Coronary artery fistulas in neonates,infants, and children: clinical findings and outcome

Ten patients were diagnosed with coronary artery fistula (CAF) between 1991 and 1998 in our department. The ages of patients ranged from 3 days to 12 years. Five patients were male and five patients were female. Nine patients had single CAF and 1 patient had dual CAFs. CAFs of 7 patients originated from the left coronary artery, and CAFs of 3 patients originated from the right coronary artery. CAFs of 7 patients terminated at the right ventricle, CAFs of 2 patients terminated at the right atrium, and the CAF of 1 patient terminated at the pulmonary artery. Four patients were diagnosed with CAF in the neonate period. All presented with congestive heart failure. Medical therapy was successful in treating congestive heart failure in 2 of these patients, but the other 2 needed operations. One patient presented with subacute bacterial endocarditis at 12 years of age requiring surgical intervention. One patient had a large left-to-right shunt that was surgically repaired. One patient with dual CAFs underwent coil embolization and the larger CAF achieved complete embolization, but the smaller CAF failed. Follow-up data revealed that 1 patient who received an operation died of sepsis due to recurrent bronchiolitis 6 months later. Nine patients were asymptomatic. Because complications including endocarditis may be encountered in later life, we suggest early closure with coil embolization.     

Surgical Outcome of Coronary Artery Fistulas Repair in Children

Coronary artery fistula (CAF) is a rare congenital anomaly that can be complicated by endocarditis, myocardial infarction, or coronary aneurysms. This article reviews the clinical characteristics and surgical outcome of 10 patients with CAF in Rajae Heart Center. From 1990 to 2000, 10 patients (age, 6 months to 15 years; mean age, 8.5 years) were diagnosed with CAF by echocardiography and cardiac catheterization. Six were female and 4 were male. All patients with isolated CAF (9) were asymptomatic. One patient with associated anomaly (mitral valve prolapse with severe mitral regurgitation) had dyspnea on exertion and palpitation. Five fistulas originated from the right coronary artery—three from the left and two from the left circumflex. Drainage was to the right ventricle (7), right atrium (2), and pulmonary artery (1). The ratio of pulmonary to systemic flow ranged between 1 and 1.6. All patients had surgical ligation. In the symptomatic patient, in addition to ligation, mitral valve replacement was performed. There was no operative or late death. Follow-up evaluation (range, 1–6 years; mean, 4.2 years) showed no evidence of recurrent or residual CAF. Surgical management of CAF is a safe and effective treatment resulting in 100% survival and clousure rate.     

儿童孤立性冠状动脉瘘的治疗及中期随访分析

目的对儿童孤立性冠状动脉瘘的治疗过程及中期随访结果进行总结分析。方法回顾性分析广州市妇女儿童医疗中心2009年7月至2017年7月诊断为孤立性冠状动脉瘘的17例患儿临床资料,其中男11例,女6例,年龄40日龄至12岁(中位值23个月),体重3.8~29 kg(中位体重11.3 kg)。2例存在气促表现,2例存在生长发育落后表现,2例存在反复呼吸道感染表现,1例主诉心悸,余10例无明显临床症状。其中3例心脏检查未发现明显杂音,14例发现心脏杂音。瘘口发生于右冠状动脉8例,左冠状动脉9例。瘘入右心房5例,右心室10例,左心室2例;17例中12例合并巨大冠状动脉瘤。结果1例行经皮冠状动脉瘘封堵术,1例在非体外循环下行瘘管结扎术,其余15例在体外循环下行冠状动脉瘘矫治术;平均住院时间(11.1±4.1)d。其中2例术后3 d内出现射血分数下降(最低者降至38%),术后1个月复查时射血分数均升至50%以上。7例术后出现一过性T波改变,出院时均已恢复正常;2例术后存在1~2 mm残余瘘的患儿在近期复查中残余瘘消失。围术期无一例死亡。随访10个月至9年,所有患儿无自觉症状和阳性体征,心电图均无心肌缺血表现,复查超声心动图均提示心脏收缩功能正常,冠状动脉直径均较术前缩小。结论大多数儿童孤立性冠状动脉瘘无明显症状,但可合并巨大冠状动脉瘤,应尽早手术,中远期预后良好;但因存在冠状动脉扩张,仍需长期随访。     

Echocardiographic Evaluation of Coronary Artery Fistula in Pediatric Patients

The management of pediatric patients with coronary artery fistula (CAF) remains controversial because the clinical course of CAF may vary greatly from spontaneous closure to severe complications. The purpose of our study was to report the outcome of CAF in pediatric patients by using echocardiography as an evaluation tool. Between January 1997 and July 2003, 17 patients (age range, 5 months to 14 years; mean, 3.8 years) with coronary angiographically proven CAF were recruited. We divided patients into two groups. Group 1 (n = 4) included patients with symptoms related to CAF (n = 3), persistent coronary artery dilatation for 6 months (n = 4), and/or a continuous waveform of the fistula detected by Doppler (n = 3). Group 2 (n = 13) included patients who were asymptomatic, with normal coronary artery size or coronary artery dilatation less than 6 months, and/or a noncontinuous waveform of the fistula detected by Doppler. Group 1 patients received interventional therapy, whereas group 2 patients were managed conservatively. There were 6 male and 11 female patients. The correlation coefficient of coronary artery diameter measured on echocardiography and angiography was 0.935 and 0.834 in groups 1 and 2, respectively. The diameter of the involved coronary artery was 5.8-9.2 mm (mean, 7.50 ± 1.85) and 1.7-3.8 mm (mean, 2.72 ± 0.59) in group 1 and group 2, respectively. After transcatheter coil embolization, group 1 patients became asymptomatic with no residual fistula and had decrement of the coronary artery diameter (p = 0.035). All group 2 patients remained asymptomatic with no significant change in coronary artery size (p = 0.846) and 3 of them showed spontaneous closure of CAF. Persistent dilatation of the diameter of proximal coronary artery may be a useful parameter for determining subsequent application of interventional therapy. In patients with nonsignificant CAF, conservative follow-up is strongly suggested and intervention procedures may be unnecessary.     

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.     

Midterm Outcome of Percutaneous Transcatheter Coil Occlusion of Coronary Artery Fistula

The mid- to long-term outcome of pediatric patients with coronary artery fistula (CAF) after transcatheter closure remains unknown. This study reports the midterm outcome of CAF closure by transcatheter Gianturco coil occlusion in pediatric patients. Eighteen patients with CAF confirmed by coronary angiography were seen in our institution. Among them, 4 patients who had persistent coronary artery dilatation received transcatheter Gianturco coil occlusion (age range, 25 months to 12 years; mean, 4.6 years). Two were closed with a single coil, and two were closed with two coils. No residual shunts were detected on follow-up angiography and echocardiography immediately after coil occlusion in all cases. Follow-up Doppler echocardiography (4/4) and angiography (3/4) showed persistent CAF occlusion in all 4 patients. Two of 3 patients with coexistent aneurysmal formation showed obliteration of the aneurysm on angiographic follow-up. In addition, 2 showed resumption of normal coronary arterial size, and 2 showed decrement of the coronary arterial diameter. All 4 patients had normal ECG findings after 26–83 months (mean, 49 months) of follow-up, and all were in NYHA functional class I. The midterm outcome of percutaneous transcatheter coil occlusion in the treatment of CAF is excellent.     

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。     

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。     

Percutaneous coronary intervention using drug‐eluting stents in pediatric heart transplant recipients

Abstract: PCI has been used for palliation of CAV in adults, but there are limited data available in children. We sought to evaluate our experience with PCIs for CAV in pediatric heart transplant recipients. Retrospective review of the medical records of all four patients who were diagnosed with CAV, including demographic data and catheterization reports was performed. Of the 149 pediatric heart transplant recipients followed at our institution, 10 were identified with CAV. Four of these 10 underwent 12 PCI procedures for CAV. One donor heart had documented coronary artery disease. Two patients had significant risk factors for coronary artery disease including morbid obesity, hyperlipidemia, and systemic hypertension. PCI involved deployment of bare metal stents (n = 2), paclitaxel‐eluting stent (n = 6), and sirolimus‐eluting stents (n = 4) with procedural success in all and no early or late mortality. One procedure was complicated by coronary dissection that was successfully treated with stent placement. One patient has been re‐transplanted while the other three are not candidates for re‐transplantation and have remained asymptomatic as palliation with PCI. PCI using coronary stents is a safe and effective palliative measure for CAV in pediatric heart transplant recipients.     

经导管应用弹簧圈封堵术治疗儿童心血管畸形

目的　探讨应用弹簧圈治疗儿童心血管畸形的指征、方法学和并发症的预防。方法　133例心血 管畸形患儿接受了经导管应用弹簧圈封堵术。术后随访,定期行心脏超声。结果　101例动脉导管未闭(PDA)中 应用Gianturco弹簧圈14例,Duct Occlud和Nit Occlud弹簧圈87例,PDA最小直径为1.6±0.6mm(0.5～ 3.8mm)。有4例植入2只弹簧圈。即刻封堵率为90.1%(91/101);1月后封堵率98.0%(99/101),1年后封堵率 99.0%(100/101)。应用Gianturco弹簧圈封堵主动脉至肺动脉侧枝血管14例,直径3.5±0.8mm(2.1～5.0mm), 5例植入1只弹簧圈,9例植入2～4只弹簧圈。14例封堵后10～15min完全封堵。封堵冠状动脉瘘共14例,直径 3.8±1.1mm(2.0～5.1mm),其中右冠状动脉右室和右房瘘8例,左冠状动脉前降支或回旋支右室瘘和右房瘘6 例,10例植入1只Gianturco弹簧圈,2例植入2～4只Gianturco弹簧圈,应用Duct Occlud弹簧圈1例,即刻封堵率 为38.5%(5/13),1月后封堵率84.6%(11/13);1例右冠状动脉右室瘘,在封堵后即刻发生弹簧圈漂移至左肺小 动脉,用异物钳取出后再外科手术。肺动静脉瘘2例,全部应用Gianturco弹簧圈,分别植入6和16只弹簧圈,封堵 后外周动脉血氧饱和度从76%上升至91%和96%。应用Nit Occlud弹簧圈各1只封堵有假性室隔     

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.     

A Tale of Two Brothers: Anomalous Coronary Arteries in Two Siblings

We report a case of a 10-year-old boy with aberrant left coronary artery from the right sinus of Valsalva, whose presenting sign was cardiac arrest. The patient's asymptomatic younger brother was found to have aberrant right coronary artery from the left sinus of Valsalva. This is the first report of a familial clustering of coronary artery anomalies in which the initial presentation of one subject was sudden cardiac death and a sibling harboring a similar lesion was identified while asymptomatic.     

Congenital Anomalies of Coronary Arteries in Children: The Evaluation of 22 Patients

Although congenital coronary artery anomalies are seen in 0.6–1 % of adult patients undergoing coronary angiography, the data for the pediatric population are few. This study of 22 children with coronary artery anomalies evaluated them in terms of demographic and clinical features and analyzed their angiographic findings and surgical results. Databases in the Department of Pediatric Cardiology at the University of Uludag were searched for all the patients with a diagnosis of congenital coronary artery anomaly who underwent coronary angiography between 1993 and 2013. Patients with coexistent congenital heart disease were excluded from the study. The study noted 22 patients (0.9 %; 10 boys and 11 girls) with coronary artery anomalies. The mean age of these patients was 58.77 ± 52.04 months (range, 1 month–16 years). Coronary arteriovenous fistula (50 %) and anomalous left coronary artery from the pulmonary artery (ALCAPA) (36 %) were the most common anomalies. In addition, the study included one patient with diffuse coronary artery hypoplasia, one patient with muscular bridge, and one patient with left main coronary artery originating from the right aortic sinus valsalva. Of the 11 patients who had coronary atrioventricular fistula, 7 were asymptomatic, whereas 75 % of the patients with ALCAPA syndrome were admitted because of heart failure. Although 13 patients had an exact diagnosis by echocardiography, 50 % of the patients with ALCAPA syndrome had their diagnosis determined by catheter angiography performed because of severe mitral regurgitation or dilated cardiomyopathy. The mortality rate for all the patients was found to be 18.1 %. Eight patients with coronary arteriovenous fistula have been followed up without surgery to the present. In contrast, seven patients with ALCAPA syndrome have undergone surgery, and three have died. Two of these patients died during the postoperative period, and the remaining patient died suddenly during the preoperative period at home. Isolated congenital coronary artery anomalies are very rare in the pediatric population. Although most congenital coronary artery anomalies are clinically silent, they may be associated with severe symptoms in children. Recognition of potentially serious anomalies such as ALCAPA syndrome is mandatory so that early surgical treatment can be prescribed.     

川崎病冠状动脉病变远期随访评估:二维超声与冠状动脉造影对照观察

目的探讨体表十二导联心电图(ECG)、二维经胸超声心动图(2DE)、64排螺旋CT冠脉成像技术(MDCT)和选择性冠状动脉造影(CAG)在川崎病(KD)严重冠状动脉病变远期随访中的应用价值。方法回顾性分析2006年7月至2010年9月于复旦大学附属儿科医院随访的15例KD并发严重冠状动脉病变(美国冠状动脉分级Ⅲ～Ⅴ级)患儿的临床特征、治疗方案以及ECG、2DE、MDCT和CAG检查结果。结果 15例KD患儿进入分析,KD发病年龄为5个月至13.5岁,平均(4.8±1.1)岁;病程3～64个月,平均(17.4±13.0)个月。2例主诉反复心绞痛发作,2例表现为充血性心力衰竭,其余患儿无明显临床症状。ECG检查发现异常Q波及ST-T波改变3例;MDCT与CAG检查结果显示,在单支、两支和三支冠状动脉中发生巨大瘤样病变的患儿分别为5例、5例和3例;6例合并远端冠状动脉瘤;2例可见冠状动脉瘤内血栓形成,但未形成狭窄;4例存在冠状动脉严重梗阻性病变,其中右冠状动脉合并左回旋支完全闭塞1例,右冠状动脉合并左前降支严重狭窄2例,右回旋支完全闭塞1例。冠状动脉病变Ⅲ级1例,Ⅳ级10例,Ⅴ级4例。与MDCT、CAG结果对照,2DE诊断完全符合率为46.7%(7/15例),其中遗漏诊断远端冠状动脉瘤6例,冠状动脉严重狭窄和(或)闭塞4例,冠状动脉内附壁血栓1例。结论对于2DE诊断为严重冠状动脉病变的KD患儿,应予以进一步MDCT或CAG检查以了解冠状动脉病变全貌,为治疗策略的制定提供更多信息。     

Percutaneous Transluminal Coronary Rotational Atherectomy for Localized Stenosis Caused by Kawasaki Disease

We report the results of percutaneous transluminal coronary rotational atherectomy (PTCRA) for localized stenosis caused by Kawasaki disease (KD) in children. Five males and a females, aged 5–15 years old (median, 9), underwent PTCRA. The interval from the onset of KD to PTCRA ranged from 5 to 12 years (median, 9). The target vessels were the left anterior descending artery (three patients), the left circumflex (two patients), and the right coronary artery (one patient). The immediate results of PTCRA were successful in all patients, and the mean stenosis degree improved from 89 ± 10% to 27 ± 12%. In follow-up coronary angiograms within 1 year, four vessels were restenosed, including two with complete occlusion. A 15-year-old male has had good patency for 4 years after undergoing re-PTCRA for restenosis using a larger burr size. PTCRA is feasible for severe localized stenosis with calcification caused by KD in children and the immediate results are good. However, restenosis often occurred within 1 year after PTCRA in small children and PTCRA is not always appropriate for them. When the use of a larger burr is possible, good patency can be expected and can be maintained by close follow-up and re-PTCRA.     

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.     

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     

Dipyridamole-provoked chest pain implies severe coronary artery disease in children

The diagnostic significance of dipyridamole-provoked chest pain was studied in 17 children with severe coronary arterial stenotic lesions (CAL) complicated with Kawasaki disease. Although dipyridamole induced chest pain in seven patients (symptomatic group), 10 reported no pain (asymptomatic group). In the asymptomatic group, seven children had one vessel disease (1VD) of right coronary artery (RCA) and the other three had two vessel disease (2VD) involving the RCA and left anterior descending artery (LAD). Four multivessel disease patients, one three vessel disease (3VD) and three 2VD of LAD and RCA, and three 1VD of LAD, were symptomatic. In the thallium scans, all patients, except two of the asymptomatic group, showed perfusion abnormalities. In addition, the extent score of the symptomatic group was significantly worse than that of the asymptomatic group (P = 0.01). While only one in six of the asymptomatic group showed abnormal ST depression on treadmill exercise electrocardiography, all patients in the symptomatic group (P = 0.02) showed ischemic ST depression. These findings suggest that the occurrence of chest pain after medication with dipyridamole closely correlates with the severity of CAL in children.     

Two-dimensional and Doppler echocardiography in the non-invasive diagnosis of coronary cardiac fistula]

Two asymptomatic children with an atypical continuous murmur and a clinical diagnosis of coronary artery fistula are reported. Cross-sectional and Doppler echocardiography led to the diagnosis of a right coronary artery-right ventricule fistula in both cases, which was confirmed by coronary angiography. Surgical closure of the fistula was realized without complications.     

川崎病并发严重冠状动脉病变患儿旁路移植术术前评估及术后随访

目的回顾性总结川崎病(KD)并发严重冠状动脉病变患儿的影像学诊断、冠状动脉旁路移植术结果及随访情况。方法复旦大学附属儿科医院2006年8月至2008年3月收治5例(男3例,女2例)KD并发严重冠状动脉病变患儿,年龄12个月至10岁4个月。所有患儿均在KD急性期予IVIG治疗,其中3例予2次。病程第15～21天均行超声心动图(ECHO)检查发现冠状动脉病变;之后常规随访ECG和ECHO,并口服阿司匹林和双嘧达莫或氯吡格雷。1例患儿行64排螺旋CT(MSCT)检查,2例行99Tcm-MIBI心肌灌注显像检查。在病程6～65个月行选择性冠状动脉造影检查,并行冠状动脉旁路移植术及冠状动脉成形术。结果 1/5例在病程中有心绞痛发作,3/5例有心功能不全,1/5例无症状。1/5例有心肌梗死发生并在恢复过程有ECG记录;2/5例ECG有ST-T改变;2/5例ECG未见异常。ECHO除均显示多发性冠状动脉瘤(CAA)外,3/5例还显示左心房和左心室增大,左室射血分数(LVEF)和短轴缩短率的降低,2/5例左室壁运动不协调,与99Tcm-MIBI检查结果一致。冠状动脉造影均可见多发性CAA且至少一处为中等或巨大CAA,并伴有血栓形成和远端闭塞。MSCT与冠状动脉造影检查结果一致。年龄最小的1例患儿(手术时22个月)在冠状动脉移植术中死亡,其余4例术后即刻效果满意。在8～24个月的随访中,1例LVEF较低,随访1年未达到正常;1例术后18个月复发KD,治疗顺利,复查ECHO和MSCT未见冠状动脉进一步损害。4例患儿目前仍在随访中。结论 KD并发冠状动脉病变应定期随访ECG、ECHO,必要时进行99Tcm-MIBI和MSCT检查;如考虑手术需行冠状动脉造影仔细评估冠状动脉病变部位和程度。有心肌缺血表现需及时行冠状动脉旁路移植术和冠状动脉成形术,手术的近期效果显著,远期疗效有待长期随访结果。