Outcome of pulmonary atresia and ventricular septal defect during infancy

We evaluated 54 patients with pulmonary atresia and ventricular septal defect who were referred during the first year of life between 1972 and 1992. Particular emphasis was given to the nature of the pulmonary blood supply and its influence on outcome. Ductal supply of confluent pulmonary arteries was present in 30 patients (55.6%, group I), whereas 24 patients (44.4%, group II) had a pulmonary blood supply that was entirely (31.4%) or predominantly (13.0%) dependent on systemic collateral arteries. Over the 20 years these was no significant difference in actuarial survival between the two groups. Corrective surgery was performed in 8 of 30 patients in group I (26.7%)-significantly more than in group II (4 of 24, 16.7%). Arborization abnormalities of the pulmonary arteries (stenosis of unbranched and intrapulmonary arteries) were almost exclusively present in patients with systemic collateral arteries (p<0.03), accounting for the lower probability of undergoing corrective surgery in group II patients. During the first decade of this study (1973–1983) corrective surgery was attempted in 9.6% of patients, with 42% mortality; and during the second decade (1983–1993) surgery was performed in 39.1% of patients, with 26% mortality, a significantly lower figure. Improving surgical results, complete preoperative demarcation of the pulmonary blood supply, and a more aggressive approach with early unifocalization of the pulmonary blood supply may invalidate comparison with retrospective data on the advisability of attempting to correct this anomaly. The present paper provides data against which treatment of infants with pulmonary atresia and ventricular septal defect presenting during the next decade can be compared.     

Constrictive pericarditis developed after childhood repair of ventricular septal defect

The patient was an 18‐year‐old man who had undergone surgical closure of ventricular septal defect at the age of 2 years. The patient was urgently hospitalized due to syncope after longstanding uneventful postoperative course, and diagnosed with atrial flutter. Echocardiography showed poor ventricular motion and markedly dilated both atriums. After termination of atrial flutter, ventricular motion improved remarkably; however, there was little improvement in heart failure. Although we suspected constrictive pericarditis, calcification observed on computed tomography was too localized to fully explain the cause of severe diastolic dysfunction. Based on the waveform of mitral annular tissue Doppler imaging, we were strongly convinced of the underlying physiology of constrictive pericarditis. Pericardial decortications markedly improved heart failure symptoms. This case emphasizes that constrictive pericarditis could be a life‐threatening complication in the remote phase after congenital heart surgery. It also emphasizes the importance of functional assessment by echocardiography to identify the cause–effect relation between imaging and clinical symptoms.     

婴幼儿主动脉缩窄伴室间隔缺损的Ⅰ期纠治

目的总结婴幼儿主动脉缩窄伴室间隔缺损胸骨正中切口Ⅰ期纠治的手术方法和临床经验。方法2000年1月~2004年5月,采用胸骨正中切口Ⅰ期纠治17例主动脉缩窄伴室间隔缺损患儿,月龄1~41个月;体重3.0~13kg,平均为(6.32±2.35)kg。主动脉缩窄位于导管前11例,邻近动脉导管处6例;其中4例伴主动脉弓发育不良。9例伴有动脉导管未闭,17例均伴有室间隔缺损。结果本组17例中,1例于矫治术后第2d死于肺动脉高压危象;余16例术后恢复良好,其中14例上下肢动脉压差消失,另2例上下肢动脉压差24h内降至15mmHg(1mmHg=0.133kPa),72h后压差消失。随访至今超声心动图检查无假性动脉瘤或主动脉再狭窄。结论采用胸骨正中切口Ⅰ期纠治主动脉缩窄伴室间隔缺损,可同时纠治并发的主动脉弓部发育不良,降低了残余梗阻的发生率。Ⅰ期矫治还具有明显降低手术费用,减少患儿二次手术痛苦的优点。Ⅰ期手术在死亡率和并发症方面与Ⅱ期手术没有显著性差异。     

合并室间隔缺损的主动脉闭锁一例

患儿男,5岁5个月,因"查体发现心脏杂音"入院.体检:血压:双上肢100/50 mm Hg(1 mm Hg=0.133 kPa),双下肢130/60 mm Hg;肺动脉瓣区第二心音亢进,胸骨左缘2～4肋间可闻及4/6级收缩期杂音,并可触及震颤;口唇及指端无发绀.经皮测四肢动脉血氧饱和度91%～93%.     

Management of asymptomatic ventricular septal defect

A patient with a small ventricular septal defect should not be subjected to operative closure of the defect. Such a ventricular septal defect is characterized by a normal second sound, normal electrocardiorgram, normal thoracic roentgenogram and echocardiogram. If the patient is catheterised, the pulmonary and right ventricular pressures are normal and the pulmonary blood flow is less than twice the systemic blood flow. If left alone a patient with such a defect is not likely to develop any difficulty throughout his life because of the defect itself, however, a periodic assessment of the patient is necessary. The patient and the parents should be advised regarding prophylaxis against infective endocarditis.     

Management of asymptomatic ventricular septal defect

A patient with a small ventricular septal defect should not be subjected to operative closure of the defect. Such a ventricular septal defect is characterized by a normal second sound, normal electrocardiorgram, normal thoracic roentgenogram and echocardiogram. If the patient is catheterised, the pulmonary and right ventricular pressures are normal and the pulmonary blood flow is less than twice the systemic blood flow. If left alone a patient with such a defect is not likely to develop any difficulty throughout his life because of the defect itself, however, a periodic assessment of the patient is necessary. The patient and the parents should be advised regarding prophylaxis against infective endocarditis.     

室间隔缺损修补术后少见并发症的超声心动图诊断病例系列报告

目的： 探讨室间隔缺损(VSD)修补术后少见并发症的超声心动图诊断要点。 方法：通过查询北京安贞医院（我院）超声影像报告系统及病案管理系统获取病例信息,纳入2013年1月至2018年4月在我院诊断或随访的VSD修补术后少见并发症的连续病例,术后时间不限,使用Philips IE33彩色多普勒超声诊断仪,选择S5-1探头,频率1~5 MHz。VSD修补术后少见并发症由我院经验丰富的医生确诊,并经团队医生共同确认。 结果：符合本文纳入标准的VSD修补术后少见并发症34例,男19例,女15例,年龄4个月至25岁,平均（4.7±5.9）岁。28例为术后左室-右房通道（LVRAC）形成,3例为术后主动脉瓣穿孔并主动脉瓣反流,1例为术后LVRAC形成合并主动脉瓣穿孔,2例为术后主动脉-左房通道形成。34例患者中6例接受外科手术矫治,5例LVRAC患者在随诊过程中自行愈合,其余23例患者继续随访观察中。LVRAC超声心动图表现：①心尖及剑突下四腔心切面显示三尖瓣隔瓣与二尖瓣前瓣之间回声中断,缺损位于三尖瓣环上方;②彩色多普勒显示从左室至右房的异常分流信号;③连续多普勒记录到收缩期高速射流频谱。主动脉瓣穿孔超声心动图表现：①左心室长轴及大动脉短轴切面显示无冠瓣或右冠瓣局部回声中断;②彩色多普勒显示源自瓣叶裂孔处的主动脉瓣反流。主动脉-左房通道超声心动图表现：①彩色多普勒显示从主动脉至左房的异常分流信号,左心室长轴、大动脉短轴及五腔心切面均可显示;②连续多普勒记录到双期连续性高速射流频谱。 结论：多切面扫查及密切结合血流频谱特点,可以明确VSD修补术后异常分流信号的诊断。     

Traumatic ventricular septal defect after a kick by a cow

Summary A case is presented of an unusual rupture of the ventricular septum due to a kick from a cow. The 7-year-old boy developed acute cardiac failure. The electrocardiogram showed anterior and apical myocardial infarction. Two-dimensional color and Doppler echocardiography revealed a rupture of the ventricular septum. The traumatic ventricular septal defect was successfully closed using a right atrial approach.     

室间隔缺损的治疗进展

室间隔缺损是儿童最常见的先天性心脏畸形之一.其临床处理方法主要有外科手术修补、经导管介入封堵及镶嵌治疗.体外循环下行外科修补为其传统治疗方法,而作为新的手术方式,经导管介入封堵和镶嵌治疗因具有创伤小、并发症少等优点,成为该领域国内外应用及研究的热点.该文就近年来室间隔缺损三种主要治疗方法的适应证、并发症及最新进展作一综述,以探讨单纯性室间隔缺损的合理治疗方案.     

膜周部室间隔缺损封堵术后Amplatzer封堵器塑形变化对近期疗效的影响

目的探讨膜周部室间隔缺损(perimembranous ventricular septal defect ,PMVSD)封堵术后Amplatzer封堵器(amplatzer asymmetric ventricular septal defect occluder ,AAVSDO)塑形变化对近期疗效的影响.方法共23例PMVSD患者接受Amplatzer封堵术,其中男10例,女13例.所有患者于术后即刻、72 h、6个月和1年行经胸超声心动图(transthoracic echocardiography examination ,TTE)及相同投照体位X线检查.TTE于左室长轴切面测定封堵器左侧盘上缘至主动脉瓣间距(L1).所采集的X线影像经计算机分析系统测量封堵器双盘上缘间距(L2)、下缘间距(L3)、封堵器厚度(L4)、封堵器双盘的直径(D1、D2)和封堵器双盘间夹角(α),以评价近期封堵疗效及可能产生的影响.结果 23例完成术后72 h及6个月随访,12例完成1年随访.术后6个月时L2、L4、α较术后72 h缩小而D1、D2增加(均P＜0.05),提示封堵器厚度变薄、剖面径(profile)减小;术后1年α较术后6个月进一步减小(P<0.05),封堵器双盘更接近平行,而其余指标的差异无显著意义(均P>0.05).L4与封堵器直径(r分别为0.47、0.33、0.39)和α(r分别为0.47、0.53、0.49)呈弱正相关,与D1(r分别为-0.27、-0.45、-0.29)呈弱的负相关.结论 Amplatzer封堵器置入患者体内后,封堵器厚度及剖面径随置入时间推移而逐渐减小,这种塑形变化于术后6个月最为显著,1年时达到稳定.近期随访显示这种变化是安全的,有利于提高完全封堵率.     

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目的探讨三维超声心动图(3DE)在圆锥动脉干畸形(CTD)室间隔缺损(VSD)定量研究中的准确性及其临床意义。方法2000年5月至2003年11月在上海儿童医学中心住院且二维超声心动图(2DE)、经胸3DE、心血管造影及手术等定量测量结果完整的CTD患儿共63例,将3DE测量结果与2DE、造影及手术测值对比。结果方差分析、SNK均数间两两比较及相关分析显示,3DEVSD长径测值为1.69cm(从右室面)和1.63cm(从左室面),虽较手术测值(1.84cm)低估,但低估程度小于2DE测值(1.27cm)及造影测值(1.12cm);3DE与手术测值的相关性(r=0.74)明显高于2DE(r=0.39)及造影测值(r=0.25)。3DEVSD短径测值与造影测值相接近,两者比较无统计学差异(P>0.05)。3DEVSD短径测值与2DE及造影测值的相关性(r=0.45,r=0.54)高于3DEVSD长径测值与2DE及造影测值的相关性(r=0.37,r=0.44)。3DEVSD面积测值与手术测值呈良好相关(r=0.57)。结论3DE可作为CTDVSD定量分析的又一无创手段,能提供较2DE更准确的定量诊断。