成人型与婴儿型左冠状动脉异常起源于肺动脉的超声诊断

目的总结不同类型左冠状动脉异常起源于肺动脉（ALCAPA）的超声心动图表现,提高对本病的诊断及鉴别诊断能力。方法回顾性分析首都医科大学附属北京安贞医院儿童心血管病中心收治并经手术证实的24例ALCAPA患儿的超声心动图结果,根据侧支循环的发育情况分为婴儿型和成人型,并总结婴儿型和成人型ALCAPA的超声心动图特征。 结果婴儿型和成人型ALCAPA相同的超声心动图特征：①左冠状动脉窦内无左冠状动脉主干开口,其主干直接与肺动脉连接;②左心室高度扩张,室间隔和左室前壁节段性运动障碍,左室收缩功能减低;③左心室广泛纤维化,以心内膜下区域最为显著;④二尖瓣腱索、乳头肌纤维化,回声显著增强;⑤右冠状动脉起源位置正常,内径增宽;⑥左冠状动脉前降支和回旋支血流为逆向灌注（向心性）;⑦收缩期二尖瓣口可见少至大量反流信号。婴儿型和成人型ALCAPA不同的超声心动图表现：彩色多普勒显示成人型侧支循环血流丰富,婴儿型侧支循环少。 结论婴儿型和成人型ALCAPA具有其特异性的超声心动图特征,结合其临床及心电图等表现,可以对本病做出正确诊断。     

实时三维经胸超声心动图诊断儿童心脏横纹肌瘤的临床价值

目的 探讨实时三维经胸超声心动图（real-time three-dimensional transthoracic echocardiography,RT-3D-TTE）诊断儿童心脏横纹肌瘤的临床价值。方法 2016年3月至2022年3月,在解放军总医院第七医学中心首诊发现的心脏横纹肌瘤患儿中,纳入19例为研究对象,其中15例诊断为结节性硬化症（tuberous sclerosis,TSC）。19例患儿均进行二维经胸超声心动图（two-dimensional transthoracic echocardiography,2D-TTE）及RT-3D-TTE检查,记录横纹肌瘤病灶数量、位置、回声描述、形状、大小等结果。从检出病灶数量、病灶大小、病灶与周边组织关系3个方面比较两者的诊断效能。统计学方法采用χ2检验和配对t检验。结果 2D-TTE和RT-3D-TTE检查均发现6例单发病例;多发者13例,每例检出病灶数目2～9个。2D-TTE共检查出病灶58个,RT-3D-TTE检出病灶56个。以2D-TTE为标准,TR-3D-TTE检出心脏横纹肌瘤的敏感性为96.5%(56/58)。RT-3...     

完全性肺静脉异位引流的超声心动图诊断价值

自１９９０年６月至１９９７年１月７年中,我院彩超室共诊断完全性肺静脉异位引流１２３例,其中４５例行心导管及造影检查并证实。其中男２８例,女１７例,年龄７天至９岁。超声心动图完全性肺静脉异位引流的敏感性为９７．７％,特异性为１００％,异位引流部位诊断的准确率为１００％,其中心内型１８例,心上型２３例,     

经胸超声心动图和螺旋CT冠状动脉成像在川崎病冠状动脉病变随访中应用价值的比较

目的　比较经胸超声心动图（TTE）和螺旋CT冠状动脉成像（CTA）在川崎病（KD）患儿冠状动脉病变（CAL）随访中的价值。方法　回顾性分析青岛大学附属妇女儿童医院2017年6月至2020年1月收治的512例KD患儿的TTE和CTA资料。512例中29例于急性期TTE检查发现CAL而恢复期TTE显示冠状动脉正常,或急性期TTE检查冠状动脉正常但恢复期随访过程中TTE检查冠状动脉可疑异常,或多人次TTE检查冠状动脉测量结果不一致者,将其分为急性期合并CAL（急性期CAL）组以及急性期未合并CAL（急性期Non-CAL）组。所有患儿于恢复期行TTE和CTA检查,分析CAL好发部位、大小、数目和风险分级,比较TTE和CTA对KD患儿CAL的评估价值。结果　29例KD患儿恢复期TTE发现冠状动脉瘤/扩张数19支,CTA发现28支,其中急性期Non-CAL组TTE较CTA漏诊7支（P＜0.05）,分别为左冠状动脉主干（LMCA） 4支,冠状动脉左回旋支（LCX） 3支;两种检查方法对急性期CAL组CAL风险分级评估差异无统计学意义（P＞0.05）,但对急性期Non-CAL组CAL分级评估差异有统计学意义（P＜0.01）,其中TTE评估9例为CAL Ⅰ级,而CTA评估仅4例为Ⅰ级,6例为Ⅲa级,其中1例为TTE冠状动脉无异常。TTE测量的29例KD患儿的LMCA Z值较CTA小（P＜0.01）,而LAD Z值较CTA大（P＜0.05）;另外,急性期CAL组TTE显示分别有5例LMCA Z值和3例RCA Z值正常,但CTA均显示其Z值异常（P＜0.05）;急性期Non-CAL组TTE显示4例患儿LMCA Z值正常,而CTA显示其Z值异常（P＜0.05）。结论　对于急性期发生CAL但恢复期TTE检查正常或急性期未发现CAL而恢复期TTE疑似CAL者不宜单纯采用TTE评估CAL,而应给予CTA验证。     

超声心动图对儿童完全性肺静脉异位引流诊断价值的探讨

目的　旨在评价超声心动图对完全性肺静脉异位引流 (TAPVD)的诊断价值。方法　对 1987年 9月至2 0 0 2年 11月收治的TAPVD患儿 70例进行分析。均有完整的临床及超声心动图资料 ,其中 35例施行了手术治疗。结果　 70例TAPVD的解剖分型为 :心内型 5 2例、心上型 16例、心下型 2例 ,未发现混合型。 35例施行手术 ,32例与术前超声心动图的诊断一致 ;3例与超声心动图诊断不完全一致 ,其超声心动图诊断分别为 :三房心伴继发孔房缺、左房隔膜伴继发孔房缺、原发孔房缺伴卵圆孔未闭 ,而手术结果均显示为心内型TAPVD伴继发孔房缺。结论　超声心动图检查是诊断TAPVD重要且准确的方法 ,并能确定其分型 ,提供精确的解剖及血流动力学资料。在应用超声心动图诊断TAPVD时 ,应注意与三房心、左房隔膜等疾病进行鉴别。     

先天性左冠状动脉主干闭锁3例超声心动图特征及文献复习

目的 通过研究先天性左冠状动脉主干闭锁（LMCAA）的超声心动图表现,提高对LMCAA诊断的准确性。方法 回顾性分析经冠状动脉造影证实的3例LMCAA患儿的超声心动图检查结果,并复习相关文献,总结LMCAA超声心动图特征。结果 LMCAA特异性超声心动图特征：①主动脉左冠状动脉窦内无左冠状动脉主干开口,左冠状动脉主干近心端闭锁呈盲端,远心端内径细窄,发育不良;②右冠状动脉内径增宽;③多切面未显示左冠状动脉与肺动脉确切连接的证据。LMCAA非特异性超声心动图特征：①左心室明显扩大,左室收缩功能可正常或减低;②二尖瓣腱索、乳头肌回声显著增强,可伴有二尖瓣脱垂。彩色多普勒超声特征： ①收缩期二尖瓣口可见中至大量反流信号;② 左、右冠状动脉之间形成细小侧支循环; ③左冠状动脉前降支和回旋支血流为逆向灌注（向心性）;④发育不良的左冠状动脉虽然在肺动脉周围分布,但彩色多普勒超声不能显示其与肺动脉连接的确切逆灌血流信号。结论 LMCAA有特异性的超声心动图特征,提高对LMCAA的全面认识是诊断本病的关键。     

彩色多普勒超声心动图对川崎病急性期冠状动脉病变的诊断价值

目的 探讨彩色多普勒超声心动图在川崎病所致的冠状动脉病变中的诊断价值。方法 对138例川崎病患儿进行二维彩色多普勒超声心动图检查，测量各冠状动脉内径及显示长度，观察冠状动脉内膜及腔内透声情况。结果 发现川崎病患儿冠状动脉不同程度病变71例，其中冠状动脉扩张52例，冠状动脉瘤15例，巨大冠状动脉瘤4例。结论 彩色多普勒超声心动图对川崎病冠状动脉病变的诊断及随访具有安全、简便、可靠、迅速、定位准确、重复性好的优点，为最佳的无创检查方法，具有重要的临床价值。     

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。     

川崎病并发冠状动脉病变的超声心动图表现的动态观察

目的 探讨川崎病(KD)所引起的冠状动脉扩张(CAD)以及冠状动脉瘤(CAA)的超声心动图特点.方法 用彩色多普勒超声心动图检测KD患儿冠状动脉开口和内径,并动态观察静脉免疫球蛋白治疗前后冠状动脉内径的变化情况.结果 本组46例KD患儿,41例患儿行超声心动图检查,合并CAD 12例,左冠状动脉(LCA)较右冠状动脉(RCA)更易受累及(P<0.05);CAA 4例,均为双侧冠状动脉病变,最大内径10 mm.治疗后6～18 d复查超声心动图,CAD组LCA较治疗前明显回缩(P<0.05),RCA内径无变化(P>0.05);CAA组LCA和RCA内径均无明显变化(P>0.05).对10例冠状动脉病变患儿进行随访,其中8例CAD冠状动脉内径均恢复正常,1例CAA冠状动脉病变加重,1例CAA完全恢复正常.结论 (1)在KD所引起的CAD中,LCA比RCA更易受累及,静脉免疫球蛋白治疗后短期内LCA明显回缩.(2)超声心动图是动态观察KD并发CAD和CAA的无创手段.     

左冠状动脉起源于肺动脉的术前诊断与治疗

目的 探讨左冠状动脉起源于肺动脉(anomalous origin of the left coronary artery from the pulmonary artery,ALCAPA)的术前诊断,手术治疗和疗效.方法 对山西省儿童医院2010年6月至2014年3月收治的9例ALCAPA患儿的术前诊断及手术方式进行总结.结果 本组患儿共9例,男3例,女6例,年龄4个月～4岁6个月,平均(1.14±1.33)岁;体重5.6～18.0 kg,平均(8.06±3.99)kg.术前行心脏彩超、心脏增强CT检查,明确诊断,其中有1例患儿行心脏造影检查.9例患儿中合并二尖瓣轻度反流2例,二尖瓣轻-中度反流5例,中-重度反流2例.心电图表现:9例患儿在导联Ⅰ、AVL及V4-6有不同程度的异常Q波,ST段改变或T波倒置.胸部X线检查示心影明显增大,心胸比例0.56～0.73,平均0.61 ±0.05,左心缘饱满隆起,向左下方突起,心脏彩超示左冠状动脉起源于肺动脉,左心室明显扩大,心肌收缩力减弱,射血分数低于50％,8例患儿左心室舒张末径均＞35 mm,1例患儿合并心尖部室壁瘤.1例患儿行心脏彩超及CT检查后怀疑ALCAPA,后经造影检查明确诊断.6例患儿术前行放射性核素心肌显像检查,均伴有不同程度的心尖、前壁、侧壁心肌梗死,部分心肌存活.本组患儿均在全身麻醉体外循环下行左冠状动脉移植术,7例患儿加做二尖瓣成形术.全组患儿早期死亡1例,死亡原因为严重低心排综合征,余均治愈出院.其中,围术期合并低心排综合征2例,肺炎3例,室上性心动过速1例.术后随访3个月～2年,超声心动图检查提示冠状动脉血流通畅,射血分数较术前提高,二尖瓣反流减轻.患儿生长发育良好,无心力衰竭表现,心功能恢复良好.结论 超声心动图对ALCAPA的诊断有重要价值,心脏增强CT及造影检查可确诊,术前核素心肌显像对存活心肌的判定、手术及预后有重要意义.冠状动脉移植术是最理想的手术方法.     

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.     

左冠状动脉起源于肺动脉的临床分析

目的：左冠状动脉起源于肺动脉（anomalous origin of the left coronary artery from the pulmomary artery, ALCAPA)是临床上一种少见的先天性冠状动脉异常性疾病，目前国内报道的资料不多。该研究对其临床特点与治疗进行探讨。方法：对10例诊断为左冠状动脉起源于肺动脉患儿的临床表现、心电图特征、心脏超声、心导管资料、手术治疗及转归进行分析。结果：患儿都有不同程度的多汗、烦躁、气促、拒食等心绞痛及心功能不全的表现；心电图、彩色超声心动图及心导管检查有较特征性的改变；4例患儿行肺动脉内隧道术，4例行直接左冠状动脉移植术，2例行左冠状动脉重建术。术后8例症状改善，2例死亡。结论：掌握ALCAPA的病变特点和相关的诊断线索，辅以彩色超声心动图和心导管检查，可提高ALCAPA的检出率，并早期手术治疗。［中国当代儿科杂志，2007，9（1）：25-27］     

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。     

Pulmonary artery “stop-flow” angiography to visualize the anomalous origin of the left coronary artery from the pulmonary artery in infants

Summary In five infants, anomalous left coronary from the pulmonary artery (ALCA) was diagnosed by selective pulmonary artery angiography using the “stop flow” technique: the left pulmonary artery was occluded by inflating a balloon and a large bolus of dye was injected proximal to the balloon at a high flow velocity. These conditions, by increasing pulmonary artery pressure, increase the chances of imaging directly an ALCA. It also leaves the left pulmonary artery branches free of contrast medium and allows easier visualization of ALCA, mainly in the antero-posterior view. When an ALCA is injected from the pulmonary artery, left heart catheterization can be avoided. However, a false-negative result is to be expected in specific conditions and was observed in one of our patients. If pulmonary angiography fails to prove a ALCA, left heart catheterization remains mandatory to exclude the diagnosis.     

Total anomalous origin of the coronary arteries from the pulmonary artery

Summary The clinicopathologic features of four patients with total anomalous origin of the coronary arteries from the pulmonary artery (TCAPA) are presented and compared with 21 previously reported cases. Patients with TCAPA usually present with left ventricular heart failure, angina, or associated cardiovascular anomalies. Of the 19 patients in whom a clinical history was available, 16 were symptomatic before three days of age. All patients died with 60% dying before two weeks of age. Longer survival was associated with additional cardiovascular anomalies that increased pulmonary arterial perfusion pressure, oxygen saturation, or both. Seventeen (68%) patients had additional cardiovascular anomalies, most commonly atrial (nine cases) or ventricular (eight cases) septal defects and tetralogy of Fallot or other variants of pulmonary atresia (four cases). Only five (22%) of 23 had noncardiovascular anomalies. The coronary arteries arose equally from either one ostium or from two, and the number of ostia was not related to either anomalous coronary artery distribution or to the presence of additional cardiovascular anomalies. Cardiomegaly was present in 56% of cases and the majority of patients had myocardial fibrosis or infarction. Embryology is reviewed and evidence is presented to support the theory of involution-persistence of coronary artery anlagen as the pathogenetic mechanism of TCAPA.     

Surgical management of anomalous left coronary artery with mitral insufficiency in infancy: Contribution of echocardiography

Summary A 6-month-old infant with anomalous left coronary artery underwent a direct left subclavian-left coronary artery anastomosis without the use of cardiopulmonary bypass. Subsequently, mitral valve replacement was performed for severe mitral insufficiency diagnosed echocardiographically to be due to isolated papillary muscle infarction. To our knowledge, this is the youngest patient to have undergone both procedures during the first year of life with excellent results on follow-up studies. The case demonstrates the advantages of using direct subclavian-coronary artery anastomosis and 2-dimensional echocardiography in the management of patients with anomalous left coronary artery.Supported in part by the A.C. Buehler Foundation, Park Ridge, Illinois, USA.     

An uncommon echocardiographic marker for anomalous origin of the left coronary artery from the pulmonary artery: visualization of intercoronary collaterals within the ventricular septum

A rare and striking echocardiographic finding with color-flow Doppler—visualization of intercoronary collaterals within the ventricular septum—in an asymptomatic 5-year-old girl is reported. It strongly suggests anomalous origin of the left coronary artery from the pulmonary artery. Several additional echocardiographic features can secure the diagnosis and allow one to proceed with surgical correction without confirmatory cardiac catheterization and angiocardiography. The ages of our patient and of the seven cases reported previously suggest that these collaterals will more likely be identified beyond infancy and in relatively asymptomatic patients with this congenital anomaly.     

Resolution of pathologic Q wave,left ventricular dysfunction and mitral regurgitation after dual coronary repair of the anomalous origin of the left coronary artery from the pulmonary artery

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital cardiac defect that usually presents as dilated cardiomyopathy in infancy. From 1984 to 2005, 13 (five males and eight females, 0.13%) out of 9,950 patients with congenital heart disease were identified as ALCAPA at our institute. Corrective surgery was performed at a median age of 9 months (range: 2 months to 5 years). Eleven patients underwent direct reimplantation of the left coronary artery (LCA) to the aorta, while two received extrapulmonary baffling. The overall survival rate was 92%. Only one patient died 5 months after reimplantation of the LCA due to acute myocardial ischaemia. Left ventricular ejection fraction (LVEF) was significantly lower in the eight (62%) patients operated during infancy than in those operated beyond 5 months (median: 35% vs. 75%). Left ventricle function was normalised in 11 patients (85%). Among the eight patients without concomitant mitral annuloplasty, mitral regurgitation (MR) improved to a mild or trivial degree in six patients and remained at the pre-operative level in two patients. Pathologic Q wave was noted in 11 patients, which eventually regressed in all except two cases. The median interval of recovery was 16 days, 6 months and 24 months for MR, LVEF and electrocardiogram (ECG) changes, respectively. In conclusion, ALCAPA is also a rare disease in Asian countries, such as Taiwan. The subsequent recovery of MR, left ventricular (LV) function and even pathologic Q wave can be expected after dual coronary repair, regardless of the age at repair.