左冠状动脉起源于肺动脉7例临床分析

左冠状动脉起源于肺动脉(anomalous origin of left coronary artery from pulmonary artery, LCAPA)是罕见的先天性心脏畸形,国外报道约占先天性心脏病的0．4％。本病预后极差,未行手术治疗者死亡率高。由于对疾病的认识和检查手段方面的原因,早期难以发现,临床误诊率高,尤其易误     

左冠状动脉起源于肺动脉二例

左冠状动脉起源于肺动脉（anomalous origin of left coronary artery from pulmonary artery,ALCAPA）是一种罕见的先天性心脏病,早期表现为心肌缺血、心力衰竭等非特异性症状,死亡率超过90％,平均死亡年龄35岁,如未予以手术治疗,极少有患者能活过50岁。因其早期症状缺乏特异性,以往对其没有很好的认识,临床上易误诊为扩张性心肌病、心内膜弹力纤维增生症、先天性的二尖瓣病变等。早期手术治疗能明显改善预后,故早期诊断有重要的临床价值。现将我院诊治的2例ALCAPA资料总结报告如下。[第一段]     

左冠状动脉起源于肺动脉6例患儿的诊断与治疗

目的分析左冠状动脉起源于肺动脉(ALCAPA)的诊断特点,评估其手术效果。方法本院2006年3月-2007年10月共收治ALCAPA患儿6例。男4例,女2例;年龄6个月～10岁,平均43.5个月。对这6例患儿的临床表现、心电图(ECG)特征、心脏超声、64排螺旋CT及心导管检查资料、手术治疗及转归进行分析。结果ALCAPA5例患儿有不同程度的多汗、烦躁、气促、拒食等临床表现,临床检查示心功能不全。ECG特异表现:4例患儿ECG存在前侧壁异常Q波及ST段改变,1例患儿存在ST段改变,1例患儿ECG大致正常。典型彩色超声心动图表现为:4例患儿出现左室扩大伴左室收缩功能减低;2例患儿存在二尖瓣轻-中量返流,2例患儿为大量二尖瓣返流;2例患儿表现为右冠状动脉扩张。增强CT及心导管检查有特征性改变。其中5例患儿行手术治疗:2例直接移植,3例行左冠状动脉重建术;2例重度二尖瓣返流患儿同时行二尖瓣成形术。1例无症状患儿未行手术治疗。术后患儿症状改善,无死亡病例。结论了解ALCAPA的病变特点及相关辅助检查,可提高ALCAPA的检出率,尽早手术治疗,改善预后。     

左冠状动脉起源于肺动脉的临床分析

目的：左冠状动脉起源于肺动脉（anomalous origin of the left coronary artery from the pulmomary artery, ALCAPA)是临床上一种少见的先天性冠状动脉异常性疾病，目前国内报道的资料不多。该研究对其临床特点与治疗进行探讨。方法：对10例诊断为左冠状动脉起源于肺动脉患儿的临床表现、心电图特征、心脏超声、心导管资料、手术治疗及转归进行分析。结果：患儿都有不同程度的多汗、烦躁、气促、拒食等心绞痛及心功能不全的表现；心电图、彩色超声心动图及心导管检查有较特征性的改变；4例患儿行肺动脉内隧道术，4例行直接左冠状动脉移植术，2例行左冠状动脉重建术。术后8例症状改善，2例死亡。结论：掌握ALCAPA的病变特点和相关的诊断线索，辅以彩色超声心动图和心导管检查，可提高ALCAPA的检出率，并早期手术治疗。［中国当代儿科杂志，2007，9（1）：25-27］     

左冠状动脉起源于肺动脉的术前诊断与治疗

目的 探讨左冠状动脉起源于肺动脉(anomalous origin of the left coronary artery from the pulmonary artery,ALCAPA)的术前诊断,手术治疗和疗效.方法 对山西省儿童医院2010年6月至2014年3月收治的9例ALCAPA患儿的术前诊断及手术方式进行总结.结果 本组患儿共9例,男3例,女6例,年龄4个月～4岁6个月,平均(1.14±1.33)岁;体重5.6～18.0 kg,平均(8.06±3.99)kg.术前行心脏彩超、心脏增强CT检查,明确诊断,其中有1例患儿行心脏造影检查.9例患儿中合并二尖瓣轻度反流2例,二尖瓣轻-中度反流5例,中-重度反流2例.心电图表现:9例患儿在导联Ⅰ、AVL及V4-6有不同程度的异常Q波,ST段改变或T波倒置.胸部X线检查示心影明显增大,心胸比例0.56～0.73,平均0.61 ±0.05,左心缘饱满隆起,向左下方突起,心脏彩超示左冠状动脉起源于肺动脉,左心室明显扩大,心肌收缩力减弱,射血分数低于50％,8例患儿左心室舒张末径均＞35 mm,1例患儿合并心尖部室壁瘤.1例患儿行心脏彩超及CT检查后怀疑ALCAPA,后经造影检查明确诊断.6例患儿术前行放射性核素心肌显像检查,均伴有不同程度的心尖、前壁、侧壁心肌梗死,部分心肌存活.本组患儿均在全身麻醉体外循环下行左冠状动脉移植术,7例患儿加做二尖瓣成形术.全组患儿早期死亡1例,死亡原因为严重低心排综合征,余均治愈出院.其中,围术期合并低心排综合征2例,肺炎3例,室上性心动过速1例.术后随访3个月～2年,超声心动图检查提示冠状动脉血流通畅,射血分数较术前提高,二尖瓣反流减轻.患儿生长发育良好,无心力衰竭表现,心功能恢复良好.结论 超声心动图对ALCAPA的诊断有重要价值,心脏增强CT及造影检查可确诊,术前核素心肌显像对存活心肌的判定、手术及预后有重要意义.冠状动脉移植术是最理想的手术方法.     

成人型与婴儿型左冠状动脉异常起源于肺动脉的超声诊断

目的总结不同类型左冠状动脉异常起源于肺动脉（ALCAPA）的超声心动图表现,提高对本病的诊断及鉴别诊断能力。方法回顾性分析首都医科大学附属北京安贞医院儿童心血管病中心收治并经手术证实的24例ALCAPA患儿的超声心动图结果,根据侧支循环的发育情况分为婴儿型和成人型,并总结婴儿型和成人型ALCAPA的超声心动图特征。 结果婴儿型和成人型ALCAPA相同的超声心动图特征：①左冠状动脉窦内无左冠状动脉主干开口,其主干直接与肺动脉连接;②左心室高度扩张,室间隔和左室前壁节段性运动障碍,左室收缩功能减低;③左心室广泛纤维化,以心内膜下区域最为显著;④二尖瓣腱索、乳头肌纤维化,回声显著增强;⑤右冠状动脉起源位置正常,内径增宽;⑥左冠状动脉前降支和回旋支血流为逆向灌注（向心性）;⑦收缩期二尖瓣口可见少至大量反流信号。婴儿型和成人型ALCAPA不同的超声心动图表现：彩色多普勒显示成人型侧支循环血流丰富,婴儿型侧支循环少。 结论婴儿型和成人型ALCAPA具有其特异性的超声心动图特征,结合其临床及心电图等表现,可以对本病做出正确诊断。     

心电图对婴儿型左冠状动脉起源于肺动脉的诊断价值

左冠状动脉起源于肺动脉 (anomalousoriginoftheleftcoronaryarteryfromthepulmonaryartery ,ALCAPA)是一种少见的先天性冠状动脉异常性疾病。因婴幼儿期临床发病一般表现为心力衰竭 (简称心衰 )症状且影像学显示心脏扩大、心内膜增厚而极易被误诊为原发性心内膜弹力纤维增生症 (简称心弹 )。我们对我院收治的 6例经手术证实的婴儿型ALCAPA和 4 4例临床诊断为心弹的年龄 <4岁的病例进行回顾性调查 ,对比分析两组病例的心电图特征 ,以评价心电图在诊断婴儿型ALCAPA和鉴别诊断中的价值。对象及方法病例分为两组。一组为我院 1993年 8…     

婴儿左冠状动脉起源于肺动脉的诊断研究

目的　研究婴儿左冠状动脉起源于肺动脉（ＡＬＣＡＰＡ） 的临床特点及诊断方法。方法　总结分析１９９３～１９９８ 年收治的４ 例ＡＬＣＡＰＡ 的临床特点。结果　４ 例ＡＬＣＡＰＡ均初诊为婴儿心内膜弹力纤维增生症,发病年龄小于３ 个月,心电图Ｉ,ａＶＬ,Ｖ４～６ 导联出现异常Ｑ 波,彩色多普勒超声心动图检查示左冠状动脉缺如。最后经导管检查及心血管造影确诊。结论　本文提出的诊断方法有助于婴儿ＡＬＣＡＰＡ的诊断,且易为儿科医师掌握。     

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。     

一侧肺动脉起源于升主动脉的诊治特点探讨

目的 探讨一侧肺动脉起源于升主动脉(AOPA)的临床诊治特点.方法 对12例AOPA患儿的临床特点、超声心动图、心血管造影及于术治疗结果 进行总结分析.结果 12例中男7例,女5例;平均年龄23个月;均有不同程度的气促、喂养困难及反复呼吸道感染史.经心导管检查证实诊断,其中9例为右肺动脉异常起源于升主动脉,为近端型;3例左肺动脉异常起源于升主动脉,1例为近端型,2例为远端型.12例患儿均合并有不同程度的肺动脉高压.超声心动图检查的符合率为83%.9例经手术治疗,其中1例术后4个月死亡.结论 超声心动图对于AOPA的诊断具有重要价值,结合心导管造影检查可确诊,心导管造影对大龄患儿尤其需要;由于存在发展为器质性肺高压的风险,本病一经诊断应尽早行于术治疗.     

Association of Common Origin of the Carotid Arteries with Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery

Common origin of the carotid arteries (COCA) is a common pattern of aortic arch vessels and is the single most common cause of tracheobronchial compression by a congenital cardiovascular anomaly. By no means all affected patients are symptomatic. Symptoms may range from recurrent pulmonary infections and “noisy respiration” to stridor and apneic spells. In our study of patients with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) we found a highly significant association of COCA with ALCAPA (85%), although no patient with ALCAPA in this study had evidence of tracheal stenosis documented in the hospital chart. As COCA is easily correctable, we suggest consideration of COCA during evaluation and surgery of patients with ALCAPA so that, if the patient also has symptoms possibly related to COCA, the artery can be suspended from the posterior wall of the sternum.     

Hoarseness as the Initial Clinical Presentation of Anomalous Left Coronary Artery from the Pulmonary Artery

Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital anomaly that, if left untreated, will most often result in severe myocardial ischemia and significant morbidity and mortality. We report an unusual presentation of this defect in a 2-month-old infant who had an initial complaint of a “hoarse cry.” We theorize that impingement of the recurrent laryngeal nerve due to dilatation of the pulmonary artery was the most likely etiology of the patient's symptoms. This case serves as an important reminder that serious congenital heart disease may present with any number of complaints and unusual findings.     

A Simple and Feasible Technique for Repair of Anomalous Origin of the Left Pulmonary Artery from the Ascending Aorta

Anomalous origin of the left pulmonary artery from the ascending aorta is a rare congenital cardiac malformation and the direct reimplantation technique has been the strategy of choice, although postoperative restenosis across the anastomosis site is frequently observed. In the described case, a simple and feasible technique was used with satisfactory results at 20-month follow-up.     

Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery Associated with Ventricular Septal Defect and Mitral Stenosis

We report a case of a child with ventricular septal defect, mitral stenosis, and patent ductus arteriosus, who was also found to have anomalous origin of the left coronary artery from the pulmonary artery. Preoperative diagnosis allowed successful surgical correction.     

Tetralogy of Fallot with Anomalous Origin of the Right Pulmonary Artery from the Ascending Aorta and Hypoplastic Left Pulmonary Artery

A case of tetralogy of Fallot associated with anomalous origin of the right pulmonary artery from the ascending aorta and hypoplastic left pulmonary artery was successfully repaired. Following the simultaneous procedure with banding of the right pulmonary artery and replacement of modified Blalock-Taussig shunt to the left pulmonary artery at 34 days of age, the intracardiac repair was performed successfully at 1 year and 10 months of age. The patient was well 6 months postoperatively, without unilateral pulmonary hypertension.     

Abnormal Flow in the Posterior Descending Artery: An Echo Doppler Clue to the Anomalous Origin of Left Anterior Descending Coronary Artery from the Pulmonary Trunk

Anomalous origin of the left anterior descending coronary (LAD) from the pulmonary trunk is a rare lesion. We describe a neonate with anomalous origin of the LAD from the pulmonary artery and the circumflex artery arising normally from the left-facing sinus. The finding of a tortuous and dilated posterior descending coronary artery suggested increased intercoronary collateral flow and provided a clue to the identification of the anomaly in an otherwise asymptomatic neonate. Interrogation of the posterior descending coronary artery should be included in the routine echocardiographic examination of children, as unusual flow patterns may help identify aberrant coronary artery connections. Electronic supplementary material  The online version of this article (doi:) contains supplementary material, which is available to authorized users.     

Anomalous Origin of the Right Coronary Artery from the Left Sinus of Valsalva

A 1-month-old boy without precedent clinical signs of heart disease died unexpectedly. At autopsy, the child was found to have origin of the right coronary artery from the left sinus of Valsalva immediately leftward to the thickening of the aortic wall at the cuspal commissure and adjacent to the ostium of the left main coronary artery. The association of this coronary anomaly with otherwise unexplained prenatal or early postnatal death has only recently been appreciated as a potential cause of sudden infant death and mimic of sepsis, dysrhythmias, congestive heart failure, or metabolic storage diseases.     

Anomalous Left Coronary Artery from the Right Sinus of Valsalva and Noncompaction of the Left Ventricle

Anomalous origin of the left coronary artery is a well-known cause of sudden death. Noncompaction of the ventricular myocardium is a cardiomyopathy characterized by prominent trabeculae and deep intertrabecular recesses. Both anomalies are rare. We report the case of a child with both anomalous origin of the left coronary artery from the right sinus of Valsalva and noncompaction of the left ventricular myocardium found during an evaluation for Kawasaki’s disease.     

Anomalous Origin of the Left Coronary Artery in Tetralogy of Fallot Associated with Abnormal Mitral Valve Pathology

The association of anomalous left coronary artery with congenital heart disease is a rare occurrence. Seven cases of anomalous left coronary artery associated with tetralogy of Fallot have been reported in the literature. We report a unique case with severe mitral valve abnormality that precluded standard surgical repair.