Objective To summarize the experience of open heart operation on neonates with critical and complex congenital heart diseases and evaluate the methods of perioperative management. Methods From May 2001 to January 2003, 12 patients of neonates with congenital heart diseases underwent emergency operation. Their operating ages ranged from 6 to 30 days, the body weights were 2.8 to 4.5 kg. Their diagnoses included D-transposition of the great arteries in 4 cases, ventricular septal defect with atrial septal defect in 5 cases, complete atrioventricular septal defect, obstructed supracardiac total anomalous pulmonary venous drainage and cardiac rhabdomyomas in 1 case respectively. 12 cases were operated under moderate or deep hypothermic cardiopulmonary bypass. Results All cases were observed in ICU for 2-11 days and discharged 7-19 days after operation. The postoperative complications included low cardiac output, mediastinal infection, respiratory distress syndrome, systemic capillary leak syndrome and acute renal failure. All cases were cured and the follow-up (from 6 months to 2 years) showed satisfactory outcome. Conclusion A particular cardiopulmonary bypass and proper perioperative management is very important to ensure the successful outcome. Peritoneal dialysis is an effective and safe method for treating acute renal failure after cardiac operation in neonates.
ObjectiveTo observe the mutation and expression of Nkx2.5 in congenital heart disease patients with diminutive pulmonary blood. We preliminarily explored the association between Nkx2.5 gene and pathogenesis of congenital heart disease patients with diminutive pulmonary blood. MethodsFifty six patients of congenital heart disease with diminutive pulmonary blood in the first affiliated hospital of Bengbu medical college and Anhui province children, s hospital between May 2012 and May 2014 were as an experimental group. Sixty three patients of ventricular septal defect were as a control group. In the trial group, there were 30 males and 26 females averagely aged 5.82± 4.23 years ranking from 6 months to 14 years. In the control group, there were 36 males and 27 females averagely aged 6.93± 4.56 years ranking from 6 months to 14 years. Before operation, peripheral venous blood of all the patients were collected. We used polymerase chain reaction combined with DNA sequencing technology to detect Nkx2.5 gene exon sequence and to analyze the association between Nkx2.5 gene mutation and congenital heart disease with diminutive pulmonary blood. And we got some hypertrophic myocardial tissue from right ventricular outflow tract in the operation, whose size was 0.5× 0.5× 0.5 cubic centimeter. And we extracted myocardial tissue RNA. The expression changes of Nkx2.5 gene mRNA were detected by real-time fluorescence quantitative polymerase chain reaction technique. ResultsThere was no mutations tested out in the peripheral venous blood in both two groups. The expression of mRNA in Nkx2.5 gene of the trial group was lower than that in the control group with a statistical difference. ConclusionNkx2.5 gene mutation may be associated with multiple factors. The occurrence of congenital heart disease with diminutive pulmonary blood may be related with a decline of Nkx2.5 gene expression in the myocardial tissue.
目的:评价先天性心脏病（CHD）不同病变类型在64层螺旋CT（64-MDCT）三维重建图像上的表现及显示效果。方法:回顾性分析36例CHD患者的64-MDCT资料,分别在轴位、冠状、矢状位及容积再现（VR）重组图像上观察病变,统计分析不同重组图像显示总体病变及房、室间隔缺损效果有无差异。结果:36例患者共有病变59处,轴位、冠状及矢状位图像在发现病变（59处,100.0%；57处,96.6%；53处,89.8%）及室间隔缺损显示（显示效果评分:41、38及39分）方面无明显差异（P＞0.05）,但轴位图像显示房间隔缺损明显优于冠、矢状位图像（显示效果评分:19、13及12分）（P＜0.05）。VR图像与轴位及冠、矢状位图像显示心外大血管病变效果无差异P＞0.05）。不同类型病变在轴位,冠、矢状位及VR图像上表现特征不尽相同,而不同重组图像有各自优势显示的病变类型。结论:64-DCT三维重建图像能够很好地显示先心病各种类型病变,了解不同重建图像上病变表现特征及显示效果有利于做出准确、全面的诊断。
ObjectiveTo evaluate the mid- and long-term outcomes of different surgical techniques for subaortic stenosis.MethodsThe clinical data of 75 patients with subaortic stenosis who underwent surgery in our hospital from January 2008 to January 2018 were retrospectively analyzed, including 48 males and 27 females, with a median age of 72 (48, 132) months and mean weight of 21.35±15.82 kg. There were 40 (53.3%) patients combined with aortic regurgitation; 38 (50.7%) patients were the first time and 37 patients were the second time to receive the operation. According to the surgical techniques, 75 patients were divided into two groups: a group A (40 patients with simple subaortic membrane resection) and a group B (35 patients with subaortic membrane and muscle resection or modified Konno procedure).ResultsTwo (2.67%) patients died in hospital. There was one late death in the group B. The average preoperative and postoperative pressure gradient of all patients was 69.96±42.02 mm Hg and 7.44±12.45 mm Hg, respectively. All patients were followed up for 51 (12, 120) months. Pressure gradient at follow-up in the group A and the group B was 8.83±14.52 mm Hg and 5.86±9.53 mm Hg, respectively with no statistical difference (P=0.294). Four patients in the group A and 2 patients in the group B needed reintervention. However, there was no statistical difference in the long-term reintervention rate between the two groups (P=0.480).ConclusionFor the different degree of lesions in the left ventricular outflow tract, our management strategy is feasible. Although there is no statistical difference between two the groups in the long-term reintervention rate after simple valvular membrane resection, prolonged follow-up is necessary to examine the long-term outcomes of different surgical techniques.