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.     

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.     

Successful operation in an old survivor of anomalous origin of the left coronary artery from the pulmonary trunk (Bland-White-Garland syndrome).

A case of anomalous origin of the left coronary artery from the pulmonary trunk is reported. The patient, a 64-year-old woman, presented with a history of angina and cardiac failure. She was known to have had a mitral systolic murmur since school age. Echocardiography showed clinically significant mitral regurgitation and highly unusual extensive calcification of the mitral valve chordae, papillary muscle, and posterior left ventricular wall--a pattern suggesting the possibility of abnormal coronary circulation. Subsequent cardiac catheterisation confirmed considerable mitral regurgitation with a dilated left ventricle, and arteriography confirmed anomalous origin of the left coronary artery from the main pulmonary trunk. The patient was surgically treated with ligation of the origin of the anomalous left coronary and mitral valve replacement. She was alive and well 2 years after operation.     

成人冠状动脉异常起源于肺动脉的临床分析

目的:探讨成人冠状动脉异常起源于肺动脉的临床特点、诊断方法、治疗及意义。方法:北京安贞医院自2004年1月至2012年12月,共收治12例成人冠状动脉起源异常患者,对其临床资料、治疗情况及随访结果进行回顾性分析。结果:全组男性2例,女性10例,年龄16~66岁,平均年龄为(39.2±17.9)岁。其中10例为左冠状动脉起源于肺动脉,2例为右冠状动脉起源于肺动脉。患者临床表现缺乏特异性,在确诊前均误诊为其他疾病。全组均经冠状动脉造影,心脏超声心动图(UCG),冠状动脉CTA等检查而确定诊断。其中9例患者行外科手术重建冠状动脉,2例患者拒绝手术,1例因严重心功能不全而失去手术时机。手术患者中2例患者因合并重度二尖瓣反流而同时行二尖瓣置换术,1例患者因合并冠心病同时行冠状动脉旁路移植术。无围手术期死亡病例及术后并发症。随访6~48个月,平均随访(27±13)个月,患者术后均症状消失或明显缓解,一般体力活动不受限。UCG提示:术后左心室舒张末径从[(47.4±7.2)mm减小到(41.8±6.5)mm,P0.01],左心室的短轴缩短率(FS)从[(24.3±11.3)%增至(36.1±11.6)%,P0.01)]。未手术治疗患者1例死于心力衰竭。1例失访,1例仍时有活动后气短、心慌等症状。结论:成人冠状动脉异常起源于肺动脉无特征性临床表现,除了冠状动脉造影,UCG和冠状动脉CT等无创检查对确定诊断具有重要意义。手术治疗重建双冠状动脉系统是目前首选的治疗方案,可以改善心脏血供,从而防止猝死、心功能不全等严重心脏事件的潜在危险。     

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。     

Anomalous origin of left coronary artery from pulmonary artery in adults

Various techniques have been described for management of anomalous origin of the left coronary artery from the pulmonary artery presenting in adults. Three patients, 1 male and 2 females, aged 27-37 years, underwent transpulmonary pericardial patch closure with concomitant left internal thoracic artery anastomosis to the left anterior descending artery, under standard cardiopulmonary bypass, thus creating a two-coronary system. One patient had concomitant mitral valve repair. All 3 survived the operation. Postoperative angiography in 2 patients revealed good antegrade flow with decreased collaterals in one and competitive inhibition with increased collaterals in the other. This procedure is considered to be the safest and simplest in this subset of patients.     

6例左冠状动脉起源于肺动脉外科治疗体会

目的 :总结左冠状动脉起源于肺动脉外科治疗经验。方法 :1993年 10月至 2 0 0 0年 1月 ,我院共收治左冠状动脉起源于肺动脉患者 6例 ,年龄最大 16岁 ,最小 3个月 ,平均 4 4岁。 4例采用肺动脉内隧道法重建左冠状动脉与主动脉连接 ,1例行左冠状动脉移植术 ,1例行单纯左冠状动脉结扎术。结果 :手术死亡 1例 ,死亡率 16 7%。随访共 16 4个月 ,平均 32 8月 人 ,无远期死亡 ,术后心功能明显改善。结论 :肺动脉内隧道法重建左冠状动脉手术方法简单 ,疗效满意。     

Severe mitral insufficiency in early infancy Associated with anomalous origin of the left coronary artery from the pulmonary artery

The clinical course, diagnostic cineangiocardiographic data and the postmortem findings of an infant with an anomalous left coronary artery arising from the pulmonary artery are presented. Mitral insufficiency and congestive heart failure were present before age 6 weeks, and severe myocardial dysfunction was documented by volume studies showing an elevated left ventricular end-diastolic volume and a depressed ejected fraction (0.36). Despite ligation of the anomalous vessel, the infant died. Postmortem injection of the coronary system with contrast media showed extensive collateral circulation between the right and left coronary arteries. The origin of the anomalous left coronary artery from the distal main pulmonary artery was a distinctly unusual finding. Both the left ventricular myocardium and the mitral valve showed extensive thickening, fibrosis and calcification. Associated mitral insufficiency may contribute significantly not only to the morbidity but also to the mortality in infants with an anomalous left coronary artery.     

Anomalous origin of the left coronary artery from the right pulmonary artery: Report of a case

Summary A 1-year-old boy was referred to our hospital for further investigation of mitral regurgitation. Color-Doppler echocardiography revealed the site of the anomalous origin of the left coronary artery which was confirmed by a cineangiographic study. This case is interesting for several reasons: the first is that the anomalous left coronary artery originated from the right pulmonary artery; the second is that two-dimensional echocardiographic findings can be misleading, i.e., a vessel-like structure which seemed to be the left coronary artery arising normally from the aorta was visualized; and the last is that a two-dimensional color-Doppler echocardiogram was useful in the diagnosis of the anomalous origin of the left coronary artery. The patient underwent implantation of the anomalous left coronary artery as well as Kay's plasty of the mitral valve, and has been doing well since then.     

Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery in Alagille Syndrome

Alagille syndrome is a dominantly inherited multisystem disorder involving multiple organs including the liver, heart, eyes, face, and skeleton. Congenital heart defects, the majority of which are right-sided, contribute significantly to the mortality of these patients. We report a patient with Alagille syndrome who presented with mitral valve regurgitation requiring valvuloplasty and subsequent mitral valve replacement. The patient was ultimately diagnosed with anomalous origin of the left coronary artery from the pulmonary artery and underwent successful reimplantation.     

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。     

Emergent coronary angioplasty in the treatment of acute ischemic mitral regurgitation: long-term results in five cases

Severe mitral regurgitation in the setting of an evolving myocardial infarction is associated with a high operative mortality rate. Five patients with acute severe mitral regurgitation secondary to ischemic posterior papillary muscle dysfunction underwent emergent percutaneous transluminal coronary angioplasty. Two patients were in cardiogenic shock and required intraaortic balloon counterpulsation. Angioplasty resulted in rapid improvement in hemodynamic variables, and all patients were discharged at a mean of 10 days after the procedure. Long-term follow-up study (mean 35 +/- 6 months) revealed normal mitral valve function angiographically and by Doppler echocardiography in four patients. Repeat angioplasty was required in one patient, and another underwent coronary artery bypass surgery without valve replacement for restenosis. One patient developed progressive mitral regurgitation and required elective mitral valve replacement 12 months after angioplasty. These preliminary findings suggest that emergent coronary angioplasty is a useful therapeutic intervention in the treatment of ischemic mitral regurgitation and is associated with a favorable long-term outcome.     

Anomalous left coronary artery arising from the pulmonary artery in infancy: is early operation better?

From January 1975 to January 1987, 21 consecutive infants aged less than six months (mean (SD) 2.6 (1.2] were admitted with anomalous origin of the left coronary artery from the pulmonary artery. In the first 12 patients, who were seen up to 1982, operation was performed after the age of one year (mean (SD) 29 (29) months) (group 1). The next nine infants, seen from 1983 to 1987, had their operations within a few weeks of the onset of symptoms (mean (SD) age 4.8 (1.4) months) (group 2). In group 2 the left coronary artery was relocated into the aorta, whereas in group 1 there was additional resection of the left ventricular wall or mitral valvoplasty or both. At presentation there were no differences in age, clinical condition, heart enlargement, and echocardiographic left ventricular dysfunction between groups 1 and 2. Seven of the 12 patients in group 1 died, five while they were awaiting operation (three died suddenly at home) and two at operation. The five survivors are doing well 6.4 (3.1) years after operation with normal left ventricular function which improved slowly over several months after operation. Two of the nine patients in group 2 died; both deaths occurred at or soon after operation. The seven survivors are doing well 1.8 (0.9) years after operation. In three, left ventricular function recovered within three weeks; and there was even partial or total regression of the Q waves in the supposedly necrotic areas. In the remaining four the pattern of improvement in left ventricular function resembled that in group 1. Operation should be undertaken early in infants with anomalous left coronary artery arising from the pulmonary artery because the procedure is relatively safe, prevents a high natural mortality, and offers a better chance of a faster recovery of left ventricular function.     

Anomalous left coronary artery arising from the pulmonary artery in infancy: is early operation better?

From January 1975 to January 1987, 21 consecutive infants aged less than six months (mean (SD) 2.6 (1.2] were admitted with anomalous origin of the left coronary artery from the pulmonary artery. In the first 12 patients, who were seen up to 1982, operation was performed after the age of one year (mean (SD) 29 (29) months) (group 1). The next nine infants, seen from 1983 to 1987, had their operations within a few weeks of the onset of symptoms (mean (SD) age 4.8 (1.4) months) (group 2). In group 2 the left coronary artery was relocated into the aorta, whereas in group 1 there was additional resection of the left ventricular wall or mitral valvoplasty or both. At presentation there were no differences in age, clinical condition, heart enlargement, and echocardiographic left ventricular dysfunction between groups 1 and 2. Seven of the 12 patients in group 1 died, five while they were awaiting operation (three died suddenly at home) and two at operation. The five survivors are doing well 6.4 (3.1) years after operation with normal left ventricular function which improved slowly over several months after operation. Two of the nine patients in group 2 died; both deaths occurred at or soon after operation. The seven survivors are doing well 1.8 (0.9) years after operation. In three, left ventricular function recovered within three weeks; and there was even partial or total regression of the Q waves in the supposedly necrotic areas. In the remaining four the pattern of improvement in left ventricular function resembled that in group 1. Operation should be undertaken early in infants with anomalous left coronary artery arising from the pulmonary artery because the procedure is relatively safe, prevents a high natural mortality, and offers a better chance of a faster recovery of left ventricular function.     

Pulmonary hypertension alters natural history of anomalous left coronary artery

A girl who was diagnosed at 8 months old with anomalous left coronary artery from the pulmonary artery and was subsequently lost to follow-up, presented at the age of 11 years with congestive heart failure and severe mitral regurgitation with supra-systemic pulmonary artery pressure. With progressive mitral regurgitation, coronary steal is reduced by the rising pulmonary artery pressure, and patients present late with severe valvular cardiomyopathy.     

Imaging the elusive anomalous origin of left coronary artery from pulmonary artery

A 7‐year‐old girl with severe mitral regurgitation and pulmonary artery hypertension with good ventricular function was planned for mitral valve repair, but was subsequently diagnosed to have anomalous left coronary artery from pulmonary artery. We describe the pitfalls in the diagnosis of this rare anomaly, particularly in the presence of severe pulmonary artery hypertension and absence of circumstantial evidence of coronary steal and ventricular dysfunction.     

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.     

Bland-White-Garland syndrome in an adult

We report about a 47-year-old woman, who presented with a history of cardiac failure. Echocardiography showed an impaired left ventricular function, clinically significant mitral regurgitation and pulmonary hypertension. Diagnosis of a Bland- White-Garland syndrome was made by coronary angiography. Subsequent therapy consisted of ligation of the anomalus origin of the left coronary artery, implantation of a Mammaria interna graft to the left coronary artery and replacement of the mitral valve by a mechanical prosthesis. One year after operation, left ventricular function was still impaired. At a 3-year follow-up, left ventricular function improved continuously.     

An alternative technique for transfer of anomalous left coronary artery from the pulmonary trunk in children and adults using autogenous aortic and pulmonary arterial flaps

BACKGROUND: Direct re-implantation of an anomalous left coronary artery into the aorta is the preferred surgical option for creating a dual coronary arterial system in patients in whom the anomalous artery originated from the pulmonary trunk. This technique, however, is applicable only when the anomalous artery arises from the right posterior pulmonary sinus. We report a new technique for re-implantation using combined autogenous aortic and pulmonary arterial flaps in situations when a direct connection was not possible. PATIENTS AND METHODS: We have treated 4 patients, aged 3 months, 6 months, 18 months, and 27 years respectively, who presented with anomalous origin of the left coronary artery from the left posterior pulmonary sinus. We used our proposed technique for transfer because lack of coronary arterial length, diminished vessel elasticity, and extensive collaterals around the pulmonary sinuses prevented direct attachment. RESULTS: There was no early or late death. Postoperatively, all patients are in functional class I, with good biventricular function at a median follow-up of 74 months, with a range from 9 to 96 months. Postoperative coronary angiography in our 4th patient showed good arterial flow, without any distortion. CONCLUSIONS: The potential benefits of this modification of the trapdoor technique are excellent operative exposure, use of autogenous and viable tissue capable of further growth, avoidance of injury to the aortic and pulmonary valvar apparatus and production of obstruction within the right ventricular outflow tract, complete elimination of use of pericardium for augmentation of the neo-aortic tube, achievement of the anastomosis with correct angling and length, and the possibility of implantation in all patients, including adults, regardless of the distance from the aorta or the coronary arterial configuration.