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诊治特点

目的探讨左冠状动脉起源于肺动脉的临床诊治特点。方法对16例左冠状动脉起源于肺动脉患者的体表心电图、超声心动图、心血管造影检查及手术治疗结果进行分析。结果①心电图:Ⅰ、Ⅱ、aVL、V5、V6导联T波倒置且ST段压低8例,其中6例伴左胸导联异常Q波;T波倒置4例。②超声心动图:16例左冠状动脉起源于肺动脉患儿中合并房间隔缺损2例,左室侧壁运动减弱及左室射血分数(LVEF)降低6例。③15例心导管造影提示左冠状动脉起源于肺动脉,合并房间隔缺损2例,合并动脉导管未闭1例。④治疗结果:所有患儿均进行手术治疗,死亡2例,余14例随访1个月～6 a,冠状动脉血流通畅。结论对于左冠状动脉起源于肺动脉的诊断,应结合其特征性心电图表现有助于诊断,超声心动图具重要价值,结合心导管造影检查可确诊,一经诊断应尽早手术治疗。     

婴儿型左冠状动脉异常起源于肺动脉的诊断与治疗北大核心CSCD

目的研究婴儿型左冠状动脉异常起源于肺动脉（anomalous origin of the left cornary artery from the pulmonary artery,ALCAPA）临床特点、诊断及治疗。方法2010年1月至2012年6月广东省人民医院小儿心外科手术治疗婴儿型ALCAPAl8例,对这些患儿的临床表现、检查诊断与治疗进行回顾性分析。结果 婴儿型ALCAPA临床表现无特异性,易误诊。ALCAPA诊断依赖心脏B型超声及心脏CT检查。18例手术治疗,死亡l例。结论掌握ALCAPA的临床特点,可提高ALCAPA检出率。婴儿型ALCAPA手术治疗病死率低,效果好。     

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。     

左室收缩功能正常的左冠状动脉异常起源于肺动脉患儿的超声诊断13例病例系列报告

目的:探讨左室收缩功能正常的左冠状动脉异常起源于肺动脉(ALCAPA)患儿的超声心动图特点。方法:回顾性分析2010年1月至2019年12月广州市妇女儿童医疗中心收治并经手术证实的13例左心室射血分数（LVEF）≥55%的ALCAPA患儿的超声心动图特征和临床资料。结果:13例ALCAPA患儿均可见右冠状动脉增宽,其他超声征象包括左冠状动脉血流逆灌12例、左冠状动脉直接开口于肺动脉9例、肺动脉内逆灌血流7例、心肌侧枝血流8例、左室明显增大8例、二尖瓣功能不全7例、左室心内膜纤维化5例。<1岁ALCAPA患儿的心肌侧枝血流信号不明显,左心室大小正常或轻度增大且二尖瓣功能良好;≥1岁患儿可见丰富侧枝血管、左室明显扩大以及二尖瓣功能不全。4例误诊的ALCAPA左冠状动脉均起源于肺动脉右后方。结论:超声心动图可准确诊断左室收缩功能正常的ALCAPA,如果超声科医生在怀疑该诊断时能全面探查冠状动脉的起源和血流方向,可减少漏诊和误诊。     

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目的 探讨小儿左冠状动脉起源于肺动脉(ALCAPA)所致心脏扩大病例的误诊原因,总结诊断经验.方法 回顾性分析1996年7月至2009年1月广东省心血管病研究所22例小儿ALCAPA所致心脏扩大的误诊病例.均行心电图、X线胸片、超声心动图检查,8例行心脏CT检查,14例行心导管检查及造影,21例进行了外科手术治疗.结果 ≤1岁患儿10例中,6例误诊为心内膜弹力纤维增生痘,3例误诊为扩张型心肌病,1例误诊为先天性二尖瓣脱垂并关闭不全.>1岁患儿12例中,4例误诊为扩张型心肌病,3例误诊为心内膜弹力纤维增生症,2例误诊为先天性二尖瓣脱垂并关闭不全,1例误诊为右冠状动脉右心室瘘,1例误诊为川崎病,1例误诊为动脉导管未闭.结论 小儿ALCAPA所致心脏增大病例容易误诊为心内膜弹力纤维增生症、扩张型心肌病、先天性二尖瓣脱垂等疾病,左冠状动脉起源于肺动脉后方偏右的病例更易误诊.提高对ALCAPA的认识、超声或CT检查中仔细探查冠状动脉及其起源对避免误诊至关重要.     

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.     

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.     

左冠状动脉异常起源于肺动脉的临床特征及治疗策略北大核心CSCD

目的 探讨左冠状动脉起源于肺动脉（ALCAPA）患儿的临床特征及治疗策略.方法 对2006年至2013年广东省心血管病研究所收治的25例诊断为ALCAPA并行手术治疗的＜1岁患儿的临床资料进行分析,术后随访超声心动图评估心功能.结果 患儿临床特征以气促、多汗、喂养困难、生长发育落后多见.心电图示异常Q波23例,ST-T改变16例.超声心动图检查示左心室扩大25例,心内膜增厚5例,右冠动脉扩张并存在左右冠状动脉交通血流11例.左心室射血分数（LVEF） （45.5±l3.9）％（25％～77％）,左心室缩短率（LVFS） （22.0±7.3）％（12％ ～ 38％）.21例患儿行高速多层心脏CT检查,提示左冠状动脉开口于肺动脉瓣左后侧窦9例,有后侧窦1例,开口于肺动脉主干二下段5例,开口于肺动脉近分叉处l例,开口位置不确定5例.本组25例患儿中17例在外院首诊后转入我院,除l例在外院行心血管导管检查确诊ALCAPA外,其余16例均被误诊;8例我院首诊的病例中,误诊2例,后经超声心动图复查及心脏CT检查后修正诊断.术前根据患儿的心功能情况予以不同程度的强心、利尿、扩血管治疗.2例结扎左冠状动脉口,23例采用左冠状动脉移植技术重建双冠状动脉系统.对于心功能Ⅳ级合并中度以上二尖瓣反流或心功能Ⅲ级合并重度以上二尖瓣反流者同期行二尖瓣成形术.对术后LVEF＜ 30％者,如果应用药物无法维持血压,乳酸呈进行性升高,则施行体外膜肺氧合（ECMO）辅助.2例患儿行左冠状动脉移植术后因顽固性室颤早期死亡;存活的23例患儿,呼吸机使用时间为7 ～500 h,其中11例＜60 h.平均住院天数（23.4±13.9）d（8～65 d）.中位随访时间为28.5个月（1～91个月）,失访1例,因感染性休克术后3个月院外死亡.随访的22例患儿心功能均较术前改善,14例左心室大小恢复至正常,20例LVEF恢复至正常.无行2次手术者.结论 结合病史、心电图、超声心动图及其他多种影像学诊断方法有利于正确诊断ALCAPA;对于危重患儿采取个体化治疗策略,并对术后低心排患儿积极进行ECMO辅助,可显著提高手术成功率.     

左冠状动脉异常起源于肺动脉的外科治疗及中期随访研究

目的总结评估单中心15年对于左冠状动脉异常起源于肺动脉(anomalous origin of the left coronary artery from the pulmonary artery,ALCAPA)的外科治疗经验及预后评估。方法回顾性分析2003年1月至2018年9月上海儿童医学中心心胸外科收治的126例ALCAPA患儿的临床资料,其中男59例,女67例,年龄为(17.8±12.6)个月,体重为(8.1±3.4)kg。所有患儿均接受ALCAPA纠治术,手术方式包括65例采用"凸窗"技术,51例利用自体组织重建冠状动脉管道(22例肺动脉组织管道,15例心包管道以及14例双活瓣延长管道),10例行"Takeuchi"技术;所有患儿中有72例合并中度以上二尖瓣反流,同时接受了二尖瓣整形手术。结果 21例患儿因急性左心衰竭于术后72 h内行机械辅助循环,辅助3～7 d,均顺利撤机。术后早期11例患儿死亡,其中4例为机械辅助撤离后死亡,死亡原因主要为左心衰竭、多器官衰竭和脑出血。3例中远期死亡发生在术后18个月内,死亡原因为心脏休克和心力衰竭。生存患儿出院后规律随访,随访时间为(6...     

左冠状动脉起源于肺动脉37例患儿各年龄段的临床特点及诊治

目的分析各年龄段左冠状动脉起源于肺动脉(ALCAPA)患儿的临床特点和诊治情况,提高对此病的认识,减少误诊,提高救治率。方法 1999年4月-2011年3月本院共收治37例ALCAPA患儿。男21例,女16例;年龄(5.0±4.8)岁。其中婴儿组(≤1岁)8例,幼儿组(>1～3岁)10例,大年龄组(>3岁)19例。37例中并二尖瓣关闭不全31例,室壁瘤2例,法洛四联症/ASD、重度肺动脉瓣狭窄/部分型肺静脉异位引流、ASD各1例。对各年龄段临床特点和诊治结果进行回顾性分析。随访结果依据门诊复查结果及电话随访。结果婴幼儿组发病年龄均早于大年龄组;ECG出现Q波及ST-T改变13例(35.1%,13/37例),其中婴儿组5例(62.5%)、幼儿组3例(30.0%)、大年龄组5例(26.3%),3组差异无统计学意义;胸部X片示心脏均明显增大,心胸比婴儿组大于幼儿组和大年龄组,幼儿组大于大年龄组;超声心动图示左心室舒张末径(LVED)3组均增大,以婴儿组增大最明显;左心室射血分数(LVEF)<50%者婴儿组多于幼儿组和大年龄组,幼儿组与大年龄组无明显差异。首次诊断时误诊或漏诊18例,占48.6%(18/37例)。婴儿组1例由于核素扫描提示存活心肌很少,等待心脏移植,另2例家长放弃手术。34例患儿行冠状动脉移植术23例,肺动脉内隧道术8例,冠状动脉搭桥2例,单纯结扎冠状动脉左主干1例。无手术早期死亡。术后有近期随访资料26例(76.5%,26/34例),随访6～130(13.6±18.4)个月,晚期死亡1例。患儿术后LVED均较术前缩小,仍有二尖瓣轻度关闭不全8例。有随访资料患儿中,8例术前LVEF<50%,术后3～6个月LVEF均恢复正常。结论 ALCAPA婴儿患儿临床表现较幼儿及年长儿患儿重。小儿ALCAPA由于缺乏特征性表现,各年龄段均易误诊。对于小儿各年龄段表现为心脏扩大、心功能不全、二尖瓣关闭不全等患儿,要注意除外小儿ALCAPA。可以结合其ECG表现,反复超声ECG探查冠状动脉,必要时加行多层螺旋CT或心血管造影以明确诊断,确诊后应尽快手术治疗。     

Electrocardiographic and Echocardiographic Features That Distinguish Anomalous Origin of the Left Coronary Artery from Pulmonary Artery from Idiopathic Dilated Cardiomyopathy

Many authors have reported noninvasive means of diagnosing anomalous left coronary artery from pulmonary artery (ALCAPA) and differentiating ALCAPA from idiopathic dilated cardiomyopathy (DCM). Systematic evaluation using these noninvasive diagnostic modalities is not available. To distinguish between ALCAPA and DCM using a systematic approach, we examined 23 patients with ALCAPA (age 1 month to 23 years, median 7 months) and 23 patients with DCM (age 5 days to 16 years, median 6.6 months). Standard 12-lead electrocardiograms (ECG) and 2-dimensional (2-D) and color Doppler echocardiograms were performed. A logistic regression model was applied using ALCAPA diagnosis as the dependent variable and ECG and echocardiographic findings as independent variables. A scoring system was created to establish the ALCAPA diagnosis based on results from the logistic regression. On the logistic regression, the ECG feature of QT pattern in aVL (Q wave ≥ 3 mm deep with an inverted T wave) and echocardiographic features of right coronary artery diameter to aortic annulus ratio ≥ 0.14, increased papillary muscle echogenicity, and Doppler color flow of LCA from aorta or pulmonary artery were the most significant differentiating features between the ALCAPA and DCM groups. A scoring system was developed using the previous five variables and assigning a score of 1 to each variable (−1 to Doppler color flow of LCA from aorta). The scoring system had sensitivity of 100% and specificity of 91% for ALCAPA diagnosis. Compared with previous reported diagnostic features in differentiating ALCAPA and DCM, the scoring system had a much higher specificity and positive predictive value. In conclusion, we selected the most useful ECG and echocardiographic features to differentiate between ALCAPA and DCM and created a scoring system to aid clinical diagnosis. This scoring system may be useful in evaluating children with acute congestive heart failure.     

左冠状动脉异常起源于肺动脉22例诊断分析

目的 探讨左冠状动脉异常起源于肺动脉的临床诊断方法.方法 收集本院2000年2月至2007年2月左冠状动脉异常起源于肺动脉患儿22例,分为婴儿组(年龄≤1岁,n=10)和少儿组(年龄＞1岁且≤15岁,n=12),回顾性分析临床表现、心电图和经胸超声心动图表现.其中心电图主要分析如下特征:(1)Ⅰ导联Q波和T波倒置,(2)aVL导联异常Q波和T波倒置,(3)V5-6导联Q波,(4)v4-6导联T波倒置或ST段变化,(5)左心室肥大.经胸超声心动图主要分析如下特点:(1)左冠状动脉与肺动脉的连接,(2)肺动脉内逆向的多普勒血流,(3)左心室乳头肌的回声强度,(4)右冠状动脉扩张,(5)室壁和室隔间侧支循环信号.结果 胸片示心影增大(18/22)、心电图示aVL导联异常Q波和T波倒置(17/22)、超声心动图示汇入肺动脉的逆向多普勒血流(20/22)、前外侧乳头肌强回声(17/22)和侧支循环信号(16/22)在两组患儿中的出现率均很高,差异无统计学意义(P＞0.05);婴儿组的临床症状和心电图示V5-6导联Q波、Ⅰ Q波和T波倒置的出现率均显著高于少儿组(P＜0.05);婴儿组右冠状动脉扩张的出现率要显著低于少儿组(p＜0.05).结论 婴儿和少儿患者的诊断特点不完全相同,胸部X线片示心影增大、心电图示aVL导联异常Q波和T波倒置对本病诊断有重要的提示作用,而超声心动图示逆向汇入肺动脉的彩色多普勒血流、前外侧乳头肌强回声和侧支循环信号等间接征象强烈提示本病的诊断,结合临床病史、心电图和超声心动图检查可以确诊本病.     

Anomalous left coronary artery from pulmonary artery (ALCAPA) in infants: a 5-year review in a defined birth cohort

Anomalous left coronary artery from pulmonary artery (ALCAPA) is a rare congenital cardiac malformation. It presents predominantly in infancy with features of myocardial ischaemia or cardiac failure and may be mistaken for common paediatric conditions such as colic, reflux or bronchiolitis. With early surgical correction the prognosis is good, but awareness of this condition is essential for prompt diagnosis and referral to a tertiary cardiac centre. In this report we review the five cases that presented during our 5-year study period and discuss the incidence and clinical presentation of ALCAPA among infants. Our observed incidence of 1 in 4243 live births – 0.023 % – is higher than previously reported. ALCAPA may be more common than previously recognised, and there should be a high index of awareness among paediatricians, paediatric trainees and general practitioners to enable early surgical intervention and improved prognosis for these children.     

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。     

儿童冠状动脉疾病32例临床分析

目的探讨儿童冠状动脉疾病的病因分布及临床特点,以提高诊断和治疗儿童冠状动脉疾病水平。方法回顾性分析2008年7月至2011年12月在首都医科大学附属北京安贞医院儿童心脏中心收治的冠状动脉疾病患儿（不包括复杂心脏畸形合并冠状动脉异常者）的临床资料,分析临床表现、辅助检查、诊断方法和治疗。结果32例冠状动脉疾病患儿进入分析,男20例,女12例,年龄1个月至18岁。经ECG、超声心动图、64排CT和心导管造影检查明确诊断。左冠状动脉异常起源于肺动脉10例(31.2%),8例以心力衰竭起病,其中长期误诊为“心内膜弹力纤维增生症”4例,“扩张性心肌病”1例,“二尖瓣重度关闭不全”2例,均行外科手术移植左冠状动脉,1例术后死亡,余9例术后随访临床症状好转,左室缩小,二尖瓣反流减轻;冠状动脉瘘13例(40.6%),9例以心脏杂音、4例以心力衰竭起病,外科手术行瘘缝扎或补片治疗12例,介入治疗1例,术后随访仅1例存在少量残余分流;川崎病并发冠状动脉瘤6例(18.8%),出现左心扩大、心肌缺血征象,予长期抗凝等治疗;家族性高胆固醇血症3例(9.4%),表现为冠状动脉粥样硬化性心脏病,予降胆固醇、改善心肌缺血等药物及饮食控制治疗,随访病情尚稳定,无明显加重。结论儿童冠状动脉疾病少见,先天性包括左冠状动脉异常起源于肺动脉和冠状动脉瘘,后天性以高胆固醇血症和川崎病累及冠状动脉多见,对于有左心扩大和心肌缺血表现者,应重视冠状动脉疾病的可能,早期诊断、合理治疗是改善预后的关键。     

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.     

Resolution of pathologic Q wave,left ventricular dysfunction and mitral regurgitation after dual coronary repair of the anomalous origin of the left coronary artery from the pulmonary artery

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital cardiac defect that usually presents as dilated cardiomyopathy in infancy. From 1984 to 2005, 13 (five males and eight females, 0.13%) out of 9,950 patients with congenital heart disease were identified as ALCAPA at our institute. Corrective surgery was performed at a median age of 9 months (range: 2 months to 5 years). Eleven patients underwent direct reimplantation of the left coronary artery (LCA) to the aorta, while two received extrapulmonary baffling. The overall survival rate was 92%. Only one patient died 5 months after reimplantation of the LCA due to acute myocardial ischaemia. Left ventricular ejection fraction (LVEF) was significantly lower in the eight (62%) patients operated during infancy than in those operated beyond 5 months (median: 35% vs. 75%). Left ventricle function was normalised in 11 patients (85%). Among the eight patients without concomitant mitral annuloplasty, mitral regurgitation (MR) improved to a mild or trivial degree in six patients and remained at the pre-operative level in two patients. Pathologic Q wave was noted in 11 patients, which eventually regressed in all except two cases. The median interval of recovery was 16 days, 6 months and 24 months for MR, LVEF and electrocardiogram (ECG) changes, respectively. In conclusion, ALCAPA is also a rare disease in Asian countries, such as Taiwan. The subsequent recovery of MR, left ventricular (LV) function and even pathologic Q wave can be expected after dual coronary repair, regardless of the age at repair.