Objective To evaluate the results of surgical treatment for patients with heterotaxy syndrome and cardiac anomaly.Methods From September 2008 to October 2013,34 patients with heterotaxy syndrome underwent 46 cardiac operations at Guangzhou Women and Children Medical Center.22 were male,and 12 female.Mean age at operation was 22 months (4 months to 14 year).There were right atrial isomerism 24 cases,left atrial isomerism 10 cases.Only one had a single VSD,and others were all with complex cardiac anomaly,including complete atrioventricular canal defect 26 cases,tricuspid atresia 4 cases,mitral atresia 1 case,anomalous pulmonary venous connection 18 cases,pulmonary venous stenosis 4 cases,double superior vena cava 17 cases,and interrupted hepatic portion of the inferior vena cava 5 cases.Fontan procedure was performed in 5 patients,bidirectional Glenn in 13,Kawashima in 3,central shunt in 1,atrioventricular valve replacement in 5,and pulmonary artery banding in 5.Results There were 4 died during hospitalization.The follow-up duration was 1 month to 5 years.There were 2 died.The early to middle term mortality was 17.6％ (6/34).In the same period the mortality for 103 patients without heterotaxy syndrome underwent univentricular repair was 4.8％ (5/103).There was significant statistical difference (P ＜ 0.05).Conclusion Right heart bypass operation remains the preferred palliative procedure for patients with heterotaxy syndrome,and the eraly and middle term results were satisfied.

Objective To evaluated the early and mid-term results of arterial switch operation (ASO) for patients with intramural coronary artery.Methods From September 2008 to March 2012,75 patients underwent ASO at Guangzhou Women and Children Medical Center for repair of transposition of the great arteries and Taussig-Bing anomaly.Among these patients,7patients (9.3％) had an intramural coronary artery.Mean age at operation was 2.4 months (2 days to 1 year) and mean body weight was(4.3 ±2.2) kg.The TGA and VSD in 3 cases,TGA/IVS in 2,and Taussig-Bing anomaly in 2.Among them 3 patients had an aortic arch anomaly,interruption of the aortic arch in 1 and coarctation of the aorta in 1.The individual coronary button technique was used in coronary transfer in 7 patients,of whom one patient required to unroof the intramural segment,an-other one required to unroof the intramural segment and enlarge with autologous pericardium the because of myocardial ischemia.There was 1 operative death because of low cardiac output syndrome.This patient underwent a coronary transfer combining aortic arch repair but without unroofing the stenotic intramural segment.The mortality was 14.2％.In the same period the mortality for 68 patients without an intramural coronary artery was 4.4％ (3/68).There was no statistical difference in mortalitv between the patients with and without an intramural coronary artery (P ＞ 0.05).Results 6 patients follow-up 4 to 47months.There was no late death.No intramural coronary artery obstruction was identified by cardiac computerize temography.All patients had normal ventricular function and were in NYHA class Ⅰ during follow-up.The intramural coronary artery is well known as a risk factor of ASO.Conclusion The technique of coronary transfer should be individually adapted to each anatomical situation.Individual technique for coronary transfer has excellent results.

Objective To review the outcomes of atrioventricular valve replacement in patients with functional single ventricle and evaluate risk factors for mortality.Methods Retrospectively reviewed our experience with atrioventricular valve (AVV replacement) in patients with functional single ventricle from January 2008 to December 2014.Outcome included mortality and valve-related complications.Results Thirteen patients required AVV replacement.Prosthetic valve type was St.Jude bileaflet mechanical mitral valve.Valve size ranged from 27 to 31 mm,including 27 mm in 2,29 mm in 7,and 31 mm in 4 patients.Median age at valve replacement was 4.5 years(range:1.2 years to 18.0 years).Concomitant procedures included bidirectional Glenn shunt in 3,pulmonary artery banding in 1,and repair of pulmonary vein stenosis in 1 patient.There were four early deaths with a perioperative mortality of 30.8％.Complications after AAV replacement included complete atrioventricuiar block in 1,intracerebral hemorrhage in 1 patient due to valve-related anticoagulation.No patient had perivalvular leakage.There was one late death during a mean follow-up of 3 years (range:0.5-5.0 years).Five-year Kaplan-Meier survival was 61.5％.Fisher exact probability test showed that lower weight(＜ 10 kg) at operation and prosthetic size/weight ratio ＞ 2.0 were risk factors for overall mortality.Of the survival patients,functional status is NYHA class Ⅰ in 3,class Ⅱ in 5.No patient developed valve-related complications.Conclusion Atrioventricular valve replacement can be performed in patients with functional single ventricle with acceptable mortality.Lower weight and increased prosthetic size/weight ratio at operation were significantly associated with worse survival.

OBJECTIVETo discuss the experience of surgical treatment of total anomalous pulmonary venous connection (TAPVC) in infants.

METHODSThe clinic data of 84 cases with TAPVC under 6 months of age underwent surgical treatment at Department of Cardiac Surgery, Guangzhou Women and Children's Medical Center from January 2012 to October 2015 were analyzed retrospectively. There were 58 male and 26 female patients. The patients were aged 1 days to 6 months with a mean of (2.4±2.2) months at surgery, including 22 newborns. Body weight was 1.8 to 6.8 kg with a mean of (4.3±1.2) kg. There were 24 cases of intracardiac type, 46 cases of supracardiac type, 10 cases of infracardiac type and 4 cases of mixed type. There were 26 cases received emergent operation. There were 14 cases used Sutureless technique in operations and 46 cases used conventional methods in the no-intracardiac type cases, and 2 cases enlarged the anastomsis with autologous pericardium. According to the condition, corrective surgeries of other anomalies were performed in the meantime, including 3 Warden operations (right side), 3 bilateral bidirectional Gleen operation, 2 correction of unroofed coronary sinus syndrome, 1 coarctation of aorta correction with deep hypothermic circulation arrest, and 1 repair of ventricular septal defect.

RESULTSThe ratio of newborn was higher in Sutureless technique group than in conventional methods group (7/14 vs. 32.6%, χ(2)=4.927, P=0.043), and mean age was less ((1.8±0.4) months vs. (2.4±2.2) months, F=4.257, P=0.042), but there were no difference in body weight, cardiopulmonary bypass time and aorta clamped time between the two groups. Followed up for 1 to 46 months, 10 cases (11.9%) died overall and the mortality of intracardiac (3/10) and mixed (2/4) type were much higher than in intracardiac (4.2%) and supracardiac (13.0%) type. The mortality were no difference between newborn and infants, or whether emergent operation, or Sutureless technique and conventional methods. The maximal pulmonary venous flow velocity was abnormal speed-up >1.8 m/s at 1 week and 1 to 3 months post-operation mostly.

CONCLUSIONSThe mortality of TAPVC was differed by different types. Intrinsic pulmonary vein stenosis maybe the main cause of mortality. The high quality of anastomsis could reduce the operative mortality.

Aortic Coarctation ; Body Weight ; Cardiac Surgical Procedures ; methods ; Circulatory Arrest, Deep Hypothermia Induced ; Female ; Heart Defects, Congenital ; mortality ; surgery ; Heart Septal Defects, Ventricular ; Humans ; Infant ; Infant, Newborn ; Male ; Postoperative Period ; Pulmonary Veins ; pathology ; surgery ; Retrospective Studies

Objective: To review the early and mid-term results of end-to-side anastomosis technique for interrupted aortic arch in neonates and infants. Methods: Clinic data of 46 patients were diagnosed as interrupted aortic arch in Department of Cardiac Surgery, Guangzhou Women and Children′s Medical Center between January 2010 and December 2016 were analyzed retrospectively. Twenty-six cases were neonates. The median age underwent surgery was 23 days (range: 2 days to 8 years). Anatomical subtypes included 36 cases of type A and 10 cases of type B. There was no type C case. The reconstruction of the aortic arch was completed by an extended end-to-side anastomosis technique between the descending aorta and the undersurface of the proximal aortic arch. In 42 patients, all with intracardiac anomalies, had concomitant complete repair of intracardiac anomalies through a median sternotomy. The remaining 4 patients, all without intracardiac anomalies, an end-to-side anastomosis was constructed through a left thoracotomy. During follow-up, aortic arch recurrent obstruction, left ventricular outflow tract obstruction (LVOTO) and tracheal stenosis were focused. Results: There were 3 surgical deaths, with amortality rate of 6.5%. The remaining 43 patients survived after surgery. In 39 of these patients, deep hypothermic cardiac arrest (DHCA) strategy was used for brain protection, and the mean time of DHCA was (16±3) minutes. Eight patients underwent delayed sternal closure. The mean mechanical ventilation time and ICU stay time were (3.4±1.6) days (range: 2 to 12 days) and (6.4±2.7) days (range: 3 to 16 days) respectively. In 16 patients, all with tracheal or bronchial stenosis before surgery, mechanical ventilation was successfully evacuated, and no new airway stenosis occurred. There was no residual pressure difference between upper and lower extremity arterial blood pressure at discharge. Echocardiography showed normal arterial blood flow velocity in aortic arch. At a mean follow-up of (36.2±18.9) months (range: 6 months to 7 years), there were two patients lost and one late date. Four patients developed a recurrent stenosis at the aortic arch, of which two were severe, and the other two were mild. In 2 patients, both with mild LVOTO before surgery, no significant increase in the degree of obstruction was found during the follow-up. Two patients developed new mild to moderate LVOTO without clinical symptoms, and continued to follow up. In all patients, the tracheal or bronchial stenosis were extenuated, and there was no new progressive airway stenosis by regular bronchoscopy. Conclusions The end-to-side anastomosis technique for the reconstruction of the aortic arch achieved excellent early and mid-term results in neonates and infants suffered from interrupted aortic arch. Reducing the anastomotic tension by extensive mobilization is the key to prevent postoperative early complications and late recurrent arch obstruction.

Objective: To investigate the efficacy of multistage surgery in patients with functional single ventricle (FSV) and risk factors of postoperative death. Methods: The clinical data of all consecutive patients with FSV undergoing multistage single ventricle palliation surgery in Guangzhou women and children's medical center from January 2008 to December 2017 were retrospectively reviewed. The study included 289 patients. The age was 10.0 (6.0, 35.4) months,and there were 198 male and 91 female patients. The patients were followed up at outpatient clinic. Survival rates were calculated with Kaplan-Meier. Multivariate Cox regression analysis was made to determine the risk factors of postoperative death. Results: Seventy-nine patients required the first stage palliation surgery, 232 patients received the Glenn shunt surgery, and 162 patients completed the Fontan procedure. Overall, postoperative death occurred in 39 patients including 21 after the first stage palliation surgery (early stage 13 cases, late stage 8 cases) , 10 after the Glenn shunt surgery (early stage 5 cases, late stage 5 cases) , and 8 following the Fontan procedure (early stage 6 cases, late stage 2 cases) . Kaplan-Meier analysis showed that survival rate of the entire cohort was 90.2% (95%CI 86.7%-93.7%) , 85.9% (95%CI 81.8%-90.0%) ,and 84.6% (95%CI 79.7%-89.5%) at 1 year, 5 years and 10 years post operation. Survival rate was 74.4% (95%CI 64.8%-84.0%) , 73.0% (95%CI 63.2%-82.8%) , and 73.0% (95%CI 63.2%-82.8%) at 1 year, 5 years, and 10 years post the first stage palliation surgery, 97.8% (95%CI 95.8%-99.7%) , 95.2% (95%CI 92.3%-98.1%) , and 95.2% (95%CI 92.3%-98.1%) at 1 year, 5 years, and 10 years post Glenn shunt surgery, 95.6% (95%CI 92.5%-98.7%) and 93.7% (95%CI 88.8%-98.6%) at 1 year and 5 years post Fontan surgery. Multivariate Cox regression analysis revealed that total anomalous pulmonary venous connection (HR=5.47, 95%CI 2.71-11.04, P<0.001), atrioventricular valve regurgitation more than moderate (HR=2.52, 95%CI 1.32-4.79, P=0.005), systemic ventricular outflow tract obstruction (HR=3.47, 95%CI 1.30-9.29, P=0.013), and required the first palliation surgery (HR=3.12, 95%CI 1.59-6.15, P=0.001) were risk factors of postoperative death. Conclusions The multistage surgery can effectively improve the survival of patientswith functional single ventricle and is associated with satisfactory long-term prognosis. Total anomalous pulmonary venous connection, atrioventricular valve regurgitation more than moderate, systemic ventricular outflow tract obstruction, and required the first palliation surgery are risk factors of postoperative death in these patients.

Objective To summarize the surgical results of patients with quadricuspid aortic valve and aortic regurgitation.Methods From June 2013 to June 2017,4 patients with incompetent quandricuspid aortic valve underwent surgical repair at Guangzhou Women and Children's Medical Center.The age at surgery was 2 months to 5 years,and body weight was 2.7-22.7 kg.3 patients were diagnosed with persistent tmncal arteriosus and underwent complete repair.Another one was diagnosed with tetralogy of Fallot and accepted complete repair 4 years age.All patients were diagnosed with more than moderate quandricuspid aortic valve regurgitation.Repair was performed by tricuspidalization of the native quadricuspid valve,using leaflet and related sinus of Valsalva excision.Results There was no mortality.The ICU stay and hospital stay after operation were 7-12 days and 10-16 days.The follow-up duration was 3 to 51 months.All patients were alive and free from significant aortic valve regurgitation.Conclusion Aortic valve remodeling by leaflet excision and reduction annuloplasty is an effective method for incompetent quadricuspid aortic valve repair.

Objective: To review current-era palliation outcomes of patients with heterotaxy and functional single ventricle in a single institution. Methods: The clinical data of 70 consecutive patients with heterotaxy undergoing multistage single ventricle palliation in Guangzhou Women and Children′s Medical Center from January 2008 to December 2017 were retrospectively reviewed, and the prognosis factors for mortality were analyzed. There were 53 male and 17 female patients.The median age was 13.3 months (range: 6 days to 150 months). Single ventricle multistage palliation included 1^st stage palliation surgery, 2^nd bidirectional Glenn shunt, and 3^rd stage modified Fontan.The Kaplan-Meier method was used to estimate the probability of survival. Multivariate analysis was performed by Cox regression model. Results: Sixty-two patients had right atrial isomerism while eight had left atrial isomerism. Eighteen patients (25.7%) required the first stage palliation. Fifty-five patients received the Glenn shunt, and the Fontan procedure was completed in 27 patients. Overall, mortality occurred in 17 patients (24.3%) including 9 after the first stage palliation, 6 after the Glenn shunt, and 2 following the Fontan procedure. Survival estimates for the entire cohort following surgery were 81% (95%CI: 72% to 90%), 74%(95%CI: 64% to 85%), and 74% (95%CI: 64% to 85%) at 1 year, 5 years and 10 years, respectively. Survival estimates following the first stage palliation were 56% (95%CI: 33% to 79%) and 49% (95%CI: 26% to 73%) at 1 year and 5 years, respectively. Multivariate Cox regression analysis revealed that total anomalous pulmonary venous connection (TAPVC) (HR=6.16, 95%CI: 1.65 to 22.95, P=0.007), atrioventricular valve regurgitation more than moderate (HR=3.81, 95%CI: 1.32 to 10.94, P=0.013) and required the first palliation surgery (HR=4.58, 95%CI: 1.34 to 15.72, P=0.016) were prognosis factors for overall mortality. Conclusions The management of heterotaxy patients with functional single ventricle remains challenging, and the outcomes are continously improving in china. TAPVC, atrioventricular valve regurgitation more than moderate and required the first palliation surgery still are prognosis factors for overall mortality in heterotaxy syndrome.

Objective: To evaluate the early- and mid-term outcomes of surgical atrioventricular valve (AVV) intervention in patients with functional single ventricle (FSV). Methods: The clinical data of 40 consecutive FSV patients who underwent surgical AVV intervention between January 2008 and December 2017 at Department of Cardiac Surgery, Guangzhou Women and Children′s Medical Center, Guangzhou Medical University were reviewed retrospectively. There were 25 male and 15 female patients. The median age at AVV intervention was 4.5 to 204.0 months (M(Q_R): 42.5 (59.7) months), and the median body weight was 6.0 to 55.4 kg (M(Q_R): 13.8 (8.1) kg). The systemic AVV morphology included common AVV in 21 patients, mitral valve in 7 patients, tricuspid valve in 3 patients, and both mitral and tricuspid valve in 9 patients. At first surgical intervention, 15 patients had severe, 25 patients had moderate AVV regurgitation. The timings of the initial surgical intervention were at the first palliative, Glenn, between Glenn and Fontan, and Fontan stage in 5, 23, 4, and 8 patients, respectively. The methods of the initial surgical intervention were AVV repair in 31 patients, AVV replacement in 9 patients. Actuarial survivals were estimated by the Kaplan-Meier method. Prognosis factors for atrioventricular valve reoperation were analyzed using the Cox regression hazard model. Results: There were 6 early deaths, with a mortality of 15.0%. Thirty-four survival patients received a follow-up of 1 to 117 months (M(Q_R): 44 (34) months). Survival of patients after the initial surgical intervention were 85.0% (95% CI: 74.0% to 95.9%), 79.3% (95% CI: 66.6% to 92.0%), and 79.3% (95% CI: 66.6% to 92.0%) at 1 year, 5 years and 10 years, respectively. In all, only 13 patients completed the Fontan procedure. Six patients underwent 8 reoperations, including AVV replacement in 5 patients, redo AVV repair in 3 patients. Freedom from AVV reoperation at 1 and 5 years was 89.8% (95% CI: 78.8% to 100%) and 79.4% (95% CI: 64.7% to 94.1%), respectively. Multivariate Cox regression analysis revealed that common AVV (HR=3.53, 95% CI: 1.63-7.67, P=0.020) was the prognosis factors for AVV reoperation. Conclusions The early- and mid-term outcomes of surgical AVV intervention in FSV patients are not satisfactory. The mortality and reoperation rate are still high, and common AVV predicts the AVV reoperation after the initial surgical intervention.

Objective We reviewed our experience with modified Fontan in patients with functional single ventricle and apicocaval juxtaposition with a special focus on route selection and outcomes.Methods From January 201 1 to April 2016,31 patients with functional single ventricle and apicocaval juxtaposition underwent modified Fontan procedure at our hospital.Mean age at operation was(58.5 ± 32.4) months(ranged 16 months to 14 year),and the mean body weight was (16.2 ± 6.0) kg (range,9.1-40.3 kg).There were right atrial isomerism 7 cases,complete atrioventricular canal defect 9 cases,≥moderate atrioventricular valve regurgitation 4 cases,total anomalous pulmonary venous connection 8 cases,and anomalous hepatic venous connection 4 cases.Fontan procedure was performed in 5 patients,bidirectional Glenn in 13,Kawashima in 3,central shunt in 1,atrioventricular valve replacement in 5,and pulmonary artery banding in 5.Results In 24 patients,the extracardiac conduit was placed between the inferior vena cava(IVC) and the ipsilateral pulmonary artery behind the ventricle.In the 5 other patients,the conduit was placed between the IVC and the contralateral pulmonary artery.In another 2 patients the tube graft was anastomosed with the contralateral pulmonary artery(intraextracardiac Fontan).After operation,the peripheral blood oxygen saturation was 0.90 ±0.06.Five patients suffered pleural effusion,and 1 patient died of infection during hospitalization.The follow-up duration was(17.5 ± 15.4) months(1-65 months).There was no late death and reoperation.Conclusion Modified Fontan procedure in functional single ventricle and apicocaval juxtaposition can be carried out with favorable midterm outcomes.Care must be taken in regard to critical conduit oppression by the ventricle or compression on pulmonary veins.