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.     

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.     

Ejection fraction in anomalous origin of the left coronary artery from the pulmonary artery

The ejection fraction of 10 patients with anomalous origin of the left coronary artery from the pulmonary artery has been studied. Five were seen at Albany Medical Center Hospital and 5 were collected from the literature. The 6 patients who died had an ejection fraction less than 0.36. The 4 survivors had an ejection fraction more than 0.55. It has been suggested that this measurement will help in the selection of patients for surgery and the follow-up of patients with this anomaly.     

Ultrasonic features of anomalous origin of the left coronary artery from the pulmonary artery

Two cases of anomalous origin of the left coronary artery from the pulmonary artery with and without marked dilatation of the right coronary artery were studied by M-mode echocardiography and cross-sectional echocardiography. The M-mode echocardiogram obtained from the case with the dilated right coronary artery demonstrated an abnormal structure anterior to the aortic root. The cross-sectional echocardiogram of the same case visualized the origin of the dilated right coronary artery. In addition, echocardiographic abnormalities suggesting the ischemia of the left ventricle were observed in both cases. Although many of the ultrasonic features are nonspecific, the combination of ultrasonic technique provides useful clues to the diagnosis of anomalous origin of the left coronary artery from the pulmonary artery.     

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.     

Management of symptomatic infants with anomalous origin of the left coronary artery from the pulmonary artery

There is disagreement concerning the optimal management of infants with congestive heart failure due to anomalous origin of the left coronary artery from the pulmonary artery. This report describes treatment in 23 such patients who had congestive heart failure before age 6 months. Eight patients had an operation in the 1st year of life and only two (25 percent) of these patients survived. Fifteen patients received initial medical treatment, and operation was delayed until an average age of 7.5 years; 13 (87 percent) of these patients survived. The two patients who survived early operation are still alive at an average age of 2.5 years. The 13 survivors in the medical group are still alive at an average age of 14.5 years. The age at onset and the severity of congestive heart failure were similar in the two groups. The frequency of the electrocardiographic pattern of myocardial infarction was similar in both groups. The radiographic cardiothoracic ratio was 0.74 in the surgical and 0.66 in the medical group. In 10 patients the left ventricular ejection fraction was less than 20 percent in the 1st year of life. In 7 of these 10 patients operation was performed before age 1 year and 2 (28 percent) survived; of the 3 patients treated medically, 1 (33 percent) survived.The outcome of surgical or medical treatment of the infant with anomalous origin of the left coronary artery from the pulmonary artery may be biased by patient selection. In the infants with depressed left ventricular function, the results of surgical and medical treatment are equally disappointing. Because coronary arterial surgery in the 1st year of life in infants with severely depressed myocardial function has an extremely high mortality rate (75 percent) and because many symptomatic infants with anomalous origin of the left coronary artery from the pulmonary artery can be treated medically and survive, we recommend delay of operative intervention until at least age 18 months.     

The noninvasive assessment of anomalous origin of the left coronary artery from the pulmonary artery

We present a patient with anomalous origin of the left main coronary artery from the pulmonary artery. We correlate the findings of echocardiography and myocardial imaging with angiography, and discuss the value of the noninvasive techniques in the diagnosis and in the followup of such patients.     

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

We found increased systolic coronary flow in transthoracic pulsed wave (PW) Doppler in a 42-year-old patient with anomalous origin of left main coronary artery from the pulmonary artery. This is a characteristic echocardiographic finding in this anomaly in the presence of collateral circulation and coronary L-R shunt. In comparison with so far used echocardiographic criteria this parameter when present allows quick recognition of anomalous origin of left coronary artery from the pulmonary artery, and its differentiation from other potentially lethal coronary anomalies.     

Early variation of left ventricular dimension and mitral regurgitation after correction for anomalous origin of the left coronary artery from the pulmonary artery

Background Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital cardiac lesion resulting in myocardial ischemia even infarction,morphological impairment and dysfunction of left ventricle,together with mitral regurgitation.Here we will introduce our experience in the surgical repair of this kind of congenital lesion and the retrospective analysis about the improvement of left ventricular dimension and mitral regurgitation in early postoperative term.Method From May 1998 to July 2012,38 consecutive patients with anomalous coronary artery from the pulmonary artery underwent surgical correction(33 received left coronary artery re-implantation,4 left coronary artery ligation or primary closure,1 Takeuchi procedure,and 10 simultaneous mitral valve plasty).Left ventricular dimension,mitral regurgitation,and ejection fraction,were measured by color Doppler echocardiography preoperatively,and 1 month after discharge.Results Hospital survival was 94.7%(2 in-hospital deaths).Ten paptients with more than moderate mitral regurgitation received simultaneous mitral plasty,one of whom was converted to mechanical prosthetic valve replacement.Mitral valve annuloplasty was applied in 9 cases of coronary re-implantation correction,3 of whom also received additional mitral leaflet cleft repair.Meanwhile 8 patients underwent other different concomitant operations.Echocardiographic results for the survivals 1 month after discharge showed that left ventricular end-diastolic,endsystolic dimension decreased from 40.05 ± 5.56 mm and 28.94 ± 6.21 mm to 33.07 ± 6.82 mm(P < 0.01) and 23.04 ± 5.87 mm(P < 0.01) respectively.The average mitral regurgitation grade was also reduced from 2.36 ± 1.08 to 1.64 ± 93(P < 0.05) in the group.All survival patients improved clinically and NYHA functional class decreased significantly from 2.37 ± 1.08 to 2.10 ± 0.54(P < 0.05).Conclusions The surgical repair of anomalous origin of the left coronary artery from the pulmonary artery is safe and effective,and can get satisfactory dimensional and functional improvement of the left ventricle in early term.Although controversial,concomitant mitral valve plasty can be helpful for critical patients with severe mitral regurgitation.     

小儿左冠状动脉起源于肺动脉临床分析

目的:总结小儿左冠状动脉起源于肺动脉(ALCAPA)的临床特点,提高对ALCAPA的认识。方法:对2006年8月至2008年12月期间我院心脏中心门诊以"心内膜弹力纤维增生症"收治但最终诊断为ALCAPA的8例患者的临床特点、检查情况进行回顾性分析。结果:8例ALCAPA的患者女性7例,男性1例。发病年龄2～10个月,平均年龄5.3个月。8例患者均表现不同程度的气促、喘息、声哑、多汗及喂养困难等心力衰竭(心衰)症状。病初均于首诊医院误诊为心内膜弹力纤维增生症。患儿心电图、心脏超声及心脏增强CT均有较为特征性的改变。结论:小儿ALCAPA病例临床常易与心内膜弹力纤维增生症混淆。对于临床诊断心内膜弹力纤维增生症的患儿应注意其心电图表现。其中符合ALCAPA心电图特点的病例,反复探查其心脏彩超并进行心脏增强CT、心脏冠状动脉造影检查有助于ALCAPA的诊断。     

Percutaneous coil closure of recanalised anomalous origin of left coronary artery from pulmonary artery

An asymptomatic boy underwent surgical correction of anomalous origin of left coronary artery from pulmonary artery (ALCAPA) with trans-pulmonary artery interruption and saphenous vein grafting to left anterior descending coronary artery. He developed a shunt through the re-canalised pulmonary artery end of the ALCAPA which was successfully embolised using a detachable PDA coil delivered into the left main coronary artery from the pulmonary artery.     

Cross-sectional echocardiographic findings of anomalous origin of left coronary artery from pulmonary artery.

Anomalous origin of the left coronary artery and left ventricular myocardial hypokinesis were visualised by cross-sectional echocardiography. Significant dilatation of the right coronary artery was another important finding. Cross-sectional echocardiography appears to be useful for evaluating patients with anomalous origin of the left coronary artery from the pulmonary artery.     

心电图对左冠状动脉异常起源于肺动脉的早期诊断价值

目的 探讨心电图对左冠状动脉异常起源于肺动脉(anomalous origin of the left coro-nary artery from the pulmonary artery,ALCAPA)的早期诊断价值.方法 选取经手术证实的ALCAPA住院患者16例,以及15例经冠状动脉造影术诊断的冠状动脉肺动脉瘘(...