Objective:To investigate the prognosis and risk factors for children diagnosed with all types of subaortic stenosis(SAS) who developed recurrent left ventricular outflow tract obstruction after surgical treatment.Methods:The study retrospectively included patients aged 0-18 years old who underwent open heart SAS surgery at Fuwai Hospital from 2016-2019. Children with hypertrophic obstructive cardiomyopathy were excluded. Detailed operative notes, medical records and ultrasound information, and follow-ups were extracted. Recurrent SAS was defined as left ventricular outflow tract gradient 30 mmHg(1 mmHg=0.133 kPa) 1 month after SAS surgical treatment.Results:A total of 137 children were included in this study. The medium age of children at the time of SAS surgery was 4.6 years old(3 months-17.8 years old). After a median follow-up of 4.36 years(3.2-5.7 years), a total of 30 patients developed recurrent LVOTO, with a recurrence rate of 21.9%, and 7(5.1%) underwent a second surgery. Compared to the non-recurrent group, children in the recurrent group were younger at the time of surgery( P=0.0443), had a smaller body surface area( P=0.0485), and a longer length of stay( P=0.0380). In Cox analysis, when only considering preoperative variables, the independent risk factor for LVOTO recurrence were a peak left ventricular outflow tract gradient higher than 50 mmHg( HR=5.25, P=0.001), a BSA less than 0.9( HR=2.5, P=0.023), and a length of SAS 5 mm( HR=2.29, P=0.050). When both preoperative and intraoperative variables were considered, preoperative peak left ventricular outflow tract gradient 50 mmHg( HR=4.91, P=0.002) and peeling from the aortic valve( HR=3.23, P=0.010) were independent risk factors for postoperative recurrence. Conclusion:Recurrent LVOTO after SAS surgical repair is common, and regular postoperative follow-up is crucial to evaluate whether a secondary intervention is required. Regular postoperative follow-up is needed for children at high risk.

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.

Objective    To explore the clinical effect of mitral valvuloplasty on children with Barlow disease combined with moderate to severe or severe mitral regurgitation. Methods    The clinical data of 10 patients with Barlow disease combined with moderate to severe or severe mitral regurgitation in Fuwai Hospital from January 2014 to August 2019 were analyzed retrospectively, including 3 males and 7 females, with a mean age of 8.7±7.9 years. Echocardiography before and during the operation confirmed that the mitral valve leaflets were long and swinging, the valve leaflets and the opposite edge protruded into the left atrium and were higher than the level of the mitral valve rings, the mitral valve rings were dilated, the papillary muscles and tendons were long, and the pathological changes after the operation showed mucoid degenertion of the valve leaflets and tendons, and some fibrous foci hyperplasia. Mitral valve repair included implantation of artificial valve ring, implantation of artificial tendon, posterior leaflets sliding, partial resection of posterior leaflets (excluding sliding), valve leaflets folding, tendon folding, papillary muscle splitting and annular valve contraction (excluding artificial valve ring implantation). The technique of mitral valve repair, early clinical results and follow-up echocardiographic data were analyzed. Results    All the patients successfully completed the mitral valve repair. The mean time of aortic occlusion was 73.2±17.4 min, and cardiopulmonary bypass time was 99.5±19.8 min. At the same time, 4 patients received tricuspid valve repair and 1 funnel chest correction. There was no reoperation in perioperative period. The 1-year and 5-year survival rates were 100.0% and 100.0%, respectively. The incidence of below moderate mitral regurgitation was 90.0% at postoperative 1 year and 72.0% at postoperative 5 years. Conclusion    For the young children who have Barlow disease and mitral regurgitation, considering the characteristics of heavy lesions, small operation space, and the need to meet the growth and development of valve, it is suggested to adopt the surgical techniques different from those of older children, such as valve ring retraction and tendon folding, if necessary, to adopt "edge to edge" suture, which can shorten aortic occlusion time and achieve good early effects, and its long-term effects still need further follow-up observation. Mitral valvuloplasty technique for Barlow disease similar to that of adults can be used in older children, including implantation of artificial valve ring and implantation of artificial tendon, etc.

Objective     To summarize the characteristics of children diagnosed with secondary subaortic stenosis after the surgical closure for ventricular septal defect and explore its potential mechanism. Methods     We retrospectively collected patients aged from 0 to 18 years, who underwent ventricular septal defect closure and developed secondary subaortic stenosis, and subsequently received surgical repair from 2008 to 2019 in Fuwai Hospital. Their surgical details, morphological features of the subaortic stenosis, and the follow-up information were analyzed. Results     Six patients, including 2 females and 4 males, underwent the primary ventricular septal defect closure at the median age of 9 months (ranging from 1 month to 3 years). After the first surgery, patients were diagnosed with secondary subaortic stenosis after 2.9 years (ranging from 1 to 137 months). Among them, 2 patients underwent the second surgery immediately after diagnosis, and the other 4 patients waited 1.2 years (ranging from 6 to 45 months) for the second surgery. The most common type of the secondary subaortic stenosis after ventricular septal defect closure was discrete membrane, which located underneath the aortic valve and circles as a ring. In some patients, subaortic membrane grew along with the ventricular septal defect closure patch. During the median follow-up of 8.1 years (ranging from 7.3 to 8.9 years) after the sencond surgery, all patients recovered well without any recurrence of left ventricular outflow tract obstruction. Conclusion     Regular and persistent follow-up after ventricular septal defect closure combining with or without other cardiac malformation is the best way to diagnose left ventricular outflow tract obstruction in an early stage and stop the progression of aortic valve regurgitation.