Objective To investigate the surgical treatment methods and effects for pulmonary atresia with ventricular septal defect (PAVSD) in elder children and adults in order to promote the treatment effects. Methods From October 1996 to October 2008, we performed stage1 or staged biventricular repair on 39 PAVSD patients including 21 males and 18 females, ranging from 8 to 27 years old with an average age of 13.43 years. There were 14 cases of type A, 11 cases of type B, and 14 cases of type C. Among them, 23 patients underwent stage1 radical repair in which either human blood vessel with valves or bovine jugular vein with valves were used to connect the pulmonary artery and the right ventricular outflow tract. In these 23 patients, 3 patients complicated with major aortopulmonary collaterals(MAPCAs) underwent unifocalization (UF) operation. The other 16 patients received staged repair, including 9 cases of systemic to pulmonary artery shunt and 7 of staged radical cure. Results There were 6 perioperative deaths with a total mortality of 15.38%(6/39), including 4 (17.39%) stage1 radical repair cases and 2 (12.50%) staged radical repair cases. The former 4 were all type C patients, dying from low cardiac output due to increased pulmonary arterial pressure. In the latter 2 deaths, 1 was a type B secondary shunt patient, and the other was a type C staged radical repair case, both of whom died of bleeding caused by aortic injury in the succeeding operations. Followup was done on 28 cases with a followup rate of 84.85%. The followup time ranged from 14.0 months to 9.2 years with 5 cases missing. No patient died during the followup, and 9 patients maintained their cardiac function at class Ⅰ, 13 at class Ⅱ, 5 at class Ⅲ and 1 at class Ⅳ. Three patients had aortic valve regurgitation of small to medium volume, the treatment of which included an administration of oral potassium diuretic medication and regular follow-up. Conclusion Pulmonary vessels of elder children and adults with PAVSD are usually injured severely and oftentimes it is complicated with MAPCAs. Standard for stage1 radical repair should be defined more strictly based on the present one.
Subvalvar aortic stenosis (SAS) refers to a type of disease in which the stenosis of the left ventricular outflow tract is below the aortic valve. It is a disease spectrum that includes multiple diseases ranging from the simple to the complex. This disease has its own characteristics according to different types. The diagnostic criteria and treatment strategies are basically unified, but there are still some disputes about the surgical treatment. Based on the evidence-based data and expert consensus, this paper makes a comprehensive summary and recommendations on the clinical classification, diagnostic criteria and surgical treatment strategies of SAS, which is more suitable for the clinical characteristics of SAS patients in China and more clinically operable.
ObjectiveTo summarize clinical experience of staged left ventricular retraining for infants with transposition of the great arteries (TGA). MethodsFrom January 2001 to December 2011, 38 TGA infants with intact ventricular septum or a small ventricular septal defect underwent left ventricular retraining in Fu Wai Hospital. There were 26 male and 12 female patients with their age of 19.1±7.7 months and body weight of 7.6±4.7 kg. Preoperative arterial oxygen saturation (SaO2)was 72.6%±9.1%. Left ventricular retraining included aortopulmonary shunt and pulmonary artery banding. Three patients received concomitant excision of the atrial septum. All survival patients were followed up after discharge. ResultsPostoperatively, SaO2 increased to 83.9%±8.1% from preoperative 72.6%±9.1%, and left ventricle-to-right ventricle pressure ratio increased to 0.75±0.09 from preoperative 0.36±0.04. Three patients (7.89%)died postoperatively. Thirty-five patients were followed up for 2 to 11 years. During follow-up, 23 patients successfully received second stage arterial switch operation (ASO). ConclusionFor TGA infants with decreased left ventricular mass who have missed the neonate period, left ventricular retraining is a safe and efficacious procedure to provide necessary preparation for second stage ASO.
ObjectiveTo determine the prevalence of aspirin (ASA) resistance in pediatric patients with congenital heart disease and evaluate whether postoperative thrombosis is associated with aspirin resistance.MethodsA total of 52 patients undergoing high-risk congenital cardiac surgery were recruited in a prospective cohort study at Fuwai Hospital from August 2016 to December 2017. There were 29 males and 23 females with a median age of 8 months (6 d to 13 years). The response to aspirin was determined using the thromboelastography with platelet mapping (TEG-PM) system several days after administration. According to the arachidonic acid (AA) inhibition< 50% or not, they were divided into an ASA resistance group (n=14) and an ASA sensitivity group (n=38). Risk factors of ASA resistance were identified using univariate and multivariate analysis. Patients were monitored prospectively for three months for the development of a thrombosis event. ResultsOf 52 children analyzed, 14 (26.9%) were ASA resistance. The prevalence of thrombosis after ASA antiplatelet therapy was 5.9%. Dose escalation based on aspirin testing was performed in 3 of 14 patients, and the ASA sensitivity was observed in 1 patient. No correlation was found between ASA resistance and postoperative thrombosis (r=0.04, P=0.80).ConclusionPostoperative thrombosis is not associated with aspirin resistance in these patients. Our findings also suggest that resistance may be due to lack of aspirin doses, monitoring of aspirin therapy and consideration of dose adjustment or alternative agents for unresponsive patients.
Objective To evaluate the efficacy of pulmonary surfactant (PS) on severe acute respiratory distress syndrome (ARDS) in different age baby with congenital heart disease. Methods We divided 43 baby patients into two separate groups including a little baby group (12 patients with age less than 3 months) and an infants group (31 patients with age of 3 months to one year). Both groups of patients were treated with intratracheal PS at the same time. The clinical data were collected and analyzed. Results The little baby group had lower body weight. There was no statistical difference in the cardiopulmonary bypass (CPB) time, operation blocking time, mechanical ventilation time, ICU stay time between the two groups (P>0.05). Before treatment, arterial partial presurre of oxygen (PaO2), fractional oxygen concentration in inspire gas (FiO2), the ratio of arterial PO2 to the inspired oxygen fraction (P/F) and arterial-alveolar N2 difference or gradient (a/A) had no difference between the two groups (P>0.05). After treatment, PaO2 and P/F of both groups were significantly lower than before (P<0.05), and FiO2 and P/F were significantly higher than before (P<0.05). After 24 h of treatment, PaO2 and P/F of the little baby group was significantly higher than that of the infants group (P<0.05), and FiO2 and P/F were significantly lower than those of the infants group (P<0.05). Conclusion PS treating severe ARDS in little baby with congenital heart disease has better effect than infants.
ObjectiveTo identify the risk factors of postoperative blood loss among pediatric patients following corrective operation of tetralogy of Fallot (TOF) and to develop nomogram predicting the risk of postoperative blood loss.MethodsA retrospective case-control study was conducted in pediatric TOF patients who underwent corrective operation in our hospital from November 2018 to June 2019. And the clinical data from each enrolled patient were gathered and analyzed. Clinically significant postoperative blood loss was defined as drainage volume from chest tube ≥16 mL/kg during the first 24 h after surgery, which corresponded to the 75th percentile of the blood loss in our population. The primary outcome was to determine the independent predictors of postoperative blood loss by the least absolute shrinkage and selection operator (LASSO) regression, univariate and multivariate logistic regression analysis. On the basis of the independent predictors of postoperative bleeding, nomogram was developed and its discrimination and calibration were estimated.ResultsA total of 105 children were selected (67 males and 38 females aged 3-72 months). The drainage volume from chest tube in the bleeding group was significantly higher than that in the non-bleeding group during the first 24 h (P<0.0001). Multivariate logistic regression analysis showed that low body weight (OR=0.538, 95%CI 0.369-0.787, P=0.001), high preoperative hemoglobin concentration (OR=1.036, 95%CI 1.008-1.066, P=0.013) and prolonged intraoperative aortic cross clamp time (OR=1.022, 95%CI 1.000-1.044, P=0.048) were independent risk factors for postoperative blood loss. In the internal validation, the model displayed good discrimination with a C-index of 0.835 (95%CI 0.745-0.926) and high quality of calibration plots in nomogram models was noticed.ConclusionThe nomogram demonstrated good discrimination and calibration in estimating the risk of postoperative blood loss among pediatric patients following corrective operation of TOF.
ObjectiveTo summarize the clinical characteristics and outcome of tricuspid valve replacement (TVR) in children aged no more than 14 years, and to discuss the selection of prosthesis.MethodsFrom September 2002 to August 2019, 14 patients aged no more than 14 years who received TVR were included in our study. There were 9 males and 5 females, with a mean age of 9.8±4.3 years.ResultsMechanical prosthesis was implanted in 8 patients, and bioprosthesis in 6 patients. The mean cardiopulmonary time and aortic-clamp time was 170.3±109.8 min and 95.1±63.1 min, respectively. The mortality within 30 days after surgery was 21.4% (3/14), and all 3 patients died of severe low cardiac output syndrome. Eleven patients were followed up for 34-199 (100.1±57.4) months. During the follow-up, mechanical prosthesis dysfunction occurred in 3 patients, 2 of whom received secondary TVR. One patient died during the follow-up.ConclusionThe bioprosthesis is the first choice for TVR in children. Some long-term complications may occur after TVR, and close follow-up and timely intervention are needed.
A 12-year-old girl with double outlet of right ventricle, complete atrioventricular septal defect, pulmonary valve stenosis, single atrium, inferior vena cava-hemiazygos vein drainage and left isomerism, who had undergone surgical left superior vena cava-pulmonary artery connection was referred to our hospital. Echocardiography revealed thickening, crimping and malalignment of common atrioventricular valve, moderate-to-severe regurgitation mainly from the left side and the main pulmonary artery was poorly developed. We preserved the autologous valve and used the 31# mechanical mitral valve which was sutured to mitral annulus. After the main pulmonary artery was transected, proximal end was sutured and a 20# Gore-tex artificial vessel was anastomosed to the distal end of the main pulmonary artery. We excised hepatic vein from the right atrium which was then anastomosed to another artificial vessel. Postoperative oxygen saturation was 95%-100%. Echocardiography and CT showed that the function of mechanical valve and heart was good before discharge. Common atrioventricular valvuloplasty is the preferred choice of the management of impaired valve. However, when dysfunction of valve is too severe and valvuloplasty is more likely to fail, the risk of barely performing a valvuloplasty will increase dramatically. On this condition, the mechanical valve replacement should be performed.
Abstract： Objective?To evaluate clinical experiences and long-term outcome of morphologic left ventricle （mLV） retraining for congenitally corrected transposition of the great arteries （cCTGA）. Methods From May 2005 to May 2011, 24 patients with cCTGA anomaly underwent left ventricle retraining by means of pulmonary artery banding in Fu Wai Hospital. There were 13 males and 11 females with their age of 0.17-22.00 （3.73±4.35） years and body weight of 5.10-61.00（15.71±10.95）kg. Major concomitant malformations included tricuspid valve insufficiency （TR）in 23 patients （mild in 11 patients, moderate in 7 patients, severe in 5 patients）, restrictive ventricular septal defect in 18 patients, atrial septal defect in 5 patients, patent foramen ovale in 5 patients, patent ductus arteriosus in 4 patients, mild pulmonary stenosis in 5 patients, and aortic coarctation in 1 patient. All the patients were preoperatively diagnosed by echocardiography, cardiovascular angiography or cardiac catheterization. The mLV end diastolic diameter （mLVEDD） was 8-32（21.56±6.60）mm, posterior wall thickness of mLV was 2-7 （4.29±1.52）mm , mLV to morphologic right ventricle （mRV） pressure ratio （mLV/mRV） was 0.12-0.65 （0.41±0.12）. Pulmonary artery banding operation was performed through upper partial sternotomy or median sternotomy without circulatory arrest. Results The mLV/mRV pressure ratio reached to 0.57-0.93 （0.76±0.10） under direct pressure monitoring after surgery. There was no in-hospital death in this group. Echocardiography before discharge showed that the structure and function of the two ventricles were good, the interventricular septum moved partially towards mRV, mLVEDD was increased slightly, and there was a tendency of reduced TR. Postoperative follows-up was from 1 to 35 months, and there was no late death during follow-up. All the patients were in good general condition with stable vital signs and New York Heart Association （NYHA） classⅠ-Ⅱ. The mLVEDD was 14-40 （26.17±7.11） mm, posterior wall thickness of mLV was 4-9 （4.95±1.44）mm, mLV/mRV pressure ratio was 0.52-0.98 （0.72±0.16） , and TR was significantly decreased. Fourteen patients successfully underwent staged complete double-switch procedure. Conclusion Left ventricle retraining is a safe and effective method to train mLV for cCTGA patients. Pressure load and posterior wall thickness of mLV are increased, mLV cavity is dilated, and TR is significantly reduced after the surgery.