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。     

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.     

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.     

Diagnostic value of parasternal pulmonary artery short-axis view for the anomalous origin of the left coronary artery from the pulmonary artery

ObjectiveTo evaluate the diagnostic value of parasternal pulmonary artery (PA) short-axis view for the anomalous origin of left coronary artery (LCA) from the pulmonary artery by echocardiography.MethodsA total of 13 patients (3 boys) aged from 2 months to 12 years were enrolled. Transthoracic echocardiography, including cross-sectional imaging and color Doppler flow imaging, were performed, and their diagnoses were confirmed by operation.ResultsAmong the 13 patients, 7 had LCA originated from the left posterior wall of PA, 2 from the posterior wall, and 4 from the right posterior wall. The PA short-axis view could visualize the anomalous origin of the LCA from left posterior or posterior wall of PA clearly. The LCA and aortic wall were overlapping at 3–4 o’clock at PA short-axis view in the patients with LCA originating from the right posterior wall of PA. It was similar with the image of the LCA originating from the aorta. But the blood flow was opposite to that of LCA with normal origin.ConclusionsThe parasternal PA short-axis view is a good view to visualize the anomalous origin of the LCA.     

The role of echocardiography in anomalous origin of coronary artery from pulmonary artery (ALCAPA): Simple tool for a complex diagnosis

Anomalous origin of coronary artery from pulmonary artery (ALCAPA) is a rare coronary anomaly usually presenting in the first year of life. Adult presentation suggests a wide range of differential diagnosis such as myocarditis, dilated cardiomyopathy, and coronary artery disease. We have presented here the major distinctive echocardiographic features of ALCAPA through 4 cases. Since echocardiography is often used as the first imaging modality in these clinical scenarios, recognizing the characteristics of ALCAPA in a routine echocardiographic examination can transform a readily available, low‐cost tool into a valuable discriminative modality.     

Anomalous left coronary artery from the pulmonary artery: A rare case diagnosed in an adult

ALCAPA syndrome (anomalous origin of left coronary artery from the pulmonary artery) is an extremely rare congenital cardiac anomaly associated with high mortality rate at young age. If undiagnosed and uncorrected, the affected individuals rarely survive beyond infancy. This article reports on a 45-year-old asymptomatic man with the ALCAPA diagnosed thanks to a detailed cardiac examination, that followed the finding of the left bundle branch block and a decreased left ventricular systolic ejection fraction. Although coronary angiography and computed tomography are considered to be the key diagnostic methods for ALCAPA, in this case the diagnosis was established based on echocardiography. Previously, the patient has been under regular review by a cardiologist with the incorrect diagnosis of multiple ventricular septal defects. In fact, this diagnosis resulted from a misinterpretation of the ultrasound image of the intercoronary connections. The presence of a separate diagonal artery originating from aorta renders this case report even more interesting.     

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 origin of left coronary artery from pulmonary artery associated with pulmonary hypertension

This is a report on a 10-year-old child with anomalous origin of left coronary artery (LCA) from pulmonary artery (ALCAPA), severe pulmonary hypertension (PH), old myocardial infarction and poor intercoronary collateralization. It discusses the echocardiographic pitfalls in this particular setting and introduces a new echocardiographic view (posterior pulmonary cusp view) for visualization of the anomalous origin of LCA from the posterior pulmonary cusp (PC) in patients with ALCAPA from the PC of the pulmonary artery. We describe three echocardiographic pitfalls that can mislead the echocardiographer and two helpful hints that guide the clinician to the correct diagnosis.The survival of this child shows that limited size of left ventricular myocardial infarction and severe mitral regurgitation in early infancy can result in a life-saving pulmonary hypertension which preserves viability and function of left ventricle despite lack of intercoronary collateral arteries. After one year follow-up, she is doing well on medical treatment.     

Anomalous origin of the left coronary artery from the pulmonary trunk. Potential for false negative diagnosis with cross sectional echocardiography

Cross sectional echocardiography can identify anomalous origin of the left coronary artery from the pulmonary trunk. It has been suggested that identification of the left coronary artery arising from the aorta using this technique excludes the diagnosis. In three such infants the anomalous origin of the left coronary artery was identified in each by cross sectional echocardiography. In all three cases, however, an echo free linear structure apparently arising from the aorta, resembling a normal left coronary artery, was imaged. Anatomical sections in one patient, simulating cross sectional echocardiographic cuts, showed that this structure was almost certainly the transverse sinus of the pericardium. False positive cross sectional echocardiographic diagnosis of this condition is also possible because of the failure to image a normally arising left coronary artery. Thus identification of the anomalous origin of the left coronary artery from the pulmonary trunk appears to be the only reliable echocardiographic finding in this condition, and contrast cineaortography remains necessary in patients in whom the diagnosis is suspected clinically or electrocardiographically.     

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.     

Anomalous origin of the left coronary artery from the pulmonary trunk. Potential for false negative diagnosis with cross sectional echocardiography.

Cross sectional echocardiography can identify anomalous origin of the left coronary artery from the pulmonary trunk. It has been suggested that identification of the left coronary artery arising from the aorta using this technique excludes the diagnosis. In three such infants the anomalous origin of the left coronary artery was identified in each by cross sectional echocardiography. In all three cases, however, an echo free linear structure apparently arising from the aorta, resembling a normal left coronary artery, was imaged. Anatomical sections in one patient, simulating cross sectional echocardiographic cuts, showed that this structure was almost certainly the transverse sinus of the pericardium. False positive cross sectional echocardiographic diagnosis of this condition is also possible because of the failure to image a normally arising left coronary artery. Thus identification of the anomalous origin of the left coronary artery from the pulmonary trunk appears to be the only reliable echocardiographic finding in this condition, and contrast cineaortography remains necessary in patients in whom the diagnosis is suspected clinically or electrocardiographically.     

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.     

A case series of left main coronary artery ostial atresia and a review of the literature

Left main coronary artery ostial atresia (LMCAOA) is a rare congenital anomaly of the coronary arteries. The published literature regarding the current diagnostic and management recommendations are limited. We present three case series of LMCAOA from our institution, including one with a unique association with anomalous origin of left coronary artery (LCA) from pulmonary artery. In addition, this report includes a review of 50 pediatric and 43 adult cases from literature. The majority of the patients were symptomatic. Sudden cardiac death occurred in 10% of pediatric patients and 7% of adult patients. Almost half of pediatric patients had additional cardiac lesions. At the time of diagnosis, 82% of patients had abnormal exercise stress test and 73% had abnormal myocardial perfusion imaging (MPI). The diagnosis of LMCAOA was suspected by echocardiography in 47% of pediatric patients, while 26% were initially misdiagnosed as anomalous origin of LCA from pulmonary artery. Coronary angiography confirmed the diagnosis in most cases and 70.5% of pediatric patients had small collaterals, while 80.5% of adult patients had large collaterals. Nine pediatric patients had no revascularization surgery with five deaths. Revascularization surgery was performed in 39 pediatric patients with four deaths. After 2005, there is a gradual shift toward performing coronary osteoplasty rather than coronary artery bypass grafting. Eighteen adult patients had revascularization surgery and all survived. Fifteen adult patients had no revascularization surgery, of which there were five deaths. In patients with LMCAOA, revascularization surgery is currently recommended in the presence of symptoms, ischemic changes on electrocardiogram or exercise stress test, myocardial perfusion defect on MPI, global left ventricular systolic dysfunction on echocardiogram, severe mitral regurgitation, or small‐sized collaterals in coronary angiography. Short‐term and mid‐term outcomes are encouraging.     

Atypical Bland-White-Garland syndrome: a 58-year-old woman with stenosis of the pulmonary origin of the left coronary artery

The anomalous origin of the left coronary artery from the main pulmonary trunk (also known as Bland-White-Garland syndrome) is a rare congenital malformation that occurs in 0.4% of patients with cardiac anomalies. We present an adult case (a 58-year-old woman) of atypical Bland-White-Garland syndrome. The patient displayed a stenosis at the ostium of the anomalous origin of the left coronary artery and an aortopulmonary fistula. Using conventional angiography, it was not possible to differentiate between an anomalous origin of the pulmonary coronary artery and total stenosis of the left main coronary artery in combination with a pulmonary fistula. However, transesophageal echocardiography (TEE) succeeded in making this differential diagnosis. CONCLUSION: If there is subtotal or total occlusion, TEE can be used for detection of coronary vessel morphology, particularly in cases of coronary anomalies.     

Anomalous origin of the left anterior descending coronary artery from the pulmonary artery

Three children were identified as having anomalous origin of the left anterior descending coronary artery (LAD) from the pulmonary artery (PA). Two had had congestive heart failure in infancy with clinical diagnosis of endocardial fibroelastosis and all had abnormal ECGs. The correct diagnosis was delayed in each case, and two patients required selective coronary angiography. Surgery was accomplished in the three children although ECG abnormalities have persisted and one child has dyskinesis of the left ventricular apex. Because this diagnosis may be difficult to make when intercoronary anastomoses are inadequate to outline the left anterior descending coronary flow into the PA, patients with clinical findings suggestive of anomalous coronary artery may require selective coronary studies to exclude this anomaly.     

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.     

Left-right shunt caused by anomalous implantation of the left coronary artery in the trunk of pulmonary artery. Detection by contrast echocardiography

The authors describe a case of an anomalous left coronary artery arising from the main pulmonary artery in an 11 year old child suffering from chest pain on effort and with a continuous murmur in the second left intercostal space. Two dimensional echocardiography (2D E) showed dilatations of the first segment of the right coronary artery and the anomalous origin of the left main coronary artery. A peripheral injection of microbubbles showed a left-to-right shunt between the left coronary artery and the pulmonary artery. Semi-quantitative evaluation of LV regional wall motion showed abnormal contraction of the anterolateral walls. Haemodynamic, angiographic data and the operative findings confirmed the diagnosis. The abnormal coronary ostium was closed and a bypass graft from the aorta to the left anterior descending artery was performed. Clinical and echocardiographic follow-up 7 months after surgery was completely normal.     

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.     

Aortopulmonary window with anomalous origin of the right coronary artery from the pulmonary artery: two cases highlighting the importance of complete pre-operative echocardiographic evaluation of the coronary arteries in all conotruncal anomalies.

This report describes two infants with an aortopulmonary window in association with anomalous origin of the right coronary artery from the pulmonary artery. In both cases the diagnosis was made pre-operatively by transthoracic echocardiography, with the initial clue being extensive collateral flow within the myocardium. In each case there was surgical confirmation of the echocardiographic findings. These two cases demonstrate that coronary artery evaluation should be an integral part of every new echocardiographic evaluation, particularly in the setting of conotruncal anomalies.     

Cardiac imaging in a patient with anomalous origin of the left coronary artery from the pulmonary artery--a case report

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is an uncommon congenital heart disease and has a high mortality rate in infancy. However, myocardial ischemia does not develop until adolescence or adulthood in about 10% of patients. Moreover, the diagnosis of ALCAPA is often difficult in cases without heart murmur or cardiac symptoms. The authors report the case of a 31-year-old man with ALCAPA. He was admitted to the hospital for evaluation of mild shortness of breath at exercise, but he had no typical chest symptoms due to myocardial ischemia or heart failure until age 31 and he had no heart murmur. Moreover, electrocardiogram did not show an old myocardial infarction or myocardial ischemia. Therefore, the authors did not suspect ALCAPA until they performed transthoracic echocardiography and exercise-stress single photon emission computed tomography (SPECT) with Tc-99m-tetrofosmin. The final diagnosis was established from the results of coronary arteriography. In the present case, a transthoracic echocardiogram showed abnormal coronary circulation, and exercise-stress SPECT revealed reversible myocardial ischemia. Transthoracic echocardiography and myocardial SPECT imaging could be a useful noninvasive tools for diagnosing the ALCAPA.