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。     

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.     

Anomalous Left Coronary Artery from the Pulmonary Artery with a Large Patent Ductus Arteriosus: Aversion of a Catastrophe

We present an infant who had an anomalous left coronary artery arising from the pulmonary artery (ALCAPA) and a large patent ductus arteriosus (PDA), who was diagnosed before a potentially catastrophic closure of PDA. In the presence of normal left ventricular function and the absence of coronary artery collaterals, it is difficult to diagnose ALCAPA. A disproportionate degree of left ventriclular dilation and severity of mitral valve regurgitation relative to the degree of PDA shunt, and echogenic papillary muscles on an echocardiogram should raise a suspicion of coronary artery anomalies. The infant underwent surgical ligation of PDA with translocation of coronary arteries and had an uneventful recovery.     

Pulmonary artery origin of the left coronary artery: diagnosis by transoesophageal echocardiography in infancy

In two infants the anomalous origin of the left coronary arteryfrom the pulmonary artery (ALCAPA) was detected prospectivelyby transoesophageal echocardiography (TEE). Colour flow mappingand pulsed Doppler ultrasound revealed a predominant right-to-leftshunt from the pulmonary artery to the left coronary artery.These findings were confirmed by angiography. Transihoracic echocardiography (TTE)failed to show the anomalousorigin of the left coronary artery or any abnormal pulmonaryflow pattern. TEE may be useful in the diagnosis of ALCAPA in selected cases,when TTE is inconclusive.     

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.     

Echocardiographic diagnosis of anomalous origin of the left coronary artery from the pulmonary artery

The objective of this study was to analyze echocardiographic characteristics of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) and to explore the diagnostic value of transthoracic echocardiography. The echocardiographic characteristics of 8 patients hospitalized with ALCAPA from 2000 to 2005 were analyzed retrospectively, including the results of real time three-dimensional echocardiography in one case, and compared with angiographic results. Eight cases included 6 older type patients and 2 infant type patients. Echocardiography showed abnormal vessel inserting into pulmonary artery (PA), continuous shunt into PA and intercoronary collateral signals within the ventricular septum in all cases and bifurcate structure of the abnormal vessel with retrograde filling in 4 cases. The morphological and functional changes and valvular regurgitation induced by insufficient myocardial perfusion were also evaluated. In former 4 patients, 2 cases were misdiagnosed as right coronary artery-PA fistula and the other 2 cases were given an uncertain diagnosis of anomalous origin of the coronary artery because of the visualization of the echo-free linear structure which apparently arose from the aorta resembling a normal left coronary artery. The latter 4 patients were correctly diagnosed by excluding the aforementioned interference. The diagnosis of ALCAPA was confirmed by angiocardiography in all patients and by intraoperative findings in 4 patients. Based on the apprehension of ultrasonic features and the enhancement of diagnostic alertness, the echocardiography can evaluate ALCAPA accurately and give more information than angiography. It may be the first diagnostic choice.     

Imaging characteristics of anomalous left coronary artery from the pulmonary artery

SUMMARY: The authors report the imaging characteristics of the anomalous origin of the left coronary artery from the pulmonary artery in a 6 1/2-month-old male infant using electrocardiography, echocardiography, angiography, and electron-beam computed tomography. The patient presented with tachycardia, prolonged and interrupted feeding, failure to thrive, and myocardial infarction, and after diagnosis of the anomalous origin of the left coronary artery, survived the reimplantation of the left coronary artery directly to the ascending aorta. When reviewed 7 months after surgery, echocardiography showed improved left ventricular function and surface electrocardiography showed regression of pathologic Q waves in leads I, V 4, V 5, and V 6.     

Respiratory Syncytial Viral Infection in an Infant with Unrepaired Anomalous Left Coronary Artery from the Pulmonary Artery

Abnormal origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare coronary anomaly in children that requires necessary and urgent repair. We report a child who was hospitalized with respiratory failure due respiratory syncytial viral (RSV) infection and was subsequently diagnosed with ALCAPA. Aggressive treatment for RSV included synagis and nebulized ribavirin prior to surgical repair. After waiting 4 weeks for the RSV infection to resolve, she underwent successful left coronary artery reimplantation on hospital day 27 and has regained normal left ventricular size and function.     

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.     

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。     

Value of coronary blood flow pattern as a predictor of functional recovery and short-term left ventricular remodeling after primary coronary angioplasty. A transthoracic Doppler study

INTRODUCTION AND OBJECTIVES: Coronary blood flow measurement using a Doppler guidewire is the most sensitive way of detecting the no-reflow phenomenon following reperfusion of a myocardial infarction (MI). New high-frequency Doppler probes enable coronary blood flow velocity to be measured noninvasively. Our aims were to study the different patterns of left anterior coronary artery blood flow observed by transthoracic Doppler echocardiography, and to describe their association with functional recovery following reperfusion of an anterior MI. METHODS: The study included 57 patients with a mean age of 60 years (range 30-85 years). An abnormal coronary blo:d flow pattern was defined as one in which there was a high peak diastolic velocity and a short deceleration time (i.e., < or = 500 ms). We compared the regional contractility, ventricular volumes, and left ventricular ejection fraction (LVEF) measured after 72 hours with those measured 1 month after MI. RESULTS: Overall, 31 patients (54%) had a normal coronary blood flow pattern (Group 1) and 26 (46%), an abnormal pattern (Group 2). After one month, regional contractility improved in Group-1 patients, as did LVEF, from 46.8 (8.6) to 52.6 (8.8)% (P=.002). In these patients, left ventricular volumes were unchanged. In contrast, regional contractility and LVEF remained unchanged in Group-2 patients whereas ventricular volumes increased, from 55.8 (12.9) to 62.9 (16.8) ml/m2 (P=.05), and from 32.2 (9.5) to 37.1 (14.9) ml/m2 (P< .05). Coronary blood flow pattern was the most important independent predictor of left ventricular remodeling, odds ratio =6.14 (95% CI, 1.56-24.17). CONCLUSIONS: Transthoracic Doppler echocardiographic assessment of coronary blood flow following reperfusion of an anterior myocardial infarction can be used to identify patients with microvascular damage who are progressing towards ventricular dilatation without recovery of myocardial function.     

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.     

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.     

Late intervention in an asymptomatic pediatric patient with anomalous left coronary artery

Anomalous left coronary artery from the pulmonary artery(ALCAPA) is most commonly diagnosed within the first year of life with congestive heart failure symptomatology reflecting left ventricle(LV) dysfunction. The late diagnosis of ALCAPA is presented in a 5-yearold without significant LV dysfunction, mild LV dilatation and only mild mitral regurgitation that did not change significantly after surgery. The timing of surgical intervention in the late diagnosis of ALCAPA remains unclear despite risks of significant ongoing myocardial injury secondary to coronary artery hypoperfusion and progressive mitral valve dysfunction. Intervention in this case allows for revascularization which may reverse ventricular and valvular dysfunction.     

婴儿左冠状动脉异常起源于肺动脉的诊疗分析

目的 探讨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）的临床特点、诊断方法、外科治疗及疗效分析。方法 2006年6月~2015年1月阜外医院诊断为ALCAPA并行手术治疗患儿29例，对这些患儿的临床资料、诊断与治疗情况及随访结果进行回顾性分析。随访结果根据包括复查结果及电话随访。结果 本组患者男性16例，女性13例，年龄2月~1（0.75±0.27）岁，体质量4.8~11.5（8.0±2.2）kg，左心室射血分数18~66(42±18)%。患儿均出现临床心功能不全症状，并通过心电图、心脏彩超及64排螺旋CT检查以明确诊断。并发二尖瓣轻度反流10例，中度反流5例，重度反流4例。手术中将左冠状动脉从肺动脉游离后，直接将左冠状动脉移植到升主动脉15例，用部分肺动脉干和主动脉壁或心包片作左冠状动脉延伸与升主动脉吻合13例，应用肺动脉内通道术（Takeuchi方法）1例。9例并发二尖瓣中度及以上反流者同期作二尖瓣成形术。术毕3例行体外膜肺氧合(extracorporeal membrane oxygenation，ECMO)辅助左心系统。无手术早期死亡。术后随访资料24例（83%，24/29），随访1~80（22±20）个月，患儿术后症状均明显改善。结论 ALCAPA的患儿应早期诊断，及早手术治疗。冠状动脉直接移植术是目前重建双冠状动脉系统的首选方案， 外科治疗效果良好。     

Anomalous Left Coronary Artery from the Pulmonary Artery in a Preterm Infant: Presentation after Ligation of Ductus Arteriosus

Surgical or medical closure of a patent ductus arteriosus (PDA) is a very common practice in premature infants in the neonatal intensive care unit, but often the coronary arteries are not delineated prior to closure. In this report, a 32‐week gestational age premature infant who underwent surgical ligation of a PDA developed new‐onset left ventricular systolic dysfunction. A repeat echocardiogram noted an anomalous left coronary artery from the pulmonary artery. Successful left coronary artery reimplantation with rapid normalization of left ventricular systolic function occurred. As previously not reported, ligation of a PDA in a premature infant can unmask anomalous left coronary artery from the pulmonary artery. Surgical left coronary artery reimplantation is the mainstay of therapy and should be performed to establish normal coronary blood flow, even in a premature, low birth weight infant.     

Improvement in left ventricular dysfunction after aortic reimplantation in 11 consecutive paediatric patients with anomalous origin of the left coronary artery from the pulmonary artery: Early results of a serial echocardiographic follow-up

To study the potential for recovery of left ventricular functionin patients with anomalous origin of the left coronary arteryfrom the pulmonary artery (ALCAPA) after aortic reimplantation,serial two-dimensional echocardiographic examinations were performedbefore and up to 9 months after operation in 11 consecutivepaediatric patients (group 1: six infants; group 2: five childrenabove the age of 1 year). End-diastolic volume (EDV), end-systolicvolume (ESV), stroke volume (SV), ejection fraction (EF), myocardialvolume (MV), ratio of myocardial volume!end-diastolic volume(MVI), and regional wall motion of the left ventricle (LV) werestudied. Pre-operativety, mean LVEDV was 339% of normal in group 1 and289% in group 2 (P<0.001); mean LVMV was about twice thenormal value in both groups (P<0.001); LVMVI was 0–79± 0–23 in group 1 and 0–83 ± 0–3in group 2; LVEF was 28 ± 10% in group 1 and 46 ±18% in group 2; regional wall motion was normal in two group2 patients, the other showed uniform reduction in segmentalshortening fraction. Postoperatively, mean LVEDV tended to become normal after 2weeks in group 1 and after 3 months in group 2. In both groupsmean LVEF reached the normal range after 3 months; LVMV as wellas LVMVI normalized after 9 months. Three months after the operation,all infants had a nearly normal pattern of regional wall motion,while in three group 2 children a residual reduced shorteningfraction could be observed in anterior or lateral segments. The study shows that (1) aortic reimplantation in patients withALCAPA results in progressive improvement left ventricular functioneven in patients with severely damaged myocardium. (2) For aquick and complete recovery of global and regional left ventricularfunction, the operation should be undertaken early in infancy.(3) Left ventricular dysfunction is probably associated with‘hibernating myocardium’, which may ‘wakeup’ after surgical repair.     

Pre- and post-operative echocardiographic evaluation of the anomalous origin of the left coronary artery from the pulmonary trunk

OBJECTIVE: Pre and postoperative Doppler echocardiographic assessment of the anomalous origin of the left coronary artery from the pulmonary trunk (OLCAPT). MATERIAL AND METHODS: We analyzed Doppler echocardiographic diagnostic signs, the potential for recovery of left ventricular (LV) function and mitral regurgitation (MR) in 4 patients with OL-CAPT, treated by direct aortic implantation. The age at surgery ranged from 1 to 10 years and the follow-up from 3 months to 6 years. Doppler echocardiographic diagnostic study was performed at the first examination whereas LV function and MR were studied before (3 to 8 days) and serially after surgery. RESULTS: The OLCAPT was visualized in 3 patients and the retrograde flow from the anomalous left coronary in 1. An important right coronary artery dilation was present in 2 patients, being moderate in a third patient. Before surgery, the LV shortening fraction ranged from 17 to 23% and the ejection fraction from 23 to 36%. The qualitative analysis of segmental LV wall motion showed septal akinesis and hypokinesis of the remaining segments. Nine to 12 months after surgery, global and regional indexes of LV function improved, reaching the normal range except for the interventricular septum. In 2 patients with more than 2 years of follow-up, complete normalization of LV function was obtained, as well as with the electrocardiographic features of an old myocardial infarction in one case and a marked decrease in Q wave depth in the other. A moderate to severe MR was detected in all patients which improved markedly after surgery in 3. In the other case, a mitral replacement was necessary. CONCLUSION: Doppler echocardiography allowed: 1. the anatomic diagnosis in 3 of 4 patients with OLCAPT; 2. to follow global and regional LV function and MR improvement after direct aortic implantation of the anomalous left coronary artery.     

Tissue Doppler echocardiography for evaluation of patients with ventricular resynchronization therapy.

INTRODUCTION AND OBJECTIVE: Ventricular resynchronization therapy optimizes cardiac function and induces reverse remodeling of the left ventricle (LV) in patients (pts) with dilated cardiomyopathy and intraventricular conduction disturbances. Improvement of LV mechanical synchrony seems to be the predominant mechanism. There is a growing interest in objective quantification of desynchronization. This study aims to evaluate the effect of ventricular resynchronization therapy on LV remodeling and on LV desynchronization, assessed by tissue Doppler echocardiography. PATIENTS: We studied ten pts, eight male, mean age 65 +/- 10 years, with dilated cardiomyopathy, intraventricular conduction disturbances and heart failure, New York Heart Association functional class III or IV. Five pts had coronary artery disease and the others idiopathic dilated cardiomyopathy. All pts had an implanted cardioverter, defibrillator with cardiac resynchronization therapy. The LV pacing electrode was placed in the lateral or posterolateral vein. METHODS: Before and one month after resynchronization therapy the following parameters were measured with conventional Doppler echocardiography: LV end-diastolic (LVd) and end-systolic (LVs) size, ejection fraction (EF) and mitral regurgitation (MR) area. For diastolic function the maximum velocity of the E wave (MV-E) and A wave (MV-A), E/A ratio, LV filling time (LV-FT) and isovolumetric relaxation time (IVRT) were meadured. Mitral longitudinal motion was studied with pulsed tissue Doppler. Maximum velocity of the systolic S wave (MV-S) and isovolumetric contraction time (IVCT) were measured in the tissue Doppler curve of the septum and lateral, inferior and anterior walls. To evaluate the degree of desynchronization the RV index was calculated for each patient, based on the difference between the maximum and minimum IVCT, normalized for the maximum IVCT. RESULTS: There was a significant reduction in LVd and MR. EF increased significantly (p = 0.003). There were no differences in diastolic function parameters. MV-S did not increase significantly. IVCT increased significantly at the lateral wall (p = 0.037). The RV index demonstrated a significant reduction in ventricular desynchronization (p = 0.001). CONCLUSIONS: Ventricular resynchronization therapy induces reverse remodeling and improves LV function in selected pts. Improvement of mechanical LV synchrony seems to be the predominant mechanism. Ventricular desynchronization can be measured by tissue Doppler echocardiography.