64层螺旋CT对冠状动脉起源异常及预后的评价

目的：探讨64层螺旋CT诊断冠状动脉起源异常的价值及对预后的评价。方法：对1211例冠状动脉CT血管成像（CTA）资料进行回顾性分析。结果：1211例完整冠状动脉CTA结果中，共检出冠状动脉起源异常18例，检出率为2.17%。包括右冠状动脉起源于左窦5例，伴前降支和回旋支单开口1例；右冠状动脉起源于升主动脉1例；圆锥动脉单开口4例；左冠状动脉主干起源于右窦3例；回旋支起源于右窦1例。前降支和回旋支单开口4例；共有8例冠状动脉分别起源于对侧窦，其中有3例该段有50%左右狭窄。结论：64层螺旋CT是冠状动脉起源异常安全和有效的确诊方法，并可对预后做出评估。     

Anomalous origin of the left circumflex coronary artery from the pulmonary artery. A very rare congenital anomaly in an adult patient diagnosed by cardiovascular magnetic resonance

Here we report for the first time on the diagnostic potential of cardiovascular magnetic resonance (CMR) to delineate the proximal course of an anomalous left circumflex coronary artery (LCX) originating from the right pulmonary artery in an adult patient with no other form of congenital heart disease. The patient was referred to our institution due to exertional chest discomfort. X-Ray coronary angiography showed a normal left anterior descending coronary artery (LAD) and right coronary artery (RCA), while the LCX was filled retrograde by collateral flow through the LAD and the RCA. The origin of the LCX was postulated to be the pulmonary artery, but the exact origin of the anomalous artery could not be depicted on conventional angiograms. CMR provided the unambiguous depiction of the origin of the anomalous LCX from the right pulmonary artery and the delineation of its proximal course in this case of a very rare coronary anomaly in adults.     

Coronary anomalies diagnosed with transesophageal echocardiography: complementary clinical value in adults

Introduction. Coronary arteriography remains a gold standard for the evaluation of coronary anatomy. In the case of anatomical anomalies, understanding of vessel course based upon a coronary angiogram may be difficult. Transesophageal echocardiography is a noninvasive method allowing tomographic visualization of proximal coronary arteries. Experience concerning its usefulness for the assessment of anomalous coronary arteries is limited. Material. Eleven patients with confirmed coronary anomalies studied between 1993-96 were identified in the cohort of those undergoing transesophageal echocardiography. Results. Transesophageal echocardiography revealed potentially serious anomalies (origin of left or right coronary artery from contralateral aortic sinus) in 3 patients and benign in 8. Coronary ostia and proximal course could be delineated in all patients. Anatomical information was consistent between methods, except for a separate origin of the left anterior descending and circumflex artery, where the angiogram missed a very short common left main coronary artery in 2 patients. The relationship between the coronary arteries, aorta and pulmonary trunk was better defined by the echocardiogram. Doppler flow analysis allowed us to exclude anomaly-related flow disturbances. Conclusions. Transesophageal echocardiography can be considered as a noninvasive technique with the potential for anatomical and functional evaluation of anomalous proximal coronary arteries and deserves a routine use whenever such a condition is suspected. This approach may simplify invasive procedures in this patient group.     

Imaging of congenital coronary anomalies with multislice computed tomography

OBJECTIVE: To describe a single-center experience of using retrospectively gated multislice computed tomographic (MSCT) coronary angiography for imaging congenital coronary anomalies. PATIENTS AND METHODS: We retrospectively reviewed the clinical information and imaging studies for 9 patients diagnosed as having congenital coronary anomalies on invasive, selective coronary angiography between February 2001 and October 2003 at the Mayo Clinic in Jacksonville, Fla. Two experienced observers classified by consensus the origin and proximal course of the abnormal coronary arteries as seen on MSCT. RESULTS: In 1 patient, MSCT showed a normal but extremely anterior origin of the right coronary artery from the right aortic sinus of Valsalva. In the other 8 patients, the origin and course of 4 anomalous right coronary arteries, 2 anomalous left circumflex coronary arteries, and 2 single coronary arteries were recognized easily on MSCT. CONCLUSION: Similar to electron beam computed tomography and magnetic resonance imaging, widely available MSCT can characterize the proximal course of congenitally abnormal coronary arteries and thus aid in clinical decision making for patients with such anomalies.     

先天性冠状动脉起源异常病理解剖及胚胎学与外科治疗

目的总结先天性冠状动脉起源异常病理解剖和胚胎学与外科治疗。方法回顾分析阜外医院1998年2月到2007年8月先天性冠状动脉起源异常并进行外科手术病例,其中24例患者冠状动脉起源于肺动脉,男13例,女11例;年龄3月到41岁。左冠状动脉起自肺动脉14例;右冠状动脉起自肺动脉10例。按Leiden冠状动脉分型方法进行冠状动脉分型。左冠状动脉起自肺动脉14例均在体外循环下进行手术,直接冠状动脉移植12例,肺动脉内隧道2例。右冠状动脉起自肺动脉10例中2例在非体外下进行冠状动脉移植,1例合并房间隔缺损进行房间隔封堵术,2例进行肺动脉内隧道,6例进行了直接移植。1997年10月~2007年8月共完成动脉掉转手术170例,采用Leiden冠状动脉分型方法,1LCx2R为冠状动脉正常分布,其余分类形式为冠状动脉异常分布,冠状动脉异常起源48例,有3例冠状动脉壁内走行;男28例,女20例,年龄4天~6岁;简单型大动脉转位中18例冠状动脉异常起源,复杂型大动脉转位中15例冠状动脉异常起源,T-B中15例冠状动脉异常起源。冠状动脉吻合主要采用带片开门技术或吊窗技术。结果冠状动脉起源于肺动脉病例中,左冠状动脉均起自肺动脉窦1,右冠状动脉均起自肺动脉窦2;异常肺动脉起源的冠状动脉冠状动脉再移植无手术死亡。动脉掉转手术冠状动脉异常起源48例中,其中1L2RCx占18例1、R2LCX 7例1、LR2Cx 6例、2LCx2R 6例、2LCxR 5例。异常主动脉起源的冠状动脉冠状动脉再移植死亡9例,死亡率为18.7%。其中2004年前死亡7例,死亡率29.2%(7/24),2004年后死亡2例,死亡率8.3%(2/24)。结论提高对先天性冠状动脉起源异常的病理解剖,胚胎发生学的认识有助于提高手术疗效。     

Anomalous left anterior descending artery diagnosed on pulmonary artery computed tomography

Typically, the left anterior descending artery (LAD) and left circumflex artery (LCX) arise from the left main coronary artery.However, uncommon coronary anomalies may be found in clinical practice. This case presents with a rare finding where the LAD originates from the right coronary artery (RCA) separately from the LCX and takes an interarterial pathway to reach its perfusion territory.A 49-year-old Hispanic female with hypertension and diabetes mellitus presented to the emergency department with a 7-day history of chest pain. She denied nausea, diaphoresis, syncope, or other symptoms. A grade 3 out of 6 systolic murmur was noted on physical examination. Computed tomography of the pulmonary arteries (CTPA) revealed that the patient had no left main coronary artery. The patient's LAD arose from the proximal RCA and took an inter-arterial course. Subsequent coronary catheterization showed no stenosis of the coronary arterial system. The patient's chest pain subsided during the course of her admission and she was deemed stable for discharge with close cardiology follow up.In general, coronary artery anomalies are an uncommon finding in clinical practice. However, it is important to realize the different pathways of coronary artery anomalies because those with the inter-arterial subtype, such as our patient, may result in sudden cardiac death. All cases of clinically suspected inter-arterial coronary artery anomalies are recommended to undergo imaging studies to help visualize anatomic features as a guide for further management. This case represents the first reported diagnosis of this type of anomalous coronary artery on CTPA.     

Coronary Artery Anomalies: a Pictorial Review

Coronary artery anomalies range in prevalence from 0.2 to 2.3 % of the population. They range from benign incidental findings to an important cause of sudden cardiac death (SCD). In fact, coronary anomalies are the second leading cause of SCD in athletes and are responsible for ～30 % of SCD in the young. Clinically, anomalous coronary arteries arising from the opposite sinus and anomalous left coronary artery arising from the pulmonary artery are the most important as they are associated with the highest risk of mortality. Several high-risk features and their pathophysiology are reviewed. Multiple imaging modalities have been utilized to study coronary artery anomalies; however, coronary computed tomography angiography (CTA) is uniquely suited to characterize coronary artery anomalies as it allows for clear elucidation of origin, course, and termination in relationship to other relevant anatomy with high spatial resolution. This paper will provide an overview of the wide spectrum of coronary artery anomalies and variants, review the most relevant coronary CTA imaging features for each, and differentiate benign from malignant varieties.     

Computed tomographic angiography identification of intramural segments in anomalous coronary arteries with interarterial course

Certain coronary anomalies are associated with high risk features. We sought to determine the diagnostic accuracy of coronary computed tomographic angiography (CTA) in determining high-risk features, particularly intramural segments. Anomalous coronary arteries can be associated with adverse clinical events. Anomalous coronaries that course between the great vessels (interarterial) have been associated with sudden death. High-risk features of interarterial vessels described in the literature include; a slit-like orifice, acute angle of origin, and intramural segments (within the wall of the aorta). Although computed tomography (CT) findings of acute angle and slit like orifice have been described previously no prior evaluations regarding CT identification of an intramural segment have been reported. An intramural segment has distinct surgical management implications. All interarterial anomalous coronary arteries do not have an intramural segment. Since October 2004, 15 patients were diagnosed by CTA as having an anomalous coronary artery with an interarterial course, which were then confirmed by intraoperative examination of their coronary origins and course during aortic root/coronary artery surgery. The CTA images were retrospectively analyzed for the presence of high-risk features by a radiologist blinded to the surgical findings. Comparison of these findings was made to the findings at surgery. The anomalous coronary was the right coronary artery in 10 patients and the left coronary artery in 5. Eleven patients had an intramural segment identified at surgery. Pre-operative coronary CTA showed that all patients with an intramural course of the anomalous artery, had slit-like orifice, an acute angle of origin (mean 18.4 ± 3.4°), and an elliptical shaped cross-section throughout the intramural segment of the anomalous vessel. The average vessel height/width ratio for anomalous coronary vessels without an intramural segment was 1.03; compared to a ratio of 2.19 for anomalous vessels with an intramural segment (P = 0.003). Coronary CTA can identify an intramural segment of an anomalous interarterial coronary artery by its elliptical shape. Identifying an intramural segment has important clinical and surgical implications.     

Gender differences in the types and frequency of coronary artery anomalies

Coronary artery anomalies are rarely encountered in general population. Gender may play a role in the types and incidence of coronary artery anomalies, although the effect of gender is not well established. In the present study, we therefore aimed to investigate the frequency and location of various types of coronary artery anomalies and their correlation with gender. We assessed retrospectively the coronary angiography movies of 7,810 patients (2,214 females and 5,596 males), the method of which is distinct from the earlier studies with angiographic archive records. We defined and classified the coronary artery anomalies according to their origin, course (myocardial bridge), and termination (fistula). The incidence of coronary artery anomalies was 3.35% (262 of 7,810): 130 individuals with anomalous origin (1.66%), 105 individuals with myocardial bridges (1.34%), and 27 with fistulas (0.35%). The frequency of the coronary artery anomalies was significantly higher in the females than the males (p = 0.001). Of the coronary artery origin anomalies, the circumflex and the left anterior descending artery originating from separate ostia in the left aortic sinus were higher in the females compared to the males (P < 0.001). In contrast, the frequency of myocardial bridges was higher in the males (P = 0.01). No gender difference was detected in fistulas. Thus, gender affects the types of coronary artery anomalies, except for fistulas. The determination of the presence of the coronary artery anomalies during the coronary angiography is critical for the planning of the treatment and for the proper clinical follow-up of patients.     

屏气法三维磁共振冠状动脉血管造影诊断先天性冠状动脉起源畸形

目的探讨屏气法三维磁共振冠状动脉造影(3D MRCA)显示畸形冠状动脉与主动脉根部、右心室流出道关系的可行性及价值.方法 5名临床拟诊患者、1例志愿者接受MRCA检查,以屏气法三维稳态进动快速成像(3D true-FISP)梯度回波序列采集图像,获得左、右冠状动脉图像,分析畸形冠状动脉近段与冠状动脉窦(Valsalva窦)、右心室流出道的位置关系.结果 6例MRCA均明确诊断先天性冠状动脉起源畸形,畸形血管近段均走行于主动脉根部与右心室流出道之间.结论屏气法3D MRCA可明确诊断先天性冠状动脉起源畸形,显示畸形冠状动脉近段的走行路经,是一种可行的影像诊断方法.     

肺动脉异常起源于升主动脉的病理分型

目的 肺动脉异常起源升主动脉的病理分型及胚胎发生机制探讨。方法 5例肺动脉异常起源于升主动脉，作心血管造影（ACG)及磁共振(MRI)检查。结果 5例病例中3例为右肺动脉异常起源于升主动脉；2例为左肺动脉异常起源于升主动脉，4例属近端型，1例远端型。结论 肺动脉异常起源于升主动脉为少见的先天性心脏病，病理分型有二种，一种右肺动脉异常起源于升主动脉，占肺动脉异常起源升主动脉的绝大部分；另一种为左肺动脉异常起源升主动脉，此种类型少见，又可根据肺动脉起源离主动脉瓣及无名动脉的距离分近端型及远端型二种亚型。二种类型的胚胎发生学的解释不尽相同。肺动脉异常起源于升主动脉以前称为半永存动脉干，从胚胎角度讲与永存动脉干不同，故目前不提倡用半永存动脉干这个术语。     

A case for routine screening of coronary artery origins during echocardiography: fortuitous discovery of a life-threatening coronary anomaly.

Anomalous origin of the left main coronary artery from the right sinus of Valsalva with retropulmonary course is a rare congenital abnormality. It is associated with a high incidence of sudden cardiac death, particularly among young, athletic individuals. Many of these individuals do not have symptoms before sudden death, and the diagnosis is usually made at postmortem examination. We present a case of a 15-year-old boy who was evaluated for a systolic click with routine 2-dimensional echocardiography. The anomalous coronary artery was serendipitously identified, allowing surgical intervention. Coronary artery origin and proximal course should be visualized on routine echocardiography in the pediatric population.     

Successful subclavian-left coronary artery anastomosis for anomalous origin of left coronary artery in infancy.

Left subclavian artery-left coronary artery end-to-end anastomosis was successfully performed on a six-month-old female infant with anomalous origin of the left coronary artery from the pulmonary artery and severe mitral regurgitation. A piece of pulmonary arterial wall including the opening of the anomalous left coronary artery (ALCA) was isolated and was used for anastomosis instead of utilizing the left coronary arterial wall itself. Her postoperative course was uneventful and mitral regurgitation disappeared 6 months after surgery. This improved technique seems to be applicable for this anomaly, regardless of the age of patient or size of the left coronary artery.     

Pulsed Doppler echocardiographic and color flow imaging detection of retrograde filling of anomalous left coronary artery from the pulmonary artery.

We report a case in which pulsed wave Doppler echocardiography and color flow imaging of blood flow direction in an anomalous coronary artery from the pulmonary artery assisted in the correct diagnosis and confirmed the adequacy of the surgical correction. Low-velocity color scales were used to show retrograde filling of the left coronary artery before surgery and antegrade filling of the left coronary artery after surgery. Detecting direction of blood flow in coronary arteries should increase the accuracy of the noninvasive diagnosis of anomalous left coronary artery from the pulmonary artery.     

Analysis of anomalous origin of coronary arteries by coronary angiography in Chinese patients with coronary artery disease

With the development of coronary angiography for the diagnosis of coronary artery disease, its clinical significance in detecting coronary artery anomalies and evaluating the seriousness is attracting more attention. In the study we aimed to assess the prevalence of anomalous origin of coronary arteries in a Chinese population who underwent coronary angiography for coronary artery disease, and explore any patterns in the common variants and typical anomalies, especially the potentially serious ones. Patients who underwent coronary angiography from January 2013 to December 2016 in Fuwai Hospital were included. Baseline characteristics and angiographic data were collected, the incidence of anomalous origin of coronary arteries was calculated, and the typical patterns were analyzed. Comparisons between the present results and those of existing reports were also conducted. A total of 110,158 patients were included in the study, among which 0.76% (835 cases) had anomalous origin of coronary arteries. Among the anomalies, the incidences of anomalous origin of the right coronary artery (RCA), the left coronary artery (LCA), both the RCA and LCA, single coronary artery (SCA) and dextrocardia were 76.76% (641 cases), 14.61% (122 cases), 1.80% (15 cases), 4.67% (39 cases) and 2.16% (18 cases), respectively. Moreover, 47.54% (397 cases) of the anomalies were shown to be potentially serious, and an RCA arising from the left sinus of Valsalva (LSV) was the most common subtype (39.28%, 328 cases). Although anomalous origin of coronary arteries is not quite common, more clinical attention should be paid to this condition due to the potential risk of serious sequelae.     

The diagnosis of anomalous origin of the left coronary artery from the pulmonary artery by echocardiography: Case report

The anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital heart disease that affects 1 of 300,000 live births. We present the case of an 18-year-old female presenting with chest pain and dyspnea after vigorous exercise, and whose two-dimensional echocardiogram initially displayed a "normal connection" between the left coronary artery with the aortic sinus of Valsalva. Using a systematic diagnostic echocardiography approach, we rightfully diagnosed it as anomalous origin of the left coronary artery from the pulmonary artery.     

Anomalous coronary arteries of aortic origin

Anomalous coronary arteries cause only uncharacteristic symptoms and are therefore often an incidental finding during conventional coronary angiography, with an incidence of 0.3-0.8%. The commonest anomaly is an aberrant origin of the main left or right coronary artery from the wrong sinus of Valsalva. Rarely there is a fistula draining into one of the cardiac cavities (right ventricle, right atrium, left ventricle or, rarely, superior vena cava) or displaced connection, as seen in anomalous origin of coronary artery from the pulmonary artery, resulting in a left-to-right shunt. In congenital heart disease, especially Fallot's tetralogy, the incidence of abnormal coronary arteries may be 2% or more. The proximal course in the former category may be misdiagnosed in up to 50% of cases. Aortic root injection with subtraction angiography, further detailed investigation with transoesophageal echocardiography or magnetic resonance angiography are therefore required as these have potential implications on subsequent surgery. Because of the abnormal course between aorta and pulmonary artery/outflow tract of the right ventricle and acute angulation there is a risk of angina, acute myocardial infarction or sudden death during or after exercise. It is therefore important to identify the exact cardiac anatomy, particularly in patients undergoing angioplasty, stenting or cardiac surgery.     

冠心病患者胸主动脉粥样硬化与冠状动脉粥样硬化的关系——经食管超声心动图的研究

采用经食管超声心动图技术在冠状动脉造影检查证实为冠状动脉正常者１１例和３２例冠心病患者中，探测了胸主动脉粥样硬化性病变并计算了动脉粥样硬化总积分（ＴＡＳ）和硬化指数（β）。结果：ＴＡＳ或β值与冠状动脉粥样硬化之间有密切关系，随冠脉病变支数的增加，ＴＡＳ和β值也明显增高，这为无创性估测冠状动脉粥样硬化开辟了新的途径     

先天性冠状动脉畸形的超声心动图诊断

目的探讨超声心动图诊断小儿先天性冠状动脉畸形的意义。方法对本院诊治的30例先天性冠状动脉畸形的超声心动图结果与心血管造影、手术结果进行分析比较。结果冠状动脉异位开口于肺动脉4例,占13%;单支冠状动脉畸形3例,占10%;冠状动脉瘘23例,占77%,累及右冠状动脉12例（52%）,其中多个瘘口1例,左冠状动脉11例（48%）,瘘入右侧心腔11例（48%）,瘘入肺动脉11例（48%）,瘘入左侧心腔1例（4%）。冠状动脉畸形中伴有其他先天性畸形6例（26%）。超声心动图诊断敏感性93%,特异性100%。其中对冠状动脉瘘及瘘口的形态、数目、瘘入的部位准确性达100%,对冠状动脉异位开口于肺动脉及单支冠状动脉畸形各有1例误诊为弹力纤维增生症。结论超声心动图对先天性冠状动脉畸形有较高的诊断价值,可作为首选诊断方法。对心脏扩大、心内膜增厚、心功能弥漫性降低的弹力纤维增生症样超声心动图改变必须保持对冠状动脉畸形的警惕性,应仔细探测左右冠状动脉及分支,并结合心电图等临床资料进行分析。     

Diagnosis and management of anomalous origin of the right coronary artery from the left coronary sinus

The anomalous origin of the right coronary artery (RCA) from the left sinus of Valsalva coursing between the aorta and the pulmonary artery or right ventricular outflow tract, is considered a potentially fatal abnormality which may require surgery. However, diagnosing the correct course with coronary arteriography may be difficult. Fast gradient echo magnetic resonance (MR) imaging can be helpful to identify and confirm the course of aberrant coronary arteries and their relationship to the surrounding tissue. In this study, diagnostic procedures and management are described of four patients in whom the RCA originated from the left sinus of Valsalva. Although reported as investigational by the Task Force document on MR imaging by the European Society of Cardiology we are of the opinion that MR coronary angiography may have an important future role in the assessment of anomalous coronary arteries.