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     

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.     

Two-dimensional and Doppler echocardiographic features of left circumflex coronary artery to right ventricle fistula: Case report and literature review

Summary We report the case of a seven-month-old infant with a clinical diagnosis of patent ductus arteriosus whose two-dimensional and Doppler echocardiographic examinations were consistent with a coronary artery fistula. At angiography, a left circumflex coronary artery to right ventricle fistula was diagnosed. Echo-Doppler techniques for the noninvasive diagnosis of this lesion are discussed and the literature is reviewed.     

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.     

A case of right coronary artery to left ventricle fistula: Two-dimensional echocardiographic study

Summary A 12-year-old boy with a decrescendo diastolic murmur maximal at the lower right sternal border was suspected of having a coronary arteriovenous fistula. Cardiac catheterization and angiography revealed a right coronary artery to left ventricle fistula with marked dilatation of the right coronary artery.This is the thirteenth case of this anomaly reported in the literature. Characteristic two-dimensional echocardiographic features of this case are presented. A markedly dilated right coronary artery was revealed by two-dimensional echocardiography to take a normal right coronary artery course and empty into the posteromedial portion of the left ventricle.This case indicates the possibility of visualizing a dilated peripheral portion of the right coronary artery using two-dimensional echocardiography.     

Pulmonary atresia and ventricular septal defect with coronary artery to pulmonary artery fistula: Case report and review of the literature

The pulmonary blood supply in patients with pulmonary atresia and ventricular septal defect is highly variable. Several cases have been reported in the literature in which a coronary artery to pulmonary artery fistula, alone or in combination with additional vessels, supplies the pulmonary circulation. We report a case of successful repair during early infancy, with unique hemodynamic, clinical, and anatomic features. The literature is reviewed.     

Surgical therapy of a coronary artery fistula draining into the left atrium

Coronary artery fistulas (CAFs) are rare vascular anomalies. We describe a case of CAF draining into the left atrium, combined with an atrial septum defect (ASD) and azygos continuity in a 3-year-old girl. Surgical therapy included closure of the ASD combined with closure of the CAF through a left atrial approach. Even though CAF can be treated by interventional means, surgical correction may be required in the presence of associated cardiac abnormalities.     

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 coronary artery abnormalities

Congenital coronary artery abnormalities are rare and account for approximately 0.1 to 2% of congenital heart defects. They may pose significant risk of mortality or morbidity to the patient. The pediatrician and the pediatric cardiologist should be aware of their subtle but very serious presentations and diagnostic steps to be undertaken to pinpoint the diagnosis. Prevention of serious complications from these abnormalities can be achieved by making the appropriate diagnosis and performing timely surgical intervention. This review will discuss the most common congenital coronary artery abnormalities and their management.     

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.     

Coronary artery fistula complicating the evaluation of Kawasaki disease

Summary Two patients clinically diagnosed with Kawasaki disease were found to have a coronary artery to pulmonary artery fistula. The dilemma of deciding the etiology of coronary artery dilatation in these patients is discussed along with management.     

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     

Percutaneous transcatheter occlusion of coronary artery fistulas using detachable balloons

Three pediatric patients underwent successful transcatheter coronary artery fistula occlusion using the Debrun system. This latex balloon system offers several advantages over other occlusion systems. First, the balloon delivery and release is controlled. Second, “test occlusions” can be performed that allow simultaneous balloon inflation, coronary cineangiography, and electrocardiographic monitoring. Third, because the balloons are flow-directed, they are easily positioned in properly chosen locations. Finally, the balloons can be constructed to suit the size of the fistula. In this study, two patients received only one balloon; in the other patient two balloons were placed in the same fistula. All fistulas drained into either the right atrium or ventricle and were successfully occluded. After a follow-up period of up to 3 years, no local or systemic reactions to the balloons were recognized. We conclude that detachable balloon occlusion of coronary artery fistulas is a safe, effective alternative to surgical ligation in selected pediatric patients.     

Echocardiographic diagnosis of fistula between the left circumflex coronary artery and the left atrium

Summary This report describes a case of fistula between the left circumflex coronary artery and the left atrium, which was identified by color flow mapping. This finding was confirmed by selective coronary arteriography.     

Transcatheter embolization of coronary artery fistula with controlled release coils

ABSTRACT The treatment for large congenital coronary cameral fistulas has been surgical but with advances in interventional catheterization techniques transcatheter embolization of these fistulas with coils or detachable balloons is now possible. This report describes occlusion of a congenital coronary arteriovenous fistula in a 6-year-old girl.     

Congenital left ventricular aneurysm coexisting with left ventricular non‐compaction in a newborn

Described herein is the case of a rare combination of congenital left ventricular (LV) aneurysm and left ventricular non‐compaction (LVNC) in a newborn. The patient developed refractory heart failure soon after birth and died at 5 months of age. The etiology of both congenital LV aneurysm and LVNC seems to be maldevelopment of the ventricular myocardium during early fetal life. Treatment should be individually tailored depending on clinical severity, and treatment options are limited. Given that this combination of congenital LV aneurysm and LVNC is significantly associated with poor prognosis, it appears that patients with congenital LV aneurysm and LVNC are candidates for early, aggressive intervention, including surgical aneurysmectomy and evaluation for transplantation. It is important to be aware of this combination of congenital LV aneurysm and LVNC, and to make earlier decisions on therapeutic strategy.     

Anomalous left coronary artery from pulmonary artery (ALCAPA) in infants: a 5-year review in a defined birth cohort

Anomalous left coronary artery from pulmonary artery (ALCAPA) is a rare congenital cardiac malformation. It presents predominantly in infancy with features of myocardial ischaemia or cardiac failure and may be mistaken for common paediatric conditions such as colic, reflux or bronchiolitis. With early surgical correction the prognosis is good, but awareness of this condition is essential for prompt diagnosis and referral to a tertiary cardiac centre. In this report we review the five cases that presented during our 5-year study period and discuss the incidence and clinical presentation of ALCAPA among infants. Our observed incidence of 1 in 4243 live births – 0.023 % – is higher than previously reported. ALCAPA may be more common than previously recognised, and there should be a high index of awareness among paediatricians, paediatric trainees and general practitioners to enable early surgical intervention and improved prognosis for these children.     

不同类型先天性冠状动脉瘘介入治疗随访研究

目的 评价儿童不同类型冠状动脉瘘(CAF)介入封堵治疗的近中期疗效、并发症及抗凝治疗方案。方法 回顾性分析2006 年1 月至2014 年1 月行CAF 介入封堵治疗的12 例患儿术前、造影及术后随访资料,记录不同类型CAF 的封堵方式、抗凝方案、术后并发症、辅助检查结果。结果 本组患儿年龄1~158 个月;近端型/中型4 例,近端型/大型5 例,远端型/中型3 例,均成功封堵;术后随访3.5±2.4 年;11 例患儿术后口服阿司匹林6 个月,1 例口服18 个月;无血栓、介入并发症,术后左心室射血分数、心胸比、肺动脉压下降,病变冠状动脉开口直径减小。结论 儿童期介入封堵治疗近端型和远端型/中型CAF 近中期疗效、安全性满意;术后阿司匹林抗凝治疗可预防近中期血栓事件,但疗程和安全性有待进一步随访研究。     

Isolated congenital coronary arteriovenous fistula

During the last eight years a total of nine cases of isolated congenital coronary arteriovenous fistula (CAVF) have been diagnosed at our centre. Age of these patients ranged from 11 days to 22 years (mean 6.4±4.2 years). There were seven male and two female patients. Right and left coronary arteries were the sites of origin in seven and three cases respectively. One patient had two fistulae, one each originating from right coronary artery and obtuse marginal artery. The drainage sites were right ventricle in seven cases and right atrium in two. The pulmonary artery pressure was normal in eight cases and elevated in one case. The pulmonary artery to systemic flow ratio ranged from 1.1∶1–2.4∶1 in all except the 11 day-old-baby in whom it was 4∶1. Pulmonary vascular resistance (PVRI) was below 3.5 in all cases (range 1.0–3.5 units). Surgical ligation of fistula was done in four cases and two patients underwent coil embolisation of fistula. Follow-up is available in five out of eight patients including three cases in whom CAVF was closed. Two other young patients are being followed-up conservatively and all the patients are asymptomatic. CAVF can present at any age; majority of the cases have small left to right shunts and more commonly CAVF arise from right coronary artery. Surgical ligation of fistula is safe in older patients, non-surgical embolisation using coils may be an alternative to surgery in selected cases.