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Objective:To analyze the characteristics and surgical outcomes of Shone′s syndrome in children, and to explore the surgical treatment strategy and technical key.Methods:Retrospective study.Children with Shone′s syndrome treated in the Pediatric Heart Center, Beijing Anzhen Hospital, Capital Medical University from May 2013 to June 2019 were retrospectively analyzed for their baseline characteristics and surgical data.The data were compared by Student t-test. Results:A total of 22 children with Shone′s syndrome were recruited, involving 15 males (68.2%) and 7 females (31.8%). There were 6 cases (27.3%) of complete form of Shone′s syndrome and 16 cases (72.7%) of incomplete form.No deaths were reported.The postoperative mitral valve velocity [(149.7±38.2) cm/s vs.(234.9±34.0) cm/s, t=7.341, P<0.05], left ventricular outflow tract velocity [(202.0±105.0) cm/s vs.(328.6±120.3) cm/s, t=6.575, P<0.05] and aortic arch coarctation velocity [(186.1±60.9) cm/s vs.(347.9±100.8) cm/s, t=7.630, P<0.05]were significantly lower than those of preoperative levels.There were no complications occurred at 1-year follow-up, and 91.7% of the patients were followed up for 3 years, and 80.2% were followed up for 5 years without complications, 2 cases needed reoperation. Conclusions:Surgical treatment of Shone′s syndrome achieved satisfactory outcomes.Early diagnosis and early intervention are beneficial to children with Shone′s syndrome, although they need to be followed up and have the risk of reoperation in the long term.
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Objective:This study aims to review two surgical techniques results of the reoperation for left atrioventricular valve(LAVV) failure in repaired atrioventricular septal defect.Methods:All consecutive patients with repaired atrioventricular septal defect(AVSD) who underwent redo-LAVV surgery from 2005 to 2019 were included. Patients with single ventricles, banding, atrial isomerism, and complex associated anomalies were excluded. Univariate analysis included repair and replacement. Data analyzed included number and year of primary AVSD and redo-LAVV operation, morphology of AVSD, mortality, and reoperation, early and long-term survival.Results:There were a total of 28 patients including 7 boys(25%) with age of 114.5 months(63.0-194.5 months). The mean body weight was 28.55 kg(15.5-55.9 kg). There were 11(39.3%) patients with complete AVSD and 17(60.7%) with partial AVSD, and 21(75%) patients with LAVV valvuloplasty(LAVVP), 7(25%) patients with LAVV replacement(LAVVR). The aortic cross clamp time was significantly longer in patients who underwent LAVVR compared to LAVVP[LAVVR 94 min(79-107)min vs. LAVVP 66 min(45-83 min), P<0.05]. The technique of cleft closure with LAVVP 18(87.5%) more than LAVVR 2(28.6%), P<0.05. Conclusion:The postoperative left atrio-ventricular valve(LAVV) regurgitation is the main reason of the reoperation. The technique of cleft closure with LVVR-Repair is favorable. At follow-up, survivors with re-LVVR-Repair have high rates with numerous operations.
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@#Objective To explore the anatomical characteristics and surgical strategies of atrioventricular septal defect (AVSD) with left ventricular outflow tract (LVOT) stenosis. Methods The clinical data of 11 AVSD patients with LVOT stenosis who underwent surgeries in our hospital from 2008 to 2019 were retrospectively analyzed, including 6 males and 5 females with a median age of 15.0 (7.6-22.0) years. Results There were 3 patients of complete AVSD and 8 patients of partial AVSD. Subaortic stenosis resulted from discrete subaortic membrane in 3 patients, diffused subaortic membrane in 4 patients, hypertrophied muscle bundles in 3 patients and distorted valve frame in 1 patient. Among these patients, 5 patients underwent LVOT stenosis and AVSD repairs simultaneously for the first time, 5 patients underwent LVOT stenosis repair for the second time and 1 patient for the third time. No postoperative death occurred. The postoperative LVOT flow velocity decreased dramatically after LVOT stenosis repair compared with preoperative one [449.0 (393.0, 507.5) cm/s vs. 212.0 (183.0, 253.5) cm/s, P<0.05]. Conclusion Surgical results of AVSD combined with LVOT stenosis are satisfactory, but the restenosis should be paid attention to via long-term follow-up.