成人型左冠状动脉起源于肺动脉的外科治疗

目的:总结成人型左冠状动脉起源于肺动脉(ALCAPA)外科手术治疗的经验。方法:回顾性分析本院2005年l O月至2017年9月,手术治疗34例ALCAPA患者的临床资料,其中男性7例,女性27例;年龄18~63岁,平均年龄(38.4±13.5)岁。手术方法包括左冠状动脉直接移植术、肺动脉内通道术(takeuchi)、肺动脉壁卷管延长移植术(tubular extension)、冠状动脉旁路移植术(CABG)和二尖瓣成形术(MVP)。术后观察手术效果,围术期病死率和并发症发生率。结果:12例患者接受Takeuchi术,9例患者接受左冠状动脉直接移植术,10例患者接受tubular extension移植术,另3例患者接受CABG术,7例患者因二尖瓣中量或大量返流接受MVP术。体外循环时间和主动脉阻断时间分别为(144.8±65.8)min和(99.7±47.2)min。术后10例患者二尖瓣少量返流,无中量以上返流。术后左心室舒张末期内径(LVEDD)[(49.0±9.0)vs.(55.2±5.6)mm,P=0.001]及左心室射血分数(LVEF)[(61.1±7.1)%vs.(57.3±8.4)%,P=0.047]与术前相比均明显改善,差异有统计学意义,术后无严重并发症及死亡。术后随访时间6个月~12年,随访期间无患者死亡,心功能正常。结论:成人型ALCAPA经外科手术治疗后效果满意,因此,目前手术是唯一的治疗手段     

应用再植技术治疗起源异常左冠状动脉的效果分析

目的:分析和讨论应用冠状动脉再植技术治疗先天性起源于肺动脉的左冠状动脉异常(ALCAPA)的外科治疗效果和经验。方法:回顾分析2008年4月~2013年4月26例应用冠状动脉再植技术治疗先天性ALCAPA患儿的临床资料。其中男14例,女12例;年龄4月~6岁。术前均经超声心动图,CT和或心血管造影检查明确诊断。结果:术前左室射血分数(LVEF)20~80(48±18)%,心胸比0.54~0.77(0.64±0.07)。并发二尖瓣关闭不全(MI)轻度以上21例,其中中度以上17例。左心室心尖部室壁瘤1例,房间隔缺损1例。同期行二尖瓣成形11例,室壁瘤切除1例,房间隔缺损修补1例。本组患儿手术均顺利完成,无手术死亡。体外循环时间91~238(150±37)min,心肌阻断时间64~200(126±36)min。随访1~61(29±19)月,患儿无远期死亡。最后一次随诊LVEF35~75(64±11)%,心胸比0.48~0.65(0.57±0.05),较术前明显改善。MI中度以下24例。结论:冠状动脉再植技术手术效果良好,对于中度以上MI应积极治疗。     

左冠状动脉异常起源于肺动脉的超声诊断及误诊分析

目的探讨超声心动图对左冠状动脉异常起源于肺动脉(ALCAPA)的诊断价值,分析误诊原因,以提高本病的诊断及鉴别诊断水平。方法选取我院2013年10月~2015年11月收治的经心外科手术证实的ALCAPA患儿18例的超声检查资料,并与CTA及手术结果进行对比分析。结果 18例患儿中,婴儿型12例,成人型6例,超声诊断14例(符合率77.8%),误诊4例(22.2%),其中1例超声诊断为心内膜弹力纤维增生症,2例超声诊断为左冠状动脉肺动脉瘘,1例超声诊断为重度二尖瓣反流。结论超声心动图检查简单方便,无创伤性,可实时动态显像,是诊断ALCAPA的最佳检查方法。     

81例左冠状动脉异常起源于肺动脉临床诊治分析

目的:分析左冠状动脉异常起源于肺动脉(ALCAPA)患者的临床特点、诊断治疗方法、手术效果及远期随访,提高临床医师对此类疾病的诊断治疗水平。方法:回顾性分析1993年10月至2019年10月北京安贞医院小儿心脏中心诊治的81例左冠状动脉异常起源于肺动脉患者的临床及随访资料,对比婴儿型和成人型的临床特点和治疗预后。结果:本组81例患者按年龄分为婴儿型(发病年龄≤12月,40例)和成人型(发病年龄12个月,41例)。65例患者行外科手术治疗,围术期死亡10例(15.4%)。手术出院后随访部分患者心电图异常Q波逐渐消失,左心室扩大及左心功能均明显改善,二尖瓣反流较术前减轻。结论:根据婴儿型和成人型ALCAPA临床表现,结合心电图、超声心动图、心脏CT及造影可明确诊断,外科手术重建双冠状动脉循环可有效改善心脏的结构和功能,术后远期预后良好。     

婴儿期左冠状动脉异常起源于肺动脉外科术后随访研究

目的:探讨婴儿期左冠状动脉异常起源于肺动脉(ALCAPA)患儿经外科矫治后心功能恢复及临床预后情况。方法:回顾性分析首都医科大学附属北京儿童医院从2007年6月至2018年8月间,经外科手术治疗的35例ALCAPA患儿超声影像及临床资料。分为两组:≤1周岁的婴儿组,1周岁的非婴儿组。所有患儿均行术前、术后6个月超声心动图检查及术后长期电话随访。结果:婴儿组22例,其中2例(2/22,9.1%)于术后围手术期死亡。非婴儿组13例,无术后死亡。术前两组患儿相比,婴儿组左心室舒张末期内径指数(LVEDDi)较大,LVEF较低,二尖瓣中量以上反流和二尖瓣腱索及乳头肌回声增强较多(P0.05)。婴儿组术后6个月与术前相比LVEDDi减小,LVEF增高,二尖瓣反流减少,二尖瓣腱索及乳头肌回声增强减少,室壁运动异常减少(P0.05)。术后6个月两组患儿相比,婴儿组LVEDDi较大(P0.05)。两组患儿出院平均电话随访时间5年,无术后再入院及相关心肌缺血事件。结论:婴儿期ALCAPA患儿病情较重手术风险高,但及时手术后近期心功能明显改善。ALCAPA患儿术后5年预后较好。     

超声心动图在左冠状动脉异常起源于肺动脉诊断中的临床应用价值及术后随访研究

目的:归纳总结左冠状动脉异常起源于肺动脉(ALCAPA)的超声心动图诊断、鉴别要点,分析婴儿型、成人型ALCAPA的直接及间接超声心动图征象,分析ALCAPA外科手术后短期及中远期超声心动图随访结果。方法:回顾性分析2009年8月至2018年2月阜外医院经超声心动图诊断ALCAPA并接受外科手术治疗的患者资料,总结患者的超声心动图典型征象,并在术后6个月内(近期)及术后12～60个月(中远期,中位随访时间31个月)分别对患者进行超声心动图随访。结果:经胸超声心动图诊断ALCAPA共73例,包括31例婴儿型ALCAPA,42例成人型ALCAPA。与婴儿型ALCAPA患者相比,术前成人型ALCAPA患者的右冠状动脉内径较宽,右冠状动脉/主动脉根部内径比值较大,经体表面积校正后的左心室舒张末期内径(LVEDD)较小,左心室射血分数(LVEF)较高,差异均有统计学意义(P均0.05)。术后近期随访发现,婴儿型ALCAPA患者LVEF较术前明显提高,但仍低于成人型ALCAPA患者(P0.05);两型患者LVEDD较术前均明显缩小,但两者间的差异无统计学意义(P0.05)。术后中远期随访显示,婴儿型ALCAPA患者的LVEF进一步明显提高,与成人型ALCAPA患者差异无统计学意义(P0.05);两型ALCAPA患者LVEDD均未进一步明显改善。两型ALCAPA患者在术前、术后短期及中远期随访中二尖瓣形态及二尖瓣反流量上的差异均无统计学意义(P均0.05)。结论:超声心动图对ALCAPA诊断、鉴别诊断、分型及术后随访中均具有重要的临床价值,对及时、有效诊断疾病并制定手术治疗策略具有指导意义。     

二尖瓣成形环瓣环环缩术在缺血性二尖瓣返流中的应用

目的 研究应用二尖瓣成形环行瓣环环缩术治疗缺血性二尖瓣返流的手术效果。方法 选择2000年1月~2015年12月在我院行二尖瓣成形术的缺血性二尖瓣返流并发室壁瘤的患者72例,根据二尖瓣病变部位及性质选择成形方案,其中33例使用二尖瓣成形环进行瓣环环缩术。回顾性分析手术的近期、远期治疗效果。结果 围手术期死亡4例（6%）。术后二尖瓣返流程度较术前明显改善,中度及中度以上二尖瓣返流1例（1%）。术后随访(5±3)年,随访期死亡11例（16%）,出现中度及中度以上二尖瓣返流12例（18%）。是否使用成形环进行瓣环环缩术后早期二尖瓣返流程度无统计学差异,但远期成形环环缩组较对照组二尖瓣返流程度有显著改善（P<0.05）。结论 缺血性二尖瓣返流并发室壁瘤的患者中,二尖瓣成形术可安全、有效地重建二尖瓣功能。使用成形环进行瓣环环缩可改善远期效果。     

一期大动脉调转术治疗完全性大动脉转位

目的:探讨一期大动脉调转术治疗完全性大动脉转位(TGA)的手术适应证及手术效果.方法:2006-05-2007-08对15例患儿[年龄0.5～14(2.51±0.76)个月,体重3～9(3.73±0.86)kg]实施一期大动脉调转手术.15例中室间隔完整TGA(TGA/IVS)13例,均并发房间隔缺损(ASD),同时并发动脉导管未闭(PDA)11例;TGA 并发室间隔缺损(TGA/VSD)2例,均同时并发PDA和ASD.12例并发中度肺动脉高压,3例并发重度肺动脉高压,术前均使用前列腺素E1.手术在全麻、中低温、低流量体外循环下完成.在2大动脉瓣上方横断,将左、右冠状动脉开口移植至肺动脉近端.经肺动脉分叉下方,吻合肺动脉近端和升主动脉远端.用自体心包修复主动脉近端缺失部分,并在新主动脉开放后吻合主动脉近端和肺动脉远端.结果:3例TGA/IVS患儿术后肺部感染,2例(分别为13个月、3个月龄)患儿术后12 h内突发心室颤动死亡,病死率为13.3%.未发生与冠状动脉移植相关并发症.术前肺动脉高压患儿,特别是重度肺动脉高压,术后肺动脉压力均明显下降.13例术后顺利恢复出院.随诊3～12个月,全部患儿心功能恢复良好,无远期并发症和死亡.结论:一期大动脉调转手术对于TGA有较好早期效果.对于年龄超过4个月部分患儿,即使并发肺动脉压,仍可取得满意治疗效果.     

体外膜肺氧合治疗左冠状动脉异常起源于肺动脉患儿术后休克一例及文献复习

【摘要】 目的 报告1例左冠状动脉起源于肺动脉(ALCAPA)患儿心脏术后休克(PCS)使用体外膜肺氧合(ECMO)治疗成功的案例及文献复习。方法 对2017年上海市儿童医院心胸外科收治的1例诊断为ALCAPA并行左冠状动脉异常起源矫治术及ECMO治疗的患儿的临床资料进行分析，并文献回顾及前景展望。结果 4个月大男孩，因“生长发育迟缓4个月，咳嗽4天”入院，入院后予以强心，利尿，控制肺部炎症等治疗，完善心脏超声及CTA，确诊为左冠状动脉异常起源于肺动脉，在体外循环下行“左冠状动脉异常起源矫治术”，术后出现反复室颤，心脏衰竭，休克，无法撤离体外循环，遂行ECMO治疗(左心耳及右心耳插管引流；升主动脉插管灌注)。生命体征平稳后逐渐下调ECMO流量，于术后第8天撤离ECMO (LVEF 37%)，术后第29天出院(LVEF 50%)。结论 尽早发现并及时手术处理对ALCAPA患者的预后至关重要。近年来国内ECMO用于治疗儿童心脏术后休克呈增加趋势，ECMO治疗成功的关键是早期实施，小儿多学科协作的ECMO管控模式有助于总体成功率的提高。     

左冠状动脉异常起源于肺动脉的超声心动图诊断价值

目的:探讨左冠状动脉异常起源于肺动脉(ALCAPA)的超声心动图诊断价值。方法:以CTA和手术证实的16例ALCAPA患儿为研究对象,回顾分析其超声心动图表现特征。结果:16例中,超声首诊11例,误诊5例,5例均误诊为心内膜弹力纤维增生症(EFE),复诊检出3例,余2例最后经CTA检出,超声首诊正确率69%。其主要超声表现为:左冠状动脉主干异常开口于肺动脉;右冠状动脉扩张;左心室扩大;心内膜、二尖瓣腱索及乳头肌及回声增强。彩色多普勒显示左冠状动脉内血流为逆向灌注,肺动脉内舒张期见异常血流进入;室间隔可见异常交通循环血流信号。结论:ALCAPA具有特征性超声心动图表现,超声是早期诊断的重要手段。     

Repair of anomalous origin of left coronary artery from the pulmonary artery

Patients with anomalous origin of the left coronary artery from the pulmonary artery often have mitral valve regurgitation. Although establishing dual coronary circulation is the procedure of choice, there remains controversy as to how the mitral valve is handled. Between April 1999 and August 2005, 8 patients underwent surgical correction at our institution. There were 4 males and 4 females, aged from 9 months to 13 years (mean, 6.4 years). Six patients underwent direct aortic reimplantation and 2 had a Takeuchi procedure. Simultaneous mitral annuloplasty was performed in 7 patients with moderate or severe mitral regurgitation. There were no deaths or postoperative complications. Follow-up ranged from 4 to 80 months (mean, 34 +/- 26 months). Left ventricular function improved significantly from a preoperative fractional shortening of 0.21 +/- 0.09 to 0.35 +/- 0.06. Mitral regurgitation decreased on follow-up in the 7 patients who had mitral annuloplasty. We recommend performing mitral annuloplasty at the time of operation in patients with moderate or severe mitral regurgitation and anomalous origin of the left coronary artery from the pulmonary artery.     

Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery: Diagnoses and Surgical Results in 12 Pediatric Patients

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital condition. It responds well to early diagnosis and treatment, but otherwise the prognosis is poor. We present our case series of 12 patients (mean age, 2 ± 2.58 yr; age range, 2 mo–8 yr), emphasizing the diagnostic process and discussing our surgical results. The diagnosis of ALCAPA should be suspected in infants who have dilated cardiomyopathy with electrocardiographic changes that suggest ischemia, and in older children who have isolated mitral regurgitation. When clinical suspicion is high, the results of 2-dimensional echocardiography combined with color-flow Doppler studies in expert hands can establish the diagnosis, thus avoiding angiography in critically ill infants. The treatment of choice in our patients was transfer and reimplantation of the left coronary artery onto the ascending aorta. There were 2 deaths: both were infants in extremis who underwent emergency surgery. An older child with severe ventricular dysfunction was given mechanical ventricular assistance and then heart transplantation. As of this report, all 10 survivors remained well and asymptomatic.     

A rare case of anomalous origin of the left anterior descending artery from the pulmonary artery

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart disease, with an incidence of <1 in 300 000 live births. We describe a rare case of a 23‐month‐old infant admitted for cough and fever. Echocardiography showed a branch of the left coronary artery originating from the pulmonary artery. The coronary computed tomographic angiography revealed anomalous origin of the left anterior descending (LAD) artery from the pulmonary artery with the left circumflex artery and right coronary artery arising normally from the aorta. The infant successfully underwent surgical reimplantation of the LAD to the ascending aorta. Our case emphasizes that echocardiography and computed tomographic angiography are valuable imaging modalities for making an accurate diagnosis and determining the precise surgical plan.     

The Management of the Older Adult Patient with Anomalous Left Coronary Artery from the Pulmonary Artery Syndrome: A Presentation of Two Cases and Review of the Literature

ALCAPA (anomalous left coronary artery from pulmonary artery) syndrome is a rare congenital abnormality that involves an anomalous insertion of the left coronary artery into the pulmonary artery. Ninety percent of patients present in the first year of life with signs and symptoms of heart failure or sudden cardiac death secondary to chronic myocardial ischemia. There have been an increasing number of reports of ALCAPA patients surviving to adulthood. There seems, however, to be a tendency to die suddenly in the third decade of life. Adult survivors are either asymptomatic or present with mitral regurgitation, cardiomyopathy, myocardial ischemia, or malignant arrhythmias. The management of the older patient presenting with symptoms resulting from ischemia and progressive left ventricular dysfunction remains a challenge. Treatment is largely based on guidelines for adult congenital heart disease management and an extrapolation of evidence from heart failure practice. Currently, surgical reimplantation of the anomalous coronary onto the aorta is the mainstay of treatment. The management of heart failure, sudden cardiac death, and ventricular arrhythmia present problems that are not addressed by reimplantation of the anomalous vessel alone. In this report, we present two cases with different modes of presentation and discuss treatment options.     

Surgery and critical care for anomalous coronary artery from the pulmonary artery

Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital cardiac malformation that accounts for 0.25-0.50% of children with congenital cardiac disease and can cause myocardial dysfunction in young infants. In any infant presenting with ventricular dysfunction, the diagnosis of anomalous origin of the left coronary artery from the pulmonary artery must be suspected and the origin of the coronary arteries must be confirmed. The diagnosis of anomalous origin of the left coronary artery from the pulmonary artery is an indication for surgical repair. A two-coronary arterial system is the goal and is almost always achievable. The goal of surgical therapy is the creation of a two-coronary arterial system, which appears to provide better long-term survival and protection from left ventricular dysfunction and mitral valvar regurgitation than does simple ligation of the anomalous coronary artery. Direct reimplantation of the anomalous coronary artery is the procedure of choice. It is straightforward and borrows from well-practised techniques commonly used in other procedures such as the arterial switch operation. For the rare patient in whom direct reimplantation is not possible, strategies to lengthen the anomalous coronary artery, or baffle it within the pulmonary root, are available. Mitral valvar regurgitation is common at presentation, but following the establishment of a two-coronary arterial system and satisfactory myocardial perfusion, regurgitation of the mitral valve resolves in the vast majority. Therefore, mitral valvuloplasty at the time of initial surgery for anomalous origin of the left coronary artery from the pulmonary artery is not indicated. Post-operative care requires careful manipulation of inotropic support and reduction of afterload. Mechanical support, with either extracorporeal membrane oxygenation or left ventricular assist device, should be available for use if necessary.     

Resynchronization and Strain Recovery Following Reimplantation of Anomalous Coronary Artery: A Tissue Doppler and Three-dimensional Echocardiographic Study

Resynchronization may play a significant role in recovery following surgical reimplantation of anomalous left coronary artery from the pulmonary artery (ALCAPA). Three-dimensional echocardiography and tissue Doppler may quantify this recovery. A 6-week-old infant presented with signs of congestive heart failure due to ALCAPA. Two-dimensional echocardiography showed a severely dilated left ventricular (LV) cardiomyopathy and the presence of ALCAPA was confirmed by catheterization. Three-dimensional echocardiography and tissue Doppler imaging showed severe abnormalities of systolic and diastolic synchrony and decreased contractility with a left ventricular ejection fraction (LVEF) of 13%. The infant underwent surgical coronary reimplantation and was discharged 5 weeks later with an LVEF of 54%. Serial quantitative assessment showed resynchronization and normalization of global LV function following reimplantation. However, segmental contractility improved significantly but remained depressed at discharge. The immediate recovery observed following reimplantation of anomalous coronary may be largely due to resynchronization.     

Anomalous Origin of Left Coronary Artery From Pulmonary Artery: Recovery of Left Ventricular Function After Dual Coronary Repair

Objectives. We reviewed our institutional experience with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) after dual coronary repair to assess preoperative variables predictive of outcome, the time course for postoperative recovery of cardiac function, the short- and long-term complications and our experience with left ventricular assist devices (LVAD) in these patients.Background. Outcome after surgical repair of ALCAPA remains incompletely defined.Methods. The surgical records and echocardiograms of 42 patients were reviewed. Left ventricular function was assessed by fractional shortening z-score (FSz) and stress-velocity index.Results. The overall survival rate was 86%. All six patients who died were <1 year old and died within 3 days of the operation. More severe preoperative mitral regurgitation (MR) was associated with increased mortality, but age, body surface area, preoperative FSz and end-diastolic dimension were not. We used an LVAD for 7 of 28 patients who underwent repair for ALCAPA since its introduction at our institution, with a survival of 5 of 7 patients. The degree of MR improved in 62% of patients and remained unchanged in 38%. Complications included supravalvar pulmonary stenosis (16 of 21 patients) and baffle leaks (11 of 21 patients) with the intrapulmonary baffling technique. Supravalvar pulmonary stenosis developed in 1 of 11 patients after direct coronary reimplantation. Left ventricular function became normalized in all 28 patients with follow-up past 1 year, regardless of preoperative FSz. Of 13 patients who underwent serial postoperative echocardiography, the average time to normalization of function was 2 to 7 months.Conclusions. The degree of preoperative MR was predictive of outcome, whereas the severity of preoperative cardiac dysfunction and ventricular dilation were not. Mild and moderate MR tended to improve without mitral valvuloplasty. Complete recovery from myocardial dysfunction is expected after dual coronary repair of ALCAPA.     

Successful intraoperative identification of an anomalous origin of the left coronary artery from the pulmonary artery using real time three-dimensional transesophageal echocardiography

Anomalous origin of the left coronary artery (LCA) from the pulmonary artery (ALCAPA) is a rare congenital defect that presents only infrequently in adults. An adult diagnosed with ALCAPA, heart failure, and mitral regurgitation underwent surgical ligation of the anomalous origin of the LCA from the pulmonary artery (PA) and coronary artery bypass grafting (CABG). The anomalous origin in the PA and proximal segment of the left anterior descending artery (LAD) was successfully delineated via real time, three-dimensional transesophageal echocardiography during surgery. This modality allows for fast assessment and novel views of complex cardiac abnormalities and can aid in perioperative monitoring.