婴幼儿危重型先天性心脏病256层CT低管电压心脏增强扫描中造影剂最小剂量分析

目的分析70 kV管电压条件下婴幼儿心脏增强CT扫描中造影剂最小用量。 方法选取行心脏CT增强扫描的96例危重型先天性心脏病患儿,随机分为A、B、C组,各32例。造影剂选择非离子型碘造影剂（含碘320 mg/mL）,A、B、C组患儿造影剂用量分别为1.0、1.5、2.0 mL/kg。另选100例2月龄以内行心脏大血管增强先天性心脏病患儿,随机分为E、F组,各50例,管电压分别为80 kV和100 kV,其他扫描条件不变。观察A、B、C组噪声、信噪比、主观评分、诊断的准确率及左、右心室的CT值、室间隔强化值（ΔHU）,比较实验组、E组、F组的辐射剂量,分析室间隔ΔHU与造影剂用量的线性关系。 结果A、B、C组的主观评分、噪声、信噪比及诊断的准确率差异均无统计学意义（P>0.05）;A、B、C组心脏左心室、右心室CT值及室间隔ΔHU逐渐升高,差异有统计学意义（P < 0.05）。E、F组与实验组相比,随着管电压的增加,辐射剂量逐渐增高（P < 0.05）。室间隔ΔHU与造影剂用量的线性回归公式为:ΔHU=89.71+2.3×造影剂剂量,在满足室间隔ΔHU ≥ 100 HU条件下,造影剂最小剂量0.75 mL/kg。 结论70 kV条件下婴幼儿心脏CT增强扫描所需造影剂最小剂量为0.75 mL/kg。     

胸腔镜在Ⅲ型食管闭锁合并气管食管瘘修补术中的应用研究

目的探讨胸腔镜在Ⅲ型食管闭锁合并气管食管瘘修补术中的应用价值。方法回顾性分析2015年6月至2018年1月采用胸腔镜手术治疗的22例Ⅲ型食管闭锁合并食管气管瘘患儿临床资料,其中男性12例,女性10例;手术年龄2~9 d,平均手术年龄(4.27±1.81)d,体质量1.5~4.05 kg,平均体质量(2.44±0.68)kg。术前均经胸片和上消化道造影明确诊断,均在电视胸腔镜下行食管气管瘘修补及食管吻合术。结果 22例中,除1例中转开胸手术以外,其余病例均在胸腔镜下顺利完成手术;平均手术时间142.3(118~165)min,术后平均住院时间15.41(8~22)d,第一次经口喂养时间平均3.78(3~5)d,平均呼吸机通气时间26.54(9~52)h。术后有8例因吻合口狭窄行胃镜下食管球囊扩张术;2例出现吻合口瘘,1例予延长禁食时间,持续胃肠减压1周后好转,1例家属放弃治疗。除放弃治疗病例外,21例获随访1个月至2年,随访中1例术后1个月出现食管气管瘘经再次手术治愈;5例随访有吻合口狭窄,经1~4次球囊扩张后改善,现无明显吞咽困难;2例有胃食管反流,现保守治疗中;1例气管稍狭窄,现观察中;其余患儿生长发育良好。结论随着新生儿麻醉及手术技术的不断提高,胸腔镜下手术成为Ⅲ型食管闭锁合并气管食管瘘患儿首选的手术方案。     

体外循环下小儿肌部室间隔缺损的镶嵌治疗

目的 探讨镶嵌模式(hybrid procedure)治疗小儿肌部室间隔缺损(Mvsd)的手术方法及临床应用.方法 2006年1月至2010年6月,在体外循环心内直视手术下采用手术及封堵相结合的镶嵌技术矫治小儿Mvsd 45例,其中男20例,女25例;年龄52天～12岁;体重3～32 kg.7例为单个Mvsd,38例为多发性VSD.同时合并大血管错位(D-TGA)1例、法洛四联症(TOF)2例、肺动脉狭窄(PS)3例、动脉导管未闭(PDA)6例、房间隔缺损(ASD)6例、主动脉缩窄1例.均在心脏停跳后直视下将导引钢丝经三尖瓣孔自心脏右室面穿过VSD至左室面,直视下置入导引器,然后送入封堵器,完成Mvsd封堵.多发性VSD 38例,予自体心包片修补膜周部等较大的VSD,心内其他畸形同期完成矫治.结果 42例置入单枚封堵器(直径4～10 mm)、3例置入双枚封堵器(直径4～7 mm).手术经过顺利,术前左室射血分数(EF)均在正常范围,术后1天小于8月龄组EF均值低于正常,大于8月龄组EF正常,两者差异有统计学意义.术后常规每天给予5 mg/kg肠溶阿司匹林3～6个月.术后随访超声检查示封堵器位置无偏移,无残余分流,无二尖瓣、主动脉瓣反流、Ⅲ度传导阻滞及新发心律失常等.术后因重症感染放弃治疗1例,无远期死亡病例.结论 体外循环下镶嵌技术治疗小儿 Mvsd明显降低了围手术期并发症及病死率,简化了手术过程,降低了手术风险,是一种安全、有效的方法.

Abstract：

Objective To summarize the technique and clinical experience of hybrid procedure under cardiopulmonary bypass (CPB) in children with muscular ventricular septal defect (mVSD). Methods From January 2006 to June 2010, 45 cases of mVSD underwent hybrid procedure with CPB. mVSDs were closed with devices under direct vision in 45 cases. Of them, there were 20 males and 25 females. They ranged from 52 days to 12 years [mean (2.05 ±2.48) year] in age and from 3 to 30 kg [(11.93 ±7.70)kg] in body weight. Preoperatively, most of children were highly susceptible to respiratory tract infections. The hybrid approach was used in all patients with CPB under the guidance of transesophageal echocardiography (TEE). The diameter of mVSDs ranged from 2 to 7 mm under TEE. Of 45 cases, 40 patients had increased rates of pulmonary blood flow. 29 patients had left axis deviation and 12 cases had sinus arrhythmia on electrocardiography (ECG). 19 had other congenital heart lesions, including transposition of great arteries in 1 case, tetralogy of Fallot in 2, pulmonary artery stenosis in 3, patent ductus arteriosus in 6, atrial septal defects in 6) and aorta coactation in 1. The quantity of VSDs were from 1 to 7 (single, in 7; two, in 24 case; three, in 8 case; four, in 5 case and seven, in lease. 37 patients were combined with pulmonary hypertension in our cohort. 38 patients with another large VSD and 19 with other congenital heart lesions were required surgical repair at sometime. Results The hybrid procedures were undertaken in all 45 cases of this cohort. All cases were successful and no deaths occurred during operation. A total of 48 devices were implanted in 45 patients, including single devices in 42 cases (device size ranged from 4 to 10 mm) and two devices in 3 cases (device size ranged from 4 -7 mm). The average time on CPB was (58.28 ±20.70) min , while aortic crossclamp time was(34. 94 ± 14.75) min. In addition, the time on mechanical ventilation postoperatively ranged from 2 hours to 6 days. Compared to the older children, 20 infante aged less than 8 monhad a significant difference in cardiac function in the early postoperative period. One infant was given up treatment because of serious infection. Anather cases recovered with the use of supportive treatment, such as using vasoactive agents, digoxin, inhaling nitric oxide, diuresis, and so on. The enteric-coated aspirin was given at dose of 5 mg ? kg -1. day -1 for a period of 3 to 6 months as usual postoperatively. All patients attended follow-up at 1 week, 1 month, 3 months, 6 months, 1 year and 2 years post-procedure. No major complications were encountered during this period. All cases were no instance of migration of any of the devices, residual shunt, aortic regurgitation, atrioventricular valve dysfunction, Ⅲo atrial-ventricular conduction block, new arrhythmia, and so on. There are no death in long-term follow-up. Conclusion Hybrid procedure is safe and effective for the closure of congenital heart defects in children.     

法鲁四联症体外循环手术对血浆内皮素的影响

对２１例法鲁四联症患儿体外循环手术前后血浆内皮素的变化进行动态检测，并以单纯室间隔缺损２３例作为对照。结果显示，除术后１年外．法鲁四联症组各期内皮素值均较室缺组为高。术后１小时两组内皮素值均达最高峰，室缺组术后３天恢复，四联症组术后７天仍高于术前。内皮素值与体外循环时间及主动脉阻断时间在四联症组呈高度相关。文章讨论了体外循环期间内皮素变化的原因及临床意义。     

婴幼儿复杂危重先心病术后心律失常的处理

目的：探讨婴幼儿复杂危重症先天性心脏病术后心律失常的发生原因及治疗方法。方法：2008年5月~2010年6月我科共为508例复杂重危先天性心脏病患儿施行了手术治疗,术后患儿均转入心胸外科监护室进行监护和治疗,通过持续心电监护,发现心律失常患儿96例,并进行相应的处理和治疗。结果：96例术后心律失常患儿中,92例痊愈,3例死亡,1例放弃治疗。结论：心律失常是婴幼儿复杂危重先天性心脏病手术后常见并发症之一,应早期发现、及时采用抗心律失常治疗并积极去除引起心律失常的病因。正确及时诊断和治疗心律失常可以有效地降低婴幼儿复杂危重先天性心脏病手术的死亡率。     

单肺Fontan手术1例

病儿 男,4岁.全身发绀4年余.查体:发育正常,营养中等,心率110次/min,血压98/65 mmHg(1 mm Hg=0.133kPa),SpO20.68,双肺未闻及于湿罗音.     

缺氧诱导因子-1α与脑缺氧缺血性损伤

缺氧诱导因子-1α(hypoxia-inducible factor-1 alpha,HIF-lα)是近来发现的广泛存在于哺乳动物和人体内的一种缺氧应答调控因子,在调节缺氧诱导的基因表达中起关键性作用。它可调节表达多种靶基因如血管内皮生长因子、促红细胞生成素等,对改善脑缺氧缺血后能量代谢障碍、促进脑血流动力学恢复、抑制兴奋性氨基酸毒性、减少细胞凋亡等起重要作用。通过进一步对HIF-lα及其靶基因的研究,可能为临床治疗脑缺氧缺血性损伤提供了一种新的治疗策略。     

波生坦治疗先天性心脏病合并肺动脉高压的研究进展

<正>肺动脉高压(pulmonary arterial hypertension,PAH)是一种少见的预后不良的疾病,以肺动脉压力和阻力增高为特征[1]。先天性心脏病(congenital heart disease,CHD,先心病)体-肺分流型是由于缺损部位大量左向右分流导致肺循环容量明显增加,肺血管处于高流量高压力状态,肺动脉压力阻力升高,引起PAH。