一侧肺动脉起源于升主动脉的诊治特点探讨

目的 探讨一侧肺动脉起源于升主动脉(AOPA)的临床诊治特点.方法 对12例AOPA患儿的临床特点、超声心动图、心血管造影及于术治疗结果 进行总结分析.结果 12例中男7例,女5例;平均年龄23个月;均有不同程度的气促、喂养困难及反复呼吸道感染史.经心导管检查证实诊断,其中9例为右肺动脉异常起源于升主动脉,为近端型;3例左肺动脉异常起源于升主动脉,1例为近端型,2例为远端型.12例患儿均合并有不同程度的肺动脉高压.超声心动图检查的符合率为83%.9例经手术治疗,其中1例术后4个月死亡.结论 超声心动图对于AOPA的诊断具有重要价值,结合心导管造影检查可确诊,心导管造影对大龄患儿尤其需要;由于存在发展为器质性肺高压的风险,本病一经诊断应尽早行于术治疗.     

彩色多普勒超声心动图对Berry综合征的诊断价值研究

目的分析归纳Berry综合征的超声声像图特点,探讨彩色多普勒超声心动图对Berry综合征的诊断价值。方法以2010年4月至2019年11月在湖南省儿童医院经超声心动图诊断为Berry综合征的8例患儿为研究对象,采用PHILIPS IE33型及PHILIPS EPIQ 7C型彩色多普勒超声心动图诊断仪,配S5-1、S8-3、X7-2探头,频率2. 5～7. 5MHz。除常规扫查切面外,重点扫查大动脉短轴切面、右室流出道肺动脉长轴切面、胸骨上窝主动脉弓长轴切面、剑突下切面等,仔细观察主动脉与肺动脉的间隔连续情况,肺动脉主干走形及左、右肺动脉分支起源,主动脉弓顶端分支结构及远端与降主动脉延续情况等。结果 8例Berry综合征患儿均进行彩色多普勒超声心动图检查,其中7例诊断为Berry综合征,1例术前超声心动图仅仅诊断为动脉导管未闭、主动脉弓缩窄,后经CT血管重建证实Berry综合征并接受手术治疗。患儿心脏畸形包括:Ⅱ型主-肺动脉间隔缺损(8例)、右肺动脉异常起源主动脉(8例)、主动脉弓离断(7例)、主动脉弓狭窄(1例)、动脉导管未闭(8例),8例室间隔连续且完整,均合并肺动脉高压、三尖瓣轻至中度反流; 7例合并卵圆孔未闭。7例经手术治疗痊愈,1例合并重度肺动脉高压、重症肺炎于术前死亡。结论彩色多普勒超声心动图作为一种无创检查方法,能较直观地显示Berry综合征的各种畸形,对于临床疑诊病例,需联合CT血管重建,做出准确判断。     

单侧肺动脉起源于升主动脉的外科治疗

目的 探讨单侧肺动脉起源于升主动脉(AOPA)的外科治疗方法.方法 2006年5月-2008年8月共8例AOPA患儿接受外科治疗.男女各4例;年龄1～96个月;体质量4～24 kg.7例为右肺动脉起源于主动脉,同时并动脉导管未闭、肺动脉高压,其中4例并卵圆孔未闭.1例左肺动脉起源于主动脉,同时并法洛四联症型肺动脉闭锁.病例术前均经心血管增强CT确诊.其中7例右肺动脉起源于主动脉的病例,在体外循环心脏停跳下,游离并切断右肺动脉,将切断的右肺动脉直接吻合于主肺动脉.1例左肺动脉起源异常,由于并法洛四联症型肺动脉闭锁,肺动脉发育较差,因此游离左肺动脉后采用7 mm Goretex管道行中央分流.结果 本组患儿均成活,术后恢复顺利,手术效果良好.术后1个月超声复查右肺动脉与主肺动脉吻合口流速(1.71±0.24)m/s,压差(1.60±0.45)kPa,均未见明显狭窄.结论 AOPA肺动脉高压产生快,应早期诊断,早期手术治疗,近期手术效果良好.但仍需远期观察,以评估手术效果.     

单纯性完全型肺静脉异位引流产前8例超声心动图诊断

摘要 目的 探讨胎儿单纯性完全型肺静脉异位引流（TAPVC）的产前超声心动图特点,提高对本病的产前诊断准确率。方法 回顾性分析2011年5月至2014年2月经新生儿超声心动图、手术或尸解证实的8例单纯性TAPVC的胎儿期超声心动图检查结果,总结超声心动图特征。结果 8例单纯性TAPVC胎儿中,心下型2例,心内型1例,心上型5例,5例存在垂直静脉或共同肺静脉腔与垂直静脉连接处梗阻;8例胎儿中,引产1例（心下型）,余7例均于出生后行手术治疗,其中1例术后死亡（心下型）,6例均恢复良好。TAPVC胎儿超声心动图特征为：①正常的左心房形态消失,呈圆形或椭圆形,左心房光滑并多变小,在孕后期较为明显;②降主动脉与左心房间距离明显增大,多数在左心房后方可见一异常的腔隙（即共同肺静脉腔）,可显示左、右侧上升（心上型）或下降（心下型）的垂直静脉;③妊娠早期左、右心系统比值早期多正常,但妊娠中、晚期（孕26周后）可出现右心系统轻度扩大;④引流入冠状静脉窦时（心内型）,冠状静脉窦可有扩张;引流入上腔静脉时（心上型）,上腔静脉扩张;引流入肝内血管时（心下型）,肝内血管可有不同程度的扩张;⑤彩色多普勒可显示引流途径及是否合并垂直静脉梗阻。结论 在孕早期左、右心系统比值正常时,TAPVC易被漏诊及误诊,应注意多角度、多切面扫查,孕晚期超声心动图检查可减少漏诊。     

主肺动脉窗合并右肺动脉起源于主动脉和主动脉弓离断患儿诊治分析

目的探讨主肺动脉窗(APW)合并右肺动脉起源于主动脉(AORPA)及主动脉弓离断(IAA)患儿的诊断与治疗。方法回顾性分析1例APW合并AORPA及IAA患儿的临床资料。结果患儿,女,4个月,外院诊断为室间隔缺损,保守治疗无效,无法撤离呼吸机,入院后超声心动图提示为APW、AORPA、动脉导管未闭、重度肺动脉高压;外科手术后患儿出现少尿,上、下肢动脉压差大,经CT检查提示合并IAA,再次外科手术矫治;术后患儿顺利恢复并出院,随访28个月,生长发育与同龄儿无明显差别。结论 APW合并AORPA及IAA患儿一经诊断需尽早手术治疗,一期外科纠治效果满意,术前应完善心血管造影等检查防止漏诊。     

儿童右肺动脉异常起源于主动脉的诊断与外科治疗

目的 评价儿童右肺动脉异常起源于主动脉(aortic origin of the right pulmonary artery,AORPA)的治疗方法及效果,并探讨AORPA的诊断与鉴别诊断方法.方法 回顾性分析1993年5月至2007年7月11例儿童AORPA术前诊断及手术治疗的围术期疗效及中长期随访结果.分别采用右肺动脉经人工血管(2例)或同种主动脉(2例)与主肺动脉吻合术、体外循环下右肺动脉与主肺动脉直接吻合术(3例)、体外循环下(主动脉环及肺动脉环片)双片法右肺动脉与主肺动脉吻合术(2例)、非体外循环下右肺动脉经人工血管与主肺动脉吻合术(2例).结果 11例中10例术前确诊,1例术前漏诊而在术中发现.5例通过右室造影及主动脉造影或多排CT/MRI检查确诊,6例单纯超声心动图检查患儿中确诊5例,1例漏诊.11例手术患儿围术期死亡2例.术后随访2.2～13年,1例于术后4年死于右心功能衰竭.生存8例患儿术后超声心动图复查射血分数(EF)值43%～52%,心功能I级5例,Ⅱ级3例.结论 右室造影及主动脉造影或多排CT/MRI检查可减少漏诊率.手术是治疗AORPA的有效方法,但应尽早手术以进一步提高手术疗效.应用自体组织加宽加长右肺动脉可减少吻合口狭窄的可能性.     

室间隔完整的肺动脉闭锁合并右室依赖冠状动脉循环胎儿超声心动图特征

目的 探讨室间隔完整的肺动脉闭锁（PA/IVS）合并右室依赖冠状动脉循环（RVDCC）胎儿超声心动图特征,提高对本病的认识及诊断的准确率。方法 回顾性分析2010年9月至2013年5月于首都医科大学附属北京安贞医院儿童心血管病中心就诊,经超声心动图确诊的4例PA/IVS合并RVDCC的胎儿超声心动图特征,并与PA/IVS未合并RVDCC的胎儿和正常胎心胎儿超声心动图表现进行比较。结果 4例PA/IVS合并RVDCC胎儿中,3例引产后经尸解证实,1例出生后经超声心动图证实。与PA/IVS未合并RVDCC胎儿及正常胎心胎儿超声心动图表现比较,PA/IVS合并RVDCC胎儿超声心动图除了具有室间隔完整的肺动脉瓣膜性闭锁的一般超声影像学特征（室间隔完整、肺动脉膜性闭锁和动脉导管逆灌等）外,还具有：①右心室显著发育不良、右室壁多显著肥厚、三尖瓣口（环）径明显缩小和Z值﹤-3;②彩色多普勒血流显像显示右室心肌内冠状窦隙开放,形成右心室与冠状动脉的异常血流交通;③异常血流方向为从右心室至冠状动脉灌注为主的双期双向血流信号。结论 胎儿超声心动图可准确诊断PA/IVS合并RVDCC,一经确诊,应尽早采取相应的干预措施。     

超声心动图诊断胎儿先天性心脏病的研究

目的了解超声心动图在胎儿先天性心脏病（简称先心病）诊断中的价值。方法从对１８００例胎儿四室心初筛中选出１６３例胎儿应用胎儿心脏各切面和多普勒测定进行超声心动图检测。５例同时作了ＭＲＩ检查。结果发现心脏异常３２例（１９．６％），其中先心病２１例（１２．９％）。５例胎儿先心病作了ＭＲＩ检查，发现其影像较超声清晰。结论两项检查合用，可提高胎儿先心病的诊断水平；胎儿超声检查中增加卵圆孔直径和血流的测定，可以作为房间隔缺损的诊断标准。     

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。     

左冠状动脉起源于肺动脉的诊治特点

目的探讨左冠状动脉起源于肺动脉的临床诊治特点。方法对16例左冠状动脉起源于肺动脉患者的体表心电图、超声心动图、心血管造影检查及手术治疗结果进行分析。结果①心电图:Ⅰ、Ⅱ、aVL、V5、V6导联T波倒置且ST段压低8例,其中6例伴左胸导联异常Q波;T波倒置4例。②超声心动图:16例左冠状动脉起源于肺动脉患儿中合并房间隔缺损2例,左室侧壁运动减弱及左室射血分数(LVEF)降低6例。③15例心导管造影提示左冠状动脉起源于肺动脉,合并房间隔缺损2例,合并动脉导管未闭1例。④治疗结果:所有患儿均进行手术治疗,死亡2例,余14例随访1个月～6 a,冠状动脉血流通畅。结论对于左冠状动脉起源于肺动脉的诊断,应结合其特征性心电图表现有助于诊断,超声心动图具重要价值,结合心导管造影检查可确诊,一经诊断应尽早手术治疗。     

Right pulmonary artery torsion following surgical reimplantation

We report the case of a 3-month-old boy with an anomalous origin of right pulmonary artery (AORPA) from the ascending aorta who presented postoperatively with torsion of the right pulmonary artery demonstrated on 3-D volume-rendered CT angiogram. To our knowledge, CT images of this entity have not been reported. This case illustrates a rare surgical complication that can be easily imaged by CT.     

Contrast echocardiography in the diagnosis of anomalous left coronary artery arising from the pulmonary artery

Summary We have demonstrated an anomalous left coronary artery originating from the main pulmonary artery by saline contrast echocardiography. The anomalous vessel was easily identified using contrast injections into the ascending aorta at catheterization and into a radial artery cannula following catheterization. Contrast echocardiography may be useful in the diagnosis of this lesion when two-dimensional and Doppler echocardiography fail to show the anomalous vessel.     

Anomalous origin of the right pulmonary artery from the abdominal aorta with aberrant right subclavian artery and left patent ductus arteriosus

Anomalous origin of the pulmonary artery (AOPA) from the aorta is a rare congenital heart malformation. This report describes a case of AOPA from the abdominal aorta in association with an aberrant right subclavian artery and a patent ductus arteriosus, which never has been reported previously in the literature.     

Severe Right Ventricular Dysfunction in a Neonate with Aortic Origin of the RPA

Anomalous origin of the right pulmonary artery from the ascending aorta (AORPA) with severe right ventricular dysfunction in a neonate was completely diagnosed by echocardiography. Cardiac catheterization was performed to explore further right ventricular dysfunction, which is extremely rare at this age. Following cardiac catheterization, the patient died. We suggest that in critically ill infants with the aforementioned condition surgical repair guided by modern echocardiography must be the chosen management.     

Noncompaction with arcus aorta anomalies

An 18-month-old girl with tetralogy of Fallot (TOF), mental retardation and multiple infarcts on brain computerized tomography (CT) is presented. Her sineangiocardiograms showed a thrombus (moving from the left ventricle to ascending and abdominal aorta), TOF, right arcus aorta with anomalous origin of left subclavian artery from the descending aorta, honeycombed appearance of the apex and half of the posterior wall of left ventricle and the apex of right ventricle. Her echocardiography (ECHO) examinations revealed depressed left ventricular systolic function and thrombus in the left ventricle apex, with noncompaction of the left ventricle apex and the middle portion of posterior wall, and in the right ventricular apex.     

Septal Course of Left Anterior Descending Artery From the Right Aortic Sinus in Tetralogy of Fallot: A Benign Anomaly and Important Lessons Learned

The proximal course of an anomalously arising coronary artery is a decisive factor in the surgical approach for tetralogy of Fallot (TOF). Studies have shown that echocardiography provides a good anatomic definition of the ostium and proximal epicardial course of coronary arteries [1, 2]. This report describes a case of TOF with an atrioventricular canal defect whose preoperative echocardiography showed abnormal origin of the left anterior descending artery (LAD) from right aortic sinus, which was interpreted as crossing the right ventricular outflow tract. Perioperative inspection did not show any abnormal vessel crossing the outflow, and corrective surgery was performed. At the echocardiographic evaluation after surgery, it was noted that the abnormal LAD arising from right aortic sinus was taking a septal course in relation to the posterior aspect of the pulmonary annulus. It is important to recognize this anomalous course because it is benign with no surgical implications.     

Magnetic resonance imaging compared with echocardiography in the evaluation of pulmonary artery abnormalities in children with tetralogy of Fallot following palliative and corrective surgery

Background. Abnormalities of the pulmonary arteries following palliative or corrective surgery for tetralogy of Fallot (TOF) are common. Our purpose was to compare the usefulness of magnetic resonance imaging (MRI) and echocardiography in the post- operative evaluation of the pulmonary arteries in children with TOF. Objective. Our hypothesis was that MRI is more sensitive than echocardiography in the detection of branch pulmonary artery abnormalities in children with TOF. Materials and methods. Pulmonary artery MRI and echocardiography were performed in 20 children following palliative and/or corrective surgery for TOF. MRI and echocardiography were compared in their ability to detect abnormalities of the pulmonary arteries. Angiographic or surgical correlation was available in 15 children. A perfusion scan for confirmation of pulmonary artery patency was available in one additional child. Results. Abnormalities of the branch pulmonary arteries identified by MRI included: absence or occlusion (2), focal stenosis (15), hypoplasia (2), aneurysm (1), and non-confluence (1). Echocardiography could not adequately visualize the right and left branch pulmonary arteries in eight and ten children, respectively. Echocardiography missed stenosis in 13 branch pulmonary arteries, patency of hypoplastic pulmonary arteries in two children, non-confluence of the pulmonary arteries in one child, and a left pulmonary artery aneurysm in one child. Abnormalities identified by MRI were confirmed in 16 children by angiography, surgery or perfusion scan. Conclusion. MRI is more sensitive than echocardiography for the evaluation of branch pulmonary artery abnormalities in children following surgery for TOF. Received: 13 January 1997 Accepted: 31 July 1997     

Diagnosis of berry syndrome in an infant by two-dimensional and color Doppler echocardiography

A 6-month-old male infant with coexistent type B interrupted aortic arch, distal aortopulmonary window, and anomalous origin of the right pulmonary artery arising from the ascending aorta was diagnosed by two-dimensional echocardiography, color Doppler, and cardiac catheterization. Review of the available literature reveals this patient to be the fourteenth reported case of this unusual association of cardiovascular defects.