肺动脉高压先天性心脏病患者降肺动脉压的预后效果

目的：探讨先天性心脏病（CHD）合并肺动脉高压（PAH）患者行降肺动脉压治疗的安全性及疗效。方法选择2014年9月至2015年1月在该院治疗的C HD合并 PA H患者34例,均在常规治疗的基础上增加内皮素受体拮抗剂波生坦治疗,观察患者行降动脉压治疗后血流动力学、心功能检测和6 min步行距离试验（6 MWD）情况。结果治疗后患者肺血管阻力（PVR）、股动脉血氧饱和度（SaO2）、肺循环／体循环血流量比值（Qp／Qs）和右心输出量（CO）分别为（218．30±91．25）dyn · s－1· cm －5、（95．16±7．84）％、（1．10±0．27）和（5．20±1．36）L／min ,均较治疗前有所改善（P＜0．05）;治疗前后肺动脉收缩压（sPAP）、肺动脉舒张压（dPAP）、平均肺动脉压力（MPAP）、心率（HR）和中心静脉压（CVP）比较,差异均无统计学意义（P＞0．05）;治疗前后右心室直径（RVD）、左心室射血分数（LVEF）、三尖瓣返流速度（VP）、三尖瓣跨瓣压差（PGTV）、左心室舒张末容积（LVEDV）和左心室舒张末直径（LVEDD）比较,差异均无统计学意义（P＞0．05）;治疗后3、6个月6 MWD步行距离分别为（378．09±40．12）、（423．07±35．32）m ,均较治疗前提高,差异有统计学意义（P＜0．05）。结论 CHD合并PAH患者行波生坦治疗安全有效,能改善肺血流动力学和6 M WD。     

FG syndrome in a premature male

A 30-week premature male infant is presented with dolichocephaly, frontal bossing, down-slanting palpebral fissures, hypertelorism, long philtrum, micrognathia, cleft palate, and imperforate anus. He is the fifth patient to be presented with FG syndrome and sensorineural deafness. The patient's syndromic manifestations became more obvious during an inpatient observation period of 3 months.     

Changes in sensorimotor regions of the cerebral cortex in congenital amusia:a case-control study

Perceiving pitch is a central function of the human auditory system;congenital amusia is a disorder of pitch perception.The underlying neural mechanisms of congenital amusia have been actively discussed.However,little attention has been paid to the changes in the motor rain within congenital amusia.In this case-control study,17 participants with congenital amusia and 14 healthy controls underwent functional magnetic resonance imaging while resting with their eyes closed.A voxel-based degree centrality method was used to identify abnormal functional network centrality by comparing degree centrality values between the congenital amusia group and the healthy control group.We found decreased degree centrality values in the right primary sensorimotor areas in participants with congenital amusia relative to controls,indicating potentially decreased centrality of the corresponding brain regions in the auditory-sensory motor feedback network.We found a significant positive correlation between the degree centrality values and the Montreal Battery of Evaluation of Amusia scores.In conclusion,our study identified novel,hitherto undiscussed candidate brain regions that may partly contribute to or be modulated by congenital amusia.Our evidence supports the view that sensorimotor coupling plays an important role in memory and musical discrimination.The study was approved by the Ethics Committee of the Second Xiangya Hospital,Central South University,China(No.WDX20180101GZ01) on February 9,2019.     

高频震荡通气治疗先天性心脏病术后重症ARDS32例效果观察

目的探讨高频震荡通气(HFOV)对小儿心脏手术后重症ARDS的治疗效果。方法对32例心脏手术后常频通气(CMV)治疗无效的重症ARDS患儿行HFOV治疗,设置相应的参数并行氧合、通气管理,每次吸痰后行肺复张。观察治疗前后血气指标变化、HFOV治疗时间、肺复张期间循环指标变化、整体治疗期间并发症发生情况及患儿存活情况。结果 HFOV治疗后通气及气体交换在较短的时间内改善,12～48 h血气相关指标PaO2、PaCO2、吸入氧浓度(FiO2)、氧合指数(PaO2/FiO2)均明显改善且稳定。HFOV治疗时间43～238(128.5±67.49)h,肺复张期间循环指标未出现异常变化,末梢血氧饱和度快速恢复至吸痰前水平,呼吸机的吸氧浓度快速降低。整体治疗期间出现气胸9例,均安置胸腔闭式引流。本组患儿存活21例(65.6%),死亡11例。结论 HFOV对CMV治疗无效的重症ARDS能在较短时间内改善通气及气体交换,可作为小儿心脏术后重症ARDS的重要抢救措施。     

Infective endocarditis in paediatrics

Infective endocarditis is a result of infection of the endocardium, particularly of the heart valves (native or prosthetic valves). The most common causative organisms in the paediatric population are: Streptococci, Staphylococci and Enterococci. The classical signs of infective endocarditis like Roth spots, Janeway lesions, splinter haemorrhages and Osler's nodes are relatively rare in children. A high index of suspicion in a febrile child with a new murmur, detailed history, meticulous examination, repeated blood cultures, and echocardiography are essential in establishing the diagnosis. Management of infective endocarditis involves a prolonged course of antibiotics, at least for 4–6 weeks depending upon the causative organism and underlying heart condition. Complications of infective endocarditis include congestive heart failure resulting from valvular damage/regurgitation, infective emboli leading to abscesses in other organs and abnormal host immunological responses. Prophylactic antibiotics for dental and other medical procedures like genitourinary tract procedures are no longer recommended in the UK. The emphasis should be on educating children and their parents in early recognition of infective endocarditis. Children at high risk of developing endocarditis should be assessed urgently after clinical suspicion.     

提上睑肌缩短术治疗中重度先天性上睑下垂的效果分析

目的探讨应用提上睑肌缩短术治疗中重度先天性上睑下垂的效果.方法对凹例（41眼）中重度先天性上睑下垂患者采用经皮肤-睑结膜入路提上睑肌缩短术,并对术后效果进行随访观察.结果本组凹例（41眼）随访2～18个月,除2例矫正不足外,余均获得良好效果.上睑缘弧度及双重睑形成良好,术后仅部分病例早期有轻度睑裂闭合不全,无暴露性角膜炎等并发症发生.结论提上睑肌缩短术可适用于中重度先天性上睑下垂患者,在矫正畸形和改善外观方面均能达到良好的效果.     

先天性肺支气管囊肿X线、CT诊断

目的 提高对失天性肺支气管囊肿的X线、CT诊断水平。方法 对19例临床确诊为先天性肺支气管囊肿病例的X线、CT表现进行回顾性分析。结果：单发含液囊肿9例，含气囊肿1例，多发性支气管囊肿9例。结论：X线、CT检查均能很好诊断本病，CT能发现更多征象。X线、CT表现不典型时，要与肺结核空洞和肺占位病变鉴别。     

临床决策支持系统在先天性心脏病诊疗及护理中的应用进展

介绍临床决策支持系统在先天性心脏病诊疗及护理中的应用进展,并指出应用过程中存在的问题,旨在为开发更加智能化、专业化、系统化的先天性心脏病诊疗及护理的临床决策支持系统提供新思路。     

椎动脉发育不良的螺旋CT血管造影诊断

目的探讨椎动脉发育不良的螺旋CT血管造影(spiralCTangiography,SCTA)表现和临床意义。方法180例临床诊断为椎-基动脉供血不足患者行SCTA检查。造影剂为浓度350mg/100ml的Omnipaque80～100ml,足背静脉注射。流率3ml/s,延迟时间由预扫描确定。层厚、间隔分别为3mm和4.5mm。重建间隔1.5mm。采用多平面容积重建(MPVR)和表面遮盖法重建(SSD),用原始横断面图像测量椎动脉直径。CT显示有椎动脉狭窄者于1周内行DSA检查。结果360根椎动脉中共发现78根(21.7%)有全程狭窄,平均直径1.3mm(1.0～1.5mm)。其中左侧24根,右侧54根。其中有20根同时伴有局限性进一步狭窄。对侧椎动脉合并局部管壁钙化13根(16.7%),合并V1段扭曲20根(25.6%),合并钩椎关节增生压迫11根(14.1%)。DSA检查结果与SCTA完全一致。结论椎动脉发育不良是椎-基动脉供血不足的常见原因之一,SCTA可以确诊并能取代诊断性DSA检查。     

心脏右侧径路纠治心下型肺静脉异位回流

目的 评估经心脏右侧径路纠治心下型完全性肺静脉异位回流方法的价值.方法 2005年9月至2007年12月,采用经心脏右侧进路方法纠治心下型完全性肺静脉异位回流7例.平均年龄(70.57 -44.67)天;平均体重(4.07±0.87)kg.其中3例肺静脉回流梗阻,2例卵圆孔未闭较小.结果 无手术死亡.术后并发症包括:低心排5例,发生肺动脉高压危象3例,呼吸机辅助7天以上2例,肺部感染2例.术后随访6个月至2年,超声检查心房内无残余分流,吻合口无明显梗阻,流速1.20～1.47m/s;心功能:射血分数0.70～0.79,短轴缩短率0.32～0.44;1例出现肺静脉回流的流速增快,其右上肺静脉2.60m/s,右下肺静脉2.12m/s,继续随访中.结论 采用经心脏右侧径路方法纠治心下型完全性肺静脉异位回流,取得了较好效果.手术成功取决于左房与垂直静脉的吻合口大小,保证肺静脉回流无梗阻.

Abstract：

Objective Infracardiac total anomalous pulmonary venous connection, a rare congenital cardiac defect, is associated with high mortality. A modification was designed for the procedure to reduce the post-operative obstruction in the pulmonary venous. Methods From September 2005 to December 2007, seven patients with infracardiac total anomalous pulmonary venous connection were treated with repair surgery through right side approach. The patients' age at operation was (70.57 ±44.67) days , the weight was (4.07 ±0.87) kg. Three patients had pulmonary venous obstruction, and 2 with small PFO. A modified right - side approach for repairing this defect was used. Results No death occurred after the operation. The postoperative complications included low cardiac output in 5 patients (71.43%), pulmonary hypertension crisis in 3patients ( 42.86% ) , mechanical ventilation for more than 7 days, which happened in 2 patients (28.58%) and pulmonary infection. All of the patients received follow-up. No residual shunt and pulmonary venous return obstruction were identified on echocardiogram(with a velocity from 1.2 m/s to 1.47 m/s). The heart function of patients was within the normal range(EF 0.70 -0.79, FS 0.32 -0.44). Conclusion The modified surgical procedure for the correction of infracardiac total anomalous pulmonary venous connection by right side approach was associated with favorite clinical outcomes, The post-operative outcomes depended on the size of anastomosis between the common vein and left atrium and the patency of the pulmonary venous return. Adequate size of anastomosis and maintenance of the spatial structures in adjacent regions were helpful in decreasing the adverse effect of postoperative obstruction.