Unroofing Technique for Anomalous Origin of the Left Coronary Artery from the Right Sinus of Valsalva: Report of a Case

Anomalous origin of the left coronary artery (AOLCA) from the right sinus of Valsalva constitutes a rare congenital coronary artery anomaly. Patients with an anomalous left main coronary artery face a significantly higher risk of sudden cardiac death compared to those with an anomalous right coronary artery. The anomalous coronary artery traversing between the ascending aorta and the pulmonary artery markedly heightens the risk of myocardial ischemia, arrhythmia, and sudden death. Symptomatic patients often exhibit a longer intramural course of the coronary artery, which may necessitate earlier intervention or influence the choice of surgical repair method. Surgical intervention is advocated for patients with this anomaly, even in the absence of symptoms. For anomalous aortic origin of a coronary artery from the opposite sinus of Valsalva with an intramural course, coronary unroofing is the preferred revascularization procedure. This report presents a case of AOLCA originating from the right sinus of Valsalva, treated surgically using the unroofing technique for the aortic intramural segment of the anomalous coronary artery traversing between the great vessels. The unroofing technique is recommended for treating AOLCA with an extensive intramural course that does not involve the commissure.

R-I subtype single right coronary artery with congenital absence of left coronary system: A case report

BACKGROUND Isolated single coronary artery is a rare congenital anomaly.R-I subtype single coronary artery is even rarer.In this subtype,a very large right coronary artery extends in the coronary sulcus to the anterior base of the heart where it produces the left anterior descending coronary artery.Currently,only a few case reports are available in the literature for this anomaly.CASE SUMMARY Here,we report the case of a 62-year-old woman who presented to the cardiology clinic with decreased exercise tolerance and poor blood pressure control.The patient underwent coronary angiography(CAG)and emission computed tomography(ECT).CAG images revealed a single gigantic right coronary artery(R-I type)arising from the right coronary sinus with branches supplying the left coronary territory.The ECT results confirmed myocardial ischemia at the location of the absent left coronary artery.The ECT findings confirmed that ischemia was consistent with the vascular loss location in CAG images.In such anomalies,there is a compensatory widening of the coronary artery lumen.Medical treatment was administered,and the patient was discharged.CONCLUSION Isolated single coronary arteries are associated with ischemia and potentially fatal acute coronary events.Hence,controlling risk factors is critical.

Congenital coronary artery fistulas complicated with pulmonary hypertension: Analysis of 211 cases

AIM To compare the behavior of pulmonary hypertension(PHT) associated with coronary artery fistulas(CAFs) between the Asian and Caucasian subjects.METHODS CAFs may be complicated with PHT secondary to leftto-right shunt. Literature review limited to the English language. A total of 211 reviewed patients were collected. Of those, 111 were of Asian and 100 were of Caucasian ethnic origin. The mean age of the Asian and the Caucasian groups of patients were 48.9(range 19-83) and 49.9 years(range 16-85), respectively. In both groups, right heart catheterization was the most commonly(95%) used method for determining pulmonary artery pressure. RESULTS From all of the reviewed subjects, PHT was found in 49 patients(23%), of which 15 were Asian and 34 were Caucasian. In 75% of PHT subjects, mild to moderate PHT was reported and 76% of the fistulas had a vascular mode of termination. Treatment was surgical in 61%, followed by percutaneous therapeutic embolization(27%) and finally conservative medical management in 12% of PHT subjects. PHT was associated with a slight female gender predominance. The majority demonstrated mild to moderate PHT. PHT was reported more frequent in the Caucasian compared with the Asian ethnicity group. The majority of fistulas in patients with PHT had a vascular mode of termination. The results of this review are intended to be indicative and require cautious interpretation.CONCLUSION The likelihood for a CAF patient to develop PHT is presented when possessing the following features, with a Caucasian female having a fistula with a vascular mode of termination.

Congenital solitary coronary artery fistulas characterized by their drainage sites

Last centuries have witnessed tremendous sophistication and progress in the detection,diagnosis and treatment of coronary artery fistulas(CAFs).In many countries,CAFs were reported to be visualized and treated using several imaging techniques and different management strategies.Reports from nearly all continents of the globe have contributed to the description of CAFs,not only in Asia and Europe but also throughout North and Latin America.However,these reports have to be cautiously analyzed as many of them were published as a case report and careful interpretation is warranted due to possible publication bias.A literature search was performed using PubMed search interface to select papers dealing with congenital CAFs in adult population between 2000-2009.A total of 233 subjects were collected,and analysed according to their drainage site and treatment modality.They were divided into two subgroups:percutaneous transluminal embolization group(PTE group,n=122)and surgical ligation group(SL group,n=111).In the SL group,atherosclerotic coronary artery disease(19%)and associated congenital lesions(23%)were more prevalent compared with the PTE group(9%and 8%),respectively.Infective endocarditis was more frequently seen in the SL group besides syncope,congestive heart failure and hemopericardium.In both groups multimodality diagnostic workup composed of several non-invasive and invasive imaging techniques for fistula visualization were performed and drainage sites into the different cardiac chambers and intrathoracic great vessels were similarly distributed in the two groups.

Coronary artery disease in congenital single coronary artery in adults:A Dutch case series

AIM:To assess the current diagnostic and therapeutic management and the clinical implications of congenital single coronary artery(SCA) in adults.METHODS:We identified 15 patients with a SCA detected from four Dutch angiography centers in the period between 2010 and 2013.Symptomatic patients who underwent routine diagnostic coronary angiography(CAG) for suspected coronary artery disease and who incidentally were found to have isolated SCA were analyzed.RESULTS:Fifteen(7 females) with a mean age of 58.5 ± 13.78 years(range 43-86) had a SCA.ConventionalCAG demonstrated congenital isolated SCA originating as a single ostium from the right sinus of Valsalva in 6 patients and originating from the left in 9 patients.Minimal to moderate coronary atherosclerotic changes were found in 4,and severe stenotic lesions in another 4 patients.Seven patients were free of coronary atherosclerosis.Runs of non-sustained ventricular tachycardia were documented in 2 patients,one of whom demonstrated transmural ischemic changes on presentation.Myocardial perfusion scintigraphic evidence of transmural myocardial ischemia was found in 1 patient due to kinking and squeezing of the SCA with an interarterial course between the aorta and pulmonary artery.Multi-slice computed tomography(MSCT) was helpful to delineate the course of the anomalous artery relative to the aorta and pulmonary artery.Percutaneous coronary intervention was successfully performed in 3 patients.Eight patients were managed medically.Arterial bypass graft was performed in 4 patients with the squeezed SCA.CONCLUSION:SCA may be associated with transient transmural myocardial ischemia and aborted sudden death in the absence of coronary atherosclerosis.The availability and sophistication of MSCT facilitates the delineation of the course of a SCA.We present a Dutch case series and review of the literature.

Congenital coronary artery anomalies silent until geriatric age： non-invasive assessment, angiography tips, and treatment

Coronary artery anomalies （CAAs） may be discovered more often as incidental findings during the normal diagnostic process for other cardiac diseases or less frequently on the basis of manifestations of myocardial ischemia. The cardiovascular professional may be involved in their angiographic diagnosis, fimctional assessment and eventual endovascular treatment. A complete angiographic definition is mandatory in order to understand the functional effects and plan any intervention in CAAs： computed tomography and magnetic resonance imaging are useful non-invasive tools to detect three-dimensional morphology of the anomalies and its relationships with contiguous cardiac structures, whereas coronary arteriography remains the gold standard for a definitive anatomic picture. A practical idea of the possible functional sig- nificance is mandatory for deciding how to manage CAAs： non-invasive stress tests and in particular the invasive pharmacological stress tests with or without intravascular ultrasound monitoring can assess correctly the functional significance of the most CAAs. Finally, the knowledge of the particular endovascular techniques and material is of paramount importance for achieving technical and clinical success. CAAs represent a complex issue, which rarely involve the cardiovascular professional at different levels. A timely practical knowledge of the main issues regarding CAAs is important in the management of such entities.

Congenital coronary artery-left ventricle direct micro-fistulas may cause effort angina and positive stress tests in Western adults

Coronary artery anomalies （CAAs） occur in 0.64 % to 5.6% of patients undergoing coronary angiography, Most pathophysiology and clinical histories involving CCAs have been fully clarified in the past 30 years. Isolated congenital coronary artery-left ventricle direct microfismlas （CVmF） have been recently described, but the clinical significance and anatomical characteristics associated with this rare type of CAA in a western population are still unclear.

Coronary artery anomalies:prevalence and clinical profile in elderly patients

Although congenital heart diseases are uncommon in the elderly,coronary artery anomalies may be incidentally discovered in old age.We sought to determine the incidence and clinical features of coronary artery anomalies (CAAs) in patients over 65 years of age.Patients and methods Medical records of patients undergoing coronary artery angiography in the years 1997-2002 at the Legnago General Hospital were retrospectively reviewed.The clinical profiles of all patients with CAAs and CAA subtypes were noted.Comparison between patients under and over 65 was performed.Data are given as mean standard deviation and as percentages.Results Sixty- six patients (1.21%,Female/Male 22/44,mean age 65.3±10.6 years) out of the 5450 who underwent coronary angingraphy in the years 1997-2002 had CAAs.In most cases (63%,41/66 patients),the patients were over 65. CAAs were discovered incidentally in these elderly patients while undergoing coronary artery angiography for dilated cardiomyopathy, ischemic heart disease,and valvular heart disease in 75% of the cases (30/41 patients).Patients over 65 had more cardiac comorbidities and a higher incidence of coronary atherosclerosis.Conclusions The angingraphic incidence of CAAs in elderly patients is increasing as the population ages and this occurrence calls for a wider knowledge of the anatomy and pathophysiology of CAAs among geriatric cardiologists.Elderly patients seem to present with lower risk coronary anomalies (separated origin of left anterior descending coronary artery and circumflex artery,origin of circumflex artery from the right sinus or the right coronary artery,double coronary artery) but have a higher risk profile compared to younger patients due to the frequency of cardiac coraorbidities and superimposed coronary artery atherosclerosis.(J Geriatr Cardiol 2004;1:40-43.)

Inter-Coronary Communication: A Rare Anomaly in Unusual Site

Intercoronary connection is a rare variant of coronary anomalies with a direct continuity between two main coronary arteries. It may function as an alternative pathway to blood flow in compromised coronary circulation. 64 years old male presented with 4 weeks history of retro-sternal chest pain and shortness of breath;his risk factors were diabetes mellitus, hypertension and smoking. Physical examination was normal and he had no audible murmur;he had elevated cardiac enzymes;in echocardiography there was inferior wall hypokinesia, cardiac catheterization revealed complete occlusion of med right coronary artery and complete occlusion of the left main (LM) trunk. Blood flow to the entire myocardium came from large abnormal coronary connection between the right coronary artery ostium and the proximal left anterior descending artery. A smaller arterial communication maintained blood flow to the distal right coronary artery. Cardiac computed tomographic angiography confirmed the presence of a patent left main ostium followed by complete occlusion. This unusual anatomical variation has saved the life of the patient and allowed time for surgical revascularization. The patient underwent successful triple coronary artery bypass grafts with no postoperative difficulties or complications. On one-year follow-up, the patient is asymptomatic, fully active with no new ECG or echocardiography changes.

Comparison of Congenital Coronary Artery Anomalies Between Uyghur and Han： A Multi-slice Computed Tomography Study in Xinjiang, China

Background：The incidence of congenital coronary artery anomalies （CCAAs） is different between ethnic groups,but there is no report about Uyghur CCAAs because of the limitation of inspection methods.This study determined the prevalence of Uyghur CCAAs and analysis the difference of CCAAs between Uyghur and Han ethnic groups by the method of multi-slice computed tomography coronary angiography （MSCTCA）.Methods：Seven thousand four hundred and sixty-nine MSCTCA were analyzed for the CCAAs retroactively,1934 were Uyghur patients while 4746 were Han patients.All the coronary artery images dates obtained by MSCTCA were evaluated for the CCAAs by two doctors.Results：Nineteen kinds of CCAAs were found：（1） The overall incidence of CCAAs was 2.72% （203/7469） among all patients,2.34% （111/4746） among Han patients whereas a significant higher 3.93% （76/1934） among Uyghur patients （χ^2 =12.780,P 〈 0.05）; （2） the incidence of CCAAs among male patients was 2.48% （76/3069） in Han while 4.33% （56/1293） in Uyghur （χ^2 =10.663,P 〈 0.05）; （3） the incidence of CCAAs on the left side was 1.07% （51/4746） among Han patients while 2.17% （42/934） among Uyghur patients （χ^2 =12.047,P 〈 0.05）; （4） among these 19 kinds of CCAAs,there were significant differences of the incidence of the following kinds of CCAAs between Uyghur and Han：Left coronary artery （LCA） high location （χ^2 =8.320,P =0.004）,right coronary artery （RCA） originate from left coronary sinus （χ^2 =5.450,P =0.020）,and RCA originate from left Coronary sinus ＋ LCA high location （P =0.024）.Conclusions：There exists some difference in CCAAs between Uyghur and Han ethnic groups.The CCAAs incidence of Uyghur is higher than that of Han,especially in male patients and on the left side; among all kinds of CCAAs,the incidence of LCA high location,RCA originate from left coronary sinus,RCA originate from left coronary sinus ＋ LCA high locations of Uyghur is higher than Han.

Aortic implantation for anomalous origin of the left coronary artery from the pulmonary artery:A ten-year single center experience

Background At present, creation of a dual coronary system with direct aortic implantation is the preferred management for anomalous origin of left coronary artery from the pulmonary artery （ALCAPA）. However, controversy still exists over the initial management of mitral regurgitation （MR） at the time of ALCAPA repair. Methods Between March 2005 and March 2015, 52 consecutive patients （20 males and 32 females） underwent direct aortic implantation of ALCAPA. Median age at surgery was 10 months （range, 2 to 193）. Mean weight was 9.7_＋7.8 kg. In order to facilitate the analysis, description of MR was given a numeric grade： 0 = none, 1 = trivial, 2 = mild, 2.5 = mild-moderate, 3 = moderate, 3.5 = moderate-severe, and 4 = severe. Results The LV shortening fraction （LVFS） demonstrated significant improvement between presentation and discharge （32.1±10.1% vs. 28.6±9.6%, P = 0.023）, and there was also significant improvement in the mean MR grade between presentation and discharge （2.40＋1.16 vs. 2.91＋ 1.19, P = 0.001）. There were 4 hospital deaths （7.7%）. The median follow-up was 21 months （range 1.5 to 111）, 3 patients （5.8%） were lost to follow-up, 1 patient required reoperation for mitral valve replacement. Significant improvement was seen in LVFS between discharge and late follow-up （38.0±6.0% vs. 32.1±10.1%, P = 0.001）, however, the degree of MR did not dem- onstrate further change （2.29_＋1.20 vs. 2.40＋1.16, P = 0.541）. Conclusion Creation of a dual coronary system with direct aortic implantation of the left coronary artery results in complete recovery of left ventricular function. Concomitant mitral valve （MV） repair for ALCAPA patients with moderate-severe and severe MR is helpful to early MV function recovery.

右冠状动脉异常起源于肺动脉的诊疗经验及术后随访研究

目的研究右冠状动脉异常起源于肺动脉(anomalous origin of right coronary artery from the pulmonary artery,ARCAPA)的临床特点,了解不同手术方式的预后情况。方法回顾性分析9例确诊为ARCAPA患儿(患者)的临床资料,包括临床表现、辅助检查、手术方式及随访情况。结果共计8例患儿(儿童),1例患者(成人);男7例,女2例;患儿中位月龄67.5个月,成人患者年龄69岁。患儿中4例因活动后胸闷气促就诊,3例因体检发现心脏杂音就诊,1例因脑梗死就诊完善心脏检查诊断,成人患者因气促胸闷行冠状动脉造影检查后诊断。心电图(ECG)检查示3例患儿无明显心肌缺血表现,1例部分下壁导联深Q波,4例(均>6岁)有不同程度右胸导联ST段抬高表现,成人患者提示完全性右束支传导阻滞(CRBBB)、右胸导联T波双向;经胸超声心动图(TTE)检查显示成人患者术前左心室舒张末期内径(LVEDD)增大明显伴左心室射血分数(LVEF)下降,房室瓣重度反流;有2例患儿术前左心室舒张末期内径增大,左心室射血分数均正常。所有患儿(患者)均完成手术治疗,成人患者行右冠状动脉(RCA)异位开口修补+冠状动脉旁路移植术,5例患儿行右冠状动脉直接移植至主动脉(AO)根部手术,1例患儿右冠状动脉延长后移植至主动脉根部,2例患儿行右冠状动脉结扎术。成人患者术后使用多巴胺5 d,米力农3 d强心治疗,3 d后转出重症监护室,术后复查经胸超声心动图示房室瓣反流好转,目前随访3年,左心室射血分数正常。患儿术后撤机时间为0.5~1 d,使用强心药多巴胺1~2 d,1~2 d后转出重症监护室。目前中位随访时间为36个月,所有术后患者均心功能正常,无需要再次手术患者,无死亡患者。结论ARCAPA患者早期无明显心功能不全症状,临床表现不典型,超声心动图可能漏诊或误诊,及时手术治疗预后效果好。

Single coronary artery arising from right sinus of valsalva

52-year-old man presented with a history of angina .pectoris over 3 months. He had a history ofhypertension and hyperlipidemia. His physical examination, blood chemistry, ECG, chest X-ray were normal. A treadmill exercise ECG test was performed using the Bruce protocol. Owing to 1 mm ST segment depressions at derivations DⅡ, DⅢ, aVE V4-V6, and typical chest pain at third stage the test was evaluated as ischemia-positive test. Therefore, he was scheduled fox diagnostic coronary angiography.

成年人冠状动脉造影先天性变异分析

目的 :探讨成年人先天性冠状动脉变异 (畸形 )的检出频度。 方法 :回顾性研究总结 4173例患者的冠状动脉造影资料 ,准确判定各种先天性冠状动脉变异。 结果 :1冠状动脉造影 4173例中检出冠状动脉开口起源异常 5 0例 ,检出率为 1.2 % ,其中各种右冠状动脉起源异常 42例 ,占 84.0 % ,左冠状动脉起源异常 7例 ,占 14.0 % ,左、右冠状动脉开口起源均异常 1例 ,占 2 .0 % ;2检出各种类型冠状动脉分支起源异常 15例 ,检出率为 0 .4% ;3检出各种类型冠状动脉瘘 2 8例 ,检出率为 0 .7% ,其中 18例(6 4.3% )为冠状动脉—肺动脉瘘 ;4其它少见冠状动脉变异 2例。 结论 :成年人先天性冠状动脉变异的临床症状和体征多不典型或缺如 ,通常在心导管检查中被偶然发现 ,冠状动脉造影是确诊各种类型冠状动脉变异最重要的检查方法。

成人冠状动脉起源异常的分类及其发生率

目的探讨成人冠状动脉起源异常（AOCA）类型及其发生率。方法对2000年10月-2006年10月在我院行冠状动脉造影（1 520例）检出的26例存在AOCA患者资料,以及国内2006年10月以前发表的有关成人AOCA研究的8篇文献资料进行综合分析。结果9组资料冠状动脉造影总例数48 158例,检出AOCA 439例,检出率0.91%。右冠状动脉起源异常发生率0.55%（267例）,明显高于左冠状动脉0.36%（174例）,差异有统计学意义（P〈0.01）。右冠状动脉起源于左冠窦为最常见类型,占41.5%（182例）;其次为前降支和回旋支独立开口,占26.0%（114例）。结论我国成人AOCA多见于右冠状动脉,其中具有潜在临床危险的右冠状动脉起源于左冠窦为最常见类型,提示加强国人AOCA研究具有重要临床意义。

双源CT血管成像诊断成人先天性冠状动脉起源变异及畸形的价值

目的:探讨双源CT血管成像(DSCTA)诊断成人先天性冠状动脉起源变异及畸形的价值。方法:803例疑冠心病患者行DSCTA,回顾性分析146例154支先天性冠状动脉起源变异及畸形的表现。结果:803例患者中,共检出成人先天性冠状动脉起源变异及畸形146例154支冠状动脉。动脉圆锥支单独开口于右冠窦118例。开口高位20例,其中左冠主干开口高位18例,包括左冠主干开口于左冠窦嵴8例,左冠主干开口于左冠窦嵴上10例;RCA开口于右冠窦嵴上2例。RCA起源于左冠窦5例及左冠窦嵴上3例,其中4例并走行异常,即开口异常的RCA走行于主动脉与肺动脉之间。LCX起源于右冠窦2例,并走行于左心房与主动脉之间。LCX单独开口于左冠窦1例。3例为2支冠状动脉起源异常,均有动脉圆锥支单独开口于右冠窦,2例合并左冠主干开口于左冠窦嵴上,1例合并左冠主干开口于右冠窦。1例为3支冠状动脉起源异常,动脉圆锥支单独开口于右冠窦合并左、右冠状动脉分别开口于左、右冠窦嵴上。结论:双源CT冠状动脉成像综合各种后处理技术是目前诊断成人先天性冠状动脉起源变异及畸形最有用的无创性检查。

儿童复杂先天性心脏病并冠状动脉畸形造影分析

目的:分析儿童复杂先天性心脏病并发冠状动脉畸形的发生率,探讨冠状动脉造影的诊断价值。方法:2006年1月～2009年6月在重庆医科大学儿童医院心脏中心行心导管检查心血管造影的患儿229例,对患儿复杂先心病的病种构成、冠状动脉畸形发生率、冠脉畸形类型及其发生率、异常冠状动脉跨过右心室流出道的病种、跨过右心室流出道的异常冠状动脉类型,以及患儿预后进行回顾性分析。结果:229例患儿共检出27例冠状动脉先天性变异,检出率为11.8%;其中左单支冠状动脉15例,发生率6.55%,占发生冠脉畸形中的55.56%。27例冠状动脉先天性变异中16例异常冠状动脉跨过右心室流出道,占儿童复杂先天性心脏病的6.99%,异常冠状动脉跨过右心室流出道的先心病类型分别为法乐氏四联症7例、右室双出口3例、肺动脉闭锁/室间隔缺损3例、完全性大动脉转位1例、完全性房室间隔缺损1例、单心室/大动脉移位1例。229例患儿照影死亡1例,占复杂先天性心脏病并冠状动脉畸形造影总数的3.70%,占复杂先天性心脏病造影总数的0.44%,手术死亡3例,占复杂先天性心脏病并冠状动脉畸形手术患儿的16.67%。结论:儿童复杂先心病冠脉畸形的发生率高于正常人群;冠状动脉造影是确诊各种冠状动脉先天性变异的重要手段。

64层螺旋CT冠脉造影在冠状动脉起源异常诊断中的应用价值

目的探讨64层螺旋CT在冠状动脉起源异常诊断中的应用价值。方法收集行64层螺旋CT冠状动脉造影检查患者10308例,判断其有无冠状动脉起源异常情况存在,并判断不同冠状动脉起源异常类型引起冠状动脉狭窄的相关性。结果10308例患者中,发现冠状动脉起源异常者73例,检出率为0.71%,其中左冠状动脉起源异常40例,右冠状动脉起源异常25例,左、右冠状动脉同时存在起源异常8例。冠状动脉起源于同侧冠状窦60例,冠状动脉起源于对侧冠状窦13例,且冠状动脉起源于对侧冠状窦患者中69.2%引起斑块形成和冠状动脉狭窄,较冠状动脉起源于同侧冠状窦患者(38.3%)更易引起斑块形成和冠状动脉狭窄。结论64层螺旋CT冠状动脉造影检查能够准确判断异常起源的冠状动脉的开口、走行,它是诊断冠状动脉起源异常的有效检查方法之一。

先天性左冠状动脉主干闭锁3例超声心动图特征及文献复习

目的通过研究先天性左冠状动脉主干闭锁(LMCAA)的超声心动图表现,提高对LMCAA诊断的准确性。方法回顾性分析经冠状动脉造影证实的3例LMCAA患儿的超声心动图检查结果,并复习相关文献,总结LMCAA超声心动图特征。结果 LMCAA特异性超声心动图特征:①主动脉左冠状动脉窦内无左冠状动脉主干开口,左冠状动脉主干近心端闭锁呈盲端,远心端内径细窄,发育不良;②右冠状动脉内径增宽;③多切面未显示左冠状动脉与肺动脉确切连接的证据。LMCAA非特异性超声心动图特征:①左心室明显扩大,左室收缩功能可正常或减低;②二尖瓣腱索、乳头肌回声显著增强,可伴有二尖瓣脱垂。彩色多普勒超声特征:①收缩期二尖瓣口可见中至大量反流信号;②左、右冠状动脉之间形成细小侧支循环;③左冠状动脉前降支和回旋支血流为逆向灌注(向心性);④发育不良的左冠状动脉虽然在肺动脉周围分布,但彩色多普勒超声不能显示其与肺动脉连接的确切逆灌血流信号。结论 LMCAA有特异性的超声心动图特征,提高对LMCAA的全面认识是诊断本病的关键。

小儿左冠状动脉起源于肺动脉临床分析

目的:总结小儿左冠状动脉起源于肺动脉(ALCAPA)的临床特点,提高对ALCAPA的认识。方法:对2006年8月至2008年12月期间我院心脏中心门诊以"心内膜弹力纤维增生症"收治但最终诊断为ALCAPA的8例患者的临床特点、检查情况进行回顾性分析。结果:8例ALCAPA的患者女性7例,男性1例。发病年龄2～10个月,平均年龄5.3个月。8例患者均表现不同程度的气促、喘息、声哑、多汗及喂养困难等心力衰竭(心衰)症状。病初均于首诊医院误诊为心内膜弹力纤维增生症。患儿心电图、心脏超声及心脏增强CT均有较为特征性的改变。结论:小儿ALCAPA病例临床常易与心内膜弹力纤维增生症混淆。对于临床诊断心内膜弹力纤维增生症的患儿应注意其心电图表现。其中符合ALCAPA心电图特点的病例,反复探查其心脏彩超并进行心脏增强CT、心脏冠状动脉造影检查有助于ALCAPA的诊断。