirty-seven cases were followed up regularly in 18 to 172 months. Ninety-one cases were treated by cone reconstruction (mild TR in 75 cases, moderate in 15 cases and severe in 1 case). Thirty-six cases were operated by tricuspid valve hoist (mild TR in 21 cases, moderate in 12 cases and severe in 3 cases). In the patients with severe TR (4 cases), 3 cases were reoperated by cone reconstruction. One case′s valve was closed because of the dysplasia of the anterior valve and then from one and a half ventricular heart function to single ventricular function, the oxygen saturation was increased. Two patients underwent tricuspid valve replacement, 1 died and the other′s mechanical valve was removed, and changed to single ventricular function repair. Conclusions Although tricuspid cone reconstruction can achieve good results, the stable hemodynamic of early postoperative can be effectively maintained by using the surgical strategy of one and a half ventricular repair. To the patients with severe tricuspid regurgitation and hypoxemia due to severe tricuspid valve dysplasia, transforming to a functional single ventricle may be the only choice when there comes to the unstable hemodynamic.

Objective To investigate the therapeutic effect of the transcatheter closure of antegrade pulmonary blood flow with high-risk Fontan operations.Methods Retrospective analysis of 6 Fontan surgical patients, 4 males and 2 females, who received surgical treatment in the cardiothoracic surgery of Shanghai Children's Medical Center from May 2016 to August 2018;Age(5.7 ±2.9) years;(19.8 ±5.5) kg weight.All 6 patients were treated with primary pulmonary artery banding operation and secondary bilateral Glenn operation(BDG) and re-PAB.Before Fontan surgery, cardiac catheterization was performed in the department of cardiology to close the antegrade pulmonary blood flow .Results These six patients received interventional therapy in the department of cardiology.The mean pulmonary artery pressure(Pp) before occlusion was(17.0 ±0.8) mmHg (1 mmHg=0.133 kPa), the ventricular diastolic pressure(VEDP) was (11.2 ±0.9) mmHg, and the mean pulmonary artery pressure after occlusion was effectively decreased to(14.2 ±0.7) mmHg and VEDP to(9.7 ±0.7) mmHg.Fontan surgery was performed 6 to 12 months after the occlusion.No death was found after the operation, and all the clinical indicators were normal after the operation.Follow-up was conducted for 2-24 months, and the cardiac function and the function of each organ recov-ered well.Conclusion For some patients with high risk Fontan before operation , this method can safely and effectively reduce the average pulmonary artery pressure and reduce the ventricular and pulmonary vascular load , which is of positive help for the successful Fontan operation.

Objective:To investigate the pathology characteristics, so as to provide treatment experience of primary cardiac tumors for pediatric patients.Methods:A retrospective study was conducted for 135 patients with primary cardiac tumor between January 2004 and December 2017 in Shanghai Children′s Medical Center.The median age was 0.54 years (range, 0-14.36 years). Single tumor was discovered in 61 cases and multiple rumors were found in 74 cases.Forty-five patients presented obvious clinical symptoms, 4 patients with mild symptoms and 86 patients without symptoms.Forty-one patients underwent surgical treatment, including complete resection of the tumor in 24 cases, partial resection in 16 cases and heart transplantation in 1 case.Concomitant valvuloplasty was required in 13 patients.Ninety-four patients did not receive surgical treatment.Results:A total of 40 patients underwent surgical treatment in Shanghai Children′s Medical Center.Three patients died of low cardiac output (in-hospital mortality: 7.5%), and 2 patients recovered from postoperative low cardiac output.The hemodynamic status was stable in the remaining 35 cases.One patient who received heart transplantation in another hospital survived and had good cardiac function during 24 months follow-ups.There were no significant differences in the survival rate between partial resection and complete resection of benign tumors.One late death was observed in patients with malignant tumors for 2 years after operation.Surveillance was kept in 94 non-surgical patients, among whom 1 case died for the abandon of surgery and 1 case died when waiting for heart transplantation.Other 2 patients were waiting for heart transplantation and other 90 patients had no hemodynamic disorder.Conclusion:Most of primary cardiac tumors are benign and long time follow-up should be able to get on for pediatric patients.When patients develop hemodynamical obstruction, arrhythmia or malignant tumor, surgical treatment is necessary.The principle of surgical therapy in these patients is to restore normal hemodynamic status, instead of completely removing tumor.Heart transplantation is potentially the only way for patients whose tumor cannot be resected.

Objective:To summarize the experience and effect of mitral valvuloplasty in the treatment of mitral valve disease in infants.Methods:The clinical data of 140 infants with mitral valve disease from June 2010 to June 2020 were retrospectively analyzed. There were 62 males and 78 females, with body weight of(6.4±1.4)kg and age of(196.6±80.1)days. Among them, 131 cases were moderately or above mitral insufficiency, and 9 cases were mitral stenosis. The perioperative and follow-up clinical data were recorded, and the therapeutic effect and prognostic factors of mitral valve plastic surgery were analyzed.Results:All 140 children were received surgery of mitral valve repair under cardiopulmonary bypass. Cardiopulmonary bypass time was(79.1±41.9)min, aortic cross clamp time was(46.8±20.0)min, 7(5%)early death. Mechanical ventilation time was(74.2±149.8)h. After surgery, mild mitral regurgitation was found in 48 cases, mild-moderate regurgitation in 53 cases, moderate regurgitation in 32 cases, moderate-severe regurgitation in 6 cases, severe regurgitation in 1 case, with no mitral stenosis. During the follow-up period of 6-126 months, 3 cases died in the long term, and 11 cases were reoperated. Freedom from >moderate mitral regurgitation at 5 years after surgery was 81.2%. Longer cardiopulmonary bypass time and postoperative mitral regurgitation were risk factors for death and moderate or above regurgitation at the follow-up.Conclusion:Mitral valvuloplasty can effectively treat the mitral regurgitation and stenosis in infants. The prognosis of children with short cardiopulmonary bypass time and good valve plastic effect is better. For infants with Carpentier type I, good shaping effect can be achieved by mitral annuloplasty alone, while for type Ⅱ and type Ⅲ, treatment should be tailored to deal with the problems at all levels of the valve, and additional annuloplasty should be performed.

Objective:To summarize the surgical outcomes of interrupted aortic arch with ventricular septal defect (IAA/VSD) in neonates and infants.Methods:This was a retrospective review of clinical data of 123 neonates and infants who received surgery for IAA/VSD from Jan 2009 to Jan 2019. Median age at repair was 48 days. Forty-four patients (36%) were neonates. One-hundred-and-twenty-two patients (99%) underwent standard aortic arch reconstruction with VSD closure, and one neonate (1%) underwent staged Yasui operation. Risk factors for early mortality was analyzed by decision tree model.Results:Early mortality after surgery was 13%. Duration of cardiopulmonary bypass longer than 135 min, surgery received during neonatal period and before 2016 was identified as higher risk group for mortality. Median follow-up time was 3.5 years (range, 1-10 years). Freedom from aortic arch obstruction at 6 months, 1 year, 5 years after surgery was 75%, 72% and 72% respectively. Freedom from left ventricular outflow tract (LVOT) obstruction at 6 months, 1 year, 5 years after surgery was 91%, 83% and 73% respectively. A total of 17 patients received 21 reoperations. The patient who received Yasui operation experienced no residual obstruction during the follow-up.Conclusion:Early outcomes after surgery for IAA/VSD in neonates and infants are satisfactory. However, patients with standard aortic arch reconstruction have a higher risk for aortic and LVOT obstruction, and require multiple reoperations.

Objective:To compare the results of surgical valvuloplasty and balloon dilation in patients with aortic stenosis (AS) at our center.Methods:We retrospectively reviewed the clinical data of children diagnosed with AS, underwent aortic repair or balloon dilation from January 2008 to December 2019. Seeking to analyze the early and long-term valvular function, evaluate mortality and freedom from reoperation by Kaplan- Meier method and figure out the risk factors by logistic regression. Results:There were 116 patients having received surgical valvuloplasty (SAV) and 64 having received balloon dilation (BD). There were no significant differences between two groups respect to median age, weight, preoperative peek gradient, aortic insufficient degree, left ventricular ejection fraction left ventricular end-diastolic dimension Z-score and aortic anulus diameter Z-score ( P>0.05). SAV achieved better gradient reduction and freedom from reoperation than did BD. The mean follow-up time was (5.2±3.1) years in SAV and (4.5±2.1) years in BD. Survival at 10 year was comparable between 2 groups ( P=0.51). Freedom from reoperation at 10 years was 59.6% in SAV and 49.7% in BD respectively ( P=0.01). Multivariate analysis revealed that BD contributed to subsequent reoperation ( P=0.05). Conclusion:SAV and BD both significantly relieved aortic stenosis and obtained good survival benefit. SAV yielded better gradient reduction and lower reoperation risk. BD might increase the hazard of reoperation.

OBJECTIVETo review the experience of left ventricular assistance device (LVAD) using for anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) in perioperative period.

METHODSThere were 29 patients with ALCAPA underwent surgical repair from May 2006 to May 2013. The mean age was 6.5 months (ranging from 3.3 to 12.1 months). The mean weight was 6.2 kg (ranging from 4.1 to 9.5 kg). Diagnosis was established by echocardiography in all patients. There were clinical symptomatic of the severe heart dysfunction and ejection fraction were 23% to 45%. Mitral insufficiency was moderate to severe in 8 patients and less than moderate in others. Surgical methods included the intrapulmonary tunnel (Takeuchi procedure) of 4 cases, direct reimplantation of the left coronary artery onto the aorta of 6 cases and reimplantation by pericardiac patch enlarge of 19 cases. Valvuloplasty were performed in 5 patients with mitral severe insufficiency. Twenty-two patients were treated only by medicine therapy. LVAD was used in 7 patients: there were 3 patients with low blood pressure at the end of surgical repair and 4 patients with low cardio output within 24 hours postoperatively.

RESULTSPostoperatively, transesophageal echocardiography demonstrated that blood flow of the left coronary artery is fluently but left ventricular is also largement. The hemodynamic of 18 patients was stable in medicine group but 3 patients were sudden died of low cardiao output and ventricular fibrillation respectively. One patient was died of diffuse intravascular coagulation at the time of 72 hours after operation. The hemodynamic was stable in 6 patients in LVAD group and the devices after using time from 72 to 108 hours was taken down except one patient died of multi-organ dysfunction. The hospital mortality was 5/29 (17.2%). Nineteen survival (19/24) was followed up of 3.5 years (ranging from 1 to 7 years). Reoperations was performed for one patient with the supravalvar pulmonary stenosis due to the Takeuchi procedure 4 years postoperatively. Echocardiographic demonstrated that the blood flow of the left coronary artery are fluently. Mitral insufficiency was moderate in 2 cases, mild to moderate in 9 cases and mild in 8 cases. The ejection fraction value were 43% to 55% and apparent arrhythmia didn't occur.

CONCLUSIONSAlthough late results are satisfactory and left ventricular function always recovery, early mortality is higher even though the protective methods are carried out during the whole cardiopulmonary bypass procedure. In order to decrease the early mortality, heart function evaluation and LVAD should be used as an effective cardiac support technique to prevent heart failure in time.

Aged ; Aorta ; Bland White Garland Syndrome ; Cardiopulmonary Bypass ; Coronary Vessel Anomalies ; surgery ; Heart ; Heart Failure ; Hospital Mortality ; Humans ; Mitral Valve Insufficiency ; Perioperative Period ; Postoperative Care ; Prostheses and Implants ; Pulmonary Artery ; abnormalities ; Reoperation ; Treatment Outcome ; Ventricular Function, Left

Objective    To explore the experience of surgical repair for congenital aortic stenosis (AS) in our center. Methods    We retrospectively reviewed the clinical data of 145 children diagnosed with AS, who underwent aortic repair from 2008 to 2019, with or without aortic insufficiency (AI), including 104 males and 41 females with a median age of 2.9 (0.6, 7.8) years. The preoperative and intraoperative data, early and long-term valvular function, long-term survival rate and freedom from reoperation and aortic valve replacment (AVR) were analyzed. Results    There were 120 patients receiving commissurotomy, 15 valvuloplasty with extra pericardium patch, and 25 AVR. The mean follow-up time was 0.25-11.20 (4.2±2.8) years. Survival rate at 10 years was 92.3%. Freedom from reoperation and AVR was 63.2% and 60.4%respectively. Multivariate analysis revealed that using a patch during surgery (P=0.036) was an independent risk factor for reoperation. A history of preoperative balloon dilation (P=0.029) and significant preoperative AI (P=0.001) contributed to AVR. Conclusion    Surgical treatment of congenital aortic valve stenosis in children is a safe and effective method that provides enough time to achieve a more definitive solution. Using a patch during surgery increases reoperation hazard. A history of previous balloon dilation and significant preoperative AI may result in AVR during the follow-up.