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.
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.
ObjectiveTo summarize the experience of surgical treatment of transposition of the great arteries with intact ventricular septal (TGA-IVS) after left ventricular regression by comparing the characteristics of rapid and long-term two-stage arterial switch operation (ASO).MethodsForty-one patients who were mainly diagnosed with TGA-IVS from January 2007 to January 2019 and underwent two-stage ASO were included. They were divided into a rapid two-stage ASO group (19 patients) and a long-term two-stage ASO group (22 patients) according to the interval of left ventricular training surgery and ASO. The clinical effectiveness of the two groups was compared.ResultsThere was a statistical difference in age, body weight, blood oxygen saturation before ASO, end diastolic diameter of left ventricle before training, and thickness of posterior left ventricular wall before ASO (P<0.05). Children older than 1 year was an independent risk factor for long-term two-stage ASO.ConclusionLong-term two-stage ASO is suitable for children who are older than 1 year and who have severe left ventricular regression.
ObjectiveTo review our experience of reoperations for pulmonary venous stenosis (PVS) after total anomalous pulmonary venous connection (TAPVC) repair for the past decade in Fuwai Hospital.MethodsNine patients underwent reoperation for PVS between 2009 and 2019 in Fuwai Hospital, including 4 males and 5 females with an average age of 5.10±5.00 years. The patients were divided into a sutureless group (n=3) and a non-sutureless group (n=6). Clinical data were reviewed and analyzed.ResultsFor primary TAPVC type, 4 patients were supracardiac, 2 patients were cardiac, 1 patient was infracardiac, and 2 patients were mixed-type anomaly. The median cardiopulmonary bypass time was 95 (63, 208) min, aortic clamping time was 58 (30, 110) min, ICU stay was 24 (24, 2 136) h. Early hospital death occured in 1 (11.1%) patient. One (11.1%) patient with single ventricle physiology had hospital comorbidity, who underwent hemofitration therapy. The follow-up time was 11.9 (2.2, 18.0) months, during which 1 patient died of restenosis of pulmonary vein and another patient died of stroke. No statistically significant difference was found between the sutureless group and non-sutureless group in postoperative or follow-up results (P>0.05). ConclusionSurgery is effective for treatment of PVS after repair of TAPVC, yet with a realatively high morbidity and mortality. The advantage of sutureless repair over conventional repair for this particular group of patients is yet to be verified.
ObjectiveTo explore the clinical effect of mitral valvuloplasty on children with Barlow disease combined with moderate to severe or severe mitral regurgitation.MethodsThe 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.ResultsAll 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.ConclusionFor 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.
ObjectiveTo investigate the best anatomical classification, surgical timing, procedure and clinical outcomes of congenital vascular ring.MethodsThe clinical data of 58 patients who underwent congenital vascular ring surgery in Pediatric Surgery Center, Fuwai Hospital between 2014 and 2019 were retrospectively analyzed. There were 32 (55.2%) males and 26 (44.8%) females with a median age of 16.5 (2-73) months. Preoperative symptoms, imaging examinations, anatomical classifications, surgical procedures and postoperative recovery were assessed.ResultsThere were 20 (34.5%) patients of double aortic arch, 22 (37.9%) patients of right aortic arch with left arterial duct or ligament, 15 (25.9%) patients of left aortic arch with aberrant right subclavian artery, and 1 (1.7%) patient of circumflex aorta with cervical aorta arch. The median ventilator supporting time was 6.0 (0-648) h, and the median hospital stay time was 14.5 (7-104) d. One patient with coarctation of aorta died of severe pulmonary infection during perioperative period, and the others survived without symptoms and reoperation after discharge. The median follow-up time was 7.0 (1-62) months.ConclusionFor children with unexplained dyspnea and dysphagia, or with right aortic arch, preoperative imaging examinations such as computed tomography or magnetic resonance imaging are required to confirm the diagnosis of vascular ring. Surgical correction of congenital vascular ring is safe and reliable, and can effectively relieve symptoms. The mortality rate and reoperation rate are low, and the follow-up results are satisfactory.
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.
Artificial chord is a mature mitral valve repair technique, especially in adult mitral valve repair. It is still challenging to repair mitral valve in children with artificial chords because the quality of mitral valve is soft and immature. There are some differences in the methods of suture, the choice of suture size and the number of artificial chords. Although the artificial chords could not grow naturally, we found through the long-term research that most children did not have mitral valve restriction or even chords rupture due to itself can compensate through the growth of the flap and papillary muscle. This article summarizes the recent research progress on the treatment of mitral valve insufficiency in children with artificial chords, providing reference for clinical treatment.