儿童左冠状动脉异常起源于右冠状窦伴动脉间行程四例分析

目的分析儿童左冠状动脉异常起源于右冠状窦伴动脉间行程(ALCA-R-IAC)的临床特征和诊治要点.方法对2015年11月至2018年6月在首都医科大学附属北京儿童医院住院确诊的4例ALCA-R-IAC患儿的临床表现、实验室检查、影像学特点、治疗及预后进行回顾性分析.结果4例患儿均为女童,发病年龄7.5~14.7岁,全部经CT冠状动脉成像(CTCA)检查确诊为ALCA-R-IAC.4例患儿均有运动性晕厥、急性心肌梗死的临床表现,伴急性左心衰竭3例、心源性休克1例、心跳骤停1例;1例患儿出现神经系统受累.所有患儿肌钙蛋白Ⅰ均明显增高(20.65~50.00μg/L);心电图显示左主干闭塞的图形特征;超声心动图发现3例射血分数降低(25%~45%),仅1例提示左冠状动脉异常起源可能.CTCA均表现为左冠状动脉异常起源于右冠状窦,并走行于主动脉和肺动脉之间,左主干受压纤细等.2例行冠状动脉去顶术治疗,2例保守对症予严格限制活动.门诊规律随访12~43个月,4例患儿均存活,无症状反复发作,预后良好.结论ALCA-R-IAC可导致青少年运动性晕厥、急性心肌梗死甚至猝死的临床表现.CTCA有助于及早明确诊断,一旦确诊应避免剧烈活动,外科手术是主要治疗手段.     

Improvement of left ventricular function after ligation of the anomalous left coronary artery during infancy

Summary Considerable controversy surrounds the optimal management of symptomatic infants with anomalous origin of the left coronary artery from the pulmonary artery; this includes the timing and type of surgical intervention. Long-term follow-up was obtained on three patients who had simple ligation of the anomalous left coronary artery at or before 8 months of age; long-term follow-up on an adolescent treated with a saphenous vein bypass graft from the aorta to the coronary artery was available for comparison. Remarkable increase in left ventricular contractility was observed in the three patients treated only by simple ligation of the anomalous left coronary artery during infancy. Thallous chloride T1 201 exercise studies disclosed no perfusion abnormalities in any of the patients despite angiographically demonstrable wall-motion abnormalities in two of the four patients. Our study suggests that simple ligation of the anomalous left coronary artery near its origin in patients with angiographically detectable left-to-right shunting can provide long-term survival with good left ventricular function and minimal clinical complaints.     

左室收缩功能正常的左冠状动脉异常起源于肺动脉患儿的超声诊断13例病例系列报告

目的:探讨左室收缩功能正常的左冠状动脉异常起源于肺动脉(ALCAPA)患儿的超声心动图特点。方法:回顾性分析2010年1月至2019年12月广州市妇女儿童医疗中心收治并经手术证实的13例左心室射血分数（LVEF）≥55%的ALCAPA患儿的超声心动图特征和临床资料。结果:13例ALCAPA患儿均可见右冠状动脉增宽,其他超声征象包括左冠状动脉血流逆灌12例、左冠状动脉直接开口于肺动脉9例、肺动脉内逆灌血流7例、心肌侧枝血流8例、左室明显增大8例、二尖瓣功能不全7例、左室心内膜纤维化5例。<1岁ALCAPA患儿的心肌侧枝血流信号不明显,左心室大小正常或轻度增大且二尖瓣功能良好;≥1岁患儿可见丰富侧枝血管、左室明显扩大以及二尖瓣功能不全。4例误诊的ALCAPA左冠状动脉均起源于肺动脉右后方。结论:超声心动图可准确诊断左室收缩功能正常的ALCAPA,如果超声科医生在怀疑该诊断时能全面探查冠状动脉的起源和血流方向,可减少漏诊和误诊。     

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.     

儿童冠状动脉疾病32例临床分析

目的探讨儿童冠状动脉疾病的病因分布及临床特点,以提高诊断和治疗儿童冠状动脉疾病水平。方法回顾性分析2008年7月至2011年12月在首都医科大学附属北京安贞医院儿童心脏中心收治的冠状动脉疾病患儿（不包括复杂心脏畸形合并冠状动脉异常者）的临床资料,分析临床表现、辅助检查、诊断方法和治疗。结果32例冠状动脉疾病患儿进入分析,男20例,女12例,年龄1个月至18岁。经ECG、超声心动图、64排CT和心导管造影检查明确诊断。左冠状动脉异常起源于肺动脉10例(31.2%),8例以心力衰竭起病,其中长期误诊为“心内膜弹力纤维增生症”4例,“扩张性心肌病”1例,“二尖瓣重度关闭不全”2例,均行外科手术移植左冠状动脉,1例术后死亡,余9例术后随访临床症状好转,左室缩小,二尖瓣反流减轻;冠状动脉瘘13例(40.6%),9例以心脏杂音、4例以心力衰竭起病,外科手术行瘘缝扎或补片治疗12例,介入治疗1例,术后随访仅1例存在少量残余分流;川崎病并发冠状动脉瘤6例(18.8%),出现左心扩大、心肌缺血征象,予长期抗凝等治疗;家族性高胆固醇血症3例(9.4%),表现为冠状动脉粥样硬化性心脏病,予降胆固醇、改善心肌缺血等药物及饮食控制治疗,随访病情尚稳定,无明显加重。结论儿童冠状动脉疾病少见,先天性包括左冠状动脉异常起源于肺动脉和冠状动脉瘘,后天性以高胆固醇血症和川崎病累及冠状动脉多见,对于有左心扩大和心肌缺血表现者,应重视冠状动脉疾病的可能,早期诊断、合理治疗是改善预后的关键。     

Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery Confirmed by 320-Slice Computed Tomography

Anomalous origin the left coronary artery from the pulmonary artery (ALCAPA) is an extremely rare congenital coronary abnormality that may be difficult to diagnose by echocardiography. Most patients present with a potentially fatal illness leading to sudden cardiac death during infancy. This report describes a 15-year-old girl who had 15-year history of cardiac murmur but with no clinical symptoms. Echocardiographic examination was normal, but a 320-slice computed tomographic (CT) scan showed the anomalous origin of the left coronary artery form the pulmonary artery. This case demonstrates that the 320-slice CT scan is a sensitive and reliable technique for establishing the diagnosis of ALCAPA in both symptomatic and asymptomatic patients when it cannot be visualized by echocardiography.     

The 64-Multislice Computed Tomogram Averts Misdiagnosis of an Anomalous Origin of the Left Main Coronary Artery

A 38-year-old woman was discovered to have a systolic murmur for an unrelated complaint. Transesophageal echocardiography showed no atrial or ventricular septal defects, but multiple large collateral vessels in inter-ventricular septum. The origin of left coronary artery was not seen at the expected site on the aortic root. The 64-multislice computed tomography confirmed the diagnosis of an anomalous origin of the left coronary artery from the pulmonary artery. Left coronary artery was revascularized with a saphenous vein graft with an uneventful recovery.     

Bland-White-Garland syndrome of anomalous left coronary artery arising from the pulmonary artery (ALCAPA): a historical review

The landmark 1933 case report from Massachusetts General Hospital by Bland, White and Garland (Am Heart J 8:787–801) described a 3-month-old child with progressive feeding problems, cardiomegaly on chest radiography, and EKG evidence of left ventricular damage. Of interest was the fact that the vigilant father of the infant was Aubrey Hampton, a radiologist and future chairman of radiology at Massachusetts General Hospital. At autopsy, the left coronary artery originated from the pulmonary artery rather than from the aorta. Effective treatment for this condition was not available until 1960 when Sabiston, Neill and Taussig showed that the blood flowed from the left coronary artery toward the pulmonary artery. The anomalous left coronary artery was ligated at its junction with the pulmonary artery and the child survived. This historical review of Bland-White-Garland syndrome, now known as anomalous left coronary artery arising from the pulmonary artery (ALCAPA), stresses the continued diagnostic significance of cardiomegaly on chest radiography and EKG changes suggesting left ventricular damage in 2- to 3-month-old infants with feeding intolerance or irritability. With a high index of suspicion, an echocardiogram can be obtained to confirm the diagnosis. Modern surgical methods involve left coronary artery translocation and afford excellent outcomes.     

儿童冠状动脉起源异常合并心肌损害临床特征及诊治分析（附72例报告）

目的　探讨冠状动脉起源异常合并心肌损害的临床特点及相应治疗策略,提高临床医师对此类疾病的认识和诊治水平。方法　回顾性分析2015-2017年首都医科大学附属北京儿童医院心脏内科诊治的72例冠状动脉起源异常合并心肌损害患儿的临床资料,总结其临床特点和诊疗方法。结果　本组42例（58.3%）患儿以胸痛、胸闷、心悸为主要临床表现,心电图异常以ST-T改变（62.5%）为主,超声心动图结果示13例（18.1%）患儿合并心脏扩大,4例（5.6%）合并左室收缩功能减低。冠状动脉CT血管造影检查（CTA）结果示左冠状动脉起源异常最为常见（73.6%）,其次为双侧冠状动脉起源异常（13.9%）,单纯右冠状动脉起源异常最少见（12.5%）。所有患儿诊治过程顺利,无死亡病例。结论　冠状动脉起源异常可导致不同程度心肌供血问题,引起缺血性心肌损害,在患者的检查中更应重视CTA对冠状动脉开口位置和走行的判断,在诊断治疗中更应注意其与儿童心肌炎的鉴别。     

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目的 探讨小儿左冠状动脉起源于肺动脉(ALCAPA)所致心脏扩大病例的误诊原因,总结诊断经验.方法 回顾性分析1996年7月至2009年1月广东省心血管病研究所22例小儿ALCAPA所致心脏扩大的误诊病例.均行心电图、X线胸片、超声心动图检查,8例行心脏CT检查,14例行心导管检查及造影,21例进行了外科手术治疗.结果 ≤1岁患儿10例中,6例误诊为心内膜弹力纤维增生痘,3例误诊为扩张型心肌病,1例误诊为先天性二尖瓣脱垂并关闭不全.>1岁患儿12例中,4例误诊为扩张型心肌病,3例误诊为心内膜弹力纤维增生症,2例误诊为先天性二尖瓣脱垂并关闭不全,1例误诊为右冠状动脉右心室瘘,1例误诊为川崎病,1例误诊为动脉导管未闭.结论 小儿ALCAPA所致心脏增大病例容易误诊为心内膜弹力纤维增生症、扩张型心肌病、先天性二尖瓣脱垂等疾病,左冠状动脉起源于肺动脉后方偏右的病例更易误诊.提高对ALCAPA的认识、超声或CT检查中仔细探查冠状动脉及其起源对避免误诊至关重要.     

左冠状动脉起源于肺动脉37例患儿各年龄段的临床特点及诊治

目的分析各年龄段左冠状动脉起源于肺动脉(ALCAPA)患儿的临床特点和诊治情况,提高对此病的认识,减少误诊,提高救治率。方法 1999年4月-2011年3月本院共收治37例ALCAPA患儿。男21例,女16例;年龄(5.0±4.8)岁。其中婴儿组(≤1岁)8例,幼儿组(>1～3岁)10例,大年龄组(>3岁)19例。37例中并二尖瓣关闭不全31例,室壁瘤2例,法洛四联症/ASD、重度肺动脉瓣狭窄/部分型肺静脉异位引流、ASD各1例。对各年龄段临床特点和诊治结果进行回顾性分析。随访结果依据门诊复查结果及电话随访。结果婴幼儿组发病年龄均早于大年龄组;ECG出现Q波及ST-T改变13例(35.1%,13/37例),其中婴儿组5例(62.5%)、幼儿组3例(30.0%)、大年龄组5例(26.3%),3组差异无统计学意义;胸部X片示心脏均明显增大,心胸比婴儿组大于幼儿组和大年龄组,幼儿组大于大年龄组;超声心动图示左心室舒张末径(LVED)3组均增大,以婴儿组增大最明显;左心室射血分数(LVEF)<50%者婴儿组多于幼儿组和大年龄组,幼儿组与大年龄组无明显差异。首次诊断时误诊或漏诊18例,占48.6%(18/37例)。婴儿组1例由于核素扫描提示存活心肌很少,等待心脏移植,另2例家长放弃手术。34例患儿行冠状动脉移植术23例,肺动脉内隧道术8例,冠状动脉搭桥2例,单纯结扎冠状动脉左主干1例。无手术早期死亡。术后有近期随访资料26例(76.5%,26/34例),随访6～130(13.6±18.4)个月,晚期死亡1例。患儿术后LVED均较术前缩小,仍有二尖瓣轻度关闭不全8例。有随访资料患儿中,8例术前LVEF<50%,术后3～6个月LVEF均恢复正常。结论 ALCAPA婴儿患儿临床表现较幼儿及年长儿患儿重。小儿ALCAPA由于缺乏特征性表现,各年龄段均易误诊。对于小儿各年龄段表现为心脏扩大、心功能不全、二尖瓣关闭不全等患儿,要注意除外小儿ALCAPA。可以结合其ECG表现,反复超声ECG探查冠状动脉,必要时加行多层螺旋CT或心血管造影以明确诊断,确诊后应尽快手术治疗。     

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.     

Anomalous origin of the left coronary artery from the pulmonary artery

Anomalous origin of the left coronary artery from the pulmonary artery (ALCA-PA) is a rare form of congenital heart disease. In this report, three cases with this anomaly are described; two patients presented in infancy with heart failure from myocardial ischemia and infarction, while the third was asymptomatic and ALCA-PA was diagnosed during evaluation of a residual murmur after surgery for associated cardiac defects (ventricular septal defect and patent arterial duct). All three cases underwent aorto-pulmonary tunnel repair (Tukeuchi procedure), and to our knowledge two of them are the first infantile cases reported in Turkey.     

Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery in Children: 15 Years Experience

This study aimed to illustrate the experience of treating children with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). The clinical data for 19 children with ALCAPA admitted to Beijing Anzhen Hospital from August 1993 to June 2009 were reviewed. According to the data, 47.4% (9/19) of the patients had a misdiagnosis of endocardial fibroelastosis, and 15.8% (3/19) had a misdisgnosis of dilated cardiomyopathy. Electrocardiography showed abnormal Q waves with T wave inversion in leads I, avL, and V4-V6 of 18 patients, especially in lead avL. The ratio of proximal right coronary artery diameter to aotic root diameter exceeded 0.20 for 15 of 16 patients. Apical ventricular aneurysm or aneurysmal dilation (52.6%,10/19), enhanced echogenicity of papillary muscles (84.2%, 16/19), and increased coronary collaterals (78.9%, 15/19) were detected frequently during echocardiography. A total of 18 patients underwent cardiac surgery including left coronary artery (LCA) ligation for 1 patient (5.6%), LCA ligation plus coronary artery bypass grafting for 1 patient (5.6%), Takeuchi operation for 7 patients (38.9%), and LCA reimplantation for 9 patients (50.0%). Five patients died in the hospital, and the remainder were asymptomatic during a follow-up period of 6 to 166 months. Their abnormal Q waves gradually regressed, and left ventricular systolic function and size returned to normal with alleviation of mitral insufficiency. The clinical features of ALCAPA are helpful for determining an accurate diagnosis. This anomaly can be treated successfully by several types of operations with good prognosis.     

Non-invasive diagnosis and management of coronary arteriovenous fistula. A case report

Coronary arteriovenous fistulas are rare anomalies resulting in abnormal communication between the coronary artery and any chamber of the heart. An asymptomatic patient was referred for evaluation of her murmur. Two-dimensional and color Doppler echocardiographic evaluation revealed an enlarged left main coronary artery. A retrograde, eccentric small jet was found within the right ventricular outflow tract at the pulmonary artery valvular level allowing us to detect the entrance site of the fistula. The diagnosis was confirmed by cardiac catheterization and angiocardiography. Although our case was asymptomatic, the decision to perform cardiac surgery was made because of the aneurysmatic appearance of the left coronary artery. In our opinion, visualization of coronary arteries by two-dimensional echocardiography, together with additional information obtained from the Doppler examination, provides an excellent technique for the noninvasive diagnosis of coronary artery fistula.     

Incidence of cardiac abnormalities in children with human immunodeficiency virus infection: The prospective P2C2 HIV study

OBJECTIVE: To describe the 5-year cumulative incidence of cardiac dysfunction in human immunodeficiency virus (HIV)-infected children.Study design: We used a prospective cohort design, enrolling children at 10 hospitals. Group I included 205 vertically HIV-infected children enrolled at a median age of 1.9 years. Group II consisted of 600 HIV-exposed children enrolled prenatally or as neonates, of whom 93 were ultimately HIV-infected. The main outcome measures were echocardiographic indexes of left ventricular dysfunction. RESULTS: In group I, the 5-year cumulative incidence of left ventricular fractional shortening 相似文献   

左冠状动脉异常起源于肺动脉22例诊断分析

目的 探讨左冠状动脉异常起源于肺动脉的临床诊断方法.方法 收集本院2000年2月至2007年2月左冠状动脉异常起源于肺动脉患儿22例,分为婴儿组(年龄≤1岁,n=10)和少儿组(年龄＞1岁且≤15岁,n=12),回顾性分析临床表现、心电图和经胸超声心动图表现.其中心电图主要分析如下特征:(1)Ⅰ导联Q波和T波倒置,(2)aVL导联异常Q波和T波倒置,(3)V5-6导联Q波,(4)v4-6导联T波倒置或ST段变化,(5)左心室肥大.经胸超声心动图主要分析如下特点:(1)左冠状动脉与肺动脉的连接,(2)肺动脉内逆向的多普勒血流,(3)左心室乳头肌的回声强度,(4)右冠状动脉扩张,(5)室壁和室隔间侧支循环信号.结果 胸片示心影增大(18/22)、心电图示aVL导联异常Q波和T波倒置(17/22)、超声心动图示汇入肺动脉的逆向多普勒血流(20/22)、前外侧乳头肌强回声(17/22)和侧支循环信号(16/22)在两组患儿中的出现率均很高,差异无统计学意义(P＞0.05);婴儿组的临床症状和心电图示V5-6导联Q波、Ⅰ Q波和T波倒置的出现率均显著高于少儿组(P＜0.05);婴儿组右冠状动脉扩张的出现率要显著低于少儿组(p＜0.05).结论 婴儿和少儿患者的诊断特点不完全相同,胸部X线片示心影增大、心电图示aVL导联异常Q波和T波倒置对本病诊断有重要的提示作用,而超声心动图示逆向汇入肺动脉的彩色多普勒血流、前外侧乳头肌强回声和侧支循环信号等间接征象强烈提示本病的诊断,结合临床病史、心电图和超声心动图检查可以确诊本病.     

Fehlabgang der linken Koronararterie aus der A. pulmonalis (ALCAPA)

Background. ALCAPA is the anomalous origin of the left coronary artery from the pulmonary artery. It leads to more or less severe myocardial ischemia.The patients may manifest as infants with severe cardiac failure and mitral regurgitation,others may reach adulthood without symptoms. Methods and patients. Five children were operated upon for this anomaly in this center during the past 2 years.At the same time 2 adults were detected. Results. The diagnosis was made in the neonatal period in one patient, the oldest patient was 58 years of age at the time of the diagnosis. The left coronary artery was reimplanted into the aorta in all children. 2 patients after extremly poor preoperative situations needed temporary extracorporeal assist devices. All patients showed improvement of myocardial function after surgery. Conclusion. Surgery for ALCAPA should be performed soon after diagnosis has been made.The postoperative course is influenced by the preoperative degree of myocardial lesion and mitral regurgitation.Even desperate cases can be treated successfully and with good long term results by efficient intensive care using ventricular assist devices or ECMO.     

Bland-White-Garland syndrome. Clinical aspects, diagnosis, therapy]

The anomalous origin of the left coronary artery from the pulmonary artery (Bland-White-Garland Syndrome) is a rare congenital malformation reported to occur in 0.25-0.5% of all congenital cardiac anomalies. The clinical and pathomorphological picture can be classified into 2 types: infantile or adult. The infantile type is thought to lack coronary collaterals; this explains the bad prognosis and symptoms of non-operated patients. In the adult type collaterals are present or have developed in time to provide adequate blood supply to the myocardium. No history of cardiac complains and a nearly normal ECG are common findings. Echocardiography and cardiac catheterisation are mandatory diagnostic tools. The value of new methods like NMR, "stop-flow" angiography and color doppler flow mapping is still in discussion. The reimplantation of the coronary artery and Takeuchi-Operation are well established surgical methods. The time for the operation and prognostic outcome are related to the preoperative underlying myocardial damage measured by left ventricular function.     

Reconstruction of coronary artery anomaly in an infant using the internal mammary artery: 10-year follow-up

Summary We report a case of tetralogy of Fallot with an unsuspected anomalous left anterior descending coronary artery arising from the right coronary artery and crossing the right ventricular outflow tract in a 16-month-old infant. During operation, the anomalous artery was severed. Successful repair of the intracardiac anomalies was performed, including left anterior descending-internal mammary artery reconstruction of the anomalous artery. This case illustrates the importance of delineation of coronary artery anatomy and even selective coronary arteriography in patients with tetralogy of Fallot, since anomalous coronary arteries occur most frequently in association with other cardiac anomalies. Arteriography 10 years later revealed a patent anastomosis to the left anterior descending artery, proving the durability of the internal mammary artery in a patient we believe to be the youngest to have undergone bypass with this particular conduit.