Cardiac Computed Tomography of Multiple Coronary Arteries to Right Ventricle Fistulas in a Newborn With Pulmonary Atresia and Intact Ventricular Septum

Right ventricle-dependent coronary circulation significantly alters the therapeutic options and prognosis in neonates with pulmonary atresia and intact ventricular septum. The contribution of cardiac computed tomography for the accurate assessment of multiple coronary arteries to right ventricle fistulas in a newborn infant with pulmonary atresia and intact ventricular septum is described.     

Coronary artery fistula embolization in an infant with pulmonary atresia intact ventricular septum: a case report.

This report presents a case of pulmonary atresia/intact ventricular septum with right ventricular-dependent coronary circulation. At 7 months of age, the infant underwent coil embolization of the connection between the right ventricle and the coronary circulation. The child is currently well following surgical decompression of the right ventricle.     

Coronary arteries from a single coronary ostium in the right coronary sinus: a previously unreported anatomy

The coronary circulation originating from a single coronary ostium is rare. All possible anatomical variations were the basis of a recent classification. This case report describes a previously unreported IID(1) pattern, comprising a solitary coronary ostium in the right coronary sinus with an anatomical course of the right coronary artery. The left circumflex coronary artery arises from the proximal right coronary artery coursing behind the aorta to the left. The left anterior descending coronary artery arises from the proximal right coronary artery coursing to the left side anterior to the right ventricle.     

Right coronary artery fistula to left ventricle complicated with huge coronary artery aneurysm

Congenital coronary artery fistula (CAF) with huge coronary artery aneurysm is a very rare condition. In this paper, we describe a 26-year-old asymptomatic male patient with right coronary artery (RCA) to the left ventricle fistula with a huge coronary artery aneurysm which was diagnosed by multidetector computed tomography and coronary angiography. The patient received surgical treatment for coronary artery after diagnosis. Both RCA and a giant aneurysm were excised; surgical closure of CAF and coronary artery bypass grafting were performed on this patient. Two months after surgery, the enlarged left ventricle returned to normal as evaluated by echocardiography.     

Outcome in infants with pulmonary atresia, intact ventricular septum, and right ventricle-dependent coronary circulation

Management of all patients with pulmonary atresia, intact ventricular septum, and right ventricle-dependent coronary circulation (n = 12) with staged surgery directed toward a Fontan palliation resulted in an 83% 5-year actuarial survival. Both deaths in the study were presumably related to coronary ischemia and occurred in the first 4 months of life.     

Noninvasive diagnosis of coronary artery fistula by Doppler color flow mapping

Sixteen patients with coronary artery fistula proved by coronary angiography or surgery were studied using two-dimensional echocardiography and Doppler color flow mapping. The coronary artery fistula drained into the right atrium in 4 patients, the right ventricle in 2 and the pulmonary artery in 10. The dilated coronary artery was visualized in 7 of the 16 patients with a fistula, as compared with none of the 40 control subjects. These 7 patients included 5 of 6 patients with a fistula draining into the right atrium or right ventricle and only 2 of 10 patients with a fistula draining into the pulmonary artery. Abnormal flow signals in the dilated coronary artery were visualized with Doppler color flow mapping in five of these seven patients. Color flow imaging visualized abnormal flow signals with mosaic appearance in the pulmonary artery in eight patients, the right atrium in four and the right ventricle in two. The chamber in which abnormal signals were detected corresponded with the entry site of the fistula by angiography. Intraoperative imaging during surgical repair was needed in two cases to confirm ligation of all arteries feeding into the fistula network. In conclusion, Doppler color flow imaging is diagnostically useful to visualize shunt flows originating from the opening or exit of a coronary artery fistula. Furthermore, intraoperative use of this technique may provide confirmation of successful surgical ligation of the fistula.     

冠状动脉瘘28例诊断及外科治疗

目的 总结冠状动脉瘘的诊断和手术治疗效果。方法 28例不同部位的冠状动脉瘘患者采用超声心动图和选择性冠状动脉造影,明确冠状动脉瘘发生位置,全部采用外科治疗,统计其疗效。结果 单纯冠状动脉瘘20例,合并其他心内畸形8例;右冠状动脉瘘18例,左冠状动脉瘘8例,双冠状动脉瘘2例。瘘入右心室13例,瘘入右心房12例,瘘入左心室1例,瘘入肺动脉2例。心腔内双瘘口及三个瘘口各有1例,余26例为单一瘘口。所有病例行手术治疗,8例合并其他心内畸形同期矫治。全组无死亡及残余瘘,效果满意。结论 心脏直视手术治疗冠状动脉瘘效果肯定,合并其他心内畸形应同期矫治。选择性冠状动脉造影对明确冠状动脉瘘发生位置和（或）瘘人心腔的位置非常必要。     

An elephant trunk appearance—what is the diagnosis?

Coronary cameral fistula is a rare congenital cardiac abnormality. An 18‐year‐old boy presented with features of right heart volume overload. Clinical examination was suggestive of hyperdynamic circulation with continuous murmur in precordium. 2D echocardiography showed dilated right coronary artery, and 3D echocardiography added information in tracing the track of the fistula which was consistent with the diagnosis of right coronary cameral fistula draining into the right ventricle. Coronary angiograms revealed an unusually dilated right coronary artery giving the appearance of an “elephant trunk” and with a fistulous tract into the right ventricle. Considering the higher risks of surgery in such difficult cases, we performed a successful transcatheter closure of the fistula using an Amplatzer vascular plug.     

Competition of coronary arteries and ventriculo-coronary arterial communications in pulmonary atresia with intact ventricular septum

Pulmonary atresia with intact ventricular septum can be complicated by the presence of large ventriculo-coronary arterial communications which disturb normal myocardial perfusion. In the selection of patients for surgery the presence of these communications provides an additional problem. On the basis of previous cineangiocardiographic study, we performed a histopathological study of 16 cases. Twelve presented with ventriculo-coronary arterial communications and four only showed myocardial sinusoids. A tripartite right ventricle was seen in the latter group but not exclusively. It is shown that subepicardial coronary arterial pathology is exclusively present in cases with ventriculo-coronary arterial communications although not solely at a connection site between a communication and an artery. The affected coronary artery is itself focally abnormal and hypoplastic and can be blocked or even absent. The interruption of such an artery can also occur after birth and not necessarily at a site of connection with a communication. This implies that infants with communications either already have a coronary circulation partly or completely dependent on ventriculo-coronary arterial communications at birth or can develop such a condition in time. This hazard to myocardial perfusion, often compromised by a large "steal" from the aorta to the right ventricle, limits the choice of surgical procedures.     

室间隔完整型肺动脉闭锁的彩色多普勒超声诊断

目的 探讨室间隔完整型肺动脉闭锁（PAIVS）的超声影像特点.方法 回顾分析7例PAIVS的超声心动图表现,并与外科手术结果进行比较.结果 PAIVS的超声心动图特征明显：（1）二维超声心动图胸骨旁左心室长轴切面显示室间隔回声完整.（2）心尖四腔切面显示2个心房正位,室间隔回声完整.（3）胸骨旁大动脉短轴切面显示2条大动脉位置关系正常,肺动脉瓣无开放运动,肺动脉瓣无血流信号通过.手术证实7例PAIVS中肺血由未闭的动脉导管供应者6例;主肺动脉及分支发育不良,仅有体肺动脉侧支供应肺血者1例.心房水平右向左分流者7例：其中5例为卵圆孔未闭,2例为房间隔缺损.合并畸形包括三尖瓣闭锁,三尖瓣发育不良伴狭窄、房间隔膨凸瘤等.结论 PAIVS患儿肺血来源多样化,肺动脉发育程度不一,超声心动图特征明显,对PAIVS有特异性诊断价值.     

Pulmonary atresia with intact ventricular septum

Opinion statement The prognosis for patients with pulmonary atresia with intact ventricular septum is poor with and without conventional surgical treatment. Because of this reason, a comprehensive program of medical, transcatheter, and surgical treatment is necessary to improve the long-term outlook of these infants. Algorithms of management plans should be developed based on the presence of right ventricular-dependent coronary circulation as well as size and morphology of the right ventricle. In a tripartite or bipartite right ventricle, transcatheter radiofrequency perforation is preferable. Alternatively, surgical valvotomy may be performed. Augmentation of pulmonary blood flow by prolonged infusion of prostaglandin E₁, stenting the ductus, or a surgical modified Blalock-Taussig shunt may be necessary in some of these patients. In patients with a unipartite or very small right ventricle or a right ventricular-dependent coronary circulation (Tricuspid valve Z score < -2.5), augmentation of pulmonary flow along with atrial septostomy should be undertaken. Follow-up studies to determine the feasibility of biventricular repair should be undertaken and, if feasible, surgical or transcatheter methods may be used to achieve the goals. If not suitable for biventricular repair, one-ventricle (Fontan) or one and one-half ventricular repair should be considered. Comprehensive and well-planned treatment algorithms may help improve survival rate.     

Transcatheter closure of right coronary artery fistula to the right ventricle

Coronary artery fistula (CAF) is an uncommon anomaly that is usually congenital but can be acquired. Although most patients are asymptomatic, some may present with congestive heart failure, infective endocarditis, myocardial ischemia or rupture. In the past, surgical ligation was the only option in the management of CAF, but since 1983, transcatheter closure of CAF has been increasing as an alternative to surgery. We report a 3-year-old boy, presented in Queen Alia Heart Institute, who underwent successful transcatheter closure of a large fistula communicating the distal part of the right coronary artery to the right ventricle. Our case differs from other CAFs in that the fistula was communicating the right coronary artery itself to the right ventricle.     

Acute coronary syndrome without ST segment elevation in a 78 year-old woman caused by critical proximal right coronary artery stenosis with concomitant chronic total occlusion of left main coronary artery

Total occlusion of the left main coronary artery is a rare finding at coronary angiography. When present, patients most often have extensive collateral circulation from the right coronary artery. The mainstay of treatment is surgical with coronary artery bypass grafting. We present a case of a 78 year-old woman admitted to our department with two days history of nonspecific, abdominal pain. Her coronarography revealed chronic total occlusion of left main with concomitant critical, proximal right coronary artery stenosis.     

The power of collateral circulation: a case of asymptomatic chronic total occlusion of the left main coronary artery

Total occlusion of the left main coronary artery predominantly presents with recurrent angina or myocardial infarction. Long-term survival and myocardial function depends on the well-developed right to left collaterals. We report a case of a 46-year-old man who was referred because of incidental finding of low ejection fraction during work-up for syncope 5 months prior. The patient denied any recurrence or any other symptom after that episode and claimed an unchanged exercise capacity. He had hypertension, hyperlipidemia, and history of 15-pack/year smoking. Except for class II morbid obesity, he had completely normal vital signs, physical examination, and lab tests on admission. The echocardiogram was suggestive of previous anterior wall myocardial infarction and demonstrated a low left ventricle ejection fraction with diffuse hypokinesis of the left ventricle. The patient underwent cardiac catheterization, which revealed total occlusion of the left main coronary artery, dominant right coronary artery with a 95% stenosis in the proximal segment, and collaterals from the right to the left coronary arteries. The patient was immediately referred for coronary artery bypass surgery. This case demonstrates the power of collateral circulation in protecting the patient from symptoms and death despite total occlusion of the left main coronary artery and severe stenosis of the proximal right coronary artery.     

Coronary‐pulmonary arterial fistula in a neonate with pulmonary atresia—ventricular septal defect and single coronary artery

In cases of pulmonary atresia with ventricular septal defect (PA‐VSD), coronary‐pulmonary arterial fistula (CPAF) as the main source of pulmonary blood supply is extremely rare. These fistulae may arise from the left coronary artery, right coronary artery, or a single coronary artery. Fistulae from a single coronary artery are unusual. We are reporting a case of PA‐VSD with single coronary artery and CPAF as the main source of pulmonary supply in addition to two major aortopulmonary collateral arteries (MAPCAS). Successful surgical correction with VSD closure and right ventricle (RV) to the pulmonary artery (PA) conduit was made.     

合并冠状动脉畸形法洛四联症的外科治疗

目的总结合并畸形冠状动脉的法洛四联症手术治疗经验,探讨有关技术的改进。方法自2002年1月至2007年12月共完成该类手术18例,其中经右房、肺动脉切口疏通右室流出道7例,改变右室切口4例,主肺动脉移位法2例,双流出道法2例,游离牵开左前降支（LAD）疏通加宽右室流出道（RVOT）2例,1个半心室矫治1例。结果无手术死亡,随访8～82个月,无晚期死亡及并发症发生。结论术中根据右室流出道狭窄程度和畸形冠状动脉走行争取个性化手术方式,疗效满意。     

Chronic effort-induced angina as presentation of a totally occluded left main coronary artery: a case report and review

In this report, the case of a 40-year-old patient with chronic total occlusion of the left main coronary artery is discussed. Total occlusion of the left main coronary artery is a rare finding at coronary angiography. When present, patients most often have extensive collateral circulation from the right coronary artery. The mainstay of treatment is surgical with coronary artery bypass grafting.     

The prevalence of coronary arterial abnormalities in pulmonary atresia with intact ventricular septum and their influence on surgical results

BACKGROUND: The relatively high mortality in patients with pulmonary atresia and intact ventricular septum may be related to the presence of significant coronary arterial anomalies. This retrospective review of cineangiocardiograms was undertaken to further elucidate the types and variety of such coronary arterial abnormalities, and to assess their effect on postoperative survival. MATERIAL AND RESULTS: Details regarding coronary arterial anatomy and abnormalities were assessed in 116 patients. We noted the site and severity of lesions, and the presence of fistulous communications from the right ventricle to the coronary arteries, assessing the proportion of left ventricular myocardium affected by coronary arterial interruptions or significant stenoses, in other words, the amount dependent on coronary circulation from the right ventricle. We also measured diameters of the tricuspid and mitral valves. Fistulas were found in 87 patients (75%), interruptions of major coronary arteries in 40 patients (34%), lack of connections between the coronary arteries and the aorta in 18 patients (16%), and single origin of a coronary artery, with the right coronary artery arising from the left, in 6 patients (5%). We found increased mortality in 47 patients (40%) who had a right ventricular-dependent coronary arterial circulation. The presence of fistulas in itself was not associated with higher mortality, but the presence of coronary arterial interruptions (p = 0.05), and a higher myocardial score (p = 0.0009), were. CONCLUSION: We encountered a higher prevalence of both coronary arterial abnormalities and right ventricular-dependent circulation than previously reported. Awareness of the severity of the coronary arterial abnormalities should assist in planning treatment.     

The blood pressure and the size of a cardiac infarct

In the cat with an otherwose normal circulation, the blood pressure is almost invariably normal three weeks after the ligation of a large coronary artery (left circumflex). This applies to infarcts of widely different sizes, ranging from a relatively small area involving the upper third of the left ventricle posteriorly to a very large one including nearly all of the left ventricle posteriorly, together with adjacent strips of the right ventricle and interventricular septum.     

少见冠状动脉畸形18例

目的 :探讨 18例少见冠状动脉 (冠脉 )畸形特征及评价选择性冠脉造影在其诊断中的作用。方法 :对17例患者进行冠脉造影 ,13例患者进行手术纠正治疗。另 1例二维超声心动图误诊为动脉导管未闭 ,术中证实为右冠脉 -右室瘘。结果 :18例患者中共发现有 9种冠脉畸形 ,包括冠脉起源异常 2例 ;左冠脉回旋支缺如 1例 ;左、右冠脉 -肺动脉瘘 3例 ;左冠脉 -肺动脉瘘 3例 ;右冠脉 -右心房瘘 3例 ;右冠脉 -右室瘘 2例 ;右冠脉 -静脉瘘 1例 ;左冠脉 -右室瘘 1例 ;左冠脉 -左心室瘘 2例。其中 13例患者经手术治疗得到再证实。结论 :冠脉畸形是少见的先天性血管畸形 ,选择性冠脉造影是确诊的重要手段 ,需与先天性心脏病和冠心病心绞痛鉴别 ,术后效果良好。