Detection of anomalous origin of left coronary artery from pulmonary artery by real-time Doppler color flow mapping in a 53-year-old asymptomatic female.

The anomalous origin of the left coronary artery from the pulmonary artery in a 53-year-old asymptomatic female was diagnosed by the color Doppler technique. Doppler color flow mapping on a modified high parasternal short axis view clearly demonstrated the blood flow from the anomalous left coronary artery into the pulmonary trunk. Aortography confirmed this finding. The anatomical factors of marked right coronary artery preponderance and a hypoplastic circumflex artery were considered to contribute to the long survival of this patient. In asymptomatic adult patients with this anomaly, Doppler color flow mapping is a sensitive diagnostic method, and should be used first in adult patients suspected of having this anomaly.     

Noninvasive diagnosis of coronary artery fistula by Doppler color flow mapping

Sixteen patients with coronary artery fistula proved by coronary angiography or surgery were studied using two-dimensional echocardiography and Doppler color flow mapping. The coronary artery fistula drained into the right atrium in 4 patients, the right ventricle in 2 and the pulmonary artery in 10. The dilated coronary artery was visualized in 7 of the 16 patients with a fistula, as compared with none of the 40 control subjects. These 7 patients included 5 of 6 patients with a fistula draining into the right atrium or right ventricle and only 2 of 10 patients with a fistula draining into the pulmonary artery. Abnormal flow signals in the dilated coronary artery were visualized with Doppler color flow mapping in five of these seven patients. Color flow imaging visualized abnormal flow signals with mosaic appearance in the pulmonary artery in eight patients, the right atrium in four and the right ventricle in two. The chamber in which abnormal signals were detected corresponded with the entry site of the fistula by angiography. Intraoperative imaging during surgical repair was needed in two cases to confirm ligation of all arteries feeding into the fistula network. In conclusion, Doppler color flow imaging is diagnostically useful to visualize shunt flows originating from the opening or exit of a coronary artery fistula. Furthermore, intraoperative use of this technique may provide confirmation of successful surgical ligation of the fistula.     

A case of anomalous origin of the right coronary artery from the pulmonary trunk: imaging of abnormal flow by Doppler color flow mapping

An asymptomatic 48-year-old woman was admitted to our hospital for evaluation of a precordial continuous murmur. The chest radiograph showed a normal cardiac silhouette. The resting electrocardiogram showed incomplete right bundle branch block. A multistage, graded treadmill exercise test did not affect the ST-T segment. A thallium-201 myocardial perfusion scan with a multistage ergometer exercise test showed no distinct perfusion defect. A two-dimensional echocardiogram disclosed a dilated left coronary artery arising from the left sinus of Valsalva, and a dilated right coronary artery crossing the aorta anteriorly to the pulmonary trunk. Real-time two-dimensional Doppler echocardiography of the same region showed an abnormal jet coming from the right coronary artery into the pulmonary trunk during diastole. A continuous wave Doppler flow study in the pulmonary trunk revealed a high-speed, disturbed flow which began in mid-systole, and continued during diastole. These findings were compatible with anomalous origin of the right coronary artery from the pulmonary trunk. An aortogram confirmed the diagnosis.     

Exercise performance after repair of anomalous origin of the left coronary artery from the pulmonary artery

Eleven patients underwent exercise testing after operative repair of anomalous origin of the left coronary artery from the pulmonary artery. Five patients repaired after 2 years of age comprised a childhood surgery group, and six patients repaired before 2 years of age comprised an infant surgery group. All patients were exercised using either a treadmill or electronically braked bicycle with simultaneous thallium 201 scintigraphy. Oxygen consumption, carbon dioxide production, pulmonary functions, and electrocardiogram were all monitored continuously. Pulmonary reserve was normal in all patients. Based on heart rate reserve, respiratory exchange ratio, and oxygen-consumption response to work load, two patients in the infant surgery group stopped exercise before achieving maximum aerobic capacity. All remaining patients achieved their maximum aerobic capacity. There was no difference in work rate or oxygen consumption during exercise between the infant and childhood surgical group. Four patients (two in each surgical group) had an impaired chronotropic response to exercise. Three of these four patients demonstrated perfusion defects by thallium scintigraphy. Thallium scintigraphy was normal in all remaining patients. Electrocardiographic abnormalities were noted in seven of 11 patients having ventricular arrhythmias or ST segment depression. It is concluded from this study that exercise performance after repair of anomalous origin of the left coronary artery from the pulmonary artery is not affected by the age at which surgery is performed. Exercise is frequently associated with electrocardiographic evidence of abnormal myocardial perfusion despite frequently negative simultaneous 201Tl scintigraphy.     

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.     

Anomalous origin of the left coronary artery from the pulmonary trunk: elucidation with colour Doppler flow mapping

Four infants and children with anomalous connection of the left coronary artery to the pulmonary trunk were studied with colour Doppler flow mapping. In three the diagnosis was only suspected when the colour Doppler study showed dilated intraseptal and epicardial vessels and an abnormal flow signal into the pulmonary artery in diastole; this latter signal localised the exact site of communication, which was not apparent on angiocardiography. Two of these patients had previously had operations for severe mitral regurgitation, the diagnosis of anomalous left coronary artery having been previously considered in one but missed despite aortic root angiography. The colour study in the fourth was largely confirmatory, operation without catheterisation being undertaken on the basis of the echocardiographic images. By contrast in two infants subsequently seen with congestive cardiomyopathy the demonstration of flow direction in the left coronary artery confirmed that it was normally connected to the aorta. Colour Doppler flow mapping can show flow direction in the left coronary artery and from the mouth of an anomalous coronary artery into the pulmonary artery, thus simplifying the diagnosis and allowing the site of the connection of the left coronary artery to the pulmonary artery to be determined with precision.     

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 origin of the left coronary artery from the pulmonary artery: diagnosis by two-dimensional Doppler color

A case of anomalous origin of the left coronary artery from the pulmonary artery (ALCA) is presented. The noninvasive diagnosis was made by 2D-color flow Doppler. Application of this technique may alleviate the necessity for angiography in patients with ALCA.     

Diagnosis of anomalous left coronary artery by Doppler color flow mapping: distinction from other causes of dilated cardiomyopathy.

BACKGROUND. Anomalous origin of the left coronary artery from the pulmonary trunk is difficult to diagnose reliably by two-dimensional echocardiography. Therefore, Doppler color flow mapping was tested in 29 patients with dilated cardiomyopathy or anomalous left coronary artery, or both. METHODS AND RESULTS. All patients with anomalous left coronary artery (10 patients) or dilated cardiomyopathy (27 patients) (excluding those with other known causes for cardiomyopathy) examined between January 1988 and May 1991 were identified. The direction of flow in the three main segments of the left coronary system was determined by Doppler color flow mapping. In all 10 patients with anomalous left coronary artery, flow mapping demonstrated an abnormal jet from the left coronary artery into the pulmonary trunk and retrograde flow in at least two segments of the left coronary system. The diagnosis was confirmed in all 10 patients at operation. Doppler color flow mapping, performed in 19 of the 27 patients with dilated cardiomyopathy, demonstrated anterograde flow in at least one segment of the left coronary system in 16 of the 19 patients; flow direction was not determined in the other 3 patients. Coronary artery anatomy was confirmed by aortic root or left ventricular angiography in 14 patients and at autopsy in 1 patient and was not directly confirmed in 4 patients. Left ventricular function spontaneously improved to normal in three of the latter four patients, a clinical course not consistent with anomalous left coronary artery. The left coronary artery appeared to arise from the aortic root by two-dimensional echocardiographic imaging alone in all patients with dilated cardiomyopathy and in 5 of 10 patients with anomalous left coronary artery (50% false negative diagnoses). CONCLUSIONS. Detection of an abnormal jet into the pulmonary trunk and retrograde flow in the left coronary system by Doppler color flow mapping is reliable for diagnosing anomalous left coronary artery whereas two-dimensional echocardiographic imaging alone is often inconclusive or misleading. Determining flow direction in the left coronary system in patients with dilated cardiomyopathy is useful for excluding anomalous left coronary artery but is technically more difficult to document in this condition than in anomalous left coronary artery.     

Anomalous origin of the left coronary artery from the pulmonary trunk: elucidation with colour Doppler flow mapping.

Four infants and children with anomalous connection of the left coronary artery to the pulmonary trunk were studied with colour Doppler flow mapping. In three the diagnosis was only suspected when the colour Doppler study showed dilated intraseptal and epicardial vessels and an abnormal flow signal into the pulmonary artery in diastole; this latter signal localised the exact site of communication, which was not apparent on angiocardiography. Two of these patients had previously had operations for severe mitral regurgitation, the diagnosis of anomalous left coronary artery having been previously considered in one but missed despite aortic root angiography. The colour study in the fourth was largely confirmatory, operation without catheterisation being undertaken on the basis of the echocardiographic images. By contrast in two infants subsequently seen with congestive cardiomyopathy the demonstration of flow direction in the left coronary artery confirmed that it was normally connected to the aorta. Colour Doppler flow mapping can show flow direction in the left coronary artery and from the mouth of an anomalous coronary artery into the pulmonary artery, thus simplifying the diagnosis and allowing the site of the connection of the left coronary artery to the pulmonary artery to be determined with precision.     

Intercoronary collateral flow detected by Doppler colour flow mapping is an additional diagnostic sign in children with anomalous origin of the left coronary artery from the pulmonary artery.

Intercoronary collateral flow within septal collaterals was detected by colour-coded Doppler echocardiography in three children with anomalous origin of the left coronary artery from the pulmonary artery. In each of the three patients angiography confirmed the presence of septal collaterals.     

Surgical repair of naturally palliated anomalous origin of the left coronary artery from the right pulmonary artery.

Anomalous origin of the left coronary artery from the pulmonary arteries is a rare and life-threatening defect, usually needing prompt surgical correction during infancy. We describe the case of a young asymptomatic patient with this defect who underwent surgical reimplantation despite the absence of signs of myocardial ischemia, due to the presence of proximal stenosis of the anomalous coronary artery.     

Anomalous origin of the left coronary artery from the pulmonary artery: direct assessment of anomalous and collateral coronary flow by pulsed Doppler echocardiography.

We directly assessed the anomalous and collateral coronary flow profiles of a 58 year-old-man with Bland-White-Garland syndrome using pulsed Doppler echocardiography. Doppler recordings in this patient document the utility of pulsed Doppler echocardiography in the assessment of a cardiac shunt associated with a congenital coronary anomaly in an adult.