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.     

Transcatheter closure of congenital coronary arterial fistula with a detachable balloon

Summary Coronary arterial fistulae are rare congenital cardiac defects that typically are treated by surgery. A case of transcatheter closure of a left anterior descending coronary artery to right ventricular fistula with a detachable balloon is described in a 16-month-old child. The fistula was easily occluded without complication. Follow-up 1.5 years later revealed normal ventricular function and no recurrence of the fistula. Detachable balloon occlusion of coronary arterial fistula is feasible in patients as young as 4 months.     

Bilateral coronary arteriovenous fistulas

Summary A 14-year-old girl with bilateral coronary arteriovenous fistulas (CAVFs) draining into the right ventricle (RV) is presented. Selective coronary angiography revealed involvement of both right and left coronary arteries in the fistulous formation, but communication into the RV was single.     

Balloon occlusion aortography

We review the validity of balloon occlusion aortography (BOA) on the basis of our personal experience with 18 patients with congenital heart disease (mean weight 4.55 g, including 8 neonates). Four of the 18 patients underwent aortic arch angiography using balloon occlusion of the descending aorta. Pulmonary angiography was also performed in 9 patients via a patent ductus arteriosus and in 3 patients via a Blalock-Taussig shunt. The remaining 2 patients underwent coronary arteriography by balloon occlusion of the ascending aorta. The information obtained was satisfactory in 17 of the 18 patients. However, in one patient with a double-outlet right ventricle and pulmonary stenosis, the pulmonary arteries were not clearly visualized because of dominant antegrade flow from the right ventricle. BOA is a safe and useful procedure which can be used to image the aortic arch, pulmonary artery, and coronary arteries in infants with congenital heart diseases. In children over 3 years of age, however, the balloon may not be able to occlude the appropriate site of the aorta, so selective angiography is required to obtain precise information.     

小儿先天性冠状动脉瘘的临床诊断与分析

目的探讨先天性冠状动脉瘘的临床诊断特点.方法回顾性分析有完整病史资料并经手术治疗的小儿先天性冠状动脉瘘21例.结果21例中右冠状动脉瘘15例占71.4%,左冠状动脉瘘6例占28.6%,19例引流到右心系统占90.5%,2例引流到左心系统占9.5%;合并其他畸形3例占14.3%.结论先天性冠状动脉瘘最重要的临床诊断特征是心前区异常位置的连续性杂音,杂音的性质与分流部位、压力相关.彩色多普勒血流显像仪和升主动脉或冠状动脉造影能够明确诊断,可显示迂曲扩张的冠状血管与其引流的心腔部位     

Doppler and 2D echocardiographic diagnosis of congenital coronary artery fistulae to the right cardiac chambers: Report of 3 cases

We present three cases of coronary artery fistulae to the right cardiac chambers. The first was a 2-day-old neonate in congestive heart failure: 2D and Doppler echocardiography revealed a dilated proximal left coronary artery and a fistulous connection to the right atrium. The other two patients, respectively 4 and 3 years old, were asymptomatic and presented with a continuous heart murmur: a left coronary artery fistula into the right ventricle was detected by ultrasound in one, and a dilated proximal right coronary artery in the other. The diagnosis was confirmed in all three patients. The first patient was operated upon at 18 months of age; the second patient is awaiting surgery, and in the third patient the fistula was ligated at the age of 3 years. The possibility of ultrasound diagnosis without invasive procedures is suggested.     

动脉导管造影联合经胸超声在动脉导管未闭封堵术治疗中的应用

目的：探讨经动脉导管造影联合经胸超声能否作为动脉导管未闭封堵术的一种简化治疗方法。方法：选择欲行动脉导管未闭介入封堵术患儿40例,随机分为观察组20例和对照组20例。对照组应用传统的动脉导管未闭介入封堵技术。观察组采用简化的动脉导管未闭介入封堵术,并借助经胸超声实时监测。结果：观察组20例患儿用简化方法行动脉导管未闭封堵术均一次封堵成功,超声显示封堵器位置良好,左、右肺动脉、降主动脉血流速度均在正常范围内。观察组患儿放射线曝露时间明显少于对照组;观察组患儿无一例与血管穿刺相关的血管并发症发生,但对照组患儿发生4例血管并发症。患儿的恢复时间和滞留于监护室的时间也相应缩短;观察组总的医疗费用少于对照组。结论：经动脉导管造影联合经胸超声行PDA封堵术是一种良好的简化封堵方法。［中国当代儿科杂志,2010,12（2）：103-105］     

经导管介入法治疗小儿先天性冠状动脉瘘9例

目的探讨经导管介入法治疗小儿先天性冠状动脉瘘的方法及效果。方法广东省人民医院2002年1月至2005年1月收治的先天性冠状动脉瘘患儿9例,先行心导管检查及选择性冠状动脉造影,显示瘘管的形态及引流情况,建立达到或通过瘘管欲堵闭处的输送轨道。6例瘘管最窄处内径≤3mm的病例选用可控弹簧圈进行堵闭,3例内径>3mm的病例应用Amplazter动脉导管堵闭器进行堵闭。结果8例堵闭成功,1例瘘管扭曲严重的病例堵闭失败而行外科手术。全部病例随诊2个月至3年,随诊中无并发症发生。结论经导管介入治疗冠状动脉瘘是一种创伤性小、疗效确切的方法,但必须严格掌握介入治疗适应证。     

Congenital left coronary artery — right ventricular fistula

ABSTRACT. The case of a neonate with heart failure and myocardial ischaemia due to a large coronary artery fistula is reported. ECG monitoring and Thallium — 201 imaging indicated pre-operative myocardial ischaemia. The diagnosis was confirmed by cardiac catheterization. Successful surgical ligation of the fistula was performed at the age of 10 days and to our knowledge is the first successful ligation in the neonatal period. Follow up Thallium — 201 imaging was normal. Repeat cardiac catheterization demonstrated complete closure of the fistula and normal left ventricular function.     

Coronary artery anomalies and clinically important anatomy in patients with congenital heart disease: multislice CT findings

In patients with congenital heart disease, coronary artery anomalies are common and have different clinical importance from individuals with structurally normal hearts. Visibility of the coronary arteries by CT has markedly improved due to high temporal resolution and ECG-synchronized data acquisition. In this article we describe current multislice CT techniques for coronary artery imaging and illustrate coronary artery anomalies and clinically important coronary artery anatomy from the point of view of congenital heart disease.     

Acute angular origin of left coronary artery from aorta: An unusual cause of left ventricular failure in infancy

Summary An unusual congenital anomaly of the left coronary artery, consisting of ostial stenosis caused by an acute angular origin of the vessel from the aorta in an infant, is presented. This anomaly resulted in progressive myocardial fibrosis and development of left ventricular congestive heart failure. The clinical state mimicked anomalous origin of the left coronary artery from the pulmonary artery.     

Spontaneous closure of a coronary artery fistula in childhood

Summary An infant with a symptomatic coronary artery fistula, documented by angiography, is presented. By age 5 years, clinical evidence of the fistula was no longer present. Repeat cardiac catheterization confirmed spontaneous closure of the coronary artery fistula. More information regarding the natural history of a coronary artery fistula is required before an elective surgical approach can be recommended in all asymptomatic children. Supported in part by Research Grants from the National Institute of Health. Training Grant HL 07413-03     

Congenital coronary artery fistula suggesting a “steal phenomenon” in a neonate

Summary The case of a neonate reported herein demonstrates a congenital fistula between the left circumflex coronary artery and the right ventricle. Intraoperative stress to the patient's heart suggested a coronary steal phenomenon.     

Failure of color flow mapping to identify coronary artery to pulmonary artery fistula

Summary A case of coronary artery to pulmonary artery fistula presented with the typical clinical findings of asymptomatic precordial continuous murmur and small left-to-right shunt. Echocardiography with color flow mapping failed to demonstrate the fistula on three occasions, including one study performed after angiographic demonstration of the fistula site. This case illustrates the importance of angiography in patients with unexplained continuous murmurs, even when echocardiographic and color flow mapping results are normal.     

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.     

The influence of ventricular morphology and coronary artery dominance and morphology on early mortality in patients with pulmonary atresia and intact ventricular septum

Coronary artery abnormalities are known to be present in a certain percentage of patients presenting with pulmonary atresia and intact ventricular septum. These abnormalities range in severity from small fistulae to complete right ventricular dependence of components in the coronary circulation. These abnormalities are known to complicate therapy preoperatively, during palliation, and during definitive correction. This study will explore the influence of right ventricular morphology and coronary artery dominance and morphology on early mortality in infants with pulmonary atresia and intact ventricular septum, something which has not been previously explored.     

Use of Balloon-expandable stents to treat experimental peripheral pulmonary artery and superior vena caval stenosis: Preliminary experience

Summary Current therapy of congenital or acquired stenoses of the peripheral pulmonary arteries and superior vena cava are frequently ineffective. This report describes our initial experience with the use of a balloon-expandable stainless steel stent to treat experimentally created branch pulmonary artery and superior vena cava stenosis. Fifteen adult mongrel dogs had surgically created stenoses of either a branch pulmonary artery and/or superior vena cava. A balloon-expandable stainless steel (0.076 mm), 3 cm long, intravascular stent was used in all animals. Stents were successfully placed in 13 of 15 dogs (nine with branch pulmonary stenosis and four with superior vena caval stenosis) with hemodynamic and angiographic relief of the stenoses in all. In three animals, successful stent placement was not accomplished because the distal right pulmonary artery was found to be totally obstructed in two and in one dog with combined vena cava and pulmonary stenosis the distal right pulmonary artery was so severely stenotic that the stenosis could not be crossed. Repeat catheterization performed 6 months following stent placement documented persistent gradient relief and angiographic evidence of unobstructed flow through the stent without thrombus formation and with patent side branch vessels. Our preliminary results suggests that balloon-expandable stents are a potential therapy for the treatment of branch pulmonary artery and superior vena cava stenoses.     

Left coronary to right ventricle fistula in a child: management strategy based on cardiac-gated 64-slice CT

Congenital coronary fistulae are a diagnostic challenge. A prerequisite for best management is accurate anatomical evaluation, traditionally provided by invasive catheter angiography. Multislice CT (MSCT) is an emerging noninvasive technique for coronary artery evaluation. We present a 3-year-old boy and highlight the clinical usefulness of new-generation MSCT to study coronary artery fistulae in children. Multiplanar and 3-D reconstruction offer invaluable information to plan the best therapeutic strategy in this setting. We provide evidence for the expanding clinical role of MSCT for coronary artery imaging in children.     

Successful percutaneous closure of a coronary artery fistula with a detachable balloon

Precutaneous transcatheter closure technique of a coronary artery fistula with a detachable balloon was performed for a 14 year old male student. Complete closure of the fistula without any complications was confirmed by angiography after the procedure. When the patient underwent a second angiography 6 months after the closure, it was confirmed that the position of the balloon had not changed, that the interruption of the flow of the fistula had been maintained and that the diameter of the left coronary artery and the fistula were reduced. Percutaneous closure technique using a detachable balloon may become the primary treatment for a coronary artery fistula in place of surgical ligation.     

Low-Dose Coronary Artery Computed Tomography Angiogram of an Infant with Tetralogy of Fallot Using a 256-Slice Multidetector Computed Tomography Scanner

The reported incidence of anomalous coronary arteries among children is 0.17%, but it is much greater (8%) among children with tetralogy of Fallot (TOF). The diagnosis of coronary artery anomalies can be difficult using invasive aortography and is not without risk. Technical improvements with multidetector computed tomography (MDCT), including a reduced effective radiation dose, make cardiac CT angiography an attractive alternative not only because the risks of an invasive examination are avoided, but also because sedation risks also are eliminated in most cases. This report highlights 256-slice MDCT imaging of the coronary arteries in a nonsedated 26-day-old infant using an effective radiation dose of only 0.51 millisieverts (mSV).